Marfan Sendromu Olan Çocuklarda Kardiyovasküler Sistemin Değerlendirilmesi

Abstract

Marfan Syndrome (MFS) is an inherited connective tissue disease that causes morbidity and mortality at early ages. Aortopathies and atrioventricular valve pathologies are the most common cardiovascular complications. In recent years, it has been reported that myocardial involvement can also be seen in Marfan syndrome. However, studies are substantially with adult patients; tissue Doppler imaging (TDI) or strain echocardiography studies with pediatric MFS patients have not been reported. In this study, myocardial functions of the children with MFS were evaluated with TDI and strain echocardiography in addition to conventional echocardiographic studies. The conduction system was evaluated with electrocardiography (ECG) and Holter monitoring. Of the 22 patients, 86% had aortic root enlargement, 95% had mitral valve prolapse, and 45% had tricuspid valve prolapse. In left ventricular (LV) evaluations, systolic function with m-mode was normal in all patients. However, TDI showed decreased systolic functions in 42% and impaired myocardial relaxation in 37% of the patients. In addition, LV peak longitudinal strain were low in 48% of the patients. Right ventricle systolic functions were detected low in 55% of the patients by TDI. ECG and Holter monitoring revealed incomplete right bundle branch block in 2 patients, left axis deviation in 1 patient, and non-sustained ventricular tachycardia in 1 asymptomatic patient with normal baseline ECG. In conclusion, we recommend evaluating myocardial functions with TDI and strain echocardiography in addition to conventional evaluations. We also recommend Holter monitoring to detect subclinical arrhythmias in asymptomatic patients with normal ECGs.