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Objective: To describe regional incidence, presentation, and outcome of idiopathic (familial) and Noonan syndrome related infant hypertrophic cardiomyopathy (HCM) between 1969 and 1994.Design: Case series.Setting: Regional cardiac referral unit of the South West Region of England and south Wales, population approximately four million.Patients: 21 cases of idiopathic (or familial) HCM, and eight infants with Noonan syndrome.Main Outcome Measures: Survival and persistence or resolution of symptoms or cardiac hypertrophy.Results: Incidence: eight cases between 1969 and 1982 (idiopathic 6, Noonan 2), 21 cases between 1982 to 1994 (idiopathic 15, Noonan 6). Mode of presentation: cardiac failure, 17 (59%); murmur, 9 (30%); cyanosis, 2 (7%); family history, 1 (7%). Age at presentation: 0-7 days, 16 (55%); 8 days-4 months, 9 (31%); 5-12 months, 4 (14%).Outcome: five deaths (17%), all < 1 year, all from progressive cardiac failure (idiopathic 3, Noonan 2). Four of these five had not received beta blockade. Among the 24 survivors (follow up 1.3-23.2 years, median 5.5 years) hypertrophy had resolved in nine (38%) (idiopathic 8, Noonan 1), was mild and asymptomatic in seven (29%), and was symptomatic or severe in eight (33%). All 10 infants presenting with septal thickness > 1.3 cm have persistent cardiac hypertrophy.Conclusions: Mortality in infant HCM is much lower than previously reported and resolution is more frequent. This may reflect increased detection of less severe forms in addition to the success of aggressive medical management including beta blockade. [ABSTRACT FROM AUTHOR]

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]

We report a case of mucocutaneous lymph node syndrome, which was diagnosed in a 13 month old boy two years after the death of his sister from the same disease. [ABSTRACT FROM AUTHOR]

A small number of patients with haemangioma need treatment for serious complications such as Kasabach-Merritt syndrome, cardiac failure and obstruction of the airway. We report on the management of an infant with Kasabach-Merritt syndrome. [ABSTRACT FROM AUTHOR]

An infant presented shortly after birth with signs suggestive of aortic coarctation. Echocardiography revealed an extensive aortic arch thrombus, not amenable to surgery. Thrombolytic agents reduced thrombus size, enabling survival, but failed to prevent neurological damage secondary to cerebral embolisation. [ABSTRACT FROM AUTHOR]