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Thirty-two patients with double outlet right ventricle (DORV) were studied between 1960 and 1976. Associated congenital defects frequently compounded the difficulty of clinical diagnosis. Cardiac catheterisation was performed in 27 patients, and the ventriculograms were studied with particular regard to the relative positions of the great vessels to each other and to the ventricular septal defect. These relationships determine which corrective operation is possible. Correction has been performed in 12 patients with a perioperative mortality of 25%. Although mitral-aortic discontinuity was demonstrated in all cases, consideration of the anatomical spectrum included in the term DORV suggests that discontinuity is not an essential feature. In common with other clinical data and in contrast with necropsy studies, none of our patients was found to show the normal relationship of the great vessels to each other, in which the aorta lies posterior and to the right of the pulmonary artery. The reason for this difference between the clinical and necropsy findings is not apparent. A similar disparity was shown with regard to pulmonary stenosis, which was demonstrated at catheterisation in 68% of the 27 patients (mean gradient 68 +/- 3 mmHg), in contrast with incidences of 18% and 25% in recent necropsy series. Patients in the necropsy studies were frequently neonates or infants, in whom death may have been the result of intractable cardiac failure secondary to excessive pulmonary blood flow. In older patients without pulmonary stenosis and with pulmonary hypertension, frequent observation is imperative so that surgical treatment can be instituted before the development of irreversible pulmonary vascular disease. [ABSTRACT FROM PUBLISHER]

, 438-443. Bacterial pericarditis was diagnosed in 28 children under the age of 13 years during a five-year period at one hospital. The diagnosis was established clinically in 26 cases; in two the condition was first recognized at necropsy. was isolated in 22 patients, including all 12 with associated osteitis. The other principal primary condition was pneumonia. While 11 patients were treated with antibiotics only, 13 required one or more pericardial aspirations. Acute constrictive pericarditis occurred in two children, both of whom survived pericardiectomy. Of the 26 children with a clinical diagnosis of bacterial pericarditis, eight died in hospital (31%). The pericarditis probably caused death in two cases and was contributory in six. Septic pericarditis tends to occur in children who are already ill because of other foci of bacterial infection and may easily be overlooked, especially in infants. The large numbers reported reflect a high incidence of osteitis in this community. Electrocardiographic and radiological changes are almost always demonstrable, but these are not diagnostic. Antibiotic therapy is imperative and drainage of the pericardium is usually indicated. Bacterial pericarditis is associated with a high mortality. However, while early recognition and treatment is important, death is more often due to the general septicaemia than to pericardial tamponade. In this respect the pericarditis is one indication of the severity of the systemic illness. [ABSTRACT FROM PUBLISHER]

OBJECTIVE--To test the hypothesis that isolated coarctation of the aorta is associated with relative hypoplasia of the mitral valve, even when the valve is morphologically normal. DESIGN--Cross sectional and Doppler echocardiography were used in a prospective, paired, case control study to compare mitral valve dimensions and diastolic transmitral flow characteristics as indices of left heart development. 40 children with isolated coarctation and 40 size matched controls were examined. Within the coarctation group 14 children with apical diastolic murmurs were compared with 14 size matched patients without murmurs. SETTING--A supraregional tertiary referral centre for paediatric cardiology. OUTCOME MEASURES--Mitral valve diameters, measured from the parasternal long axis, short axis, and apical four chamber views; mitral valve cross sectional area measured from the parasternal short axis view; peak early (E) and peak atrial (A) phase diastolic transmitral flow velocities measured by pulsed wave Doppler from the apical four chamber view; derived E/A ratio and pressure half time of decay from peak E. RESULTS--Mitral valve dimensions were significantly smaller in children with coarctation than in controls for long axis diameter (median 1.74 v 1.90cm, p = 0.0001), short axis diameter (2.21 v 2.28 cm, p = 0.027), and cross sectional area (2.37 v 3.15 cm2, p = 0.001). Peak E and A velocities were significantly higher in patients than in controls (0.9 v 0.82 ms-1, p = 0.013 and 0.61 v 0.51 ms-1, p = 0.007). The only difference between children with coarctation plus murmurs and those without murmurs was a marginally longer pressure half time. CONCLUSIONS--Smaller mitral valve dimensions and increased diastolic transmitral flow velocities in children with isolated coarctation compared with normal children suggests that coarctation may be part of a generalised hypoplasia of left heart structures. [ABSTRACT FROM PUBLISHER]

OBJECTIVE--To assess the results of balloon dilatation of the aortic valve as first line treatment for infants presenting in the first two months of life with cardiac failure caused by severe aortic stenosis. DESIGN--A prospective study of all such infants presenting to a single centre over a three year period. PATIENTS--Of 14 consecutive infants aged < 2 months presenting with heart failure due to severe stenosis of the aortic valve, 12 underwent balloon dilatation of the valve. Two infants with hypoplastic left heart syndrome were excluded. Six patients had other significant cardiac malformations, nine had endocardial fibroelastosis. Four infants were moribund at presentation. INTERVENTIONS--Balloon dilatation was performed through the femoral artery under general anaesthetic, with heparin and antibiotic prophylaxis. Maximum inflated balloon size was selected as equal to or slightly less than the diameter of the aortic valve measured echocardiographically. In two infants balloon dilatation of coexistent severe coarctation was performed at the same time. RESULTS--Dilatation was achieved in all cases. Early survival rate (up to one month) was 75%; 50% survive up to three years. The two deaths occurring < 24 hours after dilatation probably related to the infants' poor condition at presentation and the presence of significant left ventricular hypoplasia. The other early death was due to severe mitral regurgitation from papillary muscle dysfunction. All later deaths were related to associated malformations. The difference in left ventricular size (diameter and length) measured echocardiographically at presentation between survivors and those that died is significant (p < or = 0.002). 83% of deaths occurred in infants with associated malformations. Cusp perforation occurred in one infant, which may have contributed to this patient's death. Left ventricular perforation and cardiac tamponade were treated successfully by prompt pericardiocentesis in another. CONCLUSIONS--Balloon dilatation of the aortic valve is an acceptable first line treatment for neonates with severe symptomatic stenosis of the aortic valve. Outcome depends principally on left ventricular size and on the presence, severity, and timing of treatment of associated malformations. Severe endocardial fibroelastosis was not a risk factor for early death but may have implications for long term survival. The survival rate for this high risk group might be improved by a stratified approach where patients with adequate left ventricular size have balloon dilatation whereas those with severe ventricular hypoplasia are managed like patients with the hypoplastic left heart syndrome. [ABSTRACT FROM PUBLISHER]

OBJECTIVE--To assess the clinical condition of patients and the adequacy of their newly constructed venous pathways after the Senning operation for simple transposition of the great arteries. PATIENTS AND DESIGN--All 34 patients who had the Senning operation between March 1983 and December 1986 were reviewed. Survivors had detailed cardiac catheterisation studies one to two years later and clinical evaluation two to six years after surgery. RESULTS--There were two operative deaths (6%), one sudden late death (after two years), and 31 survivors (91%). No clinical evidence of obstructed venous pathways was found and there was no need for reoperation for any reason. The average mean (SD) pressure gradient at the junction of the superior vena cava and systemic venous atrium was 2.0 (1.7) mm Hg, although two patients had gradients of 7 mm Hg. The average gradient was 0.7 (1) mm Hg in the inferior vena caval pathway, and 1.4 (1.1) mm Hg between the mean pulmonary arterial wedge and pulmonary venous atrial pressures. Only the two patients with gradients of 7 mm Hg at the junction of the superior vena cava and the systemic venous atrium had considerable narrowing of the pathway and retrograde flow in the azygos vein to below the diaphragm. CONCLUSION--A small series of patients were comprehensively studied after the Senning operation for simple transposition of the great arteries. Scrupulous technique in the construction of the venous pathways has had excellent results with no need for reoperation for obstruction in either the systemic or pulmonary pathways. Clinical observation alone may fail to identify patients with venous pathway obstruction, therefore careful assessment is required, even in patients who are physically normal. [ABSTRACT FROM PUBLISHER]

OBJECTIVE--The origin of innocent systolic murmurs is uncertain. This study was designed to evaluate the relative contribution in the same subjects of the main factors implicated in the genesis of vibratory innocent systolic murmurs--that is, the presence of left ventricular bands and increased left ventricular flow indices--and to examine evidence for turbulence in relation to these findings. PATIENTS--40 children with innocent systolic murmurs and 40 paired controls without murmurs were matched for age and body surface area. DESIGN--These subjects were examined prospectively by cross sectional echocardiography with pulsed and continuous wave Doppler and colour flow mapping. SETTING--A tertiary referral centre for paediatric cardiology. RESULTS--Left ventricular bands were significantly more prevalent in patients with innocent murmurs (70%) than in controls (48%) but only if apical bands were included (p = 0.039). Cases had significantly higher aortic peak flow velocities (p less than 0.001), flow integrals (p = 0.001), and stroke volumes (p = 0.014), and significantly lower heart rates (p = 0.033) than controls. There were no significant differences between cases and controls in mean aortic diameter, cardiac output, or ejection fraction. There was no evidence of turbulence in the left ventricle or outflow tract in any individual but curvilinear periodic vibrations and audible "whoops" were detected in the left ventricle by pulsed wave Doppler echocardiography. These were significantly more common in cases than controls (p less than 0.01) but were not related to the presence of bands. CONCLUSIONS--Innocent systolic murmurs were unrelated to aortic size but they were strongly associated with higher aortic flow volume and velocity, with relative bradycardia, and with evidence of vibratory phenomena in the left ventricle. The role of left ventricular bands remains uncertain. [ABSTRACT FROM PUBLISHER]

There are as yet few clinical reports of successful treatment of bacterial endocarditis with oral antibiotics, or of the use of the rifamycins in non-tuberculous infections. A case of refractory staphylococcal endocarditis successfully treated with oral rifampicin and erythromycin is presented. The availability of effective oral antibiotic therapy avoids the serious difficulties of long term parenteral antibiotics and offers a hopeful alternative to emergency cardiac surgery in this condition. [ABSTRACT FROM PUBLISHER]

A pair of thoracopagus conjoined twins is described in whom a common heart with atrial and ventricular conjunction was demonstrated by cardiac catheterization and at autopsy. Each twin had an independent noncommunicating single ventricle with separate origins of the aorta and pulmonary arteries. A complex pattern of pulmonary and systemic venous return resulted in a fugure-of-eight circulation not previously described which precluded surgical intervention. This report demonstrates the feasibility of detailed hemodynamic assessment in thoracopagus twins and the value of selective ventricular angiography with careful observation of the venous phase in determining an accurate preoperative diagnosis.

The technique of surface-induced hypothermia, circulatory arrest and limited extracorporeal circulation was used in the surgical correction of congenital heart defects in 125 young children. Hospital mortality was 18% and no death could be attributed to the surgical technique. An analysis of risk factors demonstrated that successful corrective surgery was not significantly related to age, body weight or pulmonary vascular obstructive disease. In transposition of the great arteries, the presence of a ventricular septal defect was associated with an increased mortality. Emergency operations performed because of severe hypoxaemia carried a high mortality, especially in patients with tetralogy of Fallot.

Mitral valve replacement (MVR) is associated with higher mortality and morbidity rates in children than in adults, and the use of heterograft valves has been encouraged. The results of MVR in 56 consecutive patients, aged 2 to 12 years, presenting between January, 1972, and January 1979, were reviewed to test these beliefs. The etiology of mitral valve disease was rheumatic in 46, congenital in eight, and acute bacterial endocarditis in two. All children were seriously disabled (NYHA Classes III and IV). Cardiac catheterization in 36 patients revealed mixed valve disease in 26, pure mitral regurgitation in seven, and pure mitral stenosis in three. Seventeen Starr-Edwards (SE), five Lillehei (L), Björk-Shiley (BS), eight Hancock (H), and 25 Carpentier-Edwards (CE) mitral prosthesis were inserted. Operative mortality was 2% (1 BS) and late mortality was 10% (three SE, one L, two CE). Serious late complications occurred in 30% of survivors, including 11 instances of calcific valve stenosis (five H, six CE), one case of valve thrombosis (1 L), and two embolic episodes (1 SER, 1 H). Survival curves were similar for patients with heterograft and mechanical valves (92% and 77% at 5 years). Event-free curves showed heterograft valves to have a far higher complication rate than mechanical valves (10% complication free at 4 years compared to 84% free at 5 years). Early operative results in children are excellent, and the overall mortality (10%) compares favorably with figures for MVR in adults. However the long-term durability and choice of prostheses remain problematical, since less than 10% of heterograft valves survive beyond 5 years.

We report the clinical and biochemical course of 2 patients who underwent portacaval shunting for the relief of homozygous type II hyperlipoproteinaemia. In our first patient, the initial favourable clinical and biochemical response subsequently deteriorated and a splenoportogram revealed the presence of a blocked splenic vein with a large collateral blood supply to the liver. The second patient has responded quite dramatically; there has been relief of her preoperative anginal attacks and a significant fall in her plasma cholesterol concentration. The present role of portacaval shunts in the management of this conditions is discussed.

We report the clinical and laboratory effects of continuous-flow plasma exchange in two patients suffering from homozygous familial hypercholesterolemia. In one (Case 1) plasmapheresis was performed at fortnightly intervals over a period of 18 months; in the other (Case 2) the necessity for surgical relief of an associated supravalvular aortic stenosis resulted in premature termination of the trial. The plasma cholesterol levels in both patients fell by 35 per cent from the mean before study in the course of treatment. In Case 1 this was associated with marked regression of the patient's xanthomas, disappearance of the S-T segment depression seen on effort electrocardiograms obtained prior to the introduction of plasmapheresis, possible widening of the stenosis present at the origin of the left anterior descending coronary artery, and a marked increase in exercise tolerance and diminished frequency of anginal attacks. Cessation of cholestyramine and clofibrate administration during this study did not in any way reverse the reduction of plasma cholesterol achieved by means of plasmapheresis combined with drug therapy. We conclude that plasmapheresis has a role to play in the management of patients with homozygous familial hypercholesterolemia.

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.

Angiography of the superior systemic veins was performed on 510 patients with congenital heart disease. An analysis of these angiograms was carried out and a simple angiographic classification of superior vena caval anomalies is presented. Eleven per cent of cases had complete bilateral superior vena cavae, a frequency of occurrence higher than that previously reported. An analysis of associated congenital heart lesions revealed a higher than average association of bilateral superior vena cavae with atrioventricular septal defects and double-outlet right ventricle, but this was seen rarely in cases of transposition of the great arteries. Bilateral superior vena cavae also occurred very frequently (72%) in patients with situs abnormalities. Femoral vein catheterisation is likely to facilitate diagnosis and appropriate radiological signs are discussed.

Lymphatic abnormalities are not generally recognized as part of the Noonan syndrome. A child with this condition in whom unique and widespread lymphatic abnormalities were demonstrated by lymphography is described. Both T and B lymphocytes were detected in chylous fluid drained from the thorax. In addition, the child was found to have a protein-losing enteropathy and cardiovascular defects. The clinical spectrum of the Noonan syndrome may include animalies of the lymphatic system.

An anomalous left coronary artery from the pulmonary artery was diagnosed clinically in 12 patients (and confirmed angiographically in 11 and at autopsy in 1). A classic history of 'infantile angina' was obtained in only 1 patient, while the typical electrocardiographic findings of anterolateral ischaemia or infarction were present in 11 patients (92%). The majority had evidence of left ventricular dysfunction and mitral regurgitation. Three patients underwent surgical reimplantation of the anomalous left coronary artery into the ascending aorta. Two survive, but with persistent electrocardiographic changes and cardiomegaly. Nine patients, of whom 4 survive, were managed medically. Five of the 6 deaths occurred within 1 month of diagnosis. A conservative approach to surgical intervention is recommended, as surgery is unlikely to alter the ultimate prognosis.

Cutis laxa is manifest histologically by fragmentation of elastic tissue throughout the body. The aorta and other systemic vessels show dilatation, elongation and even aneurysm formation. We report multiple pulmonary artery branch stenoses in a child who had cutis laxa. Pulmonary artery stenoses may be the cause of the pulmonary hypertension and right heart failure observed in patients with this condition.

An infant with hypotonia, gross cardiomegaly and heart failure is described. Angiocardiography revealed a hypertrophic restrictive cardiomyopathy. The diagnosis of type II glycogenosis was confirmed by the total absence of alpha-1,4-glucosidase in cultured skin fibroblasts. It is now possible to offer prenatal diagnosis by amniocentesis to women at risk of having affected children.