Pulmonary vascular and alveolar development in tetralogy of Fallot: a recommendation for early correction.

Using quantitative morphometric techniques, we analysed pulmonary arterial and alveolar development in the lungs of seven children aged 1.2-12 years who died during or soon after repair of tetralogy of Fallot. One child had a residual ventricular septal defect and survived for five months. One other child had had a previous Waterston-Cooley anastomosis (Waterston shunt). Postmortem lung volume in relation to body surface area was generally below normal for age, the alveoli were small, and the total alveolar number was below normal in five of the seven cases. Microscopically, airway and alveolar structure appeared normal. The preacinar arteries were larger and the intra-acinar arteries were smaller than normal for age. The preacinar elastic pulmonary arteries appeared to contain less elastin and in both preacinar and intra-acinar muscular arteries the media was thinner than normal, although muscle was normally distributed along the arterial pathway. Eccentric areas of intimal fibrosis were small and uncommon. The bronchial arteries were generally more prominent than usual both macroscopically and microscopically, but no abnormal bronchopulmonary connections were present. After corrective surgery a residual ventricular septal defect and pulmonary hypertension were associated with arterial medial hypertrophy, and this change was also found in the right lung of a normotensive patient who had had a Waterston shunt. This group probably represents the most favourable clinical picture of tetralogy in patients who usually survive but, even so, pulmonary arterial and alveolar development was abnormal. The structural findings are discussed in relation to the functional outcome in patients with tetralogy who have survived. Repair of the abnormality during the first two to three years of life is recommended.     

Pulmonary artery banding: long-term results in 63 patients.

Sixty-three patients who underwent banding of the pulmonary artery between 1968 and 1975 were studied. Mortality among patients who underwent the banding procedure was 22%, and among those with ventricular septal defect it was 7%. Thirty-eight of the 49 survivors of the banding procedure were investigated for abnormalities of the pulmonary outflow tract caused by the band. Seventy-one percent of these 38 patients had an identifiable abnormality of the pulmonary valve or artery. These complications occurred more frequently in patients banded at a very young age (less than 2 months old) and in patients in whom the band was in place more than two years. Corrective operations have been performed in 24 of the 49 patients who survived banding. Seventy-six percent of the patients with ventricular septal defect survived corrective operation, while only 29% of the patients with more complex lesions survived.     

Factors that exaggerate the deleterious effects of pulmonary insufficiency on the right ventricle after tetralogy repair. Surgical implications

Postoperative cardiac catheterization data of 74 patients with pulmonary insufficiency after tetralogy repair were analyzed. Two groups were identified: Group A, 26 patients with normal right ventricular function (ejection fraction 95% +/- 5.5%, end-systolic volume 110% +/- 17% of predicted normal) and Group B, 48 patients with right ventricular dysfunction (ejection fraction 80% +/- 18% [p less than 0.001], and end-systolic volume 218% +/- 75% of predicted normal [p less than 0.001]). There was no significant difference between the two groups with respect to frequency of previous palliative procedures, age at operative repair, operative techniques, methods of myocardial protection, and follow-up period. Right ventricular dysfunction in Group B was associated with significant distal pulmonary stenosis (right ventricle-pulmonary artery pressure gradient 28 +/- 13 torr in Group A versus 55 +/- 20 torr in Group B, p less than 0.001), moderate pulmonary regurgitation (regurgitant fraction 18% +/- 11% in Group A versus 32% +/- 10% in Group B, p less than 0.001), and large transannular outflow patch (ratio of patch diameter to descending aorta diameter 1.31 +/- 0.16 in Group A versus 2.50 +/- 0.28 in Group B, p less than 0.001). Pulmonary valve insertion was performed in 42 patients in Group B. Eighteen had subsequent cardiac catheterization. Right ventricular function recovered completely (end-systolic volume 122% +/- 24%, and ejection fraction 92% +/- 7% of predicted) in five of six patients (83%) who had valve insertion within the first 2 years after tetralogy repair. In contrast, right ventricular function remained abnormal in all 12 patients who had valve insertion later than 2 years after tetralogy repair (p less than 0.05). Patients with residual pulmonary stenosis and/or a large transannular outflow patch are at risk for the development of right ventricular dysfunction from pulmonary insufficiency after tetralogy repair. Early correction of these residual lesions and control of pulmonary insufficiency may prevent long-term deterioration in right ventricular function.     

Non-invasive assessment of right and left ventricular volumes 11 to 24 years after corrective surgery on patients with tetralogy of Fallot

Patients who had corrective surgery for tetralogy of Fallot using a transannular right ventricular outflow tract (TRVOT) patch showed a higher risk of post-operative mortality and reoperation in the long-term follow-up. A total of 642 patients were operated upon for tetralogy of Fallot between 1952 and 1982. Twenty-six patients who survived for more than 10 years were selected for this study. Fifteen randomly selected patients (group 1) without outflow tract patch were compared with 11 patients (group 2) where a transannular right ventricular outflow patch had been used. Right and left ventricular volumes were assessed using combined first-pass and equilibrium radionuclide ventriculography. After administration of 25 mCi of 99mTc-pertechnetate, data were acquired with a gamma camera with a large viewing field and the patient at rest and during exercise. The patients in group 1 were able to manage a workload of 65 +/- 24 W on the bicycle ergometer while the patients in group 2 could only reach a mean maximum of 34 +/- 12 W. Right ventricular end-diastolic volume (RV-EDV) at rest was 198 +/- 67 ml in group 1 and 224 +/- 69 ml in group 2. During exercise, RV-EDV was increased to 218 +/- 75 ml in group 1 (P less than 0.01) and to 246 +/- 79 ml in group 2. Right ventricular end-systolic volumes did not change significantly during exercise. In group 1, the left ventricular (LV) volumes were comparable to the normal; in group 2, LV-EDV was diminished at rest and during the stress test.(ABSTRACT TRUNCATED AT 250 WORDS)     

Evaluation of long-term postoperative status and cardiac function in patients with tetralogy of Fallot

This study was undertaken to clarify the long-term post-operative state of patients with tetralogy of Fallot (TF). Total number of 243 patients with TF who underwent corrective surgery between 1965 and 1984 were investigated by the follow-up study. Cardiac function was evaluated by Holter ECG (for 48 patients), Treadmill exercise tolerance test (for 74 patients), and cardiac catheterization (for 56 patients). Twenty-year's follow-up revealed 13 patients (5.3%) of late deaths, and 10 of them were cardiac deaths. Three patients (1.2%) expired by sudden death. Nineteen female patients experienced 26 deliveries. No patients required artificial abortion due to heart failure during pregnancies. No baby with cardiac anomaly was born by these mothers. Holter ECG showed complex ventricular arrhythmias in 23 patients (48%). The high systolic pressure ratio of right ventricle to aorta, the old age at surgery, and the long elapsed time after surgery, were the three major aggravating factors of complex ventricular arrhythmias after the correction of TF. Treadmill tests disclosed that patients with poor exercise tolerance had low RVEF and high RVEDP, indicating that RV dysfunction limited the exercise capacity in the long-term postoperative state. Among the three methods of RV outflow tract reconstruction, the follow-up data showed that patients with right ventricular patch below pulmonary valve had better event-free rate compared to those with transannular patch or to those with extensive muscle resection without patch enlargement. Cineangiogram revealed that patients with transannular patch had biventricular enlargement, but the cardiac function did not differ among the three RV outflow reconstructive methods. Because 15 years after surgery the event-free rate decreases gradually and the arrhythmia problem becomes prominent, it is concluded that patients with TF should be followed and observed carefully even though they are currently in the healthy state.     

Repair of ventricular septal defect after pulmonary artery banding.

Since 1964, 90 patients have undergone two-stage surgical repair of ventricular septal defect (VSD) with pulmonary artery banding (PAB) in early infancy and total repair at an average age of 4 years. Reconstruction of the pulmonary artery was accomplished with a pericardial patch, woven Dacron patch, or transverse angioplasty. The VSD was closed with a knitted Dacron patch in 75 patients and by primary suture technique in 13 patients. The VSD closed spontaneously in 2 patients. The mortality rate for patients who had repair and debanding was 9 per cent (8 patients), including 4 deaths due to severe pulmonary hypertensive disease, 3 from congestive heart failure, and one from atrioventricular block. Twenty patients underwent repeat cardiac catheterization several months to 7 years after total repair. This study revealed no shunt in 16 patients and a minimal shunt not requiring operation in the other 4 children. Slight residual stenosis of the pulmonary artery was found in 2 patients and a residual infundibular stenosis in another 2 patients. We believe two-stage surgical treatment of VSD in severely ill infants under one year of age is safe and reliable.     

Ascending aortic origin of a branch pulmonary artery--surgical management and long-term outcome.

OBJECTIVE: Ascending aortic origin of a branch pulmonary artery (AOPA, hemitruncus arteriosus) is a rare congenital malformation. While there have been isolated case reports, larger series, relating to long-term outcomes following surgery are few. This article analyses the surgical results of a series of nine patients, over a period of 29 years. METHODS: Between 1974 and 2003, nine patients [neonates, 6; infants, 3; male, 5; female, 4] were operated on for AOPA. Median age at presentation was 14 days (range birth to 231 days). Six [corrected] patients (group 1) had associated simple lesions like patent ductus arteriosus or right aortic arch. Three patients (group 2) had complex lesions with right ventricular outflow tract obstruction. One patient (group 2) had DiGeorge syndrome. All patients except group 2 presented with congestive cardiac failure and, in addition one had pre-operative coronary ischemia. Diagnosis was established by angiocardiography in two patients and by echocardiography in seven [corrected] The median age at operation was 28 days (range 7-365). Follow-up period ranged from 7 months to 20.5 years (median 9 years). RESULTS: All nine patients had an anomalous right pulmonary artery (RPA) arising from the proximal ascending aorta, while the left branch was of right ventricular origin. All had evidence of pulmonary hypertension or elevated right ventricular pressure pre-operatively. There was no operative mortality. Of eight patients who had direct anastomosis of the RPA to the main pulmonary artery, one required patch enlargement and another required stenting of an anastomotic stenosis. One patient had a RV-RPA conduit, which required replacement 8, 13, and 14 years later. At follow-up, all patients were alive. All patients in group 1 had normal haemodynamic function and were in NYHA class I. In group 2, all were in NYHA class II with evidence of right ventricular hypertrophy. Four patients had post-operative ventilation-perfusion scans which showed satisfactory perfusion to both lungs. CONCLUSIONS: Early surgery is indicated in this lesion and is compatible with good long-term outlook. Surgical repair should not be deferred for corrective procedures of associated cardiac anomalies.     

Intermediate-term survival and functional results after arterial repair for transposition of the great arteries.

An assessment of late morbidity and mortality is essential before arterial repair can be considered truly corrective for patients with transposition of the great arteries. We describe the early and intermediate-term results in 126 patients who underwent arterial repair. Operation was performed at a median age of 6 days, with 76 patients operated on within the first 7 days of life. Coronary artery anatomy differed from the usual arrangement in 37 patients. Simultaneous procedures included ventricular septal defect closure (35) and repair of interrupted aortic arch (2) or coarctation (5). Hospital mortality was seven of 126 (5.5%), with three deaths among the most recent 100 patients (3%). There were one late, noncardiac death and one late death after reoperation. Reoperation for pulmonary artery stenosis was required in 10 of the first 63 patients (16%), all of whom underwent pulmonary artery reconstruction with separate patches for closure of the coronary excision sites. Of the last 63 patients, all of whom underwent pulmonary artery reconstruction with a single pantaloon-shaped pericardial patch, one (2%) required reoperation for pulmonary artery stenosis. Doppler flow studies and echocardiography performed in 115 of 119 surviving patients at a mean of 12 months after repair demonstrated normal left ventricular function, minimal left ventricular outflow gradients, and no more than trivial aortic regurgitation. Peak gradient across the right ventricular outflow tract was 19 +/- 3 mm Hg in patients with separate pulmonary artery patches and 5 +/- 2 mm Hg in those with a single pantaloon patch (p = 0.0001). Follow-up is 96% complete from 1 month to 8 years after operation (mean 2.5 years). The actuarial survival rate at 5 years, including operative mortality, was 92%. All patients are in sinus rhythm, and none requires antiarrhythmic medications. These data suggest that pulmonary artery reconstruction with a single pantaloon patch may be associated with a decreased requirement for reoperation. Intermediate-term survival and functional results are excellent after arterial repair for transposition of the great arteries.     

人同种心包补片在法洛四联症分期手术中的应用

目的比较人同种心包补片和G ore-tex补片在法洛四联症(tetra logy of F a llot,TOF)分期手术中扩大右心室流出道(righ t ven tricu lar ou tflow tract,RVOT)的效果。方法28例已行一期手术再行分期手术的TOF患者,按不同的二期或三期手术时间分为两组,G ore-tex组:13例,以G ore-tex补片扩大RVOT;人同种心包补片组:15例,以深低温冷冻保存的人同种心包补片扩大RVOT。比较两组术后临床结果和随访结果。结果G ore-tex补片组术中死亡1例(7.7%),人同种心包补片组术后早期死亡1例(6.7%)。人同种心包补片组住ICU时间、呼吸机使用时间和心包腔引流量均少于G ore-tex补片组(P<0.01)。所有患者均获得随访,随访时间0.8~4.5年,超声心动图检查提示G ore-tex补片组患者术后RVOT水平残余梗阻的比率高于人同种心包补片组(P<0.01),胸部X线片未发现钙化影。结论人同种心包组织致密,缝合后不易渗血,止血方便;且具有良好的弹性和顺应性,有助于降低术后RVOT水平的残余梗阻发生,可作为补片材料在心脏外科手术中应用。     

Corrective surgery for transposition of the great arteries.

The results of corrective surgery in 32 patients with transposition of the great arteries (TGA) are reviewed. Patients with TGA and associated defects comprised 47% of the series. The over-all long-term mortality rate was 31%. An acceptable mortality rate of 17,5% was achieved in simple TGA by using a Mustard repair. Good results were obtained with the Rastelli operation for patients with TGA, ventricular septal defect and severe pulmonary stenosis. Results were poor in patients with TGA and unrestricted ventricular septal defects, in whom palliative pulmonary artery banding or corrective surgery under 6 months of age is advocated. The major late complication was patch contraction resulting in pulmonary or systemic venous obstruction.     

Reoperation in adults with repair of tetralogy of fallot: indications and outcomes.

OBJECTIVE: The purpose of this study is to review indications, surgical procedures, and outcomes in adults with repaired tetralogy of Fallot referred for reoperation. METHOD: Sixty consecutive adults (age >/= 18 years) who underwent reoperation between 1975 and 1997 after previous repair of tetralogy of Fallot were reviewed. Mean age at corrective repair was 13.3 +/- 9.6 years and at reoperation 33.3 +/- 9.6 years. Mean follow-up after reoperation is 5.0 +/- 4.9 years. RESULTS: Long-term complications of the right ventricular outflow tract (n = 45, 75%) were the most common indications for reoperation: severe pulmonary regurgitation (n = 23, 38%) and conduit failure (n = 13, 22%) were most frequent. Less common indications were ventricular septal patch leak (n = 6) and severe tricuspid regurgitation (n = 3). A history of sustained ventricular tachycardia was present in 20 patients (33%) and supraventricular tachycardia occurred in 9 patients (15%). A bioprosthetic valve to reconstruct the right ventricular outflow tract was used in 42 patients. Additional procedures (n = 115) to correct other residual lesions were required in 46 patients (77%). There was no perioperative mortality. Actuarial 10-year survival is 92% +/- 6%. At most recent follow-up, 93% of the patients are in New York Heart Association classification I or II. Sustained ventricular tachycardia occurred in 4 patients (7%) during follow-up. CONCLUSIONS: Long-term complications of the right ventricular outflow tract were the main reason for reoperation. Mid-term survival and functional improvement after reoperation are excellent.     

Homograft replacement of the pulmonary artery bifurcation

Severe deformity or nonconfluence of the pulmonary arteries was repaired during correction of congenital heart defects by implantation of a complete, fresh, antibiotic-sterilized homograft pulmonary artery bifurcation in 17 patients, aged 15 months to 17 years. Six patients had Fallot's tetralogy; five, pulmonary atresia and ventricular septal defect; three, truncus arteriosus; two, ventricular septal defect; and one, pulmonary atresia with intact ventricular septum. All but one had previously undergone one or more palliative or corrective operations. Thirteen patients survived the operation (76%; 70% confidence interval = 61% to 88%) and were followed up from 2 to 56 months postoperatively (mean = 16 months). All had symmetrical pulmonary perfusion on chest x-ray films and satisfactory homograft function on Doppler echocardiograms. There were no late deaths or reoperations. Four patients (24%; 70% confidence interval = 12% to 39%) died in the hospital of pulmonary or cardiac dysfunction despite a technically satisfactory reconstruction. Comparison of predicted and observed ventricular pressure ratios in nine patients showed that the factor contributed by a right pulmonary artery branch stenois was cancelled in most. The use of a complete, adult-size homograft has thus facilitated reconstruction of the pulmonary artery bifurcation and given encouraging early results.     

Long-term effect of polystan monocusp ventricular outflow patch after right ventricular outflow tract reconstruction

Forty-eight patients who underwent right ventricular outflow tract reconstruction with Monocusp Ventricular Outflow Patch (MVOP) fifty-five times and survived surgery, were reviewed in this study. Mean age at surgery was 6.4 years-old and mean follow-up interval was 75.2 months. There was no late death, however reoperation was performed 7 times. Freedom from reoperation rate was 97.2% and 80.7% after 5 and 10 years after surgery, respectively. The main cause for reoperation were right ventricular outflow obstruction RVOTO (5 cases). All of the RVOTO occurred at the distal end of the anastomosis. However, there was no RVOTO in patients who underwent RVOTR with MVOP during the past ten years. So, we considered the cause of RVOTO a technical problem. Pulmonary regurgitation was one to two degree early after surgery, and had worsened by almost two or three degrees more than 5 years after surgery. Moreover, five of six patients who underwent cardiac catheterization more than 10 years after surgery had three degrees of pulmonary regurgitation as well as a large CTR. In conclusion, according to long-term results, especially more than 10 years post operatively, pulmonary regurgitation was the most important problem.     

The surgical therapy for pulmonary artery--right ventricular discontinuity

The results of operations performed at the University of Michigan for conditions characterized by pulmonary artery-right ventricular discontinuity are reviewed. In 5 infants with increased pulmonary blood flow, pulmonary artery banding was performed with unsatisfactory results. Two survived operation but later died of complications related to the bands. Fourteen children received shunts for conditions involving reduced pulmonary blood flow; 13 survived and have either undergone subsequent total repair or are doing well and are awaiting total repair when possible. Twelve patients have undergone total repair; valveless cloth tube conduits were utilized in 4, and 2 remain well 11 years later. More recently, fresh, antibiotic-preserved aortic allografts have been utilized for the conduit. Six of 8 patients survived the operation, and there was 1 late death. The aortic wall of one graft became calcified after 4 years and had to be replaced, although the valve leaflets remained functional. Immediate success of the totally corrective operation depends on the pulmonary vascular resistance being less than 60% of the systemic. Long-term success depends on the fate of the conduit. From previous experience we think fresh allografts should be more durable than the sterilized allografts used by most other groups; however, we have found that even fresh allografts can calcify.     

A technique for correction of truncus arteriosus types I and II without extracardiac conduits

A new corrective operation for truncus arteriosus without the use of an extracardiac conduit was performed in seven patients with truncus type I (six patients) or type II (one patient) aged from 2 to 9 months. The common truncus arteriosus was septated with a patch into aortic and pulmonary segments and the ventricular septal defect was closed through a ventriculotomy. A direct anastomosis between the pulmonary arteries and the right ventricle was performed, the anterior wall being constructed with a patch with a monocusp valve. There was one death in the immediate postoperative period. In the surviving six patients the postoperative right ventricular/left ventricular peak systolic pressure ratio was less than 0.51 in five and 0.60 in one with a residual ventricular septal defect. All are in functional class I between 1 and 14 months after the operation. On the basis of these results, we propose this technique for patients with truncus type I or II in the first year of life.     

First pulmonary valve replacement with Freedom Solo stentless valve in an adolescent. A case report

Tetralogy of Fallot (TOF) is a common form of cyanotic heart disease. Complete surgical correction in younger age group offers good long-term results with reasonable morbidity and improved prognosis in patients with TOF. However, following corrective surgery pulmonary valve replacement (PVR) might be required for residual pulmonary regurgitation in order to avoid irreversible right ventricular remodeling. Otherwise, residual uncorrected pulmonary regurgitation may lead to right ventricular dilatation, impaired biventricular function, ventricular arrhythmias and limited exercise capacity. We report the first case of Freedom Solo stentless valve (Sorin Group, Saluggia, Italy) implantation in the pulmonary position in an adolescent with severe pulmonary insufficiency 12 years after the repair of TOF. Pericardial stentless valves may be an alternative choice for pulmonary valve replacement to improve right ventricular contractile recovery and remodeling after PVR and may have impact on long-term survival.     

Aneurysm of pericardial right ventricular outflow tract patches.

Among 1,022 patients who underwent repair of tetralogy of Fallot, 252 received a pericardial patch of the right ventricular outflow tract; of these, 10 subsequently developed an aneurysm of the right ventricular outflow tract. Cardiac catheterization and angiography revealed moderate pulmonary insufficiency in all patients, a residual pressure gradient in the right ventricular outflow tract in 7, and a residual ventricular septal defect in 2 patients. Reoperation was indicated in 8 patients because of progressive distention of the aneurysm, residual infundibular or pulmonary artery stenosis, and recurrent ventricular septal defect. Reconstruction of the right ventricular outflow tract was accomplished by resection of the aneurysm and insertion of a woven Dacron patch in 5 patients, primary suture of the pulmonary artery in 2, and implantation of a woven Dacron conduit containing a Björk-Shiley cardiac valve prosthesis in 1 patient. There were no early or late deaths. When reconstruction of the right ventricular outflow tract is necessary, we recommend a woven Dacron patch because pericardium may form an aneurysm.     

Postoperative functional assessment of a modified surgical approach to repair of tetralogy of Fallot

Since February 1982, we have used a modified technique to repair tetralogy of Fallot with transatrial closure of the ventricular septal defect, a short infundibular incision with avoidance of muscle resection, and anterior expansion of the right ventricular outflow tract using a patch in every case. The pulmonary anulus is approached in the usual manner. Twenty-six patients have had repair with this technique without mortality, and 20 patients (Group 1) have been evaluated 1 to 3 years postoperatively. Their results are compared with those of all patients followed up in our division who had had repair by the traditional technique in the 5 years before February 1982, with the same evaluation within 4 years of operation (Group 2, n = 22). Ten of 20 patients in Group 1 and 14 of 22 in Group 2 required a transannular patch. At cardiac catheterization, right ventricular outflow tract obstruction was effectively relieved in both groups (right ventricular systolic pressure [mean +/- standard error], Group 1 versus Group 2, 45 +/- 5 versus 49 +/- 4 mm Hg). No patient with the modified technique had a residual ventricular septal defect. By M-mode echocardiography, right ventricular-left ventricular end-diastolic dimension ratio was significantly lower for Group 1 patients (0.58 +/- 0.03 versus 0.78 +/- 0.04; normal less than 0.45; p less than 0.001). With radionuclide ventriculography, right ventricular ejection fractions were significantly higher for Group 1 patients with the modified repair (38% +/- 2.2% versus 33% +/- 1.8%; normal greater than or equal to 45%; p less than 0.05). Because the incidence of obligatory pulmonary insufficiency caused by transannular patch is similar in both groups, these results cannot be directly attributed to the presence of pulmonary insufficiency alone. With ambulatory electrocardiographic monitoring, no patient in Group 1 had any ventricular premature contractions whatsoever, whereas six of 22 patients in Group 2 had demonstrable ventricular premature contractions. These results suggest that in children with tetralogy of Fallot, right ventricular outflow tract obstruction can be effectively relieved with this modified approach with improved early preservation of right ventricular function.     

ECMO and the management of congenital diaphragmatic hernia with large diaphragmatic defects requiring a prosthetic patch.

From 1977 to 1991, 136 neonates have had corrective surgery for diaphragmatic hernia at Children's Hospital of Los Angeles. A retrospective study was performed to determine how many of the 136 neonates had defects large enough to require the use of a prosthetic patch to repair the defect. Twelve were found. All 12 were symptomatic at birth for respiratory distress. Mean arterial blood gas values at birth were pH 6.95, PCO2 94.8, and PO2 47.2. The mean oxygen index (n = 10) was 61.8. Six of these patients were repaired without extracorporeal membrane oxygenation (ECMO) support while the other six received ECMO bypass perioperatively. All six of the patients who did not receive ECMO support died despite successful diaphragmatic repair. Five of six patients who received ECMO perioperatively survived (83%). These surviving infants are now between 1 month and 4 years of age. In the survivors, four of five required subsequent repair and patch enlargement for a recurrent diaphragmatic hernia. Gastroesophageal reflux, requiring a Nissen fundoplication in two infants, complicated the course of three survivors. Four survivors were discharged with supplemental oxygen therapy lasting less than 13 months. Patch disruption is predicted to occur at approximately 18 months of age in all patients, especially if little or no muscle was available at primary repair for prosthetic attachment. These children should be followed closely for feeding or respiratory symptoms. Diagnosis of patch disruption can be made by chest x-rays and confirmed by contrast studies. Patch expansion by laparotomy and careful search for additional musculature for patch attachment is recommended when reherniation occurs.     

Right ventricular outflow tract reconstruction with a bovine pericardial monocusp patch. Long-term clinical and hemodynamic evaluation

A bovine pericardial monocusp patch was used in 21 patients with a variety of congenital heart defects for reconstruction of obstructed right ventricular outflow tract. Two early postoperative deaths and one late death occurred, and these were unrelated to monocusp patch function. Survivors have completed a mean follow-up of 113 months (range 29 to 141 months). They are all asymptomatic, and their physical activity is unrestricted. Seventeen patients have completed more than 5 years of follow-up and, of these, 11 patients have completed more than 10 years of follow-up. Eleven patients have been reinvestigated by sequential postoperative hemodynamic and angiographic studies at mean periods of 16, 48, and 100 months. The mean peak systolic pressure gradient across the right ventricular outflow tract under conditions of normal flow was 13 +/- 2.4 mm Hg at 16 months, and it has shown no significant change when measured at intervals of up to 124 months after the operation. This lack of change suggests effective long-term relief of the outflow tract obstruction. Serial radiographic screening of all patients and angiographic studies in 11 patients disclosed no evidence of graft calcification, stenosis, or dilatation. The monocusp valve remained functional and effectively abolished or reduced pulmonary valve incompetence. In 11 patients who had residual pulmonary regurgitation, the regurgitation was mild and, up to 12 years after operation, had not resulted in any adverse clinical sequelae.