Maryland CC Project Ramani – Pulmonary Hypertension for the Pulmonologist
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Jun 1, 2020 
Dr. Ramani, an associate professor of medicine in cardiology who teaches pulmonary hypertension, walks through the updated definition and why PVR matters. He contrasts precapillary vs postcapillary phenotypes, covers testing like right-heart cath and vasoreactivity, and highlights therapies including inhaled treprostinil and considerations in HFpEF, ILD, COPD, and CTEPH.
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Precapillary PH Is Not The Same As PAH
- Precapillary pulmonary hypertension includes groups 1, 3, 4, and 5 and is not synonymous with pulmonary arterial hypertension (PAH).
- Mislabeling precapillary PH as group 1 PAH can misdirect workup and treatment decisions, especially therapy eligibility.
Started Routine Genetic Testing For Idiopathic PAH
- Dr. Ramani described starting genetic testing in idiopathic PAH patients, sending panels including BMPR2 to reclassify some 'idiopathic' cases as heritable.
- He noted the program began recently and paused briefly due to clinic shutdowns but reflects growing recognition of genetic causes.
Limit Vasodilator Challenge To Idiopathic Or Heritable PAH
- Use a vasodilator challenge during right heart cath only for idiopathic or heritable PAH to identify calcium-channel blocker responders.
- Define positive response as mean PA pressure drop ≥10 mmHg to <40 mmHg with unchanged/increased cardiac output and follow responders closely.