How a left-to-right shunt may protect against haemodynamic deterioration in restrictive cardiomyopathy.
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.
Van Mieghem
Nicolas
N
Department of Cardiology, University Hospitals, Leuven, Belgium.
Daenen
Wim
W
Budts
Werner
W
D000328:Adult; D006328:Cardiac Catheterization; D002313:Cardiomyopathy, Restrictive / Q000503:physiopathology*; D004108:Dilatation, Pathologic; D017809:Fatal Outcome; D005260:Female; D006352:Heart Ventricles / Q000473:pathology; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000601:surgery; D014683:Vena Cava, Superior / Q000503:physiopathology; D017725:Ventricular Pressure