Danon disease is a rare x-linked dominant multisystemic disorder with a clinical triad of severe cardiomyopathy, skeletal myopathy, and intellectual disability. It is caused by defects in the lysosome-associated membrane protein-2 () gene. Numerous different mutations in the protein have been described.
View Article and Find Full Text PDFBackground: The most common cause of pulmonary hypertension (PH) in developed countries is left heart disease (LHD, group 2 PH). The development of PH in heart failure (HF) patients is indicative of worse outcomes.
Objective: The aim of this study was to evaluate the long term outcomes of HF patients with PH in a national long-term registry.