Pulmonary Hypertension Flashcards¶
Definitions¶
Q: What is the hemodynamic definition of pulmonary hypertension (2022)? A: Mean PAP >20 mmHg at rest by catheterization Tags: ph, definition, high-yield
Q: What defines pre-capillary pulmonary hypertension? A: mPAP >20 mmHg + PAWP ≤15 mmHg + PVR >2 WU Tags: ph, definition, hemodynamics
Q: What defines post-capillary pulmonary hypertension? A: mPAP >20 mmHg + PAWP >15 mmHg Tags: ph, definition, hemodynamics
Q: What is the normal PVR in children? A: <3 Wood units (indexed to BSA) Tags: ph, normal-values
Classification¶
Q: What is Group 1 pulmonary hypertension? A: Pulmonary arterial hypertension (PAH) - idiopathic, heritable, drug-induced, CHD-associated Tags: ph, classification, high-yield
Q: What is Group 2 pulmonary hypertension? A: PH due to left heart disease Tags: ph, classification
Q: What is Group 3 pulmonary hypertension? A: PH due to lung disease/hypoxia (BPD, sleep apnea) Tags: ph, classification
Q: What is Group 4 pulmonary hypertension? A: Chronic thromboembolic PH (CTEPH) Tags: ph, classification
Q: What is Group 5 pulmonary hypertension? A: PH with unclear/multifactorial mechanisms Tags: ph, classification
CHD-Associated PAH¶
Q: What CHD lesions have highest risk of developing PAH? A: Large L→R shunts (VSD, AVSD, PDA), especially if unrepaired Tags: ph, chd, high-yield
Q: What is Eisenmenger syndrome? A: Reversal of shunt to R→L due to severe PAH from chronic L→R shunt Tags: ph, eisenmenger, high-yield
Q: Can Eisenmenger syndrome be surgically repaired? A: No - defect closure is contraindicated; causes acute RV failure Tags: ph, eisenmenger, management
Q: What is the PAH operability cutoff for CHD? A: PVR <6-8 WU (or PVRI <6 WU·m²); vasoreactivity testing helps Tags: ph, chd, surgery
Diagnostic Workup¶
Q: What is the gold standard for diagnosing PAH? A: Right heart catheterization with hemodynamic assessment Tags: ph, diagnosis, high-yield
Q: What is vasoreactivity testing? A: Acute response to pulmonary vasodilator (iNO, adenosine) - positive if mPAP drops ≥10 to <40 Tags: ph, catheterization
Q: What echo findings suggest pulmonary hypertension? A: Elevated TRJV (>2.8 m/s), flattened septum, RV dilation/dysfunction Tags: ph, echo
Q: How is PA pressure estimated by echo? A: 4V² + RAP (V = tricuspid regurgitation velocity) Tags: ph, echo, calculation
Treatment¶
Q: What is first-line treatment for acute PH crisis? A: Inhaled nitric oxide (iNO) - selective pulmonary vasodilator Tags: ph, treatment, crisis, high-yield
Q: What are the three pathways targeted by PAH medications? A: Endothelin (bosentan), Nitric oxide/cGMP (sildenafil, tadalafil), Prostacyclin (epoprostenol) Tags: ph, treatment, pathways, high-yield
Q: What is the mechanism of sildenafil? A: PDE-5 inhibitor - increases cGMP → pulmonary vasodilation Tags: ph, sildenafil, mechanism
Q: What is the mechanism of bosentan? A: Endothelin receptor antagonist - blocks vasoconstriction and proliferation Tags: ph, bosentan, mechanism
Q: What monitoring is required with bosentan? A: Monthly LFTs (hepatotoxicity risk) Tags: ph, bosentan, monitoring
Q: What is the most potent PAH medication (reserved for severe disease)? A: IV prostacyclin (epoprostenol) - requires continuous infusion Tags: ph, treatment
Q: What defines a positive vasoreactivity test response? A: ≥10 mmHg decrease in mPAP to absolute value <40 mmHg with maintained cardiac output Tags: ph, vasoreactivity, high-yield
Q: What medication can be used if positive vasoreactivity test? A: Calcium channel blockers (nifedipine, diltiazem) - rare responders only Tags: ph, treatment, ccb
Prognosis & Monitoring¶
Q: What are poor prognostic markers in pediatric PAH? A: Syncope, RV failure, elevated BNP, low 6MWD, need for combination therapy Tags: ph, prognosis
Q: What functional capacity test is used in PAH? A: 6-minute walk distance (6MWD) Tags: ph, monitoring
Q: What is the role of anticoagulation in PAH? A: Consider in idiopathic PAH; individualized decision Tags: ph, anticoagulation
Q: Is pregnancy safe in severe PAH? A: NO - maternal mortality 30-50%; pregnancy contraindicated Tags: ph, pregnancy, high-yield