RIGHT HEART FAILURE AND PULMONARY HYPERTENSION

RV sarcomere disease and treatment

Pulmonary hypertension (PH) – both primary and secondary – impose major abnormal loads on the right heart. The RV is remarkably able to adapt to this, but if and when it cannot, RV failure results in high morbidity and mortality.  Some forms of PH such as that associated with the rheumatological disease – systemic sclerosis (SSc) – have a particularly poor outcome linked to RV failure. We are studying mechanisms for RV disease in such conditions, focusing on the sarcomere where we first revealed major defects in SSc-PH. Mechanisms are being sought, and studies to test if sarcomere enhancing molecules can offset RV weakness and serve to imprve outcome and perhaps survival.  Studies in secondary PH associated with HFpEF and HFrEF are also being pursued with similar goals.