Heart Transplantation in a 14-Year-Old Boy in the Presence of Severe Out-of-Proportion Pulmonary Hypertension due to Restrictive Left Heart Disease: A Case Report
A 14-year-old boy after balloon valvuloplasty of severe aortic valve stenosis in the neonatal period was referred for heart-lung transplantation because of high grade pulmonary hypertension and left heart dysfunction due to endocardial fibroelastosis with severe mitral insufficiency. After heart catheterization, hemodynamic parameters were invasively monitored: a course of levosimendan and initiation of diuretics led to a decrease of pulmonary capillary wedge pressure (from maximum 35 to 24？mmHg). Instead of an expected decrease, mean pulmonary artery pressures (mPAP) increased up to 80？mmHg with increasing transpulmonary pressure gradient (TPG) up to 55？mmHg. Oral bosentan and intravenous epoprostenol then led to a ~50% decrease of mPAP (TPG between 16 and 22？mmHg). The boy was listed solely for heart transplantation which was successfully accomplished 1 month later. 1. Case A 14-year-old boy presented with the history of severe aortic valve stenosis; he had undergone successful balloon valvuloplasty at the age of 3 days. He was on followup elsewhere and was reportedly in stable clinical condition for 13 years, performing alpine skiing, cross country running, and mountain biking. He reportedly had normal systolic left heart function in the presence of left ventricular endocardial fibroelastosis (EFE) without signs of elevated right ventricular pressure on echocardiography. Neither heart catheterization nor cardiopulmonary exercise testing was performed. At the age of 14 years, he complained of fatigue during cycling and hiking for the first time. On echocardiography at that time severe mitral valve regurgitation and a markedly enlarged left atrium were seen besides the well-known mild aortic valve stenosis/regurgitation and endocardial fibroelastosis. Tricuspid valve regurgitation was demonstrated with a maximum velocity of 5.28？m/sec presuming a systolic pulmonary artery pressure of 111？mmHg (Figure 1). Figure 1: Echocardiography: 4-chamber view showing extremely enlarged left atrium (LA) and endocardial fibroelastosis (arrows) of left ventricle (LV) and mitral valve apparatus (a). Doppler maximum velocity of tricuspid insufficiency before initiation of medical therapy, indicating high systolic RV pressure of 111？mmHg (b). RV = right ventricle; RA = right atrium; IAS = interatrial septum. The boy was scheduled for heart catheterization. On admission, he was physically still active but had stopped sports. On physical examination, he was eupneic with a heart rate of 79 beats per minute at rest, blood pressure was 100/60？mmHg, and transcutaneous oxygen
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