No differences were found in sRV volume or EF, but patients with elevated ECV had higher levels of serum brain natriuretic peptide (BNP) and presented more cardiovascular events (new arrhythmia, arrhythmia device, HF hospitalization, listing for transplantation, mechanical support or cardiovascular death)after a median follow up of 4.2 ± 1.9 years. This evidence concerns the gene NPPB and hydrops fetalis.