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We report two patients (four and twelve days old respectively) with heart failure due to absent pulmonary valve and patent ductus arteriosus. The ductus arteriosus in both patients had unusual course arising early from the aortic arch and maintaining an acute angle with the aortic arch. The etiology of absent pulmonary valve syndrome is still not clear. We speculate on the role of arterial duct in the development of absent pulmonary valve syndrome.

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A clinical and genetic study of the Holt-Oram syndrome (HOS) has been carried out in the United Kingdom involving 55 cases designated Holt-Oram syndrome, together with their parents and sibs. Data from the clinical assessment of both familial and isolated cases were used to define the HOS phenotype and to outline the spectrum of abnormalities, especially factors affecting severity. Skeletal defects affected the upper limbs exclusively and were bilateral and asymmetrical. They ranged from minor signs such as clinodactyly, limited supination, and sloping shoulders to severe reduction deformities of the upper arm (4.5%). The radial ray was predominantly affected than the right. All affected cases showed evidence of upper limb involvement. Cardiac defects were seen in 95% of familial cases and included both atrial septal defect (ASD, 34%) and ventricular septal defect (VSD, 25%); 39% had only ECG changes. Cardiac involvement ranged from asymptomatic conduction disturbances to multiple structural defects requiring surgery in infancy. Sudden death could be caused by heart block. Inheritance was autosomal dominant with 100% penetrance and no evidence of reduced fitness. Increasing severity occurred in succeeding generations consistent with anticipation.

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The congenital anomaly of aortopulmonary window is rare. Its combination with interruption of the aortic arch has been previously recognized and reported in infants and children. Early and complete repair of such a congenital anomaly may prevent progression to cardiac failure and premature death. We describe the successful echocardiographic diagnosis and one-stage surgical repair, via a median sternotomy, of the aortopulmonary window, interrupted aortic arch, patent ductus arteriosus, and patent foramen ovale in a 3-day-old neonate. The literature is also reviewed.

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Certain forms of congenital heart disease (CHD) confer a high risk for the development of severe pulmonary hypertension before and after corrective cardiac surgery. Extracorporeal membrane oxygenation (ECMO) has theoretical benefits in the treatment of this complication in that it assures oxygenation, corrects acid-base balance and provides haemodynamic support at the same time as allowing lung rest from ventilation. We examined our experience of the 117 children and neonates supported with ECMO between November 1989 and July 1993. Of these, five received support for critical pulmonary hypertension associated with congenital heart disease. They comprised three who had undergone surgical repair of CHD, one whose total anomalous pulmonary venous drainage was diagnosed and corrected whilst on ECMO and one neonate with functional pulmonary atresia. Pulmonary artery pressure (PAP) was estimated by Doppler echocardiography in all patients and confirmed invasively in two. The median systolic PAP was 46 (range 42-65) mmHg prior to ECMO. The median ratio of pulmonary to systemic arterial pressure (PAP/SAP) was 0.75 (0.70-0.92). Following ECMO of 16-120 h duration, the median systolic PAP was 34 (30-49) mmHg with PAP/SAP 0.50 (0.35-0.60). All patients survived and there were no complications related to ECMO. Extracorporeal membrane oxygenation is an effective treatment in critical pulmonary hypertension and should be considered in all patients in whom this is refractory to conventional measures.

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Sick preterm infants may, under certain conditions, demonstrate blood pressure passive cerebral blood flow in response to changes in arterial carbon dioxide tension. Blood pressure in turn depends on cardiac output and peripheral resistance. A Doppler technique for assessing cardiac output compared favourably in terms of reproducibility to a thermodilution technique in a group of infants undergoing cardiac catheterization for congenital heart disease. Doppler was subsequently used to monitor changes in cardiac output following an increase in arterial carbon dioxide tension of 1 kPa in 25 ventilated preterm infants. Blood pressure increased significantly (p = 0.006). However, heart rate did not change significantly (p = 0.16) and, in addition, both stroke and minute volume decreased (p = 0.023, p = 0.02, respectively). This suggests that accompanying changes in components of peripheral resistance exert important effects on blood pressure in the preterm neonate in response to changes in arterial carbon dioxide tension.

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Aortic arch anomalies in infancy often cause intrathoracic airway obstruction. Airway function was assessed as part of the diagnostic evaluation in six symptomatic infants both by plethysmography and using a chest compression technique to obtain partial flow-volume loops. Two infants had normal intrathoracic airway function and their symptoms were unrelated to aortic arch abnormalities. The remaining four had complete vascular rings (three double aortic arch, one pulmonary sling) and had increased expiratory airway resistance (Raw) (mean Raw = 700% predicted) and greatly decreased maximum flow rates at functional residual capacity (VmaxFRC; mean VmaxFRC = 34% predicted) with gross shape abnormalities of the flow-volume loop. Postoperatively airway function was substantially improved (mean Raw = 175% predicted, VmaxFRC = 79% predicted) but some abnormality of flow-volume loop shape remained, suggesting that tracheal dynamics were not completely normal in the early postoperative period.

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A six-month-old girl presented with heart failure. Cardiac catheterisation showed a ventricular septal defect and an anomaly of the aortic arch complex, which is a challenge to embryologic theory.

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Infective pericarditis is rare; the characteristic presenting illness in 3 children, each aged under one year, is recorded. Early diagnosis is important since surgical drainage of the pericardium is often necessary in addition to antibiotic therapy. The clinical features may suggest pericarditis, but diagnosis is readily and reliably confirmed by echocardiography.

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Two dimensional echocardiography has been used to guide balloon atrial septostomy in five cases of transposition of the great arteries. A single lumen balloon catheter was used. The catheter can be accurately located within the left atrium before inflation of the balloon, thus avoiding some of the traumatic accidents possible during septostomy. The balloon can be observed creating the atrial defect and the approximate size of the resulting defect noted. It can also be seen if the foramen ovale is only being stretched and not torn. The use of the two dimensional echocardiogram during septostomy should significantly contribute to the speed, efficiency, and safety of the procedure.

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Optimal management of coarctation with ventricular septal defect is difficult. Should one treat the coarctation, the ventricular septal defect, or both? This dilemma was investigated by reviewing 39 successive patients, aged less than 4 months, undergoing coarctation repair without pulmonary artery banding. Twelve hospital deaths occurred. Ventricular septal defect size was graded "blind" according to preoperative angiocardiographic and haemodynamic findings. Of 14 patients classified as having a large ventricular septal defect, necropsy and operative findings in eight showed defects 7 to 12 mm in diameter. Factors associated univariately with significantly increased mortality were young age, raised atrial and ventricular end-diastolic pressures, low weight, high admission blood urea, preoperative ventilation, and a large ventricular septal defect. All but the last two were also closely associated with each other. A jack-knifed discriminant function based upon ventricular septal defect size, blood urea, pulmonary venous oxygen content, and inferior caval oxygen saturation correctly predicted outcome in 78.9% of patients. Combinations of these four giving a probability greater than 0.9 of survival were rare with blood ureas above 8 mmol/litre. Raised blood urea was associated with low descending aortic pressure and subsequent dialysis. Only patients with a large ventricular septal defect stand to benefit from pulmonary artery banding at initial operation. Preoperative treatment to increase renal blood flow (prostaglandins, dopamine) may improve overall survival.

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Despite balloon atrial septostomy within the first days of life, some patients with complete transposition of the great arteries die before reaching elective definitive surgery in the second six months of life. To discover why, we analysed the fate of 144 patients who had balloon atrial septostomy after 1966, using a modified logrank survival test with multivariate capability. Patients were withdrawn "alive" on reaching definitive surgery. The following largely independent factors were associated with a statistically significant excess mortality: pulmonary hypertension, the presence and size of a ventricular septal defect of persistent ductus arteriosus, relative anaemia, absence of left ventricular outflow tract obstruction, low arterial oxygen saturation, aortic stenosis and coarctation, and balloon atrial septostomy between 1 week and 1 month of life. Those of the above factors which can be determined at balloon atrial septostomy or at routine cardiac catheterisation at 3 months of age were then introduced into discriminant function analysis on survival to 6 months. Hence the probability of any individual patient dying in the first six months was calculated, allowing for these factors. This prediction was correct in 76 per cent of the patients studied. By offering earlier definitive correction to patients thus identified as being at high risk of premature death, it should prove possible to reduce overall mortality in transposition of the great arteries.

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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.

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The effect of the cardioselective beta-adrenoreceptor blocking compound, metoprolol, was compared with methyldopa in the long-term management of hypertension. Thirty patients given metoprolol and twenty-six given methyldopa were treated for 2 years. The maximum dose of metoprolol was 200 mg twice daily (average 308 mg) and of methyldopa 1,000 mg twice daily (average 1,120 mg). Blood pressure was similar at entry to the study (metoprolol 177/110 mmHg and methyldopa 181/111 mmHg). After 2 years of treatment the blood pressure levels were again similar (metoprolol 149/91 mmHg and methyldopa 148/91 mmHg). Erect pressures were lower in the methyldopa group, but there was no difference between supine and erect blood pressure levels in those on metoprolol. At an exercise load of 300 and 600 kpm the increase in systolic pressure was significantly less in the metoprolol group. The proportional increase in systolic and diastolic pressure in response to a standardized stress situation was reduced by treatment with metroprolol but not by methyldopa. Tolerance to therapy did not develop in either group. The main difference between metoprolol and methyldopa was in the incidence and severity of side effects. Four patients were withdrawn from the metoprolol group. Seventeen were withdrawn from the methyldopa mainly because of side effects including drowsiness, depression, skin rash, and impotence. Six patients on metoprolol and seventeen on methyldopa continued on therapy although side effects were present. It is concluded that metoprolol and methyldopa lower blood pressure to the same extent, but metoprolol is advantageous because of a lower incidence of side effects.

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In summary, many of these patients have achieved a normal working capacity, but as a group there is a statistically significant reduction when compared to healthy children (p < 0.01). There was no significant difference between the group of patients who had their Mustard operation in infancy and later. As yet, there is no suggestion that the older patients, or those with the longest time interval between the operation and the exercise test, have a progressive reduction in working capacity. The decreased working capacity and moderate increase in ventilation suggests restricted cardiac output on exercise. This could be related to the abnormal heart rate response rather than indicating poor ventricular function. Equally, a restricted cardiac output on exercise may be due to inefficiency of atrial transport, tricuspid regurgitation or unmasked pulmonary vascular obstructive disease. Further studies may clarify these points. This study demonstrated that the exercise performance of asymptomatic patients 6-13 years after Mustard's operation was somewhat diminished, compared with a group of normal children. Nevertheless, these patients did lead and enjoy a normal life. Only longitudinal studies may bring the final answer to the important question: How many patients will be alive with normal or near normal exercise tolerance 40-50 years after Mustard's operation? Until such an answer is available, we believe that the Mustard operation should be used in the treatment of TGA, while the alternative techniques are carefully explored.

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Three patients with aortic arch anomalies presented with severe heart failure, acidaemia, and poor or absent upper and lower limb pulses. Prominent carotid arterial pulsations were detected which distinguished the condition clinically from aortic atresia. In 2 patients with a left aortic arch, interruption was distal to the left common carotid artery and there was an aberrant right subclavian. In the 3rd patient with a right aortic arch, interruption was distal to the right common carotid and there was an aberrant left subclavian artery. By dilating the narrowed ductus, prostaglandin E2 infusion corrected the acidosis. Successful surgical correction was achieved in one patient by direct anastomosis of descending aorta to ascending aorta and the base of the left common carotid artery.

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