*****AMERICAN JOURNAL OF CARDIOLOGY*****
(REFERENCE 1 OF 16) 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 16) 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.
*****ANNALS OF THORACIC SURGERY*****
(REFERENCE 3 OF 16) 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 4 OF 16) 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 5 OF 16) 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 6 OF 16) 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 7 OF 16) 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.
*****CIRCULATION*****
(REFERENCE 8 OF 16) 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 9 OF 16) 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 10 OF 16) 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 11 OF 16) 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 12 OF 16) 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 13 OF 16) 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 14 OF 16) 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 15 OF 16) 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.
*****JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY*****
(REFERENCE 16 OF 16) 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.