Jerry D. Razook

Repair of Ebstein's anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up.

BACKGROUND Ebsteins anomaly in the severely symptomatic neonate is usually fatal. Until recently, successful repair has not been reported and various palliative operations have been associated with prohibitive mortality. Recently, we published our initial results with biventricular repair in 3 severely symptomatic neonates. We now update our experience with emphasis on the evolution of our surgical technique and the medium-term follow-up of these patients. METHODS Since 1994, 8 severely symptomatic neonates and young infants underwent biventricular repair by one surgeon. Six had Ebsteins anomaly and 2 had physiologically similar pathology with severe tricuspid valve dysplasia, cyanosis, and gross cardiomegaly. One Ebstein patient (2 months old) had undergone a Starnes operation elsewhere. Weight of the patients at operation ranged from 2.1 to 6.4 kg (mean 2.7 kg). Five patients had either anatomical (n = 3) or functional (n = 2) pulmonary atresia. Severe (4/4) tricuspid regurgitation was present in all except 1 (Starnes operation), and cardiothoracic ratio exceeded 0.85 in all patients. Echocardiography severity scores were >1.5 in 6 (grade 4/4) and 1.3 in 1 (grade 3/4). Repair consisted of tricuspid valve repair, reduction atrioplasty, relief of right ventricular outflow tract obstruction, partial closure of atrial septal defect, and correction of all associated cardiac defects. Technique of tricuspid valve repair evolved over time: 3 had Danielson-type repairs, 3 had DeVega-type repairs, and 2 had complex repairs. RESULTS One patient died in hospital: a 2.1 kg patient with tricuspid dysplasia, anatomical pulmonary atresia, and hypoplastic pulmonary arteries. The other 7 patients are all in functional class I and in sinus rhythm. Although 3 patients had symptomatic tachyarrhythmias before surgery, no child has experienced SVT after discharge. At recent echocardiography 4 patients had mild tricuspid regurgitation, and 2 had mild-moderate (2/4) tricuspid regurgitation. Three patients are now 7 years old, 2 are almost 2 years old, and the remaining 2 patients are 1 year old. CONCLUSIONS Surgical repair of the severely symptomatic neonate with Ebsteins anomaly is feasible and safe. The repair appears durable and with good medium-term outcome.

Neonatal repair of Ebstein's anomaly: indications, surgical technique, and medium-term follow-up.

BACKGROUND Ebsteins anomaly in the severely symptomatic neonate is usually fatal. Because the mortality for various surgical interventions has been prohibitively high, the indications for operation in these critically ill neonates are unclear. METHODS We reviewed our results with biventricular repair of three consecutive severely symptomatic neonates (2.8 to 3.2 kg) at our institution since 1994. Each had associated complex cardiac pathology, including multiple muscular ventricular septal defects (n = 1), pulmonary stenosis with functional pulmonary atresia (n = 1), and anatomic pulmonary atresia (n = 1). Preoperatively, all infants had severe tricuspid regurgitation, Great Ormond Street Ebstein echocardiogram scores greater than 1.3:1 (grade 3 or 4) and cardiothoracic ratio greater than 0.85. Two patients were severely cyanotic. Hepatic and renal insufficiency with diffuse coagulopathy was present preoperatively in two patients. Surgical repair consisted of (1) reconstruction of a competent monocuspid tricuspid valve, (2) right ventriculorrhaphy, (3) subtotal closure of atrial septal defect (ASD), (4) aggressive reduction atrioplasty, and (5) repair of all associated cardiac defects. RESULTS There were no early or late deaths. All patients are currently asymptomatic, without medications, and in sinus rhythm. At 5-year follow-up, trivial tricuspid regurgitation is present in 1 and mild regurgitation in 2 patients. On the basis of these results and review of the current literature, we propose new indications for surgical repair in the neonate with Ebsteins anomaly. CONCLUSIONS Biventricular repair of Ebsteins anomaly in the critically ill neonate is feasible and medium-term durability of the repair is excellent. Therefore, conventional management of these patients should be revised and early surgical repair encouraged.

Pulmonary autograft replacement in children. The ideal solution

Fifty-one children, aged 1.8 to 21 years (mean, 11.4) with aortic valve replacement using a pulmonary autograft are reviewed. Twenty-nine were intra-aortic implants and 22 were root replacements. There was one operative death, no late deaths, and two have required reoperation. Actuarial freedom from reoperation was 93% +/- 5.5 at 5.6 years. Freedom from progression of aortic insufficiency (AI) was 81% +/- 9 at 5.6 years in the intra-aortic implants and 86% +/- 10 in the root replacement. Enlargement of the pulmonary autograft was seen echocardiographically in both groups. This enlargement was consistent with somatic growth and not associated with progression of AI. Ten of 19 patients with aortic stenosis had an LV mass index suggestive of LV hypertrophy before operation. At 1 year, 18 of 25 had a normal LV mass index. Thirteen of 16 patients with AI had preoperative abnormal LV mass index. All but four returned to normal by 1 year. Low operative risk, excellent function, resolution of abnormal LV hemodynamics, and enlargement consistent with somatic growth suggest that the pulmonary autograft is the ideal replacement for the malfunctioning aortic valve.

Normal left ventricular function following pulmonary autograft replacement of the aortic valve in children.

To assess growth potential and hemodynamic sequelae of pulmonary autograft valves implanted into aortic outflow tracts of children, we reviewed our experience with 37 patients (2‐21 years) from August 1986 to December 1990. Twenty patients had predominantly aortic stenosis (AS), and 17 had aortic insufficiency (AI). Operative mortality was 3%. Two technical failures required reoperation. Of survivors, six (18%) have moderate AI. Pre‐ and postoperative echocardiograms were reviewed. The AS group showed increased left ventricular (LV) cavity size by > 1‐year follow‐up, and decreased LV wall and Interventricular septal thickness. In the AI group, wall and septal thickness increased by 10 days and LV cavity decreased by 10 days, 60 days, and > 1 year. Root replacements (n = 14) showed mean increases of 4.3 mm and 5.3 mm, respectively, in diameters of the aortic annulus and aortic sinuses at > 1 year. Intraaortic implants increased 3.1 mm (annulus) and 3.9 mm (sinuses) at > 1 year. The pulmonary autograft procedure is safe, and successful implantation normalizes LV dimensions and function rapidly. The autograft valve shows evidence of growth at > 1 year postoperative. The pulmonary autograft may be the ideal valve replacement in children.

Correction of truncus arteriosus with truncal valvar stenosis or insufficiency using two homografts

Surgical correction of truncus arteriosus requires the creation of right ventricular to pulmonary artery continuity and closure of the ventricular septal defect. A variety of conduits have been used including valved and nonvalved. Despite a significant incidence of truncal valvar stenosis and insufficiency, this valve has seldom been replaced. We present 4 cases of truncus arteriosus with truncal valvar stenosis or insufficiency that were repaired using two valved homografts: one to create the pulmonary outflow tract and the other to replace the abnormal truncal valve. Two of these patients are doing well after 4 months. Another child survived the operation and did well for 2 months when she died suddenly. The last child died 14 hours postoperatively from low cardiac output syndrome secondary to diabetic hypertrophic cardiomyopathy. When truncal valvar abnormalities are present, the primary repair of truncus arteriosus in an infant should include replacement of the truncal valve. Total correction can be successfully achieved using two valved homografts, resulting in long-term palliation and freedom from thromboembolic events and the use of anticoagulants.

Modified hemi-Fontan operation: An alternative definitive palliation for high-risk patients

BACKGROUND We conceptualized that by adding small amounts of prograde pulmonary blood flow to the hemi-Fontan operation, or bidirectional Glenn procedure, this modified hemi-Fontan operation could be safely done at an early age, with better oxygenation, and with less potential for pulmonary arteriovenous fistulae. METHODS Since April 1992 the hemi-Fontan operation was modified by adding some prograde flow through the native pulmonary artery in 10 high-risk infants, either by leaving the critical subpulmonary stenosis untreated (n = 6) or by tightening a previously placed pulmonary artery band (n = 4). All other sources of pulmonary blood flow were interrupted. Patients were 4 to 23 months old (3.8 to 10.3 kg). Diagnoses included isolated dextrocardia with single ventricle (3) and polysplenia syndrome (2). Cardiopulmonary bypass was needed in 5 patients. RESULTS There were no hospital deaths. Mean postoperative intensive care unit stay was 2 days, and 9 of 10 patients were discharged within 7 days of operation. One 4-month-old infant with Downs syndrome survived postoperative takedown of the hemi-Fontan repair after pneumonia and caval thrombosis developed. Eight patients are currently asymptomatic receiving minimal modification, and oxygen saturations range from 84% to 93%. CONCLUSIONS Adding small volumes of prograde pulmonary blood flow to the hemi-Fontan operation is safe, provides improved oxygenation, may encourage growth of central pulmonary arteries, and represent an alternative definitive palliation for high-risk Fontan candidates.

Pulmonary Autograft Replacement of the Aortic Valve in the Potential Parent

Aortic valve replacement in the child and young adult is often delayed, and multiple operations or invasive procedures are performed to avoid valve replacements. Prosthetic valves, bioprosthetic valves, or allograft valves have been associated with significant complications or early failure and have been a disappointing solution for the patient requiring aortic valve replacement. The pulmonary autograft replacement (PAR) of the aortic valve in children has been shown to be safe and effective with a low incidence of late valve dysfunction. The absence of thromboembolism, the avoidance of anticoagulants, and its viability with the potential for growth and repair strongly support its use for the potential parent, patients of age 35 or less. The experience with 112 patients, 32 females and 80 males, ages 1.5 to 35 years (average 16.1) are reviewed. Twenty‐four had aortic insufficiency, 34 had aortic stenosis, and 54 had both aortic stenosis and insufficiency. Actuarial survival was 95.4%± 2.0% at 7 years and freedom from reoperation or significant aortic insufficiency of the autograft valve was 92.7%± 3.7%. Freedom from all valve related complications of the autograft valve and the homograft replacement of the pulmonary valve was 90.0%± 4.0%. Re‐operation for the autograft valve was related to limited experience in one, leaflet prolapse and adherence to a VSD patch in one, associated lupus erythematosus in one, and annular and sinotubular dilatation in one. Reoperation of the homograft valve in two patients was secondary to early homograft stenosis, probably due to rejection. Normalization of ventricular function by 1 year postoperative, enlargement of the autograft proportional to somatic growth, and a low risk of valve related complications suggest that the PAR replacement of the aortic valve is the Ideal valve replacement for the potential parent. (J Card Surg 1994;9[Suppl]: 198–203)

Interruption of the Aortic Arch: Experience in 17 Infants

Between April, 1965, and August, 1982, 17 children ranging in age from 2 days to 4 years were identified as having interruption of the aortic arch and operated on at our institution. There were eight type A interruptions, eight type B interruptions, and one type C interruption. Associated intracardiac anomalies were present in all but 3 patients. These 3 children, who all had type A interruption, underwent repair by mobilization of the aorta and end-to-end reconstruction. The other 14 children had initial palliative operations. The 2 patients in Group 1 had type A interruption with associated ventricular septal defect (VSD), and underwent subclavian artery-aorta anastomosis. In Group 2, the palliative procedure consisted of placement of a Dacron tube graft in 1 patient with type A interruption and associated VSD, and placement of a polytetrafluoroethylene (PTFE) graft, division of the patent ductus arteriosus, and banding of the pulmonary arteries in 11 patients--2 with type A, 8 with type B, and 1 with type C interruption. Ten children (71%) survived initial palliation, 1 of the 2 in Group 1 and 9 of the 12 in Group 2. In Group 2, 5 children had interruption of the aortic arch (4, type B; 1, type C) with associated VSD; among the 4 who survived palliation, 3 subsequently have had successful closure of the VSD and 1 is awaiting closure. Among the patients who had palliative procedures, there are 6 long-term survivors (43%). In the total series, there are 9 long-term survivors (53%).(ABSTRACT TRUNCATED AT 250 WORDS)

Ventricular function after the arterial switch operation for transposition of the great arteries

The theoretical advantages of anatomical repair have resulted in the widespread use of the arterial switch operation for transposition of the great arteries. However, preservation of systemic ventricular performance and late functional results have not been well documented. To evaluate late postoperative ventricular function, we reviewed 53 consecutive patients undergoing arterial switch operation for transposition of the great arteries with or without a ventricular septal defect over the 8-year period from March 1985 to 1993. Forty-two patients had simple transposition of the great arteries and 11 patients had associated ventricular septal defects that were closed at operation. Mean age at operation was 1.8 months (range, 1 day to 36 months), and mean patient weight was 3.8 kg (range, 1.8 to 15.6 kg). All but 8 patients were neonates. There were six operative deaths (11.3%, 6/53) and two late deaths during a median follow-up of 23 months (range, 0.1 to 99.5 months). Actuarial survival at 8 years was 83% +/- 6%. Left ventricular outflow tract obstruction has not been identified, and 9 patients (20%, 9/45) have right ventricular outflow tract gradients exceeding 20 mm Hg, 3 of whom have required reoperation. Eighteen patients have mild neo-aortic valve regurgitation. All survivors are currently in New York Heart Association class I, and are in sinus rhythm. Systolic left ventricular function is well preserved with ejection fractions greater than 0.60 in all survivors followed up for more than 4 months (41 patients). Left ventricular end-diastolic volume index is elevated in only 1 patient, a patient who had pulmonary artery banding as a neonate.(ABSTRACT TRUNCATED AT 250 WORDS)