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

BACKGROUND: Ebstein's 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 Ebstein's anomaly. CONCLUSIONS: Biventricular repair of Ebstein's 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.

Evaluation of cryopreserved homografts in the right ventricular outflow tract after the Ross procedure: intermediate-term follow up.

BACKGROUND AND AIMS OF THE STUDY: The Ross procedure involves replacing a transplanted pulmonary valve with a cryopreserved homograft in order to re-establish right ventricle-pulmonary artery continuity. This study reviews mid-term results of such surgery in children and young adults. METHODS: Since November 1986, 114 patients have undergone the Ross procedure at the Children's Hospital of Oklahoma using cryopreserved pulmonary homografts (n = 113) and aortic homograft (n = 1) to reconstruct the right ventricular outflow tract. Graft size ranged from 16 to 32 mm. Ninety-three patients (mean age at implant 10.4 years (range: 0.8-22 years) have had complete mean follow up of 3.2 years (range: 5 months to 8.4 years) after surgery. Homograft evaluation included clinical reports and comparison of early post-implant and latest echocardiography. Measurements of homograft valve annulus and peak instantaneous Doppler gradient were compared; quality of valve leaflets, location of obstruction, and the degree of pulmonary regurgitation were assessed. RESULTS: Compared with early postoperative data, mean homograft annulus size decreased by 15% (p < 0.0001); in 88% of patients, the decrease ranged from one to nine millimeter. Peak Doppler gradient increased significantly (from 10 to 17 mmHg, p < 0.0001); 25% of patients developed gradients > 25 mmHg, and four had gradients > 50 mmHg. Significant obstruction developed most often at the supravalvular level or in the homograft conduit itself. This usually occurred within one year of implant, and was associated with calcification and contracture of the homograft wall. Significant pulmonary regurgitation developed in 19 cases (20%), but was more than mild in only three. Leaflet integrity was maintained except in those who developed severe stenosis or regurgitation. Two patients have undergone re-operation for homograft stenosis 2.8 and 5.4 years respectively after the Ross procedure; one has developed recurrent severe stenosis in the homograft four months later. CONCLUSIONS: After the Ross procedure: (i) Pulmonary homografts undergo significant annular reduction in most patients, though this is usually not associated with the development of significant obstruction. (ii) Peak Doppler gradients across the homograft increase in most patients, though only 4% develop more than mild obstruction. (iii) Mild pulmonary regurgitation is common (20%); moderate or severe regurgitation is rare and usually develops in concert with severe stenosis. (iv) Severe homograft valve degeneration usually occurs within one year of implant, and may reflect an immune-mediated response.

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 Down's 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.

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)

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%. Reoperation 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.(ABSTRACT TRUNCATED AT 250 WORDS)

Neonatal coarctation repair. Influence of technique on late results.

BACKGROUND: Coarctation repair in the neonate (< or = 28 days) is associated with higher mortality and increased incidence of restenosis compared with older infants. It has been suggested that resection of pericoarctation ductal tissue may reduce this risk of restenosis. METHODS AND RESULTS: To further clarify these issues, we reviewed our experience with 111 consecutive neonates undergoing primary repair between 1973 and 1991. Hospital mortality was 14.4% (16 of 111) and was not significantly different for the type of repair:resection and end-to-end anastomosis (RETE) 10.7% (6 of 56), subclavian flap angioplasty (SFA) 16.7% (6 of 36), and patch angioplasty (PA) 16.7% (3 of 18). Associated complex cardiac pathology was associated with higher operative risk: 25% (10 of 40) versus 8.4% (6 of 71) (P = .02). Median follow-up of 4.2 years (range, 0.1 to 18.5 years) was 99% complete. Late mortality was 13.6% (13 of 95), of which 92% occurred within 1 year of repair. Twenty percent (19 of 95) needed reintervention for restenosis, RETE 16% (8 of 50), SFA 13% (4 of 30), and PA 47% (7 of 15) (P = .02). Of these, 84.2% (16 of 19) required reintervention within 1 year of repair. Freedom from reintervention 1 and 8 years after operation was 80 +/- 4% and 77 +/- 5%, respectively. Actuarial survival 8 years after operation was 73 +/- 4%; for simple coarctation, this was 90 +/- 4%. By multivariate analysis, survival was negatively influenced only by presence of associated cardiac pathology (P = .002) and reintervention only by patch angioplasty technique of repair (P = .007). CONCLUSIONS: In the neonate, resection of coarctation (RETE) does not diminish the risk for reintervention compared with SFA. The risk for both late death and recurrent coarctation are highest within the first year after repair, and follow-up should be particularly vigilant during this period.

Successful thrombectomy for thrombosis of the right side of the heart after the Fontan operation. Report of two cases and review of the literature.

We performed operative thrombectomy for the treatment of cardiac thrombosis on the right side of the heart in two patients; thrombosis occurred after a total cavopulmonary connection (modified Fontan operation). In the first case, thrombosis occurred approximately 6 months postoperatively, and the thrombus was evacuated without cardiopulmonary bypass. In the second case, an emergency open atrial thrombectomy and revision of the stenotic inferior cavopulmonary anastomosis was performed on the tenth postoperative day. Review of the literature identified 12 previously reported cases that were similar. In these 12 cases 6 patients died, 5 of them early after diagnosis and institution of treatment. We believe that appropriate management should include thrombectomy and revision if a surgically remediable cause of the thrombosis is identified; otherwise, thrombolytic therapy should be initiated. The use of heparin and then sodium warfarin (Coumadin) has also been successful. Risk of thrombosis of the right side of the heart after the Fontan repair may be minimized by the use of prophylactic anticoagulation in high-risk patients soon after the Fontan operation.

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 greater than 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 greater than 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 greater than 1 year. Intraaortic implants increased 3.1 mm (annulus) and 3.9 mm (sinuses) at greater than 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 greater than 1 year postoperative. The pulmonary autograft may be the ideal valve replacement in children.

Delayed detection of coarctation in infancy: implications for timing of newborn follow-up.

During a recent 5-year period, 74 patients younger than 6 months of age were diagnosed with coarctation of the aorta. Coarctation was correctly diagnosed in only 22% of patients prior to referral despite readily apparent femoral pulse abnormalities in 86%. Infants whose symptoms were detected between 5 and 14 days of age were significantly more ill than infants outside this age range and had a high mortality rate (25%). The number of associated cardiac defects was not related to the severity of clinical illness in this group, suggesting that closure of the ductus arteriosus is the primary determinate of disease severity. Observations in two patients suggested that a detectable pulse discrepancy occurs between 3 and 5 days postnatally. Upper extremity hypertension was found commonly in infants after 5 days of age despite the presence of congestive heart failure. Earlier detection of coarctation in the newborn requires a diligent cardiovascular and peripheral pulse examination between 3 and 7 days of life, upper extremity and lower extremity blood pressure measurement, and a high index of suspicion.

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.

Senning repair for transposition of the great arteries in the first week of life.

Infants with D-loop transposition of the great arteries (D-TGA) may have unacceptable results after the balloon septostomy while awaiting surgery. It has been our policy to repair defects in infants with D-TGA and intact ventricular septum or small ventricular septal defect at the time of diagnosis. We report our experience with the Senning operation in 18 newborns less than 1 week of age. The mean age at operation was 3 days (range 12 hr to 7 days) and the mean weight was 3.5 kg (range 2.8 to 4.8). There were two early postoperative deaths (11%) and one late death (5%). Early mortality was associated with preoperative acidosis and congestive heart failure. Late mortality was associated with severe left ventricular outflow tract obstruction (LVOTO). The 15 long-term survivors have been followed for an average of 27 months and 11 of the 16 perioperative survivors have undergone postoperative catheterizations. There was no evidence of systemic or pulmonary venous obstruction. One patient developed LVOTO that led to his death. Two patients had residual atrial shunts. Electrocardiograms revealed no major arrhythmias. All patients are clinically asymptomatic. Good hemodynamic, electrocardiographic, and clinical results can be obtained with correction of D-TGA in the first week of life.

Reoperation in congenital aortic stenosis.

Over a 22-year period, 81 patients underwent initial operations for critical aortic stenosis at our institution. Their ages ranged from 3 days to 20 years (mean, 7.3 +/- 5.9 years). Fourteen (17%) were infants less than 1 year old. Three children died perioperatively (3.7%). We have followed the survivors and 3 children who underwent initial operations elsewhere for a mean of 9.0 +/- 6.8 years (range, 2 to 23 years). To the present, 27 patients have undergone one reoperation (24 of our initial survivors) at a mean interval of 7.3 years, with 2 perioperative deaths. Ten of these patients have required a second reoperation at a mean interval of 3.7 years, with 2 deaths perioperatively. There were 3 late cardiac deaths after the initial procedure and 1 after a third operation. Actuarial reoperation-free survival is 56.7% at 10 years. While overall survival is 88.6% at 10 years, we find a significantly poorer survival among those patients with valvular stenosis compared with those with subvalvular lesions (p = 0.03). We believe that for children with all levels of aortic stenosis, good functional results and survival can best be obtained by follow-up, recatheterization, and reoperation.

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)

Hemophilus influenzae purulent pericarditis in children: diagnostic and therapeutic considerations.

Purulent pericarditis is an unusual complication of infection in infancy and has been associated with an extremely high mortality rate. Early diagnosis followed by combined antibiotic therapy and surgical drainage of the pericardium has markedly improved survival. Between APril, 1975, and February, 1979, nine patients with purulent pericarditis secondary to Hemophilus influenzae type B were treated at the Oklahoma Children's Memorial Hospital. In every case signs and symptoms of congestive heart failure were present, and a pericardial effusion was demonstrated by echocardiography and confirmed by pericardiocentesis. The organism was identified with countercurrent immunoelectrophoresis and antibiotic sensitivity determined by rapid beta lactamase assay. All patients were treated with a combination of parenteral antibiotics and open surgical drainage of the pericardium. There were no deaths and all patients demonstrated marked improvement following operation. Follow-up echocardiography revealed no evidence of pericardial effusion or signs of constriction in any patient.