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Objective: To report recent experience of patients with complete atrioventricular septal defect and tetralogy of Fallot, with emphasis on anatomical features, diagnosis, and management., Design: Case notes were reviewed and patients were assessed at follow up by clinical examination and cross sectional and Doppler echocardiography., Setting: Tertiary cardiothoracic referral centre., Patients: Between 1987 and 1992 13 patients with atrioventricular septal defect and tetralogy of Fallot (12 with concordant and one with double outlet ventriculoarterial connections) underwent surgery; 10 underwent complete intracardiac repair. 11 patients had Down's syndrome. The complete diagnosis was established preoperatively by cross sectional echocardiography in all but one patient. A tri-leaflet left atrioventricular valve as seen in parasternal short axis views was the diagnostic feature of atrioventricular septal defect, with tetralogy of Fallot diagnosed from the presence of anterocephalad deviation of the outlet septum producing subvalvar pulmonary stenosis as seen in subcostal right anterior oblique views., Interventions: Total correction consisted of closure of the atrioventricular septal defect by a combined right atrial and ventricular approach, reconstruction of the atrioventricular valves, and relief of the obstruction within the right ventricular outflow tract. Separate patches were used to close the atrial and ventricular septal defects. Modified Blalock-Taussig shunts were performed in three patients, who await intracardiac repair. Surgical correction was carried out at mean (range) age of 5 (2 to 15) years., Main Outcome Measures: Diagnostic methods, surgical results, and functional state after complete correction., Results: The presence of an atrioventricular septal defect was missed preoperatively in one patient with tetralogy of Fallot. The characteristic goose neck deformity on the left ventriculogram was not present and the tri-leaflet nature of the left atrioventricular valve was not sought on echocardiography. Of the 10 patients who underwent complete repair, nine are alive and one died 34 days after operation with adult respiratory distress syndrome. Examination at necropsy showed an excellent surgical correction. Mean (range) follow up was 23 (8 to 48) months. All nine patients are alive and well (New York Heart Association Class 1)., Conclusion: Accurate diagnosis and staged management with improved surgical techniques have lowered mortality of this complex combination of cardiac defects. The current policy of this group is to recommend a systemic to pulmonary arterial shunt procedure for symptomatic children younger than 2 years and total correction in older children.

In a patient with the Wolff-Parkinson-White syndrome, Ebstein's anomaly of the tricuspid valve, a right atrial Chiari net and a patent foramen ovale two unusual complications developed after surgical epicardial dissection combined with cryoablation of the anomalous pathway. The first complication was that ablation of the right atrial wall led to changes in interatrial pressure gradients and the development of a right to left shunt necessitating surgical closure of the atrial septal defect. The second complication was the development of a thrombotic mass in the Chiari net simulating on intracavity tumour, which also had to be removed surgically.

A family with 3 cases of pulmonary stenosis with underdeveloped or normal right ventricle is described. In the family there have also been some spontaneous abortions and many infant deaths, and it seems possible that these were also related to congenital cardiac abnormalities. Recognized syndromes with pulmonary stenosis and reports on familial occurrence of this malformation are reviewed. The cases reported in this paper differ from these previously described syndromes. The possibility of sex-influenced monogenic inheritance is discussed.

A two week old girl with tetralogy of Fallot and atrioventricular septal defect underwent palliation, for severe hypoxaemia, by balloon dilatation to the right ventricular outflow tract. This procedure was repeated once over the next eight months without complications. Serial right ventricular angiograms showed growth of the hypoplastic pulmonary ring ("annulus"), trunk, and arteries. Balloon dilatation is an alternative palliative procedure for patients with the tetralogy of Fallot which may promote growth of the pulmonary ring, trunk, and arteries.

Eleven patients with double mitral valve orifice and atrioventricular defects were studied, and the diagnosis proven by open heart surgery. The correct preoperative diagnosis was suggested by a characteristic angiographic appearance of the medial border of the left ventricle. M-mode echocardiography may show the two orifices which are better seen on two dimensional echocardiography. Two operative deaths occurred in patients with a slightly obstructive valve, and one late death followed mitral valve replacement. Eight patients did well after minor repair to a cleft, or leaving the valve untouched. Occasionally a double mitral orifice, if disorganised and slightly stenotic, may cause early deterioration in a patient with an otherwise uncomplicated ostium primum defect. The surgeon should be forewarned of this mitral anomaly since simple repair may be difficult.

In a study of 45 cases of ventricular pre-excitation, 19 were classified as type A and 20 as type B according to Rosenbaum's criteria, which depend on the polarity of the major deflections in the right praecordial leads and not, as is commonly thought, on the direction of the delta vector. Six cases that could not be classified as type A or type B were termed intermediate. Vectorcardiograms were recorded from 29, and these showed a wide but continuous range of values for both the delta and the main QRS vectors in all three planes. Any classification based on these features must, therefore, depend on arbitrary quantitative data. Three patients in this series had associated right bundle-branch block. A review of the published reports on the association of pre-excitation and bundle-branch block failed to provide a rational basis for the classification of pre-excitation. It is emphasized that Rosenbaum's classification is empirical and its validity is questioned.

Eighty cases of Down's syndrome were found among 1504 children with congenital heart disease under the age of 15 years. The most common cardiac anomaly, ventricular septal defect, was found in 49 per cent of the 80 cases studied, while the second most frequently encountered anomaly, common atrioventricular canal, was found in 15%. Haemodynamic investigations of 24 cases of ventricular septal defect showed Eisenmenger's syndrome to be present in 10 cases; this seemed to appear at an earlier age in mongoloid children than in other children with ventricular septal defect. Right-to-left shunt was found in 5 out of 9 cases of common atrioventricular canal. Pulmonary hypertension was found in all of 24 cases of ventricular septal defect and in 7 of 9 cases of common atrioventricular canal. The cumulative survival up to 10 years was 64% for girls and 49% for boys. Death was most commonly the result of pulmonary complications which occurred in 22 out of the 34 patients who died.

Right atrial isomerism or left atrial isomerism is frequently diagnosed as situs ambiguous without further discrimination of the specific morbid anatomy. Thirty six cases of right atrial isomerism and seven cases of left atrial isomerism were collected from the records and pathological museum at the National Taiwan University Hospital. There was a necropsy report for 18 cases. In all patients one or more of the following conditions was met: (a) isomeric bronchial anatomy, (b) echocardiographic and angiocardiographic evidence of isomerism, and (c) surgical or necropsy evidence of abnormal atrial anatomy. An anomalous pulmonary venous connection was present in 55% of patients with right atrial isomerism; in left atrial isomerism one case (14%) had a partial anomalous pulmonary venous connection. Forty per cent of cases of anomalous pulmonary venous connection with right atrial isomerism had obstruction. Six (86%) of seven cases with left atrial isomerism had an ambiguous biventricular atrioventricular connection. In contrast, univentricular atrioventricular connection (26 of 36, 72%) was significantly more common in right atrial isomerism. A common atrioventricular valve was the most frequent mode of connection in both forms. Two discrete atrioventricular valves were significantly more common in left atrial isomerism. Atrioventricular valve regurgitation was detected in 14 cases. Double outlet right ventricle was the most common type of ventriculoarterial connection. The most commonly cited causes of death after either palliative or definitive operation were undetected anomalous pulmonary venous connection, pulmonary venous stricture, and uncorrected atrioventricular valve or aortic regurgitation complicated by abnormal coagulation. Although the prognosis is poor, successful operation depends on knowledge of the precise anatomical arrangement associated with atrial isomerism.

This report documents 2 patients who presented with cyanosis early in life. In both instances the necropsy showed the simultaneous occurrence of an imcomplete type of persistent atrioventricular canal and an Ebstein's malformation of the "tricuspid" valve component. In the first patient no clinical investigations were done. In the second patient the electrocardiogram was consistent with persistent atrioventricular canal and the angiocardiogram showed the characteristic goose-neck deformity. Moreover, an abnormality of the right cardiac contour was seen, which in retrospect was correlated with the right-sided Ebstein's malformation. Ebstein's anomaly may significantly alter the clinical and haemodynamic profile of atrioventricular canal and should be considered in atypical cases. The presence of Ebstein's anomaly will complicate surgical repair of the atrioventricular canal.

Twenty-eight cases of infradiaphragmatic total anomalous pulmonary venous return are presented, 17 without associated complex intracardiac anomalies (group A), and 11 with additional complex lesions (group B). The anomalous site of connection was to the portal vein in 19 cases (68%), to the inferior vena cava in 4 (14%), the ductus venosus in 2 (7%), to the left hepatic vein in 2 (7%), and unknown in one. A patent foramen ovale was present in 82 per cent of cases in group A and 40 per cent in group B and was frequently associated with a small left atrium and left ventricle. Nine cases (8 in group A; 1 in group B) had surgical correction, with 3 long-term survivors. The surgical mortality was 66 per cent. The postoperative haemodynamic status of the 3 surviving patients is very satisfactory, though 1 had a residual atrial septal defect. Factors which adversely affected the surgical outcome were: (1) a critically ill infant, (2) small left atrium and left ventricle, (3) a patent foramen ovale rather than atrial septal defect, (4) systemic arterial oxygen saturation less than 70 per cent, and (5) pulmonary arterial pressure in excess of systemic arterial pressure. The mortality for the entire series was 93 per cent.

A classification of one type of congenital malformation previously reported (de la Cruz et al., 1967) is based on the integration of an embryological theory for ventricular inversions with the embryological concepts of trunco-conal malformations (de la Cruz and da Rocha, 1956). In that classification we consider that in each situs, either solitus or inversus ventricular inversions may be associated with: (a) normally arranged great vessels (not transposed); (b) transposition of the great vessels; (c) persistent truncus arteriosus. The patients had ventricular inversion without transposition of the great vessels in situs inversus.THE CORRECT ANATOMICAL DIAGNOSIS WAS NOT FORESEEN BUT THE PHYSIOPATHOLOGICAL DIAGNOSIS WAS CORRECT: pulmonary ischaemia associated with septal defects. In one case these facts were substantiated by catheterization and by angiocardiographic findings which led us to advise surgery. Haemodynamically isolated inversion of the ventricles is as severe a malformation as complete (not corrected) transposition of the great vessels. However, an operation of the anastomotic type between a systemic vessel and the narrow pulmonary artery seemed justified in these cases, as a means to convey more blood to the lungs and improve the saturation of the arterial blood. It was unsuccessfully carried out in one patient.The anatomical, radiological, and electrocardiographic features which might aid in the diagnosis are analysed.