Investigation of cardiomyopathy in children with cirrhotic and noncirrhotic portal hypertension.

BACKGROUND: Cirrhotic cardiomyopathy (CCMP) is a functional disorder characterized by electrophysiological disturbances, and diastolic and/or systolic dysfunction in patients with liver disease. This disorder is a well-defined entity in adults, but pediatric data are limited. The aim of the study was to determine the incidence, features, and risk factors of CCMP in children with portal hypertension (PHT). METHODS: This study included 50 children with cirrhotic PHT (40/50) and noncirrhotic PHT (10/50). Fifty healthy children were also selected for the control group. Electrocardiography and echocardiography were used to evaluate cardiac functions. Corrected QT (QTc) ≥ 0.45 was accepted as prolonged on electrocardiography. The study group was divided into 3 groups: cirrhotic, noncirrhotic, and control. Then, the CCMP group was created according to the diagnostic criteria. Latent CCMP was diagnosed in the presence of prolonged-QTc along with a minor criterion (tachycardia). Manifest CCMP was diagnosed in the presence of at least 2 major criteria (prolonged-QTc along with abnormal echocardiographic findings). Moreover, in this study, the risk factors for CCMP were investigated. RESULTS: The CCMP group included 10 cases (20%). Nine of these cases had latent CCMP (18%), and the remaining one (2%) had manifest CCMP. All of the cases with CCMP had cirrhosis and ascites. None of the patients with CCMP had severe cardiac symptoms, but they were already using some cardioprotective drugs such as propanolol and spironolactone. As risk factors for CCMP, pediatric end-stage liver disease scores, Child-Pugh scores, and ascites grades were found to be significant for the determination of CCMP. The most important risk factor was ascites severity (P = 0.001, odds ratio 9.4). CONCLUSIONS: Approximately 20% of children with PHT have CCMP. A detailed cardiac examination should be carried out periodically in children with cirrhotic PHT, especially in the presence of ascites and high Child-Pugh score.

Recurrent hemiparesis due to anterior mitral leaflet myxomas.

Strokes, cardiac or noncardiac, generally affect the elderly and only occasionally occur in children. A tendency to stroke occurs in cyanotic congenital heart disease; however, this report describes two female patients, aged 6 years and 4 years, who had no cyanotic disease. The first patient was admitted to the hospital after the first stroke but suffered a second one after admission. Her initial cardiac examination was normal. The other patient was admitted after a second right-sided hemiparesis. A mild pansystolic ejection murmur was observed at the lower left sternal border on auscultation; slight late diastolic murmurs with opening snap were found in the mitral valve area, and the second heart sound was increased. Transthoracic echocardiography revealed a mass in the anterior mitral valve in both patients. These masses were completely removed surgically and pathology revealed myxomas. Hemiparesis completely resolved after surgical and medical treatment. The clinical manifestations of cardiac tumors primarily depend on their number, size, location, and histology. Strokes and anterior mitral valve masses are extremely rare in children. In the two cases reported here, anterior mitral valve myxomas caused systemic embolization as a result of their location. Echocardiography should be performed early in patients presenting with recurrent strokes to exclude such pathology.