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Tropical endomyocardial fibrosis (EMF) is a neglected disease of poverty that afflicts rural populations in tropical low-income countries, with some certain high-prevalence areas. Tropical EMF is characterized by the deposition of fibrous tissue in the endomyocardium, leading to restrictive physiology. Since the first descriptions in Uganda in 1948, high-frequency areas for EMF have included Africa, Asia, and South America. Although there is no clear consensus on a unified hypothesis, it seems likely that dietary, environmental, and infectious factors may combine in a susceptible individual to give rise to an inflammatory process leading to endomyocardial damage and scar formation. The natural history of EMF includes an active phase with recurrent flare-ups of inflammation evolving to a chronic phase leading to restrictive heart failure. In the chronic phase, biventricular involvement is the most common presentation, followed by isolated right-sided heart disease. Marked ascites out of proportion to peripheral edema usually develops as a typical feature of EMF. EMF carries a very poor prognosis. In addition to medical management of heart failure, early open heart surgery (endocardectomy and valve repair/replacement) appears to improve outcomes to some extent; however, surgery is technically challenging and not available in most endemic areas. Increased awareness among health workers and policy makers is the need of the hour for the unhindered development of efficient preventive and therapeutic strategies.

Background— Early case detection is vital in rheumatic heart disease (RHD) in children to minimize the risk of advanced valvular heart disease by preventive measures. The currently utilized World Health Organization (WHO) criteria for echocardiographic diagnosis of subclinical RHD emphasize the presence of pathological valve regurgitation but do not include valves with morphological features of RHD without pathological regurgitation. We hypothesized that adding morphological features to diagnostic criteria might have significant consequences in terms of case detection rates. Methods and Results— We screened 2170 randomly selected school children aged 6 to 17 years in Maputo, Mozambique, clinically and by a portable ultrasound system. Two different echocardiographic sets of criteria for RHD were assessed: “WHO” (exclusively Doppler-based) and “combined” (Doppler and morphology-based) criteria. Independent investigators reviewed all suspected RHD cases using a higher-resolution, nonportable ultrasound system. On-site echocardiography identified 18 and 124 children with suspected RHD according to WHO and combined criteria, respectively. After consensus review, 17 were finally considered to have definite RHD according to WHO criteria, and 66 had definite RHD according to combined criteria, giving prevalence rates of 7.8 (95% confidence interval, 4.6 to 12.5) and 30.4 (95% confidence interval, 23.6 to 38.5) per 1000 children, respectively ( P Conclusions— Important consideration should be given to echocardiographic criteria for detecting subclinical RHD because the number of cases detected may differ importantly according to the diagnostic criteria utilized. Currently recommended WHO criteria risk missing up to three quarters of cases of subclinically affected and therefore potentially treatable children with RHD.

Background— Infants and young children with certain types of heart disease are at increased risk for thromboses. Clopidogrel 75 mg/d is used in adults to prevent thrombotic events. The dose to achieve similar platelet inhibition in children is unknown. The objectives of the present study were (1) to determine the dose of clopidogrel needed in infants and young children to achieve a mean 30% to 50% inhibition of 5-μmol/L ADP–induced platelet aggregation (ie, inhibition similar to that observed with 75 mg in adults) and (2) to assess the safety and tolerability of clopidogrel in infants and young children. Methods and Results— We performed a prospective, multicenter, randomized, placebo-controlled trial evaluating the pharmacodynamics of clopidogrel in children (0 to 24 months) with a cardiac condition at risk for arterial thrombosis. Patients were randomized to clopidogrel versus placebo in a 3:1 ratio in 4 sequential groups (0.01, 0.10, 0.20, and 0.15 mg/kg) for ≥7 and ≤28 days. Platelet aggregation was assessed at baseline and steady state by light-transmission aggregometry. Of 116 patients enrolled, 92 (50% neonates, 50% infants/toddlers) were randomized, and 73 completed the study. A total of 79% of the randomized and treated patients were taking aspirin. Compared with placebo, clopidogrel 0.20 mg · kg −1 · d −1 resulted in a mean 49.3% (95% confidence interval 25.7% to 72.8%) inhibition of the maximum extent of platelet aggregation and a mean 43.9% (95% confidence interval 18.6% to 69.2%) inhibition of the rate of platelet aggregation. There was marked interpatient variability in the degree of platelet aggregation inhibition within each treatment-dose group and age group. No serious bleeding events occurred. Conclusions— Clopidogrel 0.20 mg · kg −1 · d −1 in children 0 to 24 months of age achieves a platelet inhibition level similar to that in adults taking 75 mg/d. Clopidogrel is well tolerated in infants and young children at this dose.

Background— This study was undertaken to determine outcomes and best strategies for treatment of occluded pulmonary arteries in patients with congenital heart disease. Methods and Results— Between 1998 and 2002, occlusion of a previously patent pulmonary artery was established in 23 patients. Data were obtained retrospectively. Diagnoses were pulmonary atresia and ventricular septal defect in 11, tetralogy of Fallot in 6, and other forms of pulmonary stenosis or atresia in 6. Median age and weight at diagnosis were 9 years (range, 6 days to 43 years) and 24 kg (range, 2.6 to 60 kg). Fourteen patients had had a previous surgery. The occluded pulmonary artery was visualized at angiography by wedge injection or injection into the collateral circulation. The left pulmonary artery was occluded in 20 patients and the right pulmonary artery in 3. Criteria for reconstruction were estimated duration of occlusion 0.2. Twelve patients fulfilled these criteria and underwent pulmonary artery reconstruction at a mean interval of 2 months (range, 6 days to 6 months) from evidence of occlusion. Six patients had pericardial patch reconstruction, 3 terminoterminal anastomosis, 2 thrombectomy, and 1 a Blalock-Taussig shunt. There was 1 late death. At a median follow-up of 4 years (2 months to 5 years), all patients underwent cardiac catheterization: in 8 patients the reconstructed artery was patent, in 3 reoccluded. Hypoplasia of the occluded artery was reversed in 6 patients. Conclusions— Our data show that in selected patients, reconstruction of an occluded pulmonary artery can restore pulmonary vascularization and reverse hypoplasia. Strict surveillance is mandatory to prevent pulmonary artery loss.

Background —Pulmonary regurgitation can lead to severe right ventricular dysfunction, which is a delicate postoperative problem in the long-term follow-up of patients who had surgery for congenital heart diseases. Clinical conditions of patients suffering from pulmonary valve incompetence are improved by valve replacement with a prosthetic valve. To date, the surgical approach is the only option to replace a pulmonary valve. We report the first experience of percutaneous pulmonary valve implantation. Methods and Results —A fresh bovine jugular vein containing a native valve was sutured into a vascular stent and then cross-linked with a 0.6% glutaraldehyde solution for 36 hours. After being hand-crimped onto a balloon catheter, the device was inserted percutaneously according to standard stent-placing techniques. The valved stent was finally deployed in the position of the native pulmonary valve of the lamb. Hemodynamic evaluation was carried out before and 2 months after implantation. Anatomic evaluation was finally performed. Percutaneous pulmonary valve replacement was successful in 5 lambs. No complications were noted. Early and late angiographic and hemodynamic studies confirmed a good position of the stents with a competent valve at the end of the protocol. One stent was slightly stenotic, with macroscopically visible calcifications. Conclusions —Nonsurgical implantation of pulmonary valves is possible in the lamb. This new technique is similar to standard stent implantation. Thus, it should be feasible in humans, in whom it will lead to a significant reduction of reoperations in patients in need of pulmonary valve replacement.

Background —Transposition of the great arteries (TGA) is a life-threatening malformation in neonates, but it is amenable to complete repair. Prenatal detection, diagnosis, and early management may modify neonatal mortality and mortality. Methods and Results —Preoperative and postoperative morbidity and mortality were compared in 68 neonates with prenatal diagnosis and in 250 neonates with a postnatal diagnosis of TGA over a period of 10 years. The delay between birth and admission was 2±2.8 hours in the prenatal group and 73±210 hours in the neonatal group ( P P 1 infusion, operation date). Preoperative mortality was 15 of 250 (6%; 95% CI, 3% to 9%) in the neonatal group and 0 of 68 in the prenatal group ( P P P Conclusions —Prenatal diagnosis reduces mortality and morbidity in TGA. Prenatal detection of this cardiac defect must be increased to improve early neonatal management. In utero transfer of fetuses with prenatal diagnosis of TGA in an appropriate unit is mandatory.

We thank Caruthers and Dorsch for their interest in the Platelet Inhibition in Children On cLOpidogrel (PICOLO) trial.1 The variability in inhibition of ADP-induced platelet aggregation by clopidogrel reported for pediatric patients in PICOLO (Figure 2 of our article1) is similar to the well-described variability in inhibition of ADP-induced platelet aggregation by clopidogrel observed in adult patients.2 Caruthers and Dorsch’s main concern is that because clopidogrel undergoes metabolism through CYP3A4, lower CYP3A4 activity …

A 3-month-old infant was transferred to our hospital for evaluation of a heart murmur associated with severe respiratory distress. At echocardiography, an aberrant origin of the left pulmonary artery was suspected (Figure 1, Movies I and II). Figure 1. Two-dimensional echocardiographic left parasternal short-axis view (left panel) and color Doppler mapping in high parasternal short-axis view (right panel). Please note that the left pulmonary (LPA) artery is only visualized on color Doppler mapping. Note the …

Anewborn infant (39 weeks’ gestation, birth weight 2.9 kg) was transferred urgently to our hospital with a transcutaneous oxygen saturation of 40% and a clinical suspicion of obstructed pulmonary venous return. Detailed cardiac ultrasound showed several large muscular ventricular septal defects with right to left shunting and a collecting vein behind the left atrium; however, the pulmonary venous anatomy was not clearly delineated. In particular, both the superior and inferior caval veins were enlarged, …

A newborn was referred to our department for severe cyanosis with cardiac systolic murmur. Echocardiography revealed in the right ventricle a 12-mm tumor with the typical aspects of a teratoma. The base of the tumor was implanted in the right ventricle apex and was composed of cysts …

A 12-year-old boy with pulmonary atresia and ventricular septal defect was initially treated with 2 modified Blalock-Taussig shunts. Total repair with closure of the ventricular septal defect and placement of a nonvalved 18-mm conduit from the right ventricle to the pulmonary artery had been performed when he was 5 years old. …

A10-year-old boy with aortic valve stenosis treated by surgical valvotomy when he was 6 months old underwent balloon dilatation of recurrent aortic stenosis. The procedure was performed through the right femoral artery under general anesthesia with mechanical ventilation. The aortic valve was dilated with a balloon 20 mm in diameter. The peak systolic gradient dropped from 120 to 30 …

From May 1977 to August 1982 50 patients who were 1.5 to 44 months old underwent anatomic correction of transposition of the great arteries (TGA) and closure of ventricular septal defect (VSD) at our institution. Thirty-nine patients underwent preliminary pulmonary arterial banding. Hospital mortality was 32%: four patients died as a result of technical problems, seven as a result of associated lesions, three of pulmonary hypertension, and two of left ventricular failure. Three other patients died after the first postoperative month (one of mediastinitis, one at reoperation for a residual VSD, and one of pulmonary hypertension). All 31 survivors are in excellent clinical condition and are in sinus rhythm after a mean follow-up period of 31 +/- 14 months. Twenty-five patients were reinvestigated by echocardiography (M mode and two-dimensional) and/or catheterization. Parameters of left ventricular contractility were within normal limits, but systolic aortic diameter was larger than normal (p less than .01). Seven patients had stenosis of the right ventricular outflow tract and five of these required reoperation. The two persistent problems with the anatomic correction of TGA associated with VSD are a relatively high operative mortality and secondary right outflow tract stenosis. However, use of this procedure results in better left ventricular function and fewer arrhythmias than does use of atrial repair techniques and also results in the use of the anatomically left ventricle as the systemic ventricle.