*****AMERICAN JOURNAL OF CARDIOLOGY*****
(REFERENCE 1 OF 13) 95159900
Sandhu SK Beekman RH Mosca RS Bove EL Single-stage repair of aortic arch obstruction and associated intracardiac defects in the neonate.
In: Am J Cardiol (1995 Feb 15) 75(5):370-3
ISSN: 0002-9149
The effectiveness of a single-stage anterior approach for the repair of aortic arch obstruction and associated intracardiac defects has not been well evaluated. We therefore reviewed our experience with 60 neonates (median age 8 days, range 1 to 28) who underwent a single- stage repair by way of a median sternotomy at our institution between 1986 and 1994. Nineteen (32%) had coarctation with ventricular septal defect, 18 (30%) had interrupted aortic arch with ventricular septal defect, and 23 (38%) had coarctation or interrupted aortic arch with complex intracardiac anatomy. The arch obstruction was repaired using resection and primary anastomosis in 54 patients, synthetic patch aortoplasty in 3, subclavian flap aortoplasty in 2, and an interposition gortex graft placement in 1. Total circulatory arrest time was 48 +/- 3 minutes (mean +/- SEM). There were 7 early postoperative deaths (11.7%; 70% confidence limit 8% to 16.6%). The 53 survivors were followed for a mean of 23 months (range 1 to 78), for a total of 1,219 patient-months. Recurrent arch obstruction > or = 20 mm Hg has occurred in 2 of 53 patients (3.8%; 70% confidence limit 1.9% to 7.5%); both underwent successful balloon angioplasty. There were 2 late deaths, 1 of which was noncardiac. We conclude that repair of aortic arch obstruction and intracardiac defects by a single-stage approach through median sternotomy can be accomplished with low mortality in infancy, even with associated complex intra- cardiac anatomy. Recurrent coarctation is relatively uncommon and can be successfully managed with balloon angioplasty.
Institutional address: Department of Pediatrics University of Michigan School of Medicine Ann Arbor.
(REFERENCE 2 OF 13) 97430026
Lewin MB Lindsay EA Jurecic V Goytia V Towbin JA Baldini A A genetic etiology for interruption of the aortic arch type B.
In: Am J Cardiol (1997 Aug 15) 80(4):493-7
ISSN: 0002-9149
Interrupted aortic arch (IAA) type B is a congenital heart defect believed to be caused by an anomaly of bronchial arch mesenchymal development. IAA type B has been associated with DiGeorge syndrome (DGS), which includes conotruncal heart defects, T-cell immunodeficiency, hypocalcemia, and facial abnormalities. The great majority of DGS cases are associated with hemizygous deletions at the chromosome 22q11 locus. The present study was designed to establish the involvement of the 22q11 locus in the etiology of IAA type B, independently from the typical DGS phenotype. An evaluation was performed on 73 patients with conotruncal heart defects using fluorescence in situ hybridization (FISH) analysis with probes from the 22q11 DGS locus. From this group, 7 patients were deleted (including 4 of the 11 patients with IAA type B). FISH analysis was extended to a total of 22 patients with IAA type B and 11 of these (50%) were deleted. FISH and Southern blot analyses using additional markers within the DiGeorge chromosomal region were performed on patients found not to be deleted in the initial FISH screening. No small deletions or rearrangements were detected. In our patient population, a single, specific genetic defect is the basis for one half of the IAA type B cases. These data suggest that IAA type B is one of the most etiologically homogeneous congenital heart defects. A 22q11 deletion in IAA type B may or may not be associated with the typical DGS phenotype. Therefore, IAA type B, per se, should be an indication for 22q11 deletion testing.
Institutional address: Department of Pediatrics Texas Children's Hospital Baylor College of Medicine Houston 77030 USA.
(REFERENCE 3 OF 13) 96399977
Momma K Kondo C Matsuoka R Takao A Cardiac anomalies associated with a chromosome 22q11 deletion in patients with conotruncal anomaly face syndrome.
In: Am J Cardiol (1996 Sep 1) 78(5):591-4
ISSN: 0002-9149
Among 114 cardiac patients with conotruncal anomaly face syndrome and DiGeorge syndrome, 100 patients were found to have a chromosome 22q11 deletion. Those with the deletion included 73 patients with tetralogy of Fallot, 12 with ventricular septal defect, 5 with aortic arch anomalies without intracardiac anomaly, 4 with interrupted aortic arch, 2 with double-outlet right ventricle, 2 with truncus arteriosus, 1 with complete transposition, and 1 with atrial septal defect.
Institutional address: Department of Pediatric Cardiology Tokyo Women's Medical College Japan.
*****ANNALS OF OTOLOGY, RHINOLOGY AND LARYNGOLOGY*****
(REFERENCE 4 OF 13) 93207291
Davis DA Tucker JA Russo P Management of airway obstruction in patients with congenital heart defects.
In: Ann Otol Rhinol Laryngol (1993 Mar) 102(3 Pt 1):163-6
ISSN: 0003-4894
Airway obstruction may complicate the course of infants undergoing repair of congenital heart disease. Airway obstruction was encountered in seven patients following surgery for complex congenital heart defects (two with interrupted aortic arch, one with pulmonary atresia and a ventricular septal defect, one with tetralogy of Fallot and an abnormal left anterior descending coronary artery, one with truncus arteriosus, and two with complex univentricular heart). In four patients, a conduit was implanted at the time of surgery. In all cases, bronchoscopy accurately demonstrated the cause of the airway obstruction. Two infants had hypoplasia of the left bronchus, and five had tracheobronchomalacia associated with extrinsic compression of the airway by a great vessel or conduit. All were treated conservatively with therapeutic bronchoscopy, tracheotomy, and/or stenting with prolonged mechanical ventilation. Follow-up ranged from 2 to 40 months. Four infants have been weaned from mechanical ventilation and decannulated; two are awaiting decannulation; and one was decannulated and died awaiting surgical repair. To reduce morbidity in infants undergoing surgery for congenital heart disease, the presence of preoperative airway anomalies should be sought and surgery planned to avoid airway compression. Conservative management using diagnostic and therapeutic bronchoscopy, tracheotomy, and stenting together with prolonged ventilator care is rewarding.
Institutional address: Pediatric Heart Institute Temple University School of Medicine Philadelphia Pennsylvania.
*****ANNALS OF THORACIC SURGERY*****
(REFERENCE 5 OF 13) 95150623
Kanter KR Miller BE Cuadrado AG Vincent RN Successful application of the Norwood procedure for infants without hypoplastic left heart syndrome.
In: Ann Thorac Surg (1995 Feb) 59(2):301-4
ISSN: 0003-4975
Although the first-stage Norwood procedure mostly has been used for hypoplastic left heart syndrome, there are other anomalies in which the Norwood procedure can be applied. Since 1991, 18 newborns without hypoplastic left heart syndrome underwent a first-stage Norwood procedure. All had a hypoplastic aortic annulus, ascending aorta, and transverse aorta. Ten had normally related great arteries: aortic atresia or aortic stenosis with inadequate left ventricle in 4, mitral atresia or stenosis in 4, and interrupted aortic arch in 2. The 8 others had double-outlet right ventricle with mitral atresia or complete transposition with a hypoplastic right ventricle. Age ranged from 2 to 77 days (median, 6 days) and weight from 2.4 to 4.4 kg (mean, 3.0 kg). The patients with interrupted aortic arch simultaneously underwent primary repair of the interruption. There were 17 hospital survivors (94%). There have been no late deaths in follow-up from 4 to 30 months (mean, 15.5 months). Thirteen children have had subsequent creation of a bidirectional Glenn shunt with takedown of the original systemic to pulmonary shunt. The 2 with interrupted aortic arch underwent a Rastelli-type biventricular repair. These results show that the Norwood procedure can be applied to infants without hypoplastic left heart syndrome who have hypoplastic aortas and excessive pulmonary blood flow with very low mortality and excellent palliation.
Institutional address: Emory University School of Medicine Atlanta Georgia.
(REFERENCE 6 OF 13) 96201793
Asou T Kado H Imoto Y Shiokawa Y Tominaga R Kawachi Y Yasui H Selective cerebral perfusion technique during aortic arch repair in neonates.
In: Ann Thorac Surg (1996 May) 61(5):1546-8
ISSN: 0003-4975
We describe selective cerebral perfusion techniques for repair of the aortic arch in neonates. These techniques may help protect the brain from ischemic injury caused by a cessation of cerebral perfusion for aortic arch reconstruction in patients with hypoplastic left heart syndrome or interrupted aortic arch.
Institutional address: Department of Cardiovascular Surgery Fukuoka Children's Hospital Japan.
*****ARCHIVES OF PEDIATRICS AND ADOLESCENT MEDICINE*****
(REFERENCE 7 OF 13) 95316122
Miller G Eggli KD Contant C Baylen BG Myers JL Postoperative neurologic complications after open heart surgery on young infants [see comments]
In: Arch Pediatr Adolesc Med (1995 Jul) 149(7):764-8
ISSN: 1072-4710
OBJECTIVE: To ascertain the relation between postoperative neurologic complications and variables occurring before, during, and after hypothermic cardiopulmonary bypass surgery to correct congenital heart disease in young infants. DESIGN: Prospective analysis of mortality and neurologic morbidity before hospital discharge; systematic comparison with patient characteristics, metabolic status, surgery variables; and preoperative neurologic findings of the patients. SETTING: Intensive care unit in tertiary care center. PATIENTS: Consecutive sample of 91 full-term infants who underwent 100 operations between January 1989 through December 1992. Nine infants had more than one operation during the study period. MAIN OUTCOME MEASURES: Levels of alertness, tone, focal signs, dyskinesia, pyramidal signs, seizures, and death. RESULTS: Reduced level of alertness at discharge from the hospital in 19% of patients; seizures in 15% (70% focal); severe hypotonia in 11% before surgery, and in 7% at discharge from hospital; generalized pyramidal findings in six (7%); asymmetry of tone in 5%; and chorea that did not persist in 11%. Results of cranial ultrasound tests were abnormal in 20% of patients. Of these those with abnormal cranial ultrasound examinations 55% were abnormal before surgery. Overall mortality was 18%. Of the patients who died, 59% had interrupted aortic arch or hypoplastic left heart syndrome. Mortality for patients with these lesions was 40%. Alertness (P = .005), chorea (P = .03), and hypotonia (P = .02) were associated with duration of deep hypothermia longer than 60 minutes. No association was found among other outcomes and study variables, except the relation between severe left-sided heart lesions and mortality. CONCLUSIONS: Mortality and neurologic morbidity after open heart surgery on young infants may be due to several factors, including type of lesion, preexisting brain abnormalities, duration of deep hypothermia, and strokes.
Comment in: Arch Pediatr Adolesc Med 1996 May;150(5):560-1
Institutional address: Section of Pediatric Neurology Baylor College of Medicine Houston Tex. USA.
*****CIRCULATION*****
(REFERENCE 8 OF 13) 98045886
Lacour-Gayet F Serraf A Galletti L Bruniaux J Belli E Piot D Touchot A Petit J Houyel L Planche C Biventricular repair of conotruncal anomalies associated with aortic arch obstruction: 103 patients.
In: Circulation (1997 Nov 4) 96(9 Suppl):II-328-34
ISSN: 0009-7322
BACKGROUND: Biventricular repair of conotruncal anomalies associated with aortic arch obstruction is a complex surgical procedure that combines a cardiac repair and a aortic arch reconstruction. METHODS AND RESULTS: From January 1984 to April 1996, such a repair was performed in 103 patients. The conotruncal anomalies included: 15 transpositions of the great arteries (TGAs) with intact ventricular septum, 44 TGAs with ventricular septal defect, 32 double outlet right ventricle with subpulmonary ventricular septal defect, 10 truncus arteriosus, one double outlet left ventricle, and one tetralogy of Fallot. The arch obstruction included 88 coarctation and 15 interrupted aortic arch. One-stage repair has been the favored technique since 1990 and was performed in 58 neonates, including 38 TGAs or double outlet right ventricle and ventricular septal defect, 10 TGAs with intact ventricular septum, and all of the 10 truncus arteriosus. The cardiac repair included 89 arterial switch operations, 2 Kawashima rerouting, 10 truncus arteriosus repairs, and one double-outlet left ventricle repair and one tetralogy of Fallot repair. The aortic arch was reconstructed by direct anastomosis in 85 patients, with a Gore-Tex conduit in three patients and more recently by an ascending aortic patch augmentation in 15 patients. The hospital mortality was 12% (7 of 58) for the one-stage repair and 20% (9 of 45) for the two-stage repair. There were six late deaths. Reoperations or angioplasties were mandatory for 12 right ventricle outflow tract obstructions after arterial switch, involving 10 patients with double outlet right ventricle (P=.02), 10 recurrent arch obstruction, and six miscellaneous lesions. CONCLUSIONS: One- stage biventricular repair of conotruncal anomalies associated with aortic arch obstruction can be achieved in selected patients with an 83% survival rate at 7 years.
Institutional address: Pediatric Cardiac Surgery Department of Pr Cl. Plance Marie Lannelongue Hospital Paris Sud University France. flacourg@pratique.fr
*****JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY*****
(REFERENCE 9 OF 13) 98372186
Goldmuntz E Clark BJ Mitchell LE Jawad AF Cuneo BF Reed L McDonald-McGinn D Chien P Feuer J Zackai EH Emanuel BS Driscoll DA Frequency of 22q11 deletions in patients with conotruncal defects [see comments]
In: J Am Coll Cardiol (1998 Aug) 32(2):492-8
ISSN: 0735-1097
OBJECTIVES: This study was designed to determine the frequency of 22q11 deletions in a large, prospectively ascertained sample of patients with conotruncal defects and to evaluate the deletion frequency when additional cardiac findings are also considered. BACKGROUND: Chromosome 22q11 deletions are present in the majority of patients with DiGeorge, velocardiofacial and conotruncal anomaly face syndromes in which conotruncal defects are a cardinal feature. Previous studies suggest that a substantial number of patients with congenital heart disease have a 22q11 deletion. METHODS: Two hundred fifty-one patients with conotruncal defects were prospectively enrolled into the study and screened for the presence of a 22q11 deletion. RESULTS: Deletions were found in 50.0% with interrupted aortic arch (IAA), 34.5% of patients with truncus arteriosus (TA), and 15.9% with tetralogy of Fallot (TOF). Two of 6 patients with a posterior malalignment type ventricular septal defect (PMVSD) and only 1 of 20 patients with double outlet right ventricle were found to have a 22q11 deletion. None of the 45 patients with transposition of the great arteries had a deletion. The frequency of 22q11 deletions was higher in patients with anomalies of the pulmonary arteries, aortic arch or its major branches as compared to patients with a normal left aortic arch regardless of intracardiac anatomy. CONCLUSIONS: A substantial proportion of patients with IAA, TA, TOF and PMVSD have a deletion of chromosome 22q11. Deletions are more common in patients with aortic arch or vessel anomalies. These results begin to define guidelines for deletion screening of patients with conotruncal defects.
Comment in: J Am Coll Cardiol 1998 Aug;32(2):499-501
Institutional address: The Children's Hospital of Philadelphia Department of Pediatrics University of Pennsylvania Medical Center 19104 USA. goldmuntz@email.chop.edu
(REFERENCE 10 OF 13) 96152320
Berning RA Silverman NH Villegas M Sahn DJ Martin GR Rice MJ Reversed shunting across the ductus arteriosus or atrial septum in utero heralds severe congenital heart disease.
In: J Am Coll Cardiol (1996 Feb) 27(2):481-6
ISSN: 0735-1097
OBJECTIVES. This study was performed to define the significance of Doppler color flow mapping in demonstrating reversal of the direction of the normal physiologic flow across the atrial septum and ductus arteriosus in the human fetus. BACKGROUND. Reversal of the physiologic shunting across the ductus arteriosus or atrial septum in utero (i.e., left to right) can be readily identified by Doppler color flow mapping, complemented by pulsed and continuous wave Doppler information. METHODS. We reviewed echocardiograms recorded at our three institutions from 1988 to 1993, which displayed reversal of flow by Doppler color flow in 53 fetuses of gestational age 18 weeks to term. The diagnoses were confirmed by postnatal echocardiography, operation or autopsy. Reversal of shunting was consistently associated with severe heart disease. RESULTS. Reversed atrial shunting was found with severe left heart obstructive lesions, including 19 with hypoplastic left heart syndrome, 3 with critical aortic stenosis, 2 with double-outlet right ventricle and 1 each with an interrupted aortic arch, atrioventricular septal defect and severe left ventricular dysfunction due to dilated cardiomyopathy. Reversed ductus arteriosus shunting was found with severe right heart obstructive lesions, including nine fetuses with pulmonary atresia, six with severe obstructive tricuspid valve abnormalities, five with severe tetralogy of Fallot, four with Ebstein's anomaly and two with single ventricle and pulmonary stenosis. Associated cardiac lesions were common in both groups. Only 3 of the 15 infants who were delivered alive from the reverse ductus arteriosus shunt group and 4 of 12 from the reverse atrial shunt group still survive. CONCLUSIONS. The finding of reversed flow by Doppler color flow mapping during fetal life provides a key to subsequent accurate diagnosis and denotes a spectrum of diseases with a very poor prognosis.
Institutional address: University of California San Francisco 94143-0214 USA.
*****JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY*****
(REFERENCE 11 OF 13) 97067983
Serraf A Lacour-Gayet F Robotin M Bruniaux J Sousa-Uva M Roussin R Planche C Repair of interrupted aortic arch: a ten-year experience [see comments]
In: J Thorac Cardiovasc Surg (1996 Nov) 112(5):1150-60
ISSN: 0022-5223
Eighty-two consecutive patients with interrupted aortic arch were referred to our institution between 1985 and 1995. Three died before any attempt at operation and 79 underwent surgical repair. Median age at operation was 9 days (range 1 day to 6 years) and median weight was 3.0 kg (range 1.8 to 20 kg). All but one were in severe congestive heart failure and 31.5% had oliguria or anuria. Preoperative pH varied between 6.8 and 7.4 (median 7.3). Sixty-nine received prostaglandin E1 infusion and 54 received mechanical ventilation. Aggressive preoperative ressucitation was necessary in 43 cases. Preoperative transfontanellar echography (performed routinely) since 1987 revealed intracerebral bleeding in six patients. Type A interrupted aortic arch was present in 37 cases, 41 patients had type B, and one had type C. Interrupted aortic arch was associated with single ventricular septal defect in 35 cases, 24 patients had associated complex heart defects, and 30 had significant subaortic stenosis (six had both subaortic stenosis and complex association). Aortopulmonary window was found in four patients, truncus arteriosus was found in eight, and transposition of the great arteries was found in five, double-outlet right ventricle was found in one, single ventricle was found in three, multiple ventricular septal defects were found in two and superior-inferior ventricles were found in one. Sixty-four patients underwent single-stage repair and 15 underwent multistage repair. Aortic arch repair consisted of direct anastomosis in 59 cases, patch augmentation in eight, and conduit interposition in 12. Ten patients underwent associated pulmonary artery banding and 19 underwent concomitant repair of complex associated lesions. The subaortic stenosis was addressed by four surgical techniques: myotomy or myectomy in five patients; creation of a double-outlet left ventricle, aortopulmonary anastomosis, and conduit insertion between the right ventricle and pulmonary artery bifurcation in four; no direct attempt to relieve the subaortic stenosis in six; and left-sided ventricular septal defect patch in 15. Mean duration of deep hypothermic circulatory arrest, crossclamp time, and cardiopulmonary bypass time were 38.8 +/- 15.6 min, 60.5 +/- 24.7 min, and 143 +/- 40.1 min, respectively. Postoperative mortality rate was 18.9% (70% confidence limits 14% to 24.6%), and overall mortality rate was 31% (70% confidence limits 20.9% to 42.2%). The results have improved with time, with an overall operative mortality rate of 12% since 1990. Univariate statistical analysis revealed that early survival was influenced by preoperative renal function, detection of cerebral bleeding by transfontanellar echography, the number of cardioplegic injections, and the date of operation. Multivariate analysis revealed that preoperative renal function and the number of cardioplegic injections were independent risk factors for early mortality. Echocardiographic measurements of the left heart-aorta complex with preoperative Z values as low as-4 demonstrated rapid growth after repair. In the presence of subaortic stenosis, better survival was obtained with a left-sided patch for ventricular septal defect closure (p < 0.05). Twenty-three patients underwent 26 reoperations for recoarctations (seven), left bronchial compression (two), second-stage repair (eight), right ventricle- pulmonary artery conduit replacement (three), and miscellaneous (four). One of the survivors was reoperated on for subaortic membrane. Survival at 5 years for the entire series was 70%. For isolated forms, it was 73.5% (90% for 1990 to 1995), for complex forms it was 70%, and in the presence of subaortic stenosis it was 60%. In conclusion, interrupted aortic arch remains a surgical challenge with continually improving results. Early diagnosis with preoperative resuscitation and adequate myocardial protection seem extremely important for further improvements. Associated subaortic stenosis or complex lesions
Comment in: J Thorac Cardiovasc Surg 1998 Jan;115(1):266-7
Institutional address: Department of Pediatric Cardiac Surgery Marie-Lannelongue Hospital Le Plessis-Robinson France.
(REFERENCE 12 OF 13) 95295338
al-Marsafawy HM Ho SY Redington AN Anderson RH The relationship of the outlet septum to the aortic outflow tract in hearts with interruption of the aortic arch.
In: J Thorac Cardiovasc Surg (1995 Jun) 109(6):1225-36
ISSN: 0022-5223
We examined 13 hearts with concordant atrioventricular and ventriculoarterial connections and interruption of the aortic arch to establish and describe the morphologic features of the outflow tracts in relation to axial deviation and malalignment of the outlet septum as opposed to overriding of the arterial valvular orifices. Interruption in all cases but one was between the left common carotid and left subclavian arteries; the other arch was interrupted at the isthmus. A patent arterial duct and ventricular septal defect were universally present. When its borders were viewed from the right ventricle, the ventricular septal defect was perimembranous in seven hearts, had exclusively muscular borders in four hearts, and was doubly committed and juxta-arterial in the remaining two hearts. Malalignment between the muscular ventricular septum and outlet septum, or a fibrous raphe, as judged when the heart was viewed in its short axis, was found in 12 of the hearts. Posterior and leftward axial deviation of the outlet septum in its long axis was found in 4 of the 12 hearts and also in one heart that did not have short-axis malalignment. Attachments of the leaflets of the pulmonary valve in both right and left ventricles, however, were present in only one of the specimens, this being a case with a doubly committed and juxta- arterial defect. These separate features of the outflow tract in hearts with interruption of the aortic arch, therefore, require thorough assessment when surgical management is planned. All these variable features can be assessed preoperatively by cross-sectional echocardiography, which should be directed toward defining the degree of development and alignment of the outlet septum, as well as the length of the subpulmonary infundibulum.
Institutional address: Department of Paediatrics Royal Brompton Hospital London England.
(REFERENCE 13 OF 13) 96074406
Jacobs ML Rychik J Murphy JD Nicolson SC Steven JM Norwood WI Results of Norwood's operation for lesions other than hypoplastic left heart syndrome.
In: J Thorac Cardiovasc Surg (1995 Nov) 110(5):1555-61; discussion 1561-2
ISSN: 0022-5223
Norwood's operation provides satisfactory palliation for neonates with hypoplastic left heart syndrome. The dominant physiologic features of hypoplastic left heart syndrome, ductal dependency of the systemic circulation and parallel pulmonary and systemic circulations, are shared by a multitude of other less common congenital heart malformations. Theoretically, these should be equally amenable to palliation by Norwood's operation. Between January 1990 and June 1994, 60 neonates with malformations other than hypoplastic left heart syndrome underwent initial surgical palliation by Norwood's procedure. Diagnoses included single left ventricle with levo-transposition of the great arteries (12); critical aortic stenosis (8); complex double-outlet right ventricle (8); interrupted aortic arch with ventricular septal defect and subaortic stenosis (7); ventricular septal defect, subaortic stenosis, and coarctation of the aorta (7); aortic atresia with large ventricular septal defect (6); tricuspid atresia with transposition of the great arteries (6); heterotaxy syndrome with subaortic obstruction (3); and other (3). There were 10 hospital deaths and 50 survivors (83% survival). After the introduction of inspired carbon dioxide therapy into the postoperative management protocol (1991), 42 of 47 patients survived (89% survival). Mortality was independent of diagnosis and essentially the same as that for hypoplastic left heart syndrome. With minor technical modifications, Norwood's operation provides satisfactory initial palliation for a wide variety of malformations characterized by ductal dependency of the systemic circulation in anticipation of either a Fontan procedure or a biventricular repair.
Institutional address: Division of Cardiothoracic Surgery Children's Hospital of Philadelphia PA 19104 USA.