Tuberculous pericarditis in children: a review of 44 cases.

This retrospective study reports experience in 44 children with pericardial tuberculosis seen during a 6-year period, 1986 to 1991. Thirty-seven children presented with pericardial effusion (PE), 4 with constrictive pericarditis and 3 with effusive constrictive disease. Features of cardiac tamponade were present in 90%, and in 91% radiologic cardiomegaly was found. A two-dimensional echocardiogram is diagnostic for PE. Culture and biopsy yields for Mycobacterium tuberculosis were low (18%). The Mantoux test was positive (> 10 mm) in 75%. In 14 patients doubt about etiology necessitated treatment for possible pyogenic infection with anterior pericardiotomy as opposed to diagnostic pericardiocentesis with catheter drainage. Twelve cases with PE were treated with steroids and the other 25 received only anti-tuberculous medication. Five cases of PE developed constrictive pericarditis during treatment of whom none received steroids. No statistically significant differences were demonstrated between the two groups. Only 5 of the 12 cases with constrictive pericarditis required pericardiectomy. There were no deaths and recovery has been complete in 43 cases. This study shows that tuberculous pericardial disease is common in areas with a high prevalence of tuberculosis. Prognosis is excellent for a condition in which morbidity and mortality have previously been high.

Paediatric heart transplants--should we do them?

Heart transplantation continues to evoke considerable controversy. While they are performed in adults, in South Africa children are at present denied this therapeutic option despite a very encouraging global/average 5-year survival rate of 62%. Objections to transplantation are usually based on moral and economic imperatives. These arguments will be analysed and we will present the case for the introduction of a transplant programme designed to benefit children with incurable heart disease.

Microcephaly-cardiomyopathy: a new autosomal recessive phenotype?

A distinctive phenotype of severe microcephaly and self-limiting dilated cardiomyopathy has been observed in two sibs suggesting autosomal recessive inheritance. Mental retardation, delayed developmental milestones, and minor dysmorphism were additional features.

Mitral valve replacement in infancy. A case report.

A 5-month-old infant with congenital mitral valve disease successfully managed by replacement with a mechanical prosthetic valve is reported. The incidence and management of congenital mitral valve defects is discussed.

Infective endocarditis in South African children.

The clinical and laboratory findings in 29 children with infective endocarditis over the past 10 years are reviewed retrospectively. Twenty children (70 per cent) had congenital heart disease, five (16 per cent) had rheumatic heart disease, and four (14 per cent) had no underlying cardiac abnormality prior to presentation. Twelve (41 per cent) patients had undergone cardiac surgery prior to developing endocarditis. The most common causative organisms were Staphylococcus aureus, Staphylococcus epidermidis, and viridans streptococci none of which was associated with a significantly greater mortality. There was a high case fatality rate of 35 per cent of children below the age of 6 months and children without an underlying cardiac abnormality were particularly at risk. There is a need for standardization of diagnostic criteria for infective endocarditis to facilitate accurate collaborative epidemiological investigation.

Myocardial infarction in Kawasaki disease.

A 5 1/2-month-old infant with Kawasaki disease complicated by coronary aneurysms is described. Despite myocardial infarction, the patient survived and is improving on medical management alone. Coronary aneurysms occur in 20% of cases presenting with Kawasaki disease. Medical management is aimed at preventing infarction and promoting healing of aneurysms. The use of anticoagulants and thrombolytics in Kawasaki disease is discussed.

Effect of kwashiorkor on the cardiovascular system.

In kwashiorkor the heart is clinically and radiologically small. This study utilises echocardiography, a tool not previously used in this disease, to show that this is due to decreased muscle mass.

Unusual cause of pulsatile liver. A case report.

A patient with tricuspid atresia developed a pulsatile liver after palliative cardiac surgery. This was complicated by infective endocarditis causing gross mitral incompetence; it is postulated that this was the cause of the pulsatile liver.

Management of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum with a small or hypoplastic right ventricle.

Twenty-one neonates and infants less than 3 months old undergoing cardiac surgery for an obstructed right ventricular outflow tract, intact ventricular septum and a small or hypoplastic right ventricle were retrospectively analyzed, in order to assess the effects of a change in management protocol. Seven of the 8 patients with critical pulmonary stenosis survived surgery using a transannular outflow patch, whereas only 1 of the 8 patients with pulmonary atresia survived the same operation. Two patients in the latter group died 2 and 3 months after surgery but with complications arising from surgery. Of 5 patients with pulmonary atresia who had a modified Blalock Taussig shunt, 3 patients survived the surgery and were discharged home. These results significantly indicate that there is an unacceptably high mortality for the relief of pulmonary atresia (with intact septum) using a transannular outflow patch, and a Blalock Taussig shunt is the preferred operation. The transannular outflow patch is a safe operation for neonates with critical pulmonary stenosis, irrespective of the size of the right ventricle.

The problem of compliance in rheumatic fever.

During a 12-month period 115 patients defaulted from a rheumatic fever clinic, so a study was undertaken to identify factors related to non-compliance by comparing defaulters with a group of 50 regular attenders. Those defaulting were significantly more likely to be coloured, male, and over 12 years old. They lived 10-99 km from the hospital, were on several drugs and despite more frequent appointments, usually had a record of poor attendance. The severity of the underlying heart disease and use of parenteral penicillin did not affect compliance. Since the use of regular penicillin prophylaxis for the secondary prevention of rheumatic fever is an essential step in reducing the prevalence of rheumatic heart disease, rheumatic fever clinics should be structured to address the needs of adolescents. Furthermore, the use of neighbourhood clinics for routine therapy between visits to a rheumatic fever clinic is essential to improve compliance.

Valve replacement in children.

The performance of the St. Jude prosthetic valve is reviewed in 81 patients aged 3 to 15 years. All 66 mitral (2 re-replacements), 8 aortic and 9 double valve replacements between February 1979 and August 1984 are included. The early mortality was 3.7% and actuarial analysis shows a 90% event free survival up to 5 years. Anticoagulant therapy was used in most patients, but comparison between groups receiving warfarin or aspirin or no therapy reveals no differences in the complication rate. The valve is well suited for use in children since the early degeneration seen with heterograft valves does not occur, and anticoagulation is not essential.

Hypertrophic cardiomyopathy in infancy and childhood.

Hypertrophic cardiomyopathy (HCM) presented in 10 children under 2 years of age (group 1) and in 5 between 3 and 8 years (group 2). The clinical, ECG, chest radiographic and echocardiographic features are reviewed and prognosis over a mean follow-up period of 3.5 years is reported. Patients in group 1 had more symptoms and 7 had evidence of heart failure at some stage; all had ECG abnormalities. Group 2 patients presented with murmurs and only 1 had heart failure. Medical management of these patients is discussed and the importance of accurate diagnosis stressed, since HCM may have a poor prognosis in childhood; 3 out of 15 patients have died.

Frontonasal dysplasia associated with tetralogy of Fallot.

Three children with frontonasal dysplasia associated with tetralogy of Fallot are reported. All cases had true hypertelorism and a median nasal groove with absence of the nasal tip. There was no mental deficiency. The facial anomaly is a sporadic, non-genetic interference of the normal development of the face. This is the first report of frontonasal dysplasia associated with a cardiac defect. Multifactorial inheritance of this syndrome is proposed.

Rupture of tendinous chords during acute rheumatic carditis in young children.

Four children (age range 2 3/4-6 1/2 years) presented with acute mitral regurgitation due to rupture of the tendinous chords. All required urgent replacement of their mitral valve. Subsequent histological examination confirmed acute rheumatic carditis in all. Acute rheumatic carditis is a rare cause of ruptured tendinous chords of the mitral valve especially in young children. The difficulties in diagnosis and management are discussed.

Endomyocardial biopsy technique in infants and small children.

Endomyocardial biopsy is performed when a histological diagnosis is required, e.g. in patients with cardiac failure due to unknown causes, in anthracycline toxicity, and to evaluate cardiac transplant rejection. Right ventricular endomyocardial biopsy has been carried out in 10 infants and small children (average age 14 months); the technical aspects of the procedure are discussed. Performed by a paediatric cardiologist endomyocardial biopsy in infancy is as safe as a routine cardiac catheterization.

A clinical and anatomical analysis of univentricular hearts.

Between January 1982 and May 1984 37 patients with a univentricular heart who had undergone two-dimensional echocardiography and cardiac catheterization were reviewed. The clinical, electrocardiographic, radiological and anatomical data are analysed and the controversy regarding this condition is discussed. The management and appropriate surgical procedures are presented.

Brain abscess in children due to Streptococcus milleri. A report of 2 cases.

Brain abscesses due to Streptococcus milleri occurring in 2 previously healthy children are reported. Both children had evidence of liver involvement and both responded well to antibiotic treatment.