Isolated Hypoplasia of a Right Ventricle That Showed Right Ventricular Restrictive Cardiomyopathy

抄録

We report the case of a 15-year-old girl with isolated right ventricular hypoplasia without interatrial shunt. At 13 years of age, her right ventricular end-diastolic volume was 58% of the normal and the left ventricular end-diastolic volume was within the normal range with (98% of normal) despite the absence of interatrial shunt. Doppler echocardiography showed anterograde diastolic pulmonary arterial flow, coinciding with the A wave of atrial contraction. At 15 years of age, the right ventricular end-diastolic volume remained unchanged, but the left ventricular end-diastolic volume decreased to 65% of normal. The right ventricular pressure wave form showed a clear dip and plateau and the exacerbation of the restrictive change. The fact that the deterioration of the right ventricular diastolic disorder progressed with the decrease of the left ventricular end-diastolic volume suggests that end-diastolic volume of the patient’s left ventricle was maintained with the end-diastolic forward flow to the pulmonary artery.