Pulmonary Hypertension Questions¶
Question 1¶
What hemodynamic criteria define pulmonary hypertension?
A) mPAP ≥20 mmHg B) mPAP ≥25 mmHg C) Systolic PA pressure ≥40 mmHg D) PVR ≥2 Wood units E) PA pressure ≥50% of systemic
Answer
**Correct Answer: A** 2022 updated definition: - **mPAP ≥20 mmHg** (previously ≥25) - PLUS PVR >2 Wood units for precapillary PH - PCWP ≤15 mmHg for precapillary **Board Pearl:** PH definition updated: mPAP ≥20 mmHg (not 25)Question 2¶
Which WHO Group of pulmonary hypertension includes CHD-associated PH?
A) Group 1 - PAH B) Group 2 - Left heart disease C) Group 3 - Lung disease D) Group 4 - CTEPH E) Group 5 - Multifactorial
Answer
**Correct Answer: A** **Group 1 PAH** includes: - Idiopathic PAH - Heritable PAH - Drug/toxin-induced - **CHD-associated** (Eisenmenger, systemic-to-pulmonary shunts) - Connective tissue disease - Portal hypertension **Board Pearl:** CHD-PH = WHO Group 1 PAHQuestion 3¶
A patient with a large VSD has PVR of 10 Wood units and no response to vasodilator testing. What is the appropriate management?
A) Surgical VSD closure B) Treat with PAH medications and reassess operability C) Heart-lung transplant evaluation D) Catheter VSD closure E) Observation with medical therapy
Answer
**Correct Answer: E** PVR >6-8 WU without vasoreactivity = **Eisenmenger/inoperable**: - VSD closure contraindicated (would cause RV failure) - Medical therapy with PAH medications - Transplant evaluation if progressive - Treat-to-repair strategy remains controversial **Board Pearl:** PVR >6-8 WU + no vasoreactivity = Eisenmenger, VSD closure contraindicatedQuestion 4¶
What is the mechanism of action of bosentan?
A) PDE-5 inhibitor B) Prostacyclin analog C) Endothelin receptor antagonist D) Soluble guanylate cyclase stimulator E) Calcium channel blocker
Answer
**Correct Answer: C** **Bosentan = endothelin receptor antagonist (ERA)**: - Blocks ET-A and ET-B receptors - Reduces vasoconstriction and remodeling - Requires monthly LFT monitoring - Other ERAs: ambrisentan, macitentan **Board Pearl:** Bosentan = ERA; monitor LFTs monthlyQuestion 5¶
Which pulmonary hypertension medication is administered by continuous IV infusion?
A) Sildenafil B) Bosentan C) Epoprostenol D) Tadalafil E) Riociguat
Answer
**Correct Answer: C** **Epoprostenol (Flolan)** = IV prostacyclin: - Continuous infusion via central line - Half-life 3-5 minutes - Cannot be interrupted (rebound PH crisis) - For severe, treatment-refractory PAH Other prostacyclins: treprostinil (IV, SC, inhaled), iloprost (inhaled) **Board Pearl:** Epoprostenol = continuous IV; cannot be interrupted (rebound crisis)Question 6¶
A patient with idiopathic PAH has positive vasoreactivity during catheterization. What is the appropriate therapy?
A) Epoprostenol B) Calcium channel blocker C) Combination ERA + PDE5i D) Lung transplant evaluation E) Observation
Answer
**Correct Answer: B** Positive vasoreactivity = **calcium channel blocker responder**: - Defined as: mPAP decrease ≥10 mmHg to <40 mmHg with maintained or increased CO - Only 10-15% of IPAH patients - CCB (nifedipine, diltiazem) monotherapy - Must monitor response; add therapy if fails **Board Pearl:** Vasoreactive IPAH = trial of CCB; only 10-15% qualifyQuestion 7¶
What is the prognosis driver in persistent pulmonary hypertension of the newborn (PPHN)?
A) Left ventricular function B) Right ventricular function and response to pulmonary vasodilators C) Aortic valve function D) Tricuspid valve morphology E) Atrial septal communication
Answer
**Correct Answer: B** PPHN prognosis depends on: - **RV function** - Response to inhaled NO and oxygen - Underlying etiology - Associated conditions (CDH, sepsis, MAS) Failure to respond = consider ECMO. **Board Pearl:** PPHN prognosis = RV function + response to iNO; ECMO if refractoryQuestion 8¶
What medication class should be used with caution in Eisenmenger syndrome due to risk of paradoxical hypoxemia?
A) Prostacyclins B) Endothelin receptor antagonists C) PDE-5 inhibitors D) Supplemental oxygen E) Diuretics
Answer
**Correct Answer: E** **Diuretics** can be problematic in Eisenmenger: - Reduce preload → less pulmonary blood flow - May worsen cyanosis - Use carefully if needed for edema - Avoid aggressive diuresis **Board Pearl:** Eisenmenger + aggressive diuresis → may worsen cyanosisQuestion 9¶
At what altitude restriction should be recommended for patients with significant pulmonary hypertension?
A) No altitude restriction B) Avoid altitudes >5,000 feet C) Avoid altitudes >8,000 feet D) Avoid altitudes >10,000 feet E) Altitude doesn't affect PH
Answer
**Correct Answer: C** Altitude considerations in PH: - Hypoxia worsens pulmonary vasoconstriction - **Avoid altitudes >8,000 feet** (some say >5,000) - Supplemental oxygen during air travel - Commercial cabins pressurized to ~6,000-8,000 feet equivalent **Board Pearl:** PH patients: avoid high altitude, may need O2 during flightQuestion 10¶
What is the most common cause of death in Eisenmenger syndrome?
A) Right heart failure B) Massive hemoptysis C) Arrhythmia D) Pulmonary embolism E) Stroke