Author:

Keywords:

Adult, Cardiomyopathy, Restrictive, Dilatation, Pathologic, Fatal Outcome, Female, Heart Catheterization, Heart Ventricles, Hemodynamic Processes, Humans, Hypertension, Pulmonary, Vena Cava, Superior, Ventricular Pressure, Science & Technology, Life Sciences & Biomedicine, Cardiac & Cardiovascular Systems, Cardiovascular System & Cardiology, restrictive cardiomyopathy, left-to-right shunt, congenital heart disease, CLINICAL PROFILE, CHILDHOOD, Cardiac Catheterization, Hemodynamics, 1102 Cardiorespiratory Medicine and Haematology, Cardiovascular System & Hematology, 3201 Cardiovascular medicine and haematology

Abstract:

Today, more and more children with complex heart lesions and underlying cardiomyopathies reach adulthood. This results in a wide range of new clinical problems encountered in later life. In particular, idiopathic restrictive cardiomyopathy is initially treated by medication to reduce symptoms, but at end-stage disease, heart or heart-lung transplantation becomes unavoidable. We describe the case of a patient with restrictive cardiomyopathy and a persistent extra-cardiac left-to-right shunt, where we hypothesize that the shunt may protect against haemodynamic deterioration in end-stage restrictive cardiomyopathie.