Caso 4/2012: homem de 25 anos com fibroma obstrutivo na via de saída do ventrículo direito

Abstract

Clinical data: During routine examination, cardiac murmur was heard in an 8-year-old patient. At the time, infundibular pulmonary stenosis with pressure gradient of 69 mmHg was diagnosed by echocardiography. Progressive fatigue on efforts has been noticed for two years, as well as unstable chest pain. No specific medication was administered during this period. Physical examination:The patient was in good general condition, eupneic, with normal pulse and normal color. Weight: 42.6 kg; height: 160 cm; BP: 110/70 mmHg; HR: 80 bpm, O2 saturation: 96%. The aorta could not be felt at suprasternal notch.Precordial systolic impulsions were discrete. Apical impulse was not palpable. The heart sounds were normal and systolic murmur ++ was harsh, across the left sternal border. The liver was not palpable and the lungs were clear. Complementary tests:Electrocardiogram showed sinus rhythm with signs of severe right ventricular overload with Rs morphology and negative T wave in V1, AP: +60 o , AQRS: +110 o , AT: +60 o ( Fig. 1).Chest radiography shows cardiac area within normal limits, with rounded morphology. The middle arch is rectified and the pulmonary vasculature is slightly increased (Fig. 1). (Fig. 2) showed hypertrophy of the right ventricular wall. There was a mass in the ventricular septum protruding into the right ventricular outflow tract causing a pressure gradient of 70 mmHg. The pulmonary valve was preserved, as well as biventricular function. The measures corresponded to RV =32 and LV = 34. EchocardiographyNuclear magnetic resonance imaging showed the same aspects with marked obstruction in the right ventricular outflow tract (Fig. 2).Cardiac catheterization also showed obstruction in the right ventricular outflow tract and pressure values were RV = 80/13, right ventricular outflow tract = 27/8, PT 27/15-19, LV = 100/14 mmHg.