Severe aortic regurgitation and pulmonary hypertension in an 18-year-old patient after balloon aortic valvuloplasty (RCD code: IV-5.A2)

Abstract

Aortic stenosis is the most common form of congenital left ventricle outflow tract obstruction. It may be a life threatening ductal-dependent condition in newborns and necessitating urgent percutaneous balloon aortic valvuloplasty (BAV). Although early results of valvuloplasty are usually satisfactory, long-term follow-up show frequent incidence of gradually progressing aortic insufficiency that requires valve replacement surgery. The case report presented below concerns a young patient with a history of BAV in childhood who developed severe aortic regurgitation accompanied by combined post-and pre-capillary pulmonary hypertension. JRCD 2016; 2 (6): [192][193][194][195] Key words: aortic valve replacement, bioprosthesis, echocardiography, heart catheterization, iliac artery occlusion, congestive heart failure, sildenafil Case presentationThe report concerns an 18-year-old patient with severe aortic regurgitation (AR) and combined post-capillary and pre-capillary pulmonary hypertension (CpcPH). In infancy he was diagnosed with severe aortic stenosis (AS) and underwent urgent percutaneous balloon valvuloplasty (BAV) (1997). Since then he had remained under care of regional outpatient clinics due to obesity, allergic rhinitis and arterial hypertension. In 2011 he began to experience decrease in physical capacity. In June 2012 after developing atypical pneumonia he was admitted to Cardiology Department of University Children's Hospital in Krakow with signs of congestive heart failure. Transthoracic echocardiography (TTE) demonstrated cardiac enlargement, severe AR, significant tricuspid regurgitation (TR), estimated right ventricle systolic pressure (RVSP) of 88 mm Hg and preserved left ventricular ejection fraction (LVEF). Right heart catheterization (RHC) confirmed severe pulmonary hypertension (PH) in the course of left heart disease. Mean pulmonary artery pressure (mPAP) was 46 mm Hg, pulmonary wedge pressure (PWP) was 27 mm Hg, intracardiac shunt was ruled out. Angiography detected chronic occlusion of the right external iliac artery and common femoral artery with well-developed collateral circulation. Renal angiograms did not show important abnormalities. Magnetic resonance imaging revealed nonischemic myocardial damage of both ventricles in the form of subendocardial fibrosis. High-resolution computed tomography (HRCT) of the lungs disclosed diffused ground-glass opacities with air traps (prominent in the lower pulmonary lobes), bronchiectases and consolidations with tree-in-bud appearance in the right lower lobe. Spirometry results were within normal references. Pulmonology consultant pointed to PH as the most probable cause of HRCT findings. Due to high operative risk associated with severe PH he was disqualified from surgical aortic valve replacement (AVR). He was given sildenafil at the dose 30mg daily as a trial and was scheduled for reevaluation after 6 months. Cardiac catheterization performed in January 2013 showed no relevant hemodynamic improvement and the drug was discontinued. Additional ...