*****AMERICAN JOURNAL OF CARDIOLOGY*****
(REFERENCE 1 OF 53) 98293747
Lane GK Lucas VW Sklansky MS Kashani IA Rothman A Percutaneous coil occlusion of ascending aorta to pulmonary artery shunts.
In: Am J Cardiol (1998 Jun 1) 81(11):1389-91
ISSN: 0002-9149
Two patients with pulmonary atresia and intact ventricular septum each underwent early palliative surgery with a pulmonary valvotomy and an ascending aorta to pulmonary artery shunt. Adequate right ventricular growth and relief of pulmonary stenosis rendered the shunts unnecessary. The shunts were successfully occluded percutaneously with Gianturco coils.
Institutional address: Department of Pediatrics University of California San Diego USA.
(REFERENCE 2 OF 53) 98252601
McElhinney DB Reddy VM Tworetzky W Silverman NH Hanley FL Early and late results after repair of aortopulmonary septal defect and associated anomalies in infants <6 months of age.
In: Am J Cardiol (1998 Jan 15) 81(2):195-201
ISSN: 0002-9149
The Richardson classification system for aortopulmonary septal defect (APSD) includes simple defects between the ascending aorta and pulmonary trunk (type I), defects extending distally to include the origin of the right main pulmonary artery (type II), and anomalous origin of the right main pulmonary artery from the ascending aorta with no other aortopulmonary communication (type III). These are rare lesions that must be repaired in early infancy to avoid development of pulmonary vascular disease. Few reports have focused on patients with complex, associated lesions who underwent repair in early infancy. Between 1972 and 1995, 24 patients with Richardson type I (n = 11), II (n = 7), or III (n = 6) defects underwent repair at ages ranging from 2 to 172 days (median 34). Twelve patients had complex, associated anomalies, including interrupted or hypoplastic arch (n = 9), tetralogy of Fallot with (n = 1) or without (n = 1) pulmonary atresia, and transposition of the great arteries (n = 1). The most recent 7 patients were diagnosed by echocardiography without cardiac catheterization. There were no early or late deaths among the 12 patients with simple APSD. Four patients with complex, associated lesions died in the early postoperative period and another died 4 months after surgery. All 6 surviving patients with interrupted arch have had recurrent obstruction at the arch repair site, although reintervention for this reason has been performed in only 2 patients. Altogether, 6 early survivors have required reintervention, and all survivors are in New York Heart Association class I at follow-up ranging from 2 to 25 years. Thus, long-term survival after repair of APSD in early infancy is excellent. Late sequelae are likely to be related either to associated lesions or to obstruction at the APSD repair site. Almost all cases of APSD in young infants can be diagnosed and evaluated by echocardiography without catheterization.
Institutional address: Division of Cardiothoracic Surgery University of California San Francisco
USA.
(REFERENCE 3 OF 53) 98122452
Garcia JA Zellers TM Weinstein EM Mahony L Usefulness of Doppler echocardiography in diagnosing right ventricular coronary arterial communications in patients with pulmonary atresia and intact ventricular septum and comparison with angiography.
In: Am J Cardiol (1998 Jan 1) 81(1):103-4
ISSN: 0002-9149
Direct and indirect evidence of right ventricular coronary arterial communications has been detected by use of 2-dimensional and Doppler echocardiography in patients with pulmonary atresia and intact ventricular septum. We describe additional consistent and reproducible Doppler echocardiographic findings that were useful for identifying patients in whom abnormal coronary arterial flow patterns were detected angiographically.
Institutional address: Department of Pediatrics University of Texas Southwestern Medical Center at Dallas 75235-9063 USA.
(REFERENCE 4 OF 53) 98060657
Weber HS Cyran SE Effectiveness of an umbilical artery "snare assisted" approach for critical pulmonary valve stenosis or atresia in the neonate.
In: Am J Cardiol (1997 Dec 1) 80(11):1502-5
ISSN: 0002-9149
Thirteen neonates with critical pulmonary valve stenosis/atresia underwent successful transcatheter balloon valvuloplasty using an umbilical artery "snare assisted" approach. This technique simplifies the procedure and avoids femoral artery injury by using the umbilical artery, reduces fluoroscopy exposure, and eliminates the need for a gradational approach which reduces costs.
Institutional address: Department of Pediatrics (Cardiology) The Pennsylvania State University Children's Hospital Hershey 17033 USA.
(REFERENCE 5 OF 53) 96313946
Coe JY Chen RP Dyck J Byrne P Transaortic balloon valvoplasty of the pulmonary valve.
In: Am J Cardiol (1996 Jul 1) 78(1):124-6
ISSN: 0002-9149
An unconventional transaortic to transductal approach was performed to perforate and dilate the pulmonary valve in pulmonary atresia. Ductal arteriosus patency was maintained by prostaglandin.
Institutional address: Children's Health Center University of Alberta Edmonton Canada.
(REFERENCE 6 OF 53) 96259453
Digilio MC Marino B Grazioli S Agostino D Giannotti A Dallapiccola B Comparison of occurrence of genetic syndromes in ventricular septal defect with pulmonic stenosis (classic tetralogy of Fallot) versus ventricular septal defect with pulmonic atresia.
In: Am J Cardiol (1996 Jun 15) 77(15):1375-6
ISSN: 0002-9149
Our results confirm that classic TF can be associated with many genetic conditions, but most patients are nonsyndromic. In contrast, children with TF-PA have a high incidence of genetic syndromes, particularly those related to CATCH22 syndrome.
Institutional address: Department of Pediatric Cardiology Bambino Gesu Hospital Rome Italy.
(REFERENCE 7 OF 53) 96259451
Wright SB Radtke WA Gillette PC Percutaneous radiofrequency valvotomy using a standard 5Fr electrode
catheter for pulmonary atresia in neonates.
In: Am J Cardiol (1996 Jun 15) 77(15):1370-2
ISSN: 0002-9149
Radiofrequency energy applications with a standard 5Fr 3 mm tip electrode catheter facilitated opening of the pulmonary valve in neonates with pulmonary atresia. Shortening of the catheter tip electrode would probably improve efficiency.
Institutional address: South Carolina Children's Heart Center Medical University of South Carolina Charleston 29425 USA.
(REFERENCE 8 OF 53) 97204859
Connolly HM Warnes CA Outcome of pregnancy in patients with complex pulmonic valve atresia.
In: Am J Cardiol (1997 Feb 15) 79(4):519-21
ISSN: 0002-9149
Fifteen women with complex pulmonary atresia who had pregnancies were retrospectively reviewed. Although no pregnancy-related deaths occurred, complications were noted in 3 patients and risk of fetal loss and premature birth were increased, none of the 10 offspring had congenital heart disease.
Institutional address: Division of Cardiovascular Diseases and Internal Medicine Mayo Clinic Rochester Minnesota 55905 USA.
*****ANNALS OF THORACIC SURGERY*****
(REFERENCE 9 OF 53) 97219082
Najm HK Williams WG Coles JG Rebeyka IM Freedom RM Pulmonary atresia with intact ventricular septum: results of the Fontan procedure.
In: Ann Thorac Surg (1997 Mar) 63(3):669-75
ISSN: 0003-4975
BACKGROUND: Children with pulmonary atresia and an intact ventricular septum show a heterogeneous spectrum of cardiac anomalies. A biventricular repair is attainable in some; a Fontan procedure or a one-and-a-half ventricle is the only possible repair for others. Children with right ventricle-to-coronary artery connections, with or without right ventricle-dependent coronary artery blood flow, are a high-risk group. METHODS: Between May 1980 and December 1994, 22 children underwent a Fontan operation for the treatment of pulmonary atresia with an intact ventricular septum at The Hospital for Sick Children, Toronto. The mean age was 5.8 years (median, 4.9 years). All children had had at least one pre-Fontan palliative procedure; 19 had two, and 7 of these had three or more. Right ventricle-to- coronary artery connections were present in 15 children, including 5 with right ventricle-dependent coronary artery blood flow. Thromboexclusion of the right ventricle was done in 10 children, with 7 undergoing it before and 3 at the time of the Fontan procedure. RESULTS: There were three early deaths (13.6%) and one late death. The actuarial survival at 10 years after the Fontan operation was 80%. Early postoperative complications occurred in 4 children. Follow- up was completed in all children at a mean of 4 years (range, 1 to 12.5 years) after the Fontan operation. Atrial arrhythmia occurred in 3 children, and permanent pacemakers were required in 4. CONCLUSIONS: Results of the Fontan operation for the treatment of pulmonary atresia with an intact ventricular septum are satisfactory. Thromboexclusion of the right ventricle is indicated in the presence of right ventricle-to-coronary artery connections without right ventricle-dependent coronary artery blood flow. The right ventricle should not be decompressed or thromboexcluded in children with right ventricle-dependent coronary artery blood flow, and at the Fontan operation, saturated blood must enter the right ventricle.
Institutional address: Department of Surgery Hospital for Sick Children Toronto Ontario Canada.
(REFERENCE 10 OF 53) 96420421
Nomura F Kadoba K Ichikawa H Akedo H Matsuda H Direct anastomosis of pulmonary artery-to-right ventricular outflow for correction of tetralogy of Fallot with pulmonary atresia.
In: Ann Thorac Surg (1996 Oct) 62(4):1199-202
ISSN: 0003-4975
A case of tetralogy of Fallot with well-developed infundibulum and pulmonary atresia was successfully repaired with direct anastomosis to reconstruct the pulmonary arterial trunk without using an extracardiac conduit. This technique may give the maximum chance for growth of the new pulmonary trunk and vasculature.
Institutional address: First Department of Surgery Osaka University Medical School Japan.
(REFERENCE 11 OF 53) 95289784
Shah AS Michler RE Successful heart transplantation with acquired pulmonary artery atresia.
In: Ann Thorac Surg (1995 Jun) 59(6):1557-9
ISSN: 0003-4975
Orthotopic cardiac transplantation was performed successfully in a patient with acquired atresia of the left pulmonary artery 19 years after repair of tetralogy of Fallot. Only the right lung could be incorporated into the cardiopulmonary circulation at transplantation, resulting in transient right ventricular dysfunction, which resolved with vasodilator therapy. Perfusion of a single pulmonary vascular bed does not preclude successful heart transplantation, provided there is a low pulmonary vascular resistance and pulmonary artery architecture free of stenoses.
Institutional address: Division of Cardiothoracic Surgery College of Physicians and Surgeons Columbia University New York New York USA.
(REFERENCE 12 OF 53) 95251463
Kitagawa T Katoh I Chikugo F Hori T Fukumura Y Mori K Matsuoka S Technique for constructing the pulmonary trunk for tetralogy of Fallot with pulmonary atresia.
In: Ann Thorac Surg (1995 May) 59(5):1245-8
ISSN: 0003-4975
In expectation of the growth of a new pulmonary arterial trunk in total correction of tetralogy of Fallot with pulmonary atresia, we used pedicled autologous pericardium combined with left atrial appendage as the posterior wall of a new pulmonary arterial trunk. In cases of long discontinuity between the right ventricular infundibulum and left pulmonary artery, our technique could be recommended for early repair of tetralogy of Fallot with pulmonary atresia.
Institutional address: Department of Cardiovascular Surgery University of Tokushima Japan.
(REFERENCE 13 OF 53) 95209467
Waldman JD Karp RB Lamberti JJ Sand ME Ruschhaupt DG Agarwala B Tricuspid valve closure in pulmonary atresia and important RV-to- coronary artery connections [see comments]
In: Ann Thorac Surg (1995 Apr) 59(4):933-40; discussion 940-1
ISSN: 0003-4975
Mortality is high for children with pulmonary atresia, intact ventricular septum, and important connections between the right ventricle and the coronary arteries because of myocardial ischemia: in systole, suprasystemic right ventricular pressure delivers deoxygenated blood to the coronary artery (or arteries) and in diastole, the right ventricle provides a lower resistance alternative to coronary perfusion of the myocardium. Tricuspid valve closure was performed in 10 such children. None had stenosis of native coronary arteries. A trial of tricuspid valve closure (by balloon) was performed in the cardiac catheterization laboratory in 5 of 10 patients. Seven of 10 children survived surgical closure of the tricuspid valve plus concurrent procedures; none had heart block. Two of the 3 nonsurvivors were probably in inoperable condition due to preoperative myocardial ischemia. Before operation, 4 patients had ischemic changes on electrocardiograms; these changes were abolished after operation. Three of 10 patients have had a Fontan operation with 2 survivors. We conclude that children with pulmonary atresia, intact ventricular septum, important connections between the right ventricle and the coronary arteries, and normal native coronary arteries should have surgical closure of the tricuspid valve within the first year of life and treated thereafter as patients with "tricuspid atresia."
Comment in: Ann Thorac Surg 1996 Sep;62(3):949-50
Institutional address: Section of Pediatric Cardiology University of Chicago Illinois.
(REFERENCE 14 OF 53) 95194091
Ando M Imai Y Hoshino S Ishihara K Autologous reconstruction of pulmonary trunk at reoperation after extracardiac conduit repair.
In: Ann Thorac Surg (1995 Mar) 59(3):621-5
ISSN: 0003-4975
Between 1991 and 1993, 5 patients underwent reoperation for critical stenosis of extracardiac conduit. Indication for extracardiac conduit repair was pulmonary truncal atresia in 3 patients and coronary anomaly including single left coronary artery and left anterior descending artery from right coronary artery in 2 patients. Age at reoperation ranged from 8 to 23 years (mean, 16.2 years). Preoperative systolic pressure ratio of right to left ventricles ranged from 0.83 to 1.05 (mean, 0.93), with the pressure gradient across the conduit ranging from 52 to 100 mm Hg (mean, 74.4 mm Hg). At reoperation, stenotic conduit was completely removed and central pulmonary artery was extensively mobilized. In 4 patients who had a relatively short distance (15 to 25 mm) between the pulmonary arterial stump and the right ventriculotomy incision, the distal pulmonary arterial stump was anastomosed directly to the cranial margin of the right ventriculotomy incision to serve as a floor mode of autologous tissue. In 1 patient with a long distance (40 mm), right ventricular-pulmonary arterial continuity was restored with a tailored autologous pericardial tube. There were no early or late deaths. Postoperative catheterization study revealed a satisfactory reduction of right ventricular pressure with the systolic pressure ratio ranging from 0.42 to 0.51 (mean, 0.47) and the pressure gradient across the right ventricular outflow tract ranged within 13 mm Hg (mean, 5 mm Hg). Restoration of right ventricular-pulmonary arterial continuity was successfully achieved by introducing the concept of autologous tissue repair even at reoperation instead of the insertion of new extracardiac conduit in patients with tetralogy of Fallot after extracardiac conduit repair.
Institutional address: Heart Institute of Japan Tokyo Women's Medical College
(REFERENCE 15 OF 53) 95150630
Laks H Gates RN Grant PW Drant S Allada V Harake B Aortic to right ventricular shunt for pulmonary atresia and intact ventricular septum [see comments]
In: Ann Thorac Surg (1995 Feb) 59(2):342-7
ISSN: 0003-4975
Acute or chronic myocardial ischemia may develop in patients with pulmonary atresia with intact ventricular septum and right ventricular-dependent coronary circulation. In such cases an aorta to right ventricle shunt may be used to reverse this ischemia. This report summarizes our experience with the placement of an aortic to right ventricular shunt in 5 patients. The shunts were made of Gore- Tex and ranged from 4 mm to 8 mm. Associated procedures were bidirectional Glenn (n = 2) and Fontan (n = 2). All 5 patients survived the procedure with documented early graft patency and no evidence of ischemia.
Comment in: Ann Thorac Surg 1996 Sep;62(3):949-50
Institutional address: Department of Surgery University of California Los Angeles 90024-1741.
(REFERENCE 16 OF 53) 98257246
Malm TK Holmqvist C Olsson CG Johansson J Olsson AK Sandstrom S Bennhagen R Jogi P Successful thrombolysis of an occluded modified Blalock shunt three days after operation.
In: Ann Thorac Surg (1998 May) 65(5):1453-5
ISSN: 0003-4975
A 10-day-old boy with pulmonary atresia received a right-sided aortopulmonary polytetrafluoroethylene shunt. Three days after the operation he became cyanotic and was reintubated. Shunt occlusion was confirmed with angiography. Recombinant tissue plasminogen activator was given locally into the proximal end of the shunt. The thrombus was completely resolved after 2 days. When administration of recombinant tissue plasminogen activator was stopped, heparin infusion was started for 5 days. Shunt patency was demonstrated by angiography at 3 months postoperatively.
Registry Numbers: EC 3.4.21.- (Plasminogen Activators) EC 3.4.21.68 (Tissue Plasminogen Activator) 9002-84-0 (Polytetrafluoroethylene) 9005-49-6 (Heparin)
Institutional address: Department of Cardiothoracic Surgery University Hospital Lund Sweden.
(REFERENCE 17 OF 53) 98097087
McElhinney DB Reddy VM Feuerstein BG Marx GR Hanley FL Intraoperative discovery of neuroblastoma in an infant with pulmonary atresia.
In: Ann Thorac Surg (1997 Dec) 64(6):1827-9
ISSN: 0003-4975
There have been 28 previously reported cases of neuroblastoma associated with congenital heart disease. Because many of these have been defects of the conotruncal region, it has been proposed that abnormal neural crest cell migration or maturation may be a factor that links these normally disparate pathologic conditions. Most neuroblastomas in these cases have been detected at autopsy or by radiologic studies conducted in the evaluation of the cardiac anomalies. Recently, we discovered an occult posterior mediastinal neuroblastoma in a patient undergoing a unifocalization procedure for tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals. The tumor was resected, and the patient has demonstrated no evidence of residual or metastatic neuroblastoma.
Institutional address: Department of Laboratory Medicine University of California San Francisco USA.
(REFERENCE 18 OF 53) 96266229
Waldman JD Karp RB Gittenberger-de Groot AC Agarwala B Glagov S Spontaneous acquisition of discontinuous pulmonary arteries.
In: Ann Thorac Surg (1996 Jul) 62(1):161-8
ISSN: 0003-4975
BACKGROUND. Discontinuous pulmonary arteries have been considered a rare complication of systemic-to-pulmonary shunt operations. We report a series of children who spontaneously acquired pulmonary artery discontinuity. METHODS. All children from 1989 through 1995 with congenital pulmonary atresia were reviewed. RESULTS. Pulmonary artery discontinuity developed in 29% (15 patients), none related to shunt operation. In 6 of 15 patients, the neonatal angiogram showed a pattern that seemed to predict subsequent discontinuity; in 9 of 15, pulmonary arteriography was normal at birth. Two clinical patterns were identified: an early rapid acquisition of discontinuity within hours to days, and a delayed, more subtle development that occurred over months. Eight of 15 have died. Pathologic studies in 6 children showed ductal tissue extending along and into the pulmonary artery wall as well as intimal hypertrophic reaction and maladaptive remodeling. CONCLUSIONS. Children with congenital pulmonary atresia may experience spontaneous acquisition of pulmonary artery discontinuity. Ductal tissue is responsible for local pulmonary artery distortion and discontinuity; this may be exacerbated by previous prostaglandin E1 administration. Clinical algorithms are suggested for patients with pulmonary atresia.
Registry Numbers: 745-65-3 (Alprostadil)
Institutional address: University of Chicago Illinois
USA.
(REFERENCE 19 OF 53) 96266225
Godart F Houyel L Lacour-Gayet F Serraf A Sousa-Uva M Bruniaux J Petit J Piot JD Binet JP Conte S Planche C Absent pulmonary valve syndrome: surgical treatment and considerations [see comments]
In: Ann Thorac Surg (1996 Jul) 62(1):136-42
ISSN: 0003-4975
BACKGROUND. The operative management of absent pulmonary valve syndrome remains controversial regarding the need for pulmonary valve implantation and remodeling of pulmonary arteries. Moreover, symptomatic infants are considered to have a poor prognosis. This retrospective report summarizes the experience of a single institution. METHODS. Between May 1977 and May 1995, 37 consecutive patients underwent repair of absent pulmonary valve syndrome. Patients were divided into two groups according to age at operation: group A (10 infants less than 1 year old) and group B (27 patients older than 1 year). Mean age at operation was 5 +/- 4 months in group A and 72 +/- 42 months in group B. Initially, repair consisted of ventricular septal defect closure and relief of right ventricular outflow tract obstruction combined with pulmonary valve implantation. More recently, the concept of treatment has evolved with pulmonary arterioplasty without pulmonary valve insertion, except in patients with elevated pulmonary artery pressure. RESULTS. Of the 37 patients, 34 had successful repair. The overall in-hospital mortality rate was 8% (two deaths in group A and one in group B). No hemodynamic data were correlated with operative death. Death was associated with longer extracorporeal circulation time (p = 0.005) and longer aortic cross-clamping time (p = 0.019). In fact, these were clearly related to more complex anatomy (p = 0.001): multiple ventricular septal defects in 1, left pulmonary artery arising from the ductus in
another, and left pulmonary artery arising from the aorta in the remainder. Follow-up was available in 22 of the 34 survivors. Mean follow-up time was 30 +/- 47 months in group A and 38 +/- 33 months in group B. All but 1 had no restriction of exercise, and most of them had pulmonary incompetence of Doppler echocardiography. One developed severe exercise intolerance because of pulmonary valve stenosis (xenograft), leading to uneventful reoperation 123 months after initial repair. One infant died suddenly of complete atrioventricular block 3 months after repair. The late mortality rate was 5%. CONCLUSIONS. Surgical treatment of absent pulmonary valve syndrome should include pulmonary arterioplasty to reduce bronchial obstruction, with no need for pulmonary valve insertion. This procedure is feasible and is recommended especially in markedly symptomatic infants.
Comment in: Ann Thorac Surg 1997 Apr;63(4):1216-7
Institutional address: Department of Pediatric Cardiac Surgery Marie Lannelongue Hospital Paris Sud University Le Plessis Robinson France.
(REFERENCE 20 OF 53) 96160239
Mignosa C Comas JV Kitayama H Karl TR Avoiding hypoxemia during unifocalization.
In: Ann Thorac Surg (1996 Feb) 61(2):715-7
ISSN: 0003-4975
An 11-month-old child underwent unifocalization of the major aortopulmonary collateral arteries, but did not tolerate occlusion of both vessels simultaneously. Using a Y-shaped homograft tube, we translocated the vessels sequentially and avoided severe hypoxemia.
Institutional address: Victorian Paediatric Cardiac Surgical Unit Royal Children's Hospital Melbourne Australia.
(REFERENCE 21 OF 53) 96160223
Moritz A Marx M Wollenek G Domanig E Wolner E Complete repair of PA-VSD with diminutive or discontinuous pulmonary arteries by transverse thoracosternotomy [see comments]
In: Ann Thorac Surg (1996 Feb) 61(2):646-50
ISSN: 0003-4975
BACKGROUND. Optimal treatment and the optimal sequence of surgical and interventional steps to correct pulmonary atresia with ventricular septal defect and hypoplastic or discontinuous intrapericardial pulmonary arteries is still under discussion. Collateral arteries may be hardly accessible through median sternotomy at total correction. Bilateral transsternal thoracotomy gives wide access to the heart, both pleural spaces and hilar structures. METHODS. We used this incision for total correction of pulmonary atresia with ventricular septal defect in 6 patients. Three had Blalock-Taussig shunts placed previously, and intrapericardial pulmonary arteries were absent in all patients but 1, in whom they were hypoplastic. Central pulmonary arteries were enlarged with pericardial patches or replaced with tube grafts; the number of unifocalized collateral arteries varied between two and eight. RESULTS. One patient died of respiratory failure and sepsis (16.7%). Oxygen saturation increased from 76% (range, 65% to 88%) preoperatively to 96% (range 91% to 99%) postoperatively. Mean postoperative pulmonary artery pressure was 30 mm Hg (range, 28 to 34 mm Hg). One patient had to be reoperated on through the same incision due to scarring and shrinkage of the peripheral anastomoses. Six months after operation 2 patients are in New York Heart Association functional class I and 2 are in class II. CONCLUSIONS. Transverse thoracosternotomy gives excellent access to the anatomical structures necessary to correct complex cases of pulmonary atresia with ventricular septal defect and may reduce the number of surgical procedures.
Comment in: Ann Thorac Surg 1996 Oct;62(4):1247-8
Institutional address: Department of Cardiothoracic Surgery University of Vienna Austria.
(REFERENCE 22 OF 53) 96146323
Toyoda Y Yamaguchi M Ohashi H Imai M Oshima Y Yoshimura N Yamashita T Staged repair of tetralogy of Fallot and pulmonary atresia without central pulmonary arteries.
In: Ann Thorac Surg (1996 Jan) 61(1):210-3
ISSN: 0003-4975
A 15-year-old boy with tetralogy of Fallot and pulmonary atresia without central pulmonary arteries who was successfully treated using a staged approach is presented. The first stage consisted of the creation of central pulmonary arteries. In the second stage, the continuity between the right ventricle and the pulmonary arteries was established with closure of the ventricular septal defect. Some patients previously considered to be unsuitable candidates for correction may be successfully repaired using this approach.
Institutional address: Department of Cardiothoracic Surgery Kobe Children's Hospital Japan.
(REFERENCE 23 OF 53) 96110235
Miyaji K Shimada M Sekiguchi A Ishizawa A Isoda T Tsunemoto M Pulmonary atresia with intact ventricular septum: long-term results of "one and a half ventricular repair".
In: Ann Thorac Surg (1995 Dec) 60(6):1762-4
ISSN: 0003-4975
BACKGROUND: Between 1982 and 1984, we successfully performed "one and a half ventricular repair" using a Glenn shunt for 3 patients with pulmonary atresia with intact ventricular septum. Here we review the 10-year follow-up results. METHODS: In these patients, the preoperative Z scores of the tricuspid valve diameters ranged from - 5.2 to -6.5. Right ventricular outflow tract reconstruction combined with a Glenn shunt were performed in all patients. Cardiac catheterization was done at least 10 years post-operatively. RESULTS: All 3 patients have maintained New York Heart Association functional class I status for more than 10 years. Angiography in 2 patients confirms sufficient left pulmonary artery pressure with pulsatile blood flow and good right ventricular contraction. A pulmonary arteriovenous fistula has developed in 1 patient. CONCLUSIONS: Although the lower limits of the tricuspid valve diameter for "one and a half ventricular repair" using a cavopulmonary shunt have not yet been determined, we successfully performed this procedure in 3 patients with severely hypoplastic right ventricles and tricuspid valve diameter Z scores of less than -5.0. The results up to 10 years postoperatively are acceptable.
Institutional address: Department of Cardiovascular Surgery National Children's Hospital Tokyo Japan.
(REFERENCE 24 OF 53) 96064539
van Son JA Vander Woude JC Cheng W Silverman NH Cahalan MK Hanley FL Surgical closed atrial septotomy under transesophageal guidance.
In: Ann Thorac Surg (1995 Nov) 60(5):1403-4
ISSN: 0003-4975
An infant with pulmonary atresia and intact ventricular septum is presented who, after initial patch reconstruction of the right ventricular outflow tract and bidirectional cavopulmonary anastomosis through a fifth median sternotomy, underwent an echocardiographically guided closed atrial septotomy, which resulted in marked long-term clinical improvement. The technique of intraoperative transesophageal echocardiography as used in the presented case represents an expanded role for this diagnostic modality in congenital cardiac surgery.
Institutional address: Division of Cardiothoracic Surgery University of California at San Francisco 94143-0118 USA.
*****BRITISH HEART JOURNAL*****
(REFERENCE 25 OF 53) 95001121
Ries M Singer H Hofbeck M Thrombolysis of a modified Blalock-Taussig shunt with recombinant tissue plasminogen activator in a newborn infant with pulmonary atresia and ventricular septal defect.
In: Br Heart J (1994 Aug) 72(2):201-2
ISSN: 0007-0769
A 10 day old infant with pulmonary atresia, ventricular septal defect, and collateral pulmonary blood supply through a left sided ductus arteriosus developed complete shunt thrombosis four days after the creation of a modified Blalock-Taussig shunt. Recombinant tissue plasminogen activator was given locally into the proximal end of the shunt as two bolus injections of 0.1 mg/kg and two bolus injections of 0.2 mg/kg over 10 minutes, followed by a continuous infusion of 1.4 mg/kg/day for 16 hours and 0.7 mg/kg/day for 18 hours with systemic low dose heparin 5 IU/kg/h. This resulted in complete clot dissolution and reperfusion without haemorrhagic complications and without laboratory signs of systemic fibrinolytic activation.
Registry Numbers: EC 3.4.21.68 (Tissue Plasminogen Activator) 9005-49-6 (Heparin)
Institutional address: Department of Paediatrics University of Erlangen-Nuernberg Germany.
*****CIRCULATION*****
(REFERENCE 26 OF 53) 97080446
Redington AN Somerville J Stenting of aortopulmonary collaterals in complex pulmonary atresia.
In: Circulation (1996 Nov 15) 94(10):2479-84
ISSN: 0009-7322
BACKGROUND: The optimal treatment of patients with complex pulmonary atresia remains controversial. Surgical unifocalization programs are increasing popular but have not previously or currently gained universal acceptance. Furthermore, not all patients are suitable for attempts at biventricular correction. These patients may become increasingly symptomatic and require palliation. METHODS AND RESULTS: We attempted to palliate 12 patients with progressive symptomatic hypoxemia. Each had at least one stenotic but balloon-dilatable collateral supplying at least three lung segments. It was impossible to traverse the stenotic area with the stent in 1 patient, despite two attempts. Twelve stents were thus deployed in 11 patients. There was no effect in 1 patient who had multiple stenoses distal to the stented area. There was excellent palliation in the remainder, arterial oxygen saturation 45% to 79% before stenting (mean, 64 +/- 12%) rising to 67% to 90% (mean, 78 +/- 10%, P < .01) at discharge from hospital. One patient was referred for surgery to secure blood flow to a nearly totally occluded side branch to the right upper lobe traversed by the stent. There was an excellent symptomatic response in the remainder, with an early increase in exercise duration (P < .01). Late arterial desaturation occurred in 2 patients. In 1, there was pulmonary arterial hypertension in the lung segments supplied by the stented vessel. A stenosis had developed within the stent in the other patient, who was noncompliant with anticoagulation therapy. CONCLUSIONS: Stenting of stenotic aortopulmonary collaterals can achieve excellent palliation in the majority of this highly selected subgroup of patients with complex pulmonary atresia.
Institutional address: Department of Pediatric Cardiology Royal Brompton Hospital London England.
(REFERENCE 27 OF 53) 95188336
Oosthoek PW Moorman AF Sauer U Gittenberger-de Groot AC Capillary distribution in the ventricles of hearts with pulmonary atresia and intact ventricular septum.
In: Circulation (1995 Mar 15) 91(6):1790-8
ISSN: 0009-7322
BACKGROUND: Pulmonary atresia and intact ventricular septum (PA-IVS) can be complicated by the presence of a severely hypoplastic thick- walled right ventricle with or without ventriculo-coronary arterial communications. A variable amount of myocardial pathology has been described in these hearts, probably the result of ischemic conditions and a high pressure in the right ventricle. We studied whether the capillary network is still intact, allowing a sufficient perfusion of the myocardium, which will be important for the success of palliative surgery. METHODS AND RESULTS: We studied the distribution of capillaries in the myocardium of hearts with PA-IVS and compared the results with normal hearts. The capillaries were detected by immunohistochemistry using a monoclonal antibody (408) against endothelium. Remarkable abnormalities in capillary distribution were found in the right ventricle of hearts with PA-IVS and reflect the arrangement of the myocytes. Thus, disorganization of capillaries, which is found to be the most common pattern, always paralleled the myocardial disarray. A low density of capillaries is always found in areas with a low density of myocytes, ie, with hypertrophied myocytes, compact fibrotic tissue, or diffuse fibrosis. Disarray and other disturbances in orientation of capillaries and myocytes are present in hearts with PA-IVS, a hypoplastic right ventricle, and ventriculo-coronary arterial communications. These disturbances are more extensive when interruptions of the coronary arteries are also present. In hearts with PA-IVS and a hypoplastic right ventricle only, extensive regions with low capillary densities and severe myocyte pathology are observed. On the contrary, hearts with PA-IVS and a normal-size right ventricle show minor abnormalities in capillary and myocyte organization. CONCLUSIONS: In hearts with PA- IVS, various abnormal capillary distribution patterns are found. Our findings correlate well with clinical data that reported the best surgical results in hearts in which the major part of the myocardium showed a normal capillary distribution and myocyte morphology. This suggests that the capillary distribution may be an important parameter for the function of the heart. Because the distribution of the capillaries is found to be a good reflection of the arrangement of the myocytes, antibody 408 is also a useful tool in detecting abnormalities of the myocardium in a fast and easy way.
Institutional address: Department of Anatomy and Embryology University of Leiden The Netherlands.
(REFERENCE 28 OF 53) 95043234
Gentles TL Keane JF Jonas RA Marx GE Mayer JE Jr Surgical alternatives to the Fontan procedure incorporating a hypoplastic right ventricle.
In: Circulation (1994 Nov) 90(5 Pt 2):II1-6
ISSN: 0009-7322
BACKGROUND: Frequently the definitive operation for patients with a right ventricle (RV) that is too small to support full cardiac output is a modified Fontan operation. However, other surgical options exist that incorporate a small RV in the atriopulmonary pathway when biventricular repair is not feasible because of RV or tricuspid valve hypoplasia. The risks and benefits of these options have not been well defined. METHODS AND RESULTS: Between 1988 and 1993, 8 patients (6 with pulmonary atresia and intact ventricular septum and 2 with tricuspid valve stenosis and RV hypoplasia) underwent a cavopulmonary connection, which allowed right atrial blood to flow either to the pulmonary artery via the RV or directly via the cavopulmonary anastomosis. Age at surgery ranged from 1.5 to 9 years. The proximal right pulmonary artery was ligated in 5 patients, and the atrial septal defect was closed during the same procedure in 7 of the 8 patients. The echocardiographic right ventricular-left ventricular volume ratio ranged from 9% to 25%, and tricuspid valve z-scores ranged from 0 to -4. There were no deaths at a median follow-up of 24 months (range, 7 to 61 months). Mild exertional limitation was evident in only one patient. Postoperative echocardiograms demonstrated pulsatile systolic flow across the RV outflow tract in 5 patients and low-velocity diastolic-systolic flow in a sixth patient with extreme tricuspid valve hypoplasia. At postoperative cardiac catheterization (6 patients) right atrial mean pressures ranged from 7 to 13 mm Hg and mixed venous saturations from 62% to 70%. CONCLUSIONS: Right atrial decompression via a superior vena cava-to- pulmonary artery anastomosis allows incorporation of a small RV into the pulmonary circulation and closure of the atrial septum, with excellent results to date.
Institutional address: Department of Cardiology Children's Hospital Boston MA.
(REFERENCE 29 OF 53) 98377916
Daubeney PE Sharland GK Cook AC Keeton BR Anderson RH Webber SA Pulmonary atresia with intact ventricular septum: impact of fetal echocardiography on incidence at birth and postnatal outcome. UK and Eire Collaborative Study of Pulmonary Atresia with Intact Ventricular Septum.
In: Circulation (1998 Aug 11) 98(6):562-6
ISSN: 0009-7322
BACKGROUND: Fetal echocardiography is widely established in the United Kingdom for prenatal diagnosis of congenital heart disease. This may result in a substantial reduction in incidence at birth because of selected termination of pregnancy. The objective of this population-based study was to determine the incidence of pulmonary atresia with intact ventricular septum (PAIVS) at birth, the impact of fetal echocardiography on this incidence, and to compare the outcome of cases with and those without prenatal diagnosis. METHODS AND RESULTS: From 1991 to 1995, all infants born with PAIVS and all fetal diagnoses in the United Kingdom and Eire were studied. There were 183 live births (incidence 4.5/100,000 live births). The incidence was 4.1 cases per 100,000 live births in England and Wales, 4.7 in Scotland, 6.8 in Eire, and 9.6 in Northern Ireland (P=0.01). There were 86 fetal diagnoses made at a mean of 22.0 weeks of gestation leading to 53 terminations of pregnancy (61%), 4 intrauterine deaths (5%), and 29 live births (34%). The incidence at birth would be 5.6 per 100,000 births in England and Wales, 5.3 in Scotland, and unchanged in Eire and Northern Ireland, if there were no terminations of pregnancy and assuming no further spontaneous fetal deaths (P=0.28). An initial diagnosis of critical pulmonary stenosis was made in 6 cases, at a mean of 22.3 weeks of gestation with progression to PAIVS by 31.4 weeks. Probability of survival at 1 year was 65% and was the same for live-born infants whether or not a fetal diagnosis had been made. CONCLUSIONS: PAIVS is rare, occurring in 1 in 22,000 live births in the United Kingdom and Eire. Termination of pregnancy has resulted in an important reduction in the live-born incidence in mainland Britain.
Institutional address: Wessex Cardiothoracic Centre Southampton General Hospital UK.
*****HEART*****
(REFERENCE 30 OF 53) 97339863
Walsh KP Abdulhamed JM Tometzki JP Importance of right ventricular outflow tract angiography in distinguishing critical pulmonary stenosis from pulmonary atresia.
In: Heart (1997 May) 77(5):456-60
ISSN: 1355-6037
OBJECTIVE: To investigate the spectrum of pulmonary atresia and critical pulmonary stenosis using right ventricular outflow tract angiography and explore its implications for catheter interventional treatment. DESIGN: Prospective clinical study. SETTING: Two paediatric cardiology centres. SUBJECTS: 11 neonates or infants (aged 1 day to 8 months; weighing 2.3 to 7.8 kg) with pulmonary atresia or where the differentiation of pulmonary atresia from critical pulmonary stenosis was unclear on either echocardiography or angiography. METHODS: Right ventricular outflow tract angiography was performed on all patients to distinguish pulmonary atresia from critical pulmonary stenosis before opening the right ventricular outflow tract. RESULTS: Right ventricular outflow tract angiography showed that three of seven patients diagnosed as pulmonary atresia by echocardiography had pin hole jets across the pulmonary valve; another had a probe patent valve that appeared imperforate on both echocardiography and right ventricular outflow tract angiography. Three of the four patients diagnosed by echocardiography as critical pulmonary stenosis were found on right ventricular outflow tract angiography to have pulmonary atresia. The remaining patient had such a tiny orifice that a second orifice had to be created with a radiofrequency catheter. The right ventricular outflow tract was opened successfully in 10 of the 11 patients, six of whom required application of radiofrequency energy. The right ventricular to aortic systolic pressure ratio fell from 1.4 (0.9 to 1.9) to 0.6 (0.2 to 1.1) (P < 0.05). All 11 patients were alive and well with transcutaneous oxygen saturations ranging from 84% to 95% at a median follow up duration of nine months. CONCLUSIONS: Critical pulmonary stenosis and pulmonary atresia cannot always be accurately distinguished by echocardiography. Right ventricular outflow tract angiography helps to distinguish the two groups. In most cases the right ventricular outflow tract can be opened without mortality and with short to medium term survival.
Institutional address: Heart Clinic Royal Liverpool Children's Hospital United Kingdom.
(REFERENCE 31 OF 53) 97246893
Gibbs JL Blackburn ME Uzun O Dickinson DF Parsons JM Chatrath RR Laser valvotomy with balloon valvoplasty for pulmonary atresia with intact ventricular septum: five years' experience.
In: Heart (1997 Mar) 77(3):225-8
ISSN: 1355-6037
OBJECTIVE: To assess immediate and medium term results of transcatheter laser valvotomy with balloon valvoplasty in selected infants with pulmonary atresia and intact ventricular septum. DESIGN: Prospective study. SETTING: Tertiary cardiac unit. PATIENTS: All infants with pulmonary atresia and intact septum with no more than minor tricuspid valve hypoplasia referred between November 1990 and June 1995. Laser valvotomy was attempted in nine infants of median age 4-5 days and median weight 3.6 kg. INTERVENTION: The pulmonary valve was perforated using a 0.018 inch fibreoptic guidewire attached to a NdYag laser and introduced through a catheter positioned beneath the valve. After perforation the valve was dilated with progressively larger balloons. MAIN OUTCOME MEASURES: Successful laser valvotomy and balloon dilatation, complications, pulse oximetry, right ventricular outflow velocities, and need for surgical treatment. RESULTS: Valvotomy was successful in all but one case, the failure being due to laser breakdown. After perforation the valve was dilated to 6-8 mm diameter. Prostaglandin E was withdrawn immediately in six of the eight duct dependent infants, and 28 and 49 days later in two. No patient required an aortopulmonary shunt. Two patients had repeat valvoplasty at 20 days and three months of age, respectively; one required infundibular resection and closure of the atrial septum at age four and one is awaiting similar treatment. CONCLUSIONS: Laser valvotomy with balloon valvoplasty is safe and effective treatment for selected patients with pulmonary atresia and intact ventricular septum and should be considered as first line treatment in place of surgical valvotomy.
Institutional address: Killingbeck Hospital Leeds West Yorkshire.
(REFERENCE 32 OF 53) 97221357
Mildner RJ Kiraly L Sreeram N Pulmonary atresia, "intact ventricular septum", and aortopulmonary collateral arteries.
In: Heart (1997 Feb) 77(2):173-5
ISSN: 1355-6037
In muscular pulmonary atresia, major aortopulmonary collateral arteries are characteristic of pulmonary atresia with ventricular septal defect and are rarely seen in pulmonary atresia with intact ventricular septum. Two unusual cases of muscular pulmonary atresia are reported, one with an intact septum and one with a perimembranous ventricular septal defect, closed in utero by aneurysmal tricuspid tissue. In both cases the pulmonary blood supply came entirely from aortopulmonary collaterals. In case 1 a collateral artery connected the left subclavian artery and hypoplastic pulmonary arteries, and several aortopulmonary collaterals arose from the descending aorta, without overlap between these two circulations. In case 2 the pulmonary trunk and arterial duct were absent and the pulmonary blood supply came entirely from collateral arteries. The right ventricle was of normal size and tripartite with a closed perimembranous ventricular septal defect, discovered only at postmortem examination. These observations suggest right ventricular outflow tract obstruction early in fetal development, with involution of the pulmonary trunk and sixth arch derivatives, and persistence of primitive aortopulmonary connections. The morphology in case 1 is at odds with the theoretical division of pulmonary atresia with intact septum and pulmonary atresia with ventricular septal defect into two separate pathological entities that occur at different stages in fetal development.
Institutional address: Heart Unit Birmingham Children's Hospital.
(REFERENCE 33 OF 53) 97435729
Neumayer U Somerville J Outcome of pregnancies in patients with complex pulmonary atresia.
In: Heart (1997 Jul) 78(1):16-21
ISSN: 1355-6037
OBJECTIVE: To evaluate the outcome of pregnancies in patients with complex pulmonary atresia, comparing those with and without previous radical surgical repair. DESIGN: A retrospective study of all pregnancies in women with complex pulmonary atresia registered on the Grown-up Congenital Heart Unit database between 1977 and 1994. SETTING: Referral centre for adolescents and adults with congenital heart disease. PATIENTS: Forty one pregnancies occurred in 15 patients. They were divided into two groups; group I, 26 pregnancies in nine patients before radical repair (cyanotic); group II, 15 pregnancies in seven women after radical surgical repair. RESULTS: In group I there were three terminations, 13 miscarriages, eight healthy children, and two neonatal deaths. Five children were born prematurely and all had low birthweights. In group II there were two miscarriages, 11 normal children, and two children with congenital heart disease. None was premature and all had normal birthweights. There were major complications in both groups: in group I there were two thromboembolic complications and one episode of heart failure; in group II there was one pulmonary embolism and one arrhythmic complication, five pregnancies in three patients were complicated by left ventricular failure that was persistent in one case and progressive in another, leading to death 13 months after delivery. CONCLUSIONS: Patients with complex pulmonary atresia, with or without surgical repair, who have no or mild symptoms, can have successful pregnancies. Surgical repair decreases fetal complications significantly. In both groups thrombotic disorders and heart failure must be prevented. Patients with residual systemic-pulmonary collaterals after surgical repair are particularly at risk of left ventricular failure.
Institutional address: Grown-Up Congenital Heart Unit Royal Brompton Hospital London UK.
(REFERENCE 34 OF 53) 98199114
Hofbeck M Rauch A Buheitel G Leipold G von der Emde J Pfeiffer R Singer H Monosomy 22q11 in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries.
In: Heart (1998 Feb) 79(2):180-5
ISSN: 1355-6037
OBJECTIVE: To describe the morphology of the pulmonary arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries with and without monosomy 22q11. DESIGN: A retrospective analysis of all patients with this congenital heart defect who are being followed at the University Children's Hospital Erlangen. SETTING: A tertiary referral centre for paediatric cardiology and paediatric cardiac surgery. PATIENTS: 21 patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Monosomy 22q11 was diagnosed by fluorescent in situ hybridisation using the D22S75 probe (Oncor). The morphology of the pulmonary arteries was assessed on the basis of selective angiograms. RESULTS: 10 patients (48%) were shown to have a microdeletion in 22q11 (group I). There was no difference with respect to the presence of confluent central pulmonary arteries between these patients (80%) and the remaining 11 patients (group II) without monosomy 22q11 (91%). Patients of group I, however, more often had arborisation anomalies of the pulmonary vascular bed (90% in group I v 27% in group II). Because of the more severe abnormalities of the pulmonary arteries, a biventricular repair had not been possible in any of the children with monosomy 22q11, though repair had been carried out in 64% of the children in group II. CONCLUSION: The developmental disturbance caused by the monosomy 22q11 seems to impair the connection of the peripheral pulmonary artery segments to the central pulmonary arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries, resulting in a lower probability of biventricular repair.
Institutional address: Department of Paediatric Cardiology University Children's Hospital Erlangen Germany.
(REFERENCE 35 OF 53) 98166830
Magee AG Rosenthal E Bostock J Gill J Unguarded tricuspid orifice with pulmonary atresia: successful radiofrequency ablation of an accessory pathway in an infant.
In: Heart (1998 Jan) 79(1):101-3
ISSN: 1355-6037
A male infant with the rare lesion of unguarded tricuspid orifice in the setting of pulmonary valve atresia, intact ventricular septum, and a hypoplastic right ventricle is described. The patient presented with cyanosis at 1 day old; transcutaneous oxygen saturations were between 20% and 30% in room air, and 60% in 100% inspired oxygen. Pre- excitation was found incidentally on the ECG and the potential for rapid antegrade conduction of atrial tachyarrhythmias, after eventual extended palliation with the Fontan procedure, was demonstrated at electrophysiological study. By 11 months old the patient was becoming increasingly cyanosed and interim palliation with a bidirectional cavopulmonary shunt was proposed. Successful radiofrequency ablation of the accessory pathway was performed before bidirectional cavopulmonary shunt, which would have prevented access to the heart via the superior vena cava. Difficulty with femoral venous access because of previous occlusion of a femoral vein was overcome by the use of 2 F pacing electrodes and a 5 F ablation catheter.
Institutional address: Department of Paediatric Cardiology Guy's Hospital London UK.
*****JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY*****
(REFERENCE 36 OF 53) 97283102
Mair DD Julsrud PR Puga FJ Danielson GK The Fontan procedure for pulmonary atresia with intact ventricular septum: operative and late results.
In: J Am Coll Cardiol (1997 May) 29(6):1359-64
ISSN: 0735-1097
OBJECTIVES: The goals of the study were to evaluate the operative and late mortality associated with the Fontan procedure in patients with pulmonary atresia and an intact ventricular septum and to obtain follow-up information on the current clinical status of surviving patients. BACKGROUND: Between 1979 and October 1, 1995, 40 patients with the anomaly had a nonfenestrated Fontan procedure performed at the Mayo Clinic. Because there are no previously published reports involving a series of this size in which the Fontan approach was used for this condition, a review of patient outcomes was thought to be of value. METHODS: The medical records of the 40 patients were reviewed retrospectively, and 34 were determined to be alive. The status of the survivors as of late 1995 was then ascertained by direct examination, questionnaire or telephone follow-up. RESULTS: There were three operative deaths and three late deaths. The current ages of the 34 survivors ranged from 4 to 30 years (median 13). Thirty- three of the 34 survivors were thought to be in New York Heart Association functional class I or II, and all but three of these patients, of school age or older, were either full-time students or working full time. The three adults who were not employed thought they were capable of working but were not doing so because of socioeconomic reasons. More than half of the patients were not receiving cardiovascular medications. CONCLUSIONS: These overall gratifying early and late results encourage continued application of this operation for appropriately selected patients with this complex congenital cardiovascular anomaly.
Institutional address: Department of Diagnostic Radiology Mayo Clinic Rochester Minnesota 55905 USA.
(REFERENCE 37 OF 53) 97045909
Acherman RJ Smallhorn JF Freedom RM Echocardiographic assessment of pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect.
In: J Am Coll Cardiol (1996 Nov 1) 28(5):1308-13
ISSN: 0735-1097
OBJECTIVES: The purpose of this study was to determine the role of high resolution two-dimensional echocardiographic imaging and color flow Doppler study in assessing the pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect (VSD). BACKGROUND: Although echocardiography is a well established technique for assessing central pulmonary arteries in pulmonary atresia VSD and for determining the presence or absence of a patent arterial duct, few data are available on its role in patients whose source of blood supply is from collateral vessels. METHODS: Forty-two patients aged a few hours to 19 months (mean 29 days) were prospectively assessed by high resolution echocardiography, including color flow Doppler study, during a 4-year period ending in 1994, before any intervention other than intravenous administration of prostaglandins. Angiographic confirmation was available in 29 patients, including 18 (95%) of 19 with aortopulmonary collateral channels. RESULTS: A patent arterial duct was correctly identified as the sole source of pulmonary blood supply in 23 patients, whereas aortopulmonary collateral channels were detected in 19, with one of these having a small patent arterial duct and collateral channels. The patent arterial duct originated from the undersurface of the aorta in 16 (67%) of 24 patients and from the base of the brachiocephalic trunk in 7 (33%) of 24. All patients with a patent ductus as the sole source of pulmonary blood supply had confluent pulmonary arteries. Nonconfluent pulmonary arteries were present in six patients, with all but one having aortopulmonary collateral channels as the sole source of pulmonary flow. Aortopulmonary collateral channels were direct in 17 (89%) of 19 patients, whereas in 2 (11%) of 19, both direct and indirect collateral channels were present. Color flow Doppler study was accurate in determining the presence or absence, the side and the origin of the collateral channels in all patients, with the correct number being determined in 12 (67%) of 18. "Wash-in" to the hilar pulmonary arteries (retrograde color flow) was seen in 12 (92%) of 13 patients with collateral channels and confluent pulmonary arteries. Failure to identify a tiny central pulmonary artery occurred in one patient. CONCLUSIONS: High resolution imaging and color flow Doppler study provide good appreciation of the source of pulmonary blood supply in neonates and young infants with pulmonary atresia VSD.
Institutional address: Division of Cardiology Hospital For Sick Children Toronto Ontario Canada.
(REFERENCE 38 OF 53) 95130881
Bull K Somerville J Ty E Spiegelhalter D Presentation and attrition in complex pulmonary atresia [see comments]
In: J Am Coll Cardiol (1995 Feb) 25(2):491-9
ISSN: 0735-1097
OBJECTIVES. This study summarized patterns of presentation and attrition in complex pulmonary atresia. BACKGROUND. Assessment of the potential impact of surgical strategies for managing complex pulmonary atresia requires information about variability in age and physiology at presentation of the condition. METHODS. We performed a retrospective review of age at presentation, referral source, pulmonary artery and collateral anatomy and surgical history of 218 patients from two institutions dealing with congenital heart disease throughout life. RESULTS. Approximately 65% of pulmonary atresia appears in infancy, with 50% of patients severely symptomatic from cyanosis and 25% from heart failure. Compared with those presenting undiagnosed, patients referred secondarily for specialist management tend to be older when first seen, and care must be taken when generalizing about the natural history of the condition from their survival experience. Overall actuarial survival, including the effects of operation, suggests that 60% (95% confidence limits [CL] 43 to 73) of patients presenting in infancy survive to their first birthday, 65% (95% CL 51 to 74) of those alive at 1 year old survive to the age of 10, and 16% (95% CL 5 to 31) of those alive at 10 years old survive to age 35. CONCLUSIONS. Novel surgical approaches have generally been applied beyond infancy in patients selected by their survival through the period of greatest attrition for this disease. Unless successful application in symptomatic infants is demonstrated, we cannot assume that these serial and complicated operations will have a major impact on the outlook of most patients with complex pulmonary atresia.
Comment in: J Am Coll Cardiol 1996 Aug;28(2):539-40
Institutional address: Cardiothoracic Unit Hospital for Sick Children London United Kingdom.
(REFERENCE 39 OF 53) 95130877
Fedderly RT Lloyd TR Mendelsohn AM Beekman RH Determinants of successful balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum.
In: J Am Coll Cardiol (1995 Feb) 25(2):460-5
ISSN: 0735-1097
OBJECTIVES. This study reviewed our experience with percutaneous balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum and defined the anatomic and hemodynamic characteristics of infants in whom this procedure is successful and provides definitive therapy. BACKGROUND. Unlike children with valvular pulmonary stenosis, the follow-up of infants with critical pulmonary stenosis undergoing percutaneous balloon valvotomy is limited. METHODS. Between December 1987 and August 1992, percutaneous balloon valvotomy was attempted in 12 infants with critical pulmonary stenosis (n = 10) or pulmonary atresia with intact ventricular septum (n = 2). Two outcome groups were identified: Group A patients are acyanotic, have mild residual pulmonary stenosis and have not required operation; Group B patients have required operation. RESULTS. Of the 12 infants, 11 had a successful balloon valvotomy procedure. Group A patients (n = 7) have a residual gradient of 22 +/- 18.7 mm Hg (mean +/- SD) at follow-up of 3.2 years (range 1.2 to 5.0). In Group B (n = 5), operation was required for inability to cross the pulmonary valve (n = 1) or persistent severe hypoxemia for > or = 2 weeks after valvotomy (n = 4). Significant differences (p < or = 0.01) between the two groups (Group A vs. Group B) were identified in pulmonary valve annulus (Z value) 8.1 mm (-1.1) versus 5.5 mm (-3.4); tricuspid valve annulus (Z value) 14.0 mm (0.8) versus 8.8 mm (-1.8); right ventricular volume 65 versus 29 ml/m2; and Lewis index 10.9 versus 8.9. CONCLUSIONS. Percutaneous balloon valvotomy is effective and likely to provide definitive therapy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum who have a tricuspid valve annulus > 11 mm, pulmonary valve annulus > or = 7 mm and right ventricular volume > 30 ml/m2.
Institutional address: Department of Pediatrics C.S. Mott Children's Hospital University of Michigan Medical Center Ann Arbor.
(REFERENCE 40 OF 53) 96121108
Momma K Kondo C Matsuoka R Tetralogy of Fallot with pulmonary atresia associated with chromosome 22q11 deletion.
In: J Am Coll Cardiol (1996 Jan) 27(1):198-202
ISSN: 0735-1097
OBJECTIVES. The purpose of this study was to clarify characteristics of tetralogy of Fallot and pulmonary atresia associated with chromosome 22q11 deletion. BACKGROUND. DiGeorge syndrome and conotruncal anomaly facies syndrome are associated with chromosome 22q11 deletion (hemizygosity). Associated cardiac anomalies include tetralogy of Fallot, truncus arteriosus and interrupted aortic arch. METHODS. Twenty-three patients with tetralogy of Fallot and pulmonary atresia were proved to have chromosome 22q11 deletion with fluorescent in situ hybridization using N25 probe (Oncor). Cardiovascular anomalies were compared with those in 26 patients with tetralogy of Fallot and pulmonary atresia without the deletion. Cardiovascular anomalies were studied with cardiac catheterization, cineangiography and echocardiography. RESULTS. In patients with 22q11 deletion, additional anomalies of the aortic arch, ductus arteriosus and pulmonary artery were more common as follows: right aortic arch (70% with deletion vs. 23% without deletion), high aortic arch reaching third rib (43% vs. 15%), aberrant left subclavian artery (35% vs. 0%), absent ductus arteriosus (83% vs. 46%), major aortopulmonary collateral arteries (91% vs. 50%), absent confluent central pulmonary arteries (48% vs. 4%). CONCLUSIONS. In patients with tetralogy of Fallot and pulmonary atresia, additional anomalies of the aortic arch, ductus arteriosus and pulmonary arteries are more common in patients with than in those without the 22q11 deletion.
Institutional address: Department of Pediatric Cardiology Heart Institute of Japan Tokyo Women's Medical College Tokyo Japan.
(REFERENCE 41 OF 53) 96083549
Gournay V Piechaud JF Delogu A Sidi D Kachaner J Balloon valvotomy for critical stenosis or atresia of pulmonary valve in newborns.
In: J Am Coll Cardiol (1995 Dec) 26(7):1725-31
ISSN: 0735-1097
OBJECTIVES. Percutaneous balloon valvotomy was studied retrospectively in newborns with critical pulmonary valve stenosis or atresia to assess its potential role as an alternative therapy to operation. BACKGROUND. Severe right ventricular outflow tract obstructions are life-threatening conditions requiring prostaglandin infusion immediately after birth and then relief of the valvular obstruction. To avoid surgical hazards at this age, it would be useful to extend to newborns the balloon valvotomy so effective in older patients. METHODS. Ninety-seven newborns (82 with critical pulmonary valve stenosis, 15 with atresia) underwent balloon valvotomy, provided that they had a well developed right ventricle, including an infundibulum close to the pulmonary artery. In patients with atresia, the outflow tract membrane had to be perforated with a wire needle or a radiofrequency probe. RESULTS. Balloon valvotomy could be performed in 81 patients and was effective in 77. It caused 3 fatal and 16 nonfatal complications. Ten patients with persistent poor right ventricular compliance despite an effective valvotomy required a surgical shunt. Among the 81 patients in whom the procedure could be performed, right ventricular surgery was avoided in 5 (55%) of the 9 patients with atresia (95% confidence interval [CI] 28% to 80%) and 55 (76%) of the 72 patients with stenosis (95% CI 66% to 86%) at the end of the follow-up period (9.7 years). CONCLUSIONS. Balloon pulmonary valvotomy is not always feasible in newborns, but it is relatively safe and effective and should be considered a valid alternative to operation.
Institutional address: Service de Cardiologie Pediatrique Hopital Necker/Enfants-Malades Paris France.
*****JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY*****
(REFERENCE 42 OF 53) 97303294
Reddy VM Petrossian E McElhinney DB Moore P Teitel DF Hanley FL One-stage complete unifocalization in infants: when should the ventricular septal defect be closed?
In: J Thorac Cardiovasc Surg (1997 May) 113(5):858-66; discussion 866-8
ISSN: 0022-5223
BACKGROUND: The decision whether to close the ventricular septal defect at the time of unifocalization in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals may be difficult. The purpose of this study was to develop morphologic and physiologic methods to aid in deciding whether to close the ventricular septal defect in patients undergoing one-stage unifocalization. METHODS: Between July 1992 and April 1996, 27 infants with pulmonary atresia, ventricular septal defect, and aortopulmonary collaterals were treated at our institution. Midline complete unifocalization was performed in 25 patients-the ventricular septal defect was closed in 17 and left open in eight. Two patients with severe distal collateral stenoses underwent staged unifocalization. Pulmonary artery and collateral sizes were measured from preoperative angiograms and used to calculate the indexed cross- sectional area of the total neopulmonary artery bed. An intraoperative pulmonary flow study previously validated with experiments in neonatal lambs was performed in six patients: the unifocalized neopulmonary arteries were perfused with a known flow and pulmonary artery pressures were recorded. RESULTS: The neopulmonary artery index was greater in patients who underwent ventricular septal defect closure than in those who did not (p = 0.001), although the values did overlap. This index correlated with the postoperative right ventricular/left ventricular pressure ratio (p = 0.037). Mean pulmonary artery pressures obtained during the intraoperative flow study and after bypass were comparable. CONCLUSION: The total neopulmonary artery index correlates with postrepair right ventricular/left ventricular pressure ratio and is useful in deciding when to close the ventricular septal defect if it is larger than 200 mm2/m2. The pulmonary flow study is helpful in deciding whether to close the ventricular septal defect in all patients.
Institutional address: Division of Cardiothoracic Surgery University of California San Francisco USA.
(REFERENCE 43 OF 53) 97258812
Dodds GA 3rd Warnes CA Danielson GK Aortic valve replacement after repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot.
In: J Thorac Cardiovasc Surg (1997 Apr) 113(4):736-41
ISSN: 0022-5223
OBJECTIVE: Little attention has been paid to the occurrence of aortic regurgitation after complete repair in patients with pulmonary atresia and ventricular septal defect or tetralogy of Fallot. To highlight the development of aortic regurgitation or aortic root dilation severe enough to necessitate aortic valve replacement with or without aortic aneurysmorrhaphy or aortic root replacement, we retrospectively reviewed the records of patients who underwent aortic valve operation at our institution subsequent to repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot. METHODS: We searched the Mayo Clinic database for patients with pulmonary atresia and ventricular septal defect or tetralogy of Fallot who subsequently had aortic valve or aortic root operations. The degree of aortic regurgitation before operation was noted. Aortic sinus and root dimensions were measured. RESULTS: Sixteen patients underwent complete repair at a median age of 17 years, followed by an aortic operation a median of 13.5 years later. All 16 patients had dilated aortic sinuses at the time of the aortic valve operation. These 16 patients had aortic valve replacement: 11 with mechanical prostheses and 5 with bioprostheses. Five of the 16 also had reduction of aortic dilation by lateral aneurysmorrhaphy, and 1 had graft replacement of the ascending aorta. Five patients had associated conditions (evidence of valvular damage, recurrent ventricular septal defect, or history of endocarditis) discovered at the aortic valve operation that have been reported to be related to the development of aortic regurgitation. The remaining 11 patients had progressive aortic regurgitation despite complete, uncomplicated repair. CONCLUSIONS: Progressive aortic regurgitation and aortic root dilation can occur despite complete repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot.
Institutional address: Division of Cardiovascular Diseases Mayo Clinic Rochester Minn. 55905 USA.
(REFERENCE 44 OF 53) 97236176
Luciani GB Wells WJ Khong A Starnes VA The clamshell incision for bilateral pulmonary artery reconstruction in tetralogy of Fallot with pulmonary atresia.
In: J Thorac Cardiovasc Surg (1997 Mar) 113(3):443-52
ISSN: 0022-5223
BACKGROUND: Patients with tetralogy of Fallot/pulmonary atresia often have bilateral pulmonary artery lesions, including diminutive central and peripheral vessels, major aortopulmonary collaterals, and distortion from previous operations. Staged procedures through lateral thoracotomies and median sternotomies have traditionally been used for repair. METHODS: Between October 1993 and December 1995, 10 patients 3 months to 15 years old with complex tetralogy of Fallot/pulmonary atresia underwent repair via a clamshell approach. Nine had undergone a mean of 2.8 +/- 0.8 previous operations (range 1 to 4). Indications for operation were repair of pulmonary artery arborization anomalies in 10 (4 unilateral, 6 bilateral), with unifocalization in 6 (2 unilateral, 4 bilateral). RESULTS: Eight of 10 patients had concomitant complete repair. There were no deaths at a mean follow-up of 17.1 +/- 4.0 months (range 12 to 26). Mean ventilation time was 3.7 +/- 2.1 days (range 1 to 14) and hospital stay 8.7 +/- 4.6 days (range 4 to 19). At follow-up, the peak right ventricular/left ventricular pressure ratio in patients who received complete repair was 0.44 +/- 0.13 (0.30 to 0.67). One patient (10%) required reoperation because of pseudoaneurysm of the main pulmonary artery 14 months after repair, and one had successful stent placement because of recurrent left and right pulmonary artery stenosis 8 months after repair. Two infants who underwent complete unifocalization and central pulmonary artery reconstruction are awaiting completion of repair. CONCLUSIONS: The clamshell approach to complex tetralogy of Fallot/ pulmonary atresia provides simultaneous exposure of bilateral central and peripheral pulmonary artery lesions and intracardiac pathologic conditions. This procedure appears safe and may decrease the number of operations required to complete repair of tetralogy of Fallot/pulmonary atresia in selected patients.
Institutional address: Division of Cardiothoracic Surgery Children's Hospital Los Angeles Calif USA.
(REFERENCE 45 OF 53) 98043237
Tchervenkov CI Salasidis G Cecere R Beland MJ Jutras L Paquet M Dobell AR One-stage midline unifocalization and complete repair in infancy versus multiple-stage unifocalization followed by repair for complex heart disease with major aortopulmonary collaterals.
In: J Thorac Cardiovasc Surg (1997 Nov) 114(5):727-35; discussion 735-7
ISSN: 0022-5223
BACKGROUND: Patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have traditionally required multiple unifocalization staging operations before undergoing complete repair. Recently, the feasibility of a single-stage unifocalization and repair was demonstrated by Hanley. In this report, we describe our experience with each approach. METHODS AND RESULTS: Since 1989, 11 of 12 patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone complete surgical correction. The first seven patients were subjected to staged bilateral unifocalizations, with repair being achieved in six (group I). The last five patients have undergone a single-stage midline unifocalization and repair via a sternotomy (group II). Four of these were infants (2 weeks to 9 months) and one was 13 years old. All patients in group I had tetralogy of Fallot, whereas in group II three patients had tetralogy of Fallot, one patient had double-outlet right ventricle, and one patient had complete atrioventricular canal and transposition. In group I, the median age at the first operation was 43 weeks. Complete repair was performed at a median age of 3.5 years, with a mean number of 3.3 operations required. In group II, only one operation was required to achieve complete repair at a median age of 28 weeks. The postoperative right ventricular/left ventricular pressure ratio was 0.49 in group I and 0.45 in group II. One intraoperative death and one late death occurred in group I and no early or late deaths in group II. Currently, four patients in group I and all five patients in group II are alive and well. CONCLUSIONS: Early intervention with both surgical approaches can lead to complete biventricular repair in most patients. Because the single-stage midline unifocalization and repair can achieve a completely repaired heart in infancy with one operation, it is currently our approach of choice.
Institutional address: Division of Cardiovascular Surgery Montreal Children's Hospital McGill University Quebec Canada.
(REFERENCE 46 OF 53) 99054774
Rychik J Levy H Gaynor JW DeCampli WM Spray TL Outcome after operations for pulmonary atresia with intact ventricular septum.
In: J Thorac Cardiovasc Surg (1998 Dec) 116(6):924-31
ISSN: 0022-5223
OBJECTIVE:Pulmonary atresia with intact ventricular septum is an anatomically heterogeneous anomaly with a variety of surgical strategies possible. We sought to compare the outcome of patients with a single ventricle approach to those with a biventricular repair and to compare outcome of patients with coronary abnormalities to those with normal coronary arteries. METHODS: A retrospective review of our surgical database revealed 67 patients with pulmonary atresia with intact ventricular septum operated on between 1981 and 1998. Patients were categorized on the basis of initial surgical strategy: strategy A, aortopulmonary shunt alone (n = 31); strategy B, right
ventricular recruitment (n = 32); strategy C, heart transplantation (n = 4). Tricuspid valve size (Z-score) and coronary anatomy were determined. Right ventricular-coronary artery dependency was noted in 8 patients. RESULTS: Overall actuarial survivals at 1, 5, and 8 years were 82%, 76%, and 76%. Mortality was highest in infancy (10 of 16 deaths). Outcome was equivalent for all 3 strategies. There was no difference in tricuspid valve size between survivors and nonsurvivors (mean Z-score -2.0 (2.5) vs -2.0 (1.9), P =.83). There was no difference in survival based on severity of coronary abnormality. Only one third of patients had a successful biventricular repair, and the tricuspid valve was significantly larger in these than in patients who had Fontan operation (mean Z-score -0.53 [1.6], range - 3.5 to 1, versus mean Z-score -3.03 [2.7], range -5.5 to 0, P =.002). CONCLUSIONS: Surgical outcome for patients born with pulmonary atresia with intact ventricular septum is satisfactory. The strategies of biventricular repair, single ventricle palliation, and heart transplantation allow for equal outcome among all anatomic subtypes.
Institutional address: Divisions of Cardiology and Cardiothoracic Surgery Children's Hospital of Philadelphia PA 19104 USA.
(REFERENCE 47 OF 53) 99054773
Carotti A Di Donato RM Squitieri C Guccione P Catena G Total repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: an integrated approach.
In: J Thorac Cardiovasc Surg (1998 Dec) 116(6):914-23
ISSN: 0022-5223
OBJECTIVE: Rapid reperfusion may be injurious to the ischemic lung. Our aim was to confirm that slow reperfusion improves postischemic pulmonary function and to elucidate the ultrastructural changes associated with slow versus rapid reperfusion. METHODS: We used an ex vivo perfused rat lung transplant model to study the effect of slow versus rapid reperfusion on subsequent lung function and morphologic condition. Functional assessment was performed in (1) fresh lung, slowly reperfused; (2) fresh lung, rapidly reperfused; (3) ischemic lung (4 hours at 22 degreesC), slowly reperfused; and (4) ischemic lung, rapidly reperfused. RESULTS: In group 4, the shunt fraction (P =.001), airway pressure (P =.001), and wet/dry ratio (P =.01) were significantly higher than in groups 1 through 3. Light and electron microscopy of slowly reperfused ischemic lungs (n = 4) appeared normal. Rapidly reperfused ischemic lungs (n = 4) demonstrated massive alveolar edema, hemorrhage, and epithelial "blebbing" by light microscopy. Electron microscopy identified the blebbing as separation of the epithelial layer from an intact basement membrane by edema fluid. The epithelial layer was disrupted in numerous locations. Complete disruption of all layers of the blood-gas barrier was occasionally present. CONCLUSION: Rapid reperfusion of the ischemic lung is an important contributing factor to reperfusion lung injury resulting in mechanical stress failure of the alveolar/capillary barrier. Gradual reintroduction of blood flow to the ischemic lung improves oxygenation.
Institutional address: Division of Pediatric Cardiac Surgery Department of Pediatric Cardiology and Cardiac Surgery Bambino Gesu Hospital Rome Italy.
(REFERENCE 48 OF 53) 95257598
Reddy VM Liddicoat JR Hanley FL Midline one-stage complete unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals.
In: J Thorac Cardiovasc Surg (1995 May) 109(5):832-44; discussion 844-5
ISSN: 0022-5223
Traditionally patients with pulmonary atresia, ventricular septal defect, diminutive or absent central pulmonary arteries, and multiple aortopulmonary collaterals have been managed by staged procedures necessitating multiple operations. We have taken a different approach to this lesion. Between August 1992 and March 1994, ten patients aged 1.43 months to 37.34 years (median 2.08 years) at the severe end of the morphologic spectrum of this lesion underwent a one-stage complete unifocalization and repair from a midline sternotomy approach. The median Nakata index of true pulmonary arteries was 50.0 (range 0 to 103.13) and they provided vascular supply to up to nine lung segments (median 5 segments). The number of collaterals per patient ranged from two to five with a median of four. The collaterals provided vascular supply to a median of 15 lung segments per patient (range 11 to 20). Complete unifocalization was achieved in all patients with emphasis on native tissue-to-tissue connections via anastomosis of collaterals to other collaterals and to the native pulmonary arteries. In only one patient (37.34 years old) was it necessary to use a non-native conduit for peripheral pulmonary artery reconstruction. The ventricular septal defect was left open in one patient (5 years old) because of diffuse distal hypoplasia and stenosis of the pulmonary arteries and the collaterals. The postrepair peak systolic right ventricular/left ventricular pressure ratio ranged from 0.31 to 0.58 (median 0.47). There were no early deaths. Complications were bleeding necessitating reexploration in one patient, phrenic nerve palsy in three patients, and severe bronchospasm in three patients. Follow-up (median 8 months, range 2 to 19 months) was complete in all patients. One patient was reoperated on for pseudoaneurysm of the central homograft conduit and
then again for stenosis of the left lower lobe collateral. After this last operation at 13 months after the initial repair she died of a preventable cardiac arrest caused by pneumothorax. The patient with open ventricular septal defect underwent balloon dilation of the unifocalized pulmonary arteries, with a current pulmonary/systemic flow ratio of 1.4 to 1.8:1, and is awaiting ventricular septal defect closure. One other patient underwent balloon dilation of the reconstructed right pulmonary artery, with a good result. All survivors (9/10) are clinically doing well. This approach establishes normal cardiovascular physiology early in life, eliminates the need for multiple systemic-pulmonary artery shunts and use of prosthetic material, and minimizes the number of operations required.(ABSTRACT TRUNCATED AT 400 WORDS)
Institutional address: Division of Cardiothoracic Surgery University of California at San Francisco USA.
(REFERENCE 49 OF 53) 95019747
DeRuiter MC Gittenberger-de Groot AC Bogers AJ Elzenga NJ The restricted surgical relevance of morphologic criteria to classify systemic-pulmonary collateral arteries in pulmonary atresia with ventricular septal defect.
In: J Thorac Cardiovasc Surg (1994 Oct) 108(4):692-9
ISSN: 0022-5223
Now that systemic-pulmonary collateral arteries are used for unifocalization in patients with pulmonary atresia and ventricular septal defect, the question arises whether morphologic criteria of these collateral arteries could help to provide better results. In an attempt to classify the morphologic features of systemic-pulmonary collateral arteries, we studied 31 heart-lung autopsy specimens with pulmonary atresia and ventricular septal defect. The course of the systemic-pulmonary collateral arteries (origin, branching pattern, and connections with systemic and central pulmonary arteries) was related to their histologic characteristics. The results show that systemic-pulmonary collateral arteries cannot be classified according to their course related to the trachea and the main branches of the bronchi. The histologic features of these collateral arteries vary along their course to the lungs. Nearly all systemic-pulmonary collateral arteries contain a muscular or a musculoelastic segment. One type of collateral artery (complex loop anastomoses) is completely muscular and resembles a bronchial artery. Nutritive branches (bronchial arteries) arise from all histologic types of systemic-pulmonary collateral artery segments. The size and number of intimal proliferations in muscular, elastic, and musculoelastic segments did not differ significantly. In 29 of 31 cases a ductus arteriosus did not coexist with large collateral arteries (two cases unknown). It is concluded that a classification of large systemic- pulmonary collateral arteries based on morphologic features results in a highly variable system, which does not facilitate decisions for the suitability of these arteries for unifocalization procedures. The variability of the systemic-pulmonary collateral arteries corresponds with the recent embryologic finding that during development, collateral artery formation is possible during extended periods.
Institutional address: Department of Anatomy and Embryology University of Leiden The Netherlands.
(REFERENCE 50 OF 53) 98266111
Ovaert C Qureshi SA Rosenthal E Baker EJ Tynan M Growth of the right ventricle after successful transcatheter pulmonary valvotomy in neonates and infants with pulmonary atresia and intact ventricular septum.
In: J Thorac Cardiovasc Surg (1998 May) 115(5):1055-62
ISSN: 0022-5223
OBJECTIVES: Since 1990, transcatheter pulmonary valvotomy has become an alternative to surgical valvotomy in the management of neonates and infants with pulmonary atresia and intact ventricular septum. We sought to determine whether right ventricular growth after transcatheter pulmonary valvotomy is commensurate with body growth. METHODS: Laser or radiofrequency-assisted balloon valvotomy was attempted in 12 neonates and infants with pulmonary atresia and intact ventricular septum. Tricuspid and mitral valve dimensions were measured retrospectively on the cross-sectional echocardiograms performed before the procedure and during follow-up. Z-values were used to standardize tricuspid valve dimensions with body size. RESULTS: The atretic pulmonary valve was successfully perforated and dilated in nine of 12 patients. Five of these nine patients required additional transcatheter or surgical procedures to augment the pulmonary blood flow. Of six survivors, five are regularly followed up with a median follow-up of 60 months (range 37 to 68 months). All five have two-ventricle circulation, two of the five patients requiring surgical enlargement of the right ventricular outflow tract with or without closure of the atrial septal defect. Echocardiographic tricuspid valve dimensions and Z-values before transcatheter valvotomy tended to be smaller in the patients who died than in the survivors. In the survivors, the absolute tricuspid valve dimensions increased after valvotomy but the Z-values tended to decrease or stayed constant. CONCLUSIONS: Transcatheter valvotomy is a good alternative to surgical valvotomy in patients with pulmonary atresia and intact ventricular septum. Two-ventricle circulation can be achieved despite subnormal right ventricular growth.
Institutional address: Department of Paediatric Cardiology Guy's Hospital London United Kingdom.
(REFERENCE 51 OF 53) 96350090
Yagihara T Yamamoto F Nishigaki K Matsuki O Uemura H Isizaka T Takahashi O Kamiya T Kawashima Y Unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.
In: J Thorac Cardiovasc Surg (1996 Aug) 112(2):392-402
ISSN: 0022-5223
To extend the indications for corrective operation in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries, surgical procedures were done to unify the blood sources for pulmonary perfusion. Since December 1985, 50 patients have undergone unifocalization at ages from 2 months to 26 years with a mean of 6 +/- 7 years. In total, 84 staged unifocalization procedures and 5 other palliative procedures were done in 49 patients. These included several operative procedures: simple ligation of major aortopulmonary collateral arteries in 8; pulmonary angioplasty in 29 including reconstruction of the pulmonary arterial tree by direct anastomosis or interposition between the central pulmonary arteries and the intrapulmonary arteries; construction of artificial central pulmonary arteries with use of a xenograft pericardial tube graft in 36 with no native central pulmonary arteries detected; and construction of supplemental central pulmonary arteries also with use of a pericardial tube graft in 10. The pericardial tube graft, if used, was anastomosed to the intrapulmonary arteries on one end and connected to a prosthetic tube on the other end so as to perfuse the reconstructed pulmonary arteries. The anastomosis was made inside the lung through the divided interlobar fissure. Five patients died after operation among those undergoing these 89 preparative operative procedures. Deaths were related either to bleeding caused by anticoagulation therapy administered to prevent thrombosis within the xenograft pericardial tube graft used or to progressive congestive heart failure as a result of an excessive amount of pulmonary blood flow. Twenty-six patients have undergone intracardiac repair after previous unifocalization. In 16 patients the artificial central pulmonary
arteries surgically constructed were connected to each other and then an external conduit was placed. In another patient, intracardiac repair and unifocalization could be concomitantly achieved via a median sternotomy. The right ventricle to left ventricle systolic pressure ratio immediately after intracardiac repair in 27 patients ranged from 0.24 to 0.91 with a mean of 0.54 +/- 0.17. One patient (4%) died shortly after intracardiac repair because of thrombosis within the pulmonary arteries. Postoperative catheterization showed that pulmonary vascular resistance was correlated significantly with the number of pulmonary vascular segments functioning rather than with the condition of the central pulmonary arteries. We conclude that surgical unifocalization is a feasible procedure before subsequent intracardiac repair, even in patients with critically hypoplastic or absent central pulmonary arteries.
Institutional address: Department of Cardiovascular Surgery National Cardiovascular Center Osaka
Japan.
(REFERENCE 52 OF 53) 96202377
McKay R Anderson RH Smith A The coronary arteries in hearts with discordant atrioventricular connections.
In: J Thorac Cardiovasc Surg (1996 May) 111(5):988-97
ISSN: 0022-5223
Gross morphologic study of 14 hearts with congenitally corrected transposition or discordant atrioventricular connections and double- outlet right ventricle or pulmonary atresia disclosed consistently the origins of the coronary arteries from two aortic sinuses. With usual atrial arrangement, the artery arising in sinus 1 (right-hand facing), as seen from the noncoronary aortic sinus, supplied the morphologically right ventricle. Coronary blood supply to the morphologically left ventricle usually came from sinus 2 (left-hand facing), although in two hearts the circumflex branch was a continuation of the artery from sinus 1. The sinus nodal artery arose from the circumflex coronary artery, and histologic studies of two hearts demonstrated that blood supply to the anterior atrioventricular node also came from this vessel. Early branching and entrapment in fat or right ventricular muscle, as well as malalignment of aortic and pulmonary sinuses, occurred frequently. These findings may have implications for coronary arterial transfer in the double switch operation in hearts with discordant atrioventricular connections.
Institutional address: Institute of Child Health Alder Hey Children's Hospital Liverpool England.
(REFERENCE 53 OF 53) 96074403
Pagani FD Cheatham JP Beekman RH 3rd Lloyd TR Mosca RS Bove EL The management of tetralogy of Fallot with pulmonary atresia and diminutive pulmonary arteries.
In: J Thorac Cardiovasc Surg (1995 Nov) 110(5):1521-32; discussion 1532-3
ISSN: 0022-5223
Since September 1991, 14 consecutive patients with tetralogy of Fallot, pulmonary atresia, and diminutive pulmonary arteries have undergone staged repair. All patients had multiple aortopulmonary collateral arteries and the ductus arteriosus was absent in 11. Mean sizes of the right and left pulmonary arteries were 2.2 +/- 0.7 mm and 1.9 +/- 0.8 mm, respectively (range 0.5 to 3.0 mm). Eight patients (57%) have subsequently received complete repair. Age at initial procedure (shunt, right ventricle-pulmonary artery conduit, or direct aorta-pulmonary artery anastomosis) in this group was 5.3 +/- 6.8 months. The number of operative procedures to achieve complete repair was 2.9 +/- 0.8 per patient (range 2 to 4). Intraoperative postrepair peak right ventricle-left ventricle pressure ratio was 0.57 +/- 0.17. Six of 8 patients (75%) required additional interventional procedures (mean 1.5 +/- 1.2 per patient) for angioplasty of peripheral pulmonary artery stenoses, coil embolization of aortopulmonary collateral arteries, or intra- operative insertion of intravascular pulmonary artery stents. Mean follow-up from complete repair was 8.7 +/- 8.3 months (range 0.5 to 23.8 months) and is complete. There was one in-hospital death at 45 days, and one late cardiac death at 20.3 months. Six patients had initial palliative operations (unifocalization, right ventricle- pulmonary artery conduit, direct aorta-pulmonary artery anastomosis, or transannular outflow patch) but have not undergone complete repair. Age at initial procedure in this group was 27.9 +/- 56.9 months (range 0.27 to 155 months), and mean follow-up from initial procedure was 10.9 +/- 11.2 months (range 0 to 31.4 months). The operative mortality rate was 33% (2 of 6 patients). There was one late noncardiac death at 5.3 months. Three patients are awaiting further intervention or repair. This experience suggests that complete repair is feasible even in patients with extremely diminutive pulmonary arteries (< or = 3.0 mm). Pulmonary artery growth is facilitated by early (3 to 6 month) establishment of central pulmonary artery flow by right ventricle-pulmonary artery conduit (pulmonary arteries > 1.5 mm) or by direct ascending aorta- pulmonary artery anastomosis (pulmonary arteries < 1.5 mm). Subsequent interventional catheterization and operative procedures as
required for pulmonary artery stenoses and coil embolization of collateral arteries allow continued recruitment of central pulmonary arteries and may obviate or minimize the need for unifocalization procedures.
Institutional address: Department of Surgery C. S. Mott Children's Hospital University of Michigan School of Medicine Ann Arbor 48109 USA.