Electrocardiographic features of arrhythmogenic right ventricular cardiomyopathy in school-aged children.

It can be difficult to distinguish children with early-stage arrhythmogenic right ventricular cardiomyopathy (ARVC) from those with benign premature ventricular contraction (PVC). We retrospectively evaluated six school-aged children with ARVC and compared with those of 20 with benign PVC. The median age at initial presentation was 11.4 and 10.2 years in ARVC and benign PVC, respectively. None of the ARVC patients fulfilled the diagnostic criteria of ARVC at initial presentation. At ARVC diagnosis, the treadmill exercise test and Holter monitoring showed provoked PVC during exercise and pleomorphic PVC in all ARVC cases, respectively. During the observation period, terminal activation duration (TAD) was prolonged in all ARVC patients. In addition, ΔTAD (5.5 [3-10] ms) were significantly longer than those with benign PVC (p < 0.001). A new notched S-wave in V1 appeared in four (67%) ARVC patients, who had myocardial abnormalities in the right ventricle, and in zero benign PVC. Our electrocardiographic findings, such as provoked PVC during exercise, pleomorphic PVC, prolonged TAD, and a new notched S-wave in V1 could contribute to the early detection of ARVC in school-aged children.

Relationship between electrocardiographic signs and shunt volume in atrial septal defect.

BACKGROUND: The aim of this study was to determine whether electrocardiographic signs correlate with hemodynamics and the magnitude of the intracardiac shunt in children with ostium secundum atrial septal defects (ASD). METHODS: A total of 100 ASD patients (median age, 6 years 4 months; 54 girls) underwent cardiac catheterization between August 1980 and April 2010. We retrospectively investigated the relationship between electrocardiographic signs and the pulmonary/systemic blood flow ratio (Qp/Qs) in these patients. We also compared 63 postoperative electrocardiograms with those recorded before surgery. RESULTS: The mean Qp/Qs ratio of the 100 patients was 2.46 ± 0.81 (range, 1.1-5.0). The Qp/Qs ratio in patients with and without right bundle branch block (RBBB) was 2.57 ± 0.82 (n = 73) and 2.15 ± 0.72 (n = 27), respectively (P = 0.016). The Qp/Qs ratio in patients with and without isolated negative T-wave was 2.85 ± 0.87 (n = 38) and 2.22 ± 0.68 (n = 62), respectively (P = 0.0003). None of the patients with low Qp/Qs ratio (Qp/Qs ratio ≤ 1.5) had both RBBB and isolated negative T-wave. The prevalence of these two signs decreased from 73.0% (n = 46) and 36.5% (n = 23) to 15.9% (n = 10) and 15.9% (n = 10) after surgical repair, respectively. CONCLUSIONS: RBBB and isolated negative T-wave in the precordial leads are well correlated with high Qp/Qs ratio in ASD patients.

The histological findings in transposition of the great artery with severe persistent pulmonary hypertension of the newborn.

The combination of persistent pulmonary hypertension of the newborn (PPHN) and transposition of the great arteries (TGA) has serious impacts on treatment and prognosis, often with adverse outcomes. We report the case of a male full-term newborn with TGA with intact ventricular septum and severe PPHN who died 2 h after birth; further, we examined his vascular histology. On autopsy, lung histology showed mild fibrous hypertrophy in the intima and moderate medial hypertrophy of the minimal pulmonary artery. Hypoplasia of the pulmonary artery was not detected. Pulmonary congestion was detected and pneumatization was poor. Debris was present in the alveoli. Hemosiderin deposition was detected, suggesting prenatal hemostasis or hemorrhage. Severe PPHN may have occurred because of pulmonary arterial spasm accompanying pulmonary congestion which had been in the fetal stage. A wide range of lesions can be present in the pulmonary vascular bed in TGA. The pathologies of pulmonary vascular tissues with TGA and PPHN are not uniform. .

Steroid Pulse Therapy for Kawasaki Disease Complicated with Myocarditis.

BACKGROUND: The clinical management of intravenous immunoglobulin-resistant Kawasaki disease shock syndrome (KDSS) is obscure. CASE CHARACTERISTICS: Three children presented with intravenous immunoglobulin-resistant KDSS complicated with myocarditis. OUTCOME: All cases were successfully managed with steroid pulse therapy. MESSAGE: Steroid pulse therapy is effective in immunoglobulin-resistant KDSS.