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Complete congenital heart block is associated with the presence of maternal autoantibodies to small ribosomal nucleoproteins (such as anti-Ro) which cross the placenta and may be deposited at the site of cardiac damage. Ten such cases of congenital heart block, their mothers, and their siblings were studied. The seropositive mother of one case had a similar conduction defect (bifascicular block) to that in her affected child. None of the siblings examined had cardiac lesions. Six mothers had Ro or La antibody five to 17 years after the birth of the affected child. Four mothers examined 11-32 years after the birth of an affected child were seronegative. Three of these mothers had evidence of a connective tissue disorder. This evidence is consistent with a hypothesis that a maternal viral infection, associated with autoantibody production, leads to virus crossing the placenta, damaging the fetal heart, and eliciting local deposition of maternal antibody.

Doppler ultrasound was used to assess the pressure drop between the ventricles in 109 infants and children (61 less than two years old) with a ventricular septal defect who underwent cardiac catheterisation. The pressure in both ventricles was measured at catheterisation in 103 patients either simultaneously through two catheters (41) or with a single catheter withdrawn across the septum or removed from one ventricle to the other (62). When pressure was measured simultaneously with two catheters (41 patients) the peak to peak and instantaneous gradients showed a maximum difference of 20 mm Hg with levels within 10 mm Hg of each other in 36. Comparison of the difference in the gradients with the average of the measurements demonstrated a tendency for Doppler to underestimate the difference when it was high (greater than 50 mm Hg) and overestimate it when it was low. A Doppler estimate of a low pressure difference between the ventricles indicates pulmonary arterial hypertension and a high one low pulmonary artery pressure, but in the intermediate group Doppler is as yet not sufficiently sensitive to allow selection of those patients who require further investigation and possible operation. Doppler ultrasound was found to be a sensitive method of detecting a very small ventricular septal defect. Thus although Doppler is a very useful means of assessing and following patients with a ventricular septal defect, further studies are required to determine its exact place in clinical practice.

Hypertrophic cardiomyopathy was diagnosed in identical twin boys in early childhood. One underwent myomectomy at the age of 12 years because of progressive severe exertional dyspnoea accompanied by considerable obstruction of the left ventricular outflow tract shown on both echocardiography and cardiac catheterisation. Seven years later, at the age of 19, he remains incapacitated to a moderate degree. By contrast, the other twin has led a relatively normal life to date and no left ventricular outflow obstruction has been shown.

The development of complete heart block is described in a patient with polymyositis. The implantation of a permanent pacemaker has controlled the heart block, but the progression of the underlying disease despite treatment with relatively high dosage of corticosteroids makes the outlook uncertain.

109 children who survived surgical treatment for isolated pulmonary valve stenosis were followed for up to 17 years. In all the postoperative status was assessed as satisfactory. Cardiac catheterization repeated in 43 gave a resting valve gradient below 40 mmHg. The 22 children whose pulmonary valves had been excised were as healthy as the 87 who had undergone pulmonary valvotomy. Consideration was given to the desirable length of postoperative review. Except for the few children with symptoms before operation, a postoperative increase in exercise tolerance was not a feature.

Congenital complete atrioventricular block is uncommon, and the outlook is usually regarded as favourable. Thirty-five patients with congenital heart block are presented. There was no obvious sex difference and their ages ranged from 12 days to 85 years, though most were under the age of 20 years when first seen. Accompanying heart disease was noted in six, but presentation with symptoms in early infancy was a more serious risk factor in our experience. Permanent pacing was required in 21, three of whom were neonates. One of the 14 unpaced patients died unexpectedly at the age of 5 years. Long term supervision is necessary, as most will require permanent pacing before their 50th birthday.

Unilateral pulmonary vein stenosis is a rare congenital anomaly. A case is described in a girl who first presented at the age of four years with recurring haemoptysis but in whom diagnosis was not established until she was 16 years old. Pulmonary angiography demonstrated a minimally hypoplastic right pulmonary artery, and the laevophase showed normal pulmonary venous return from the left lung, but none from the right. Surgical treatment was necessary because of life threatening haemoptysis, and pneumonectomy was required in the light of the findings at operation.

Of 129 patients with either mitral or aortic valve disease angina was present in 55 (42%). It was more frequent in aortic (60%) than in mitral valve disease (33%). The standard 12-lead electrocardiogram was not helpful in distinguishing underlying occlusive coronary artery disease. Coronary arteriography demonstrated coronary artery disease in 26 patients (20%), only 2 of whom had no angina. The incidence of coronary artery disease was almost identical in both the mitral and aortic groups (22% and 17%, respectively), but the percentage of those with demonstrable coronary artery disease accompanying angina was much higher in the mitral group (67% as against 29%). Angina in mitral valve disorders is thus much more likely to be the result of disease of the coronary arteries. Coronary arteriography is mandatory in all patients in both groups who have angina. Otherwise it seems unnecessary as coronary artery disease was found in only 2 patients who did not have angina.

A patient is presented in whom the heart reverted spontaneously to sinus rhythm 11 years after surgical closure of a ventricular septal defect complicated by complete heart block. It seems unlikely that regeneration of fibres in the bundle of His, if these had indeed been destroyed, could account for the restoration of sinus rhythm after so long an interval.