J F Taylor

Pulmonary atresia and intact ventricular septum: surgical management based on a revised classification.

Sixty patients with pulmonary atresia and intact ventricular septum (PA:IVS) presenting from 1970 to 1980 are reviewed. Three groups of patient are discussed: those with tripartite right ventricles, those with no trabecular portion to the cavity, and those with neither trabecular nor infundibular portions. The decrease in early mortality for neonates with PA:IVS since 1977 (one death in 15 patients) supports our current management policy of preoperative prostaglandin El infusion with transpulmonary valvotomy (for patients with an infundibular cavity) combined with a left modified Blalock‐Taussig shunt using a Gore‐Tex prosthesis. Tricuspid valve growth, estimated by serial angiograms in 12 patients, was greater if right ventricle‐to‐pulmonary artery continuity was established. Later definitive repair was attempted in nine patients, with two early deaths; five underwent right ventricular outflow tract reconstruction and four had modified Fontan procedures. Neonates with critical pulmonary stenosis are also discussed. Their neonatal mortality (nine deaths in 20 patients) was similar to that of comparable patients with PA:IVS, but their actuarial survival at 5 years (55%) was superior (36% at 5 years).

Percutaneous balloon valvuloplasty for pulmonary valve stenosis in infants and children.

Pulmonary valve stenosis was relieved by balloon dilatation during cardiac catheterisation on 27 occasions in 23 infants and children aged 7 days to 12 years, median 31 months (three aged less than 2 weeks). Pulmonary valve diameter was estimated by cross sectional echocardiography to assist in the choice of balloon size. Before dilatation the right ventricular systolic pressure ranged from 41 to 190 (median 92) mm Hg and was suprasystemic in 10 patients. There were significant reductions in the ratio of right ventricular to systemic systolic pressure and pulmonary systolic pressure gradients immediately after balloon dilatation. Twelve patients underwent recatheterisation (11 at six months and one at one week after balloon dilatation), which showed further improvement with significant reductions in right ventricular pressure or pulmonary valve gradient or both, particularly in the patients with the least satisfactory initial results. This improvement was attributed to resolution of the obstruction at infundibular level. Repeat pulmonary valve dilatation was successfully performed in four patients who had poor results after initial dilatation. Balloon pulmonary valvotomy appears to provide good short term and medium term relief of pulmonary valve stenosis and may obviate the need for surgery in many cases. An apparently poor immediate haemodynamic result does not preclude a good longer term result.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Effect of systemic pulmonary anastomosis.

In nine patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries, the effect of a systemic-pulmonary anastomosis on the lung was studied by comparison of pre- and postoperative cine angiograms. Selective injections into the collateral arteries were performed in all patients and the source of blood supply to each bronchopulmonary segment was sought. Central pulmonary arteries were present in six children and absent in three, as confirmed at thoracotomy. After insertion of a shunt, central pulmonary arteries increased in size, but the intrapulmonary vessels with which they connected remained abnormally small and were frequently stenosed. In addition, in each case the central pulmonary arteries increased flow to only five to 11 segments of lung. In the absence of central pulmonary arteries, shunting to a hilar or a lobar pulmonary artery increased perfusion to five to nine segments of lung per case. In all these three cases the pulmonary arteries at lobar level showed aneurysmal dilatation proximal to a severe stenosis. Sixty-eight per cent of collateral arteries were stenosed. The findings suggest that in this anomaly, when the central pulmonary arteries are hypoplastic, the intrapulmonary branches are also hypoplastic, emphasising the need for early surgical intervention to increase blood flow while the lung still has growth potential. Further, one collateral artery may connect with at least as many bronchopulmonary segments as does a central pulmonary artery, and the peripheral intrapulmonary arteries with which it connects may appear at least as normal angiographically as do vessels connected to central pulmonary arteries. These observations suggest that segments of lung connected to collaterals alone should, and sometimes can, be connected to central pulmonary arteries, the aim being to produce a unifocal blood supply as a prelude to total correction.

Postoperative Angiographic Assessment of Modified Blalock-Taussig Shunts Using Expanded Polytetrafluoroethylene (Gore-Tex)

Thirty-six of 87 modified Blalock-Taussig shunts done with expanded polytetrafluoroethylene (Gore-Tex) were restudied angiocardiographically. In 7 patients the study was carried out within 1 month of the shunt operation because the patients failed to make satisfactory clinical progress. Two shunts were occluded and 1 ws stenosed; all 3 were in neonates. The remaining 29 patients were reinvestigated electively between 5 and 29 months postoperatively and had a 97% shunt patency rate. Because of the rather high incidence of irregular or stenosed shunts among neonates with 4 mm conduits, we now prefer to use a larger conduit even in this age group.

Late outcome of survivors of intervention for neonatal aortic valve stenosis

BACKGROUND This study examined the late outcome after intervention for neonatal aortic valve stenosis. METHODS Seventy-three neonates (59 boys and 14 girls) underwent intervention for critical aortic valve stenosis during the first 30 days of life at two institutions, The Hospital for Sick Children, London, and Duke University Medical Center, Durham, North Carolina. Procedures performed include closed valvotomy (n = 12), open valvotomy with inflow occlusion (n = 14), open valvotomy with cardiopulmonary bypass (n = 33), balloon valvotomy (n = 12), and other procedures (n = 2). The mean age at the first intervention was 8 +/- 1 days. RESULTS The hospital mortality was 52.1%. The mean duration of follow-up for the hospital survivors (n = 35) was 8.3 +/- 1.1 years. The actuarial survival for the hospital survivors was 93.3% +/- 4.7% at 10 years and 83.9% +/- 9.8% at 15 years, whereas event-free survival (reintervention, endocarditis, or early death) was 61.8% +/- 9.3% at 5 years, 34.2% +/- 10.8% at 10 years, and 27.4% +/- 10.6% at 15 years. Three patients have died and 11 patients have required aortic valve replacement during the follow-up period. The age at the initial intervention, the type of initial intervention, and the year of initial intervention were not predictive of early death or need for reintervention. At last follow-up, 26 of the long-term survivors (n = 32) were in functional class I and 6 were in functional class II. CONCLUSIONS Aortic stenosis in the neonatal period is a difficult problem with a high initial mortality. Late survival and functional class are excellent for patients surviving the initial hospitalization, but most require further intervention within 10 years.

Infective endocarditis in children with congenital heart disease: comparison of selected features in patients with surgical correction or palliation and those without.

The diagnostic and prognostic features of 44 episodes of infective endocarditis in 42 children with congenital heart disease were reviewed. Endocarditis occurred in 18 patients who had not had surgical correction or palliation of the defect (non-operated group). There were 26 episodes in 24 patients who had been treated surgically (operated group) (16 open and eight closed cardiac operations). Endocarditis occurred soon after open heart surgery in eight patients and as a late complication in the other 16. It recurred in two patients (operated group). Invasive monitoring and low cardiac output were consistent features in those patients who had endocarditis soon after open heart surgery whereas dental treatment was a common feature in non-operated cases and after closed cardiac operations. Late cases of endocarditis after open heart surgery had various microbiological features that were not typical of infection after dental problems. Gram positive infections occurred in non-operated patients and in those who had had closed cardiac operations. The group that had open heart surgery had infections caused by Gram positive, Gram negative, and anaerobic bacteria and fungi. Fever, anaemia, leucocytosis, and positive blood cultures were the only consistent findings. Vegetations were seen in nine of 12 patients at cross sectional echocardiography. All 12 (four non-operated, one closed, and seven open cases) needed acute surgical treatment. The mortality from infective endocarditis was 17% for non-operated cases, 0% for those who had had closed heart surgery, and 50% for those who had had open heart surgery. Infective endocarditis after open heart surgery differs from that in the other subgroups in terms of microbiology, source of infection, and outcome and its early diagnosis depends on a thorough investigation of minimal symptoms and signs.

Use of high kilovoltage filtered beam radiographs for detection of bronchial situs in infants and young children.

Determination of atrial situs is of cardinal importance in the analysis of complex congenital heart lesions, and is best predicted from bronchial situs. Previous methods for assessing bronchial morphology, however, are unsuited to the very young patient. To assess bronchial morphology, 100 consecutive patients under 18 months of age (medium 57.5 days) with suspected congenital heart disease were studied by high kilovoltage filtered beam radiographs, before cardiac catheterisation. This low radiation dose technique clearly defined bronchial anatomy in 95 patients. The lengths of the left and right main bronchi were compared and 10 cases (10%) had a ratio less than 1.5 suggesting bronchial isomerism. Discriminant function analysis based on tracheal width and bronchial length enabled clear distinction of right from left bronchi. Four patients had bilateral right and six had bilateral left bronchi. Four of these 10 cases died and had necropsy confirmation of the radiological diagnosis. Practical prediction about cardiac anatomy, particularly the systemic and pulmonary venous return, may be made when bronchial morphology is known.

Correction of isolated secundum atrial septal defect in infancy.

Although it is a common congenital cardiac lesion, secundum atrial septal defect (ASD) rarely presents in infancy. Its natural history in the first two decades of life is generally benign. Reports of symptomatic ASD in early childhood stress the place of conservative management, and recommend that the operation should be deferred until school age. Few accounts of the indications for surgery in infancy exist. We present our experience of 6 patients who failed to respond adequately to conservative treatment and required operation for intractable heart failure due to isolated secundum ASD in the first year of life. As their presentation and physical signs were not typical of ASD seen in later childhood, they presented problems both of diagnosis and management.

Rastelli procedure for transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction. Early and late results in 41 patients (1971 to 1978).

Forty-one children with transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction underwent a Rastelli operation between 1971 and 1978. A homograft valve preserved in an antibiotic solution and extended with A dacron tube was the conduit of choice. Alternatively, conduits with porcine heterografts or valves constructed from calf pericardium were used. They were positioned to the left of the aorta whenever possible. The intraventricular tunnel from the left ventricle to the aorta was constructed from Dacron velour. There were four early and seven late deaths. The last 13 consecutive patients have survived. Early deaths were related to unfavourable anatomy, conduit compression, and sepsis. Residual ventricular septal defects and postoperative infection were the main factors contributing to the late deaths.

Total anomalous pulmonary venous drainage in infancy.

Between May 1971 and December 1975, 39 infants had operations for correction of total anomalous pulmonary venous drainage. Fourteen of the 39 patients were under 1 month of age at the time of operation. Twenty-four patients had supracardiac, 7 intracardiac, and 6 infracardiac total anomalous pulmonary venous drainage, and 2 had a mixed type. The overall hospital mortality was 36 per cent. There have been no late deaths. The improvement in survival rate in this series is attributed to: (1) earlier recognition and prompt referral, (2) an aggressive approach to diagnosis involving complete cardiac catheterisation and angiocardiography, (3) vigorous preoperative care, (4) early complete correction including construction of a large anastomosis and enlargement of the left atrium when indicated, and (5) intensive postoperative management paying particular attention to fluid balance and treatment of pulmonary complications. Operative mortality was highest in patients with total anomalous pulmonary venous drainage directly to the superior vena cava, and in those with infradiaphragmatic drainage of whom all had pulmonary venous obstruction. Mortality was not closely related to age, body weight, or severity of pulmonary hypertension.