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Pulmonary hypertension associated with heart failure with preserved ejection fraction — SCE Respiratory MCQ

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HardCardiovascular DiseasePulmonary hypertension associated with heart failure with preserved ejection fractionSCE Respiratory

A 74-year-old woman is referred for suspected pulmonary arterial hypertension because of progressive exertional dyspnoea. She has obesity, hypertension, type 2 diabetes and permanent atrial fibrillation. She reports orthopnoea and intermittent ankle swelling, which improved after commencing furosemide. Echocardiography shows a left ventricular ejection fraction of 60%, concentric left ventricular hypertrophy, a left atrial volume index of 52 mL/m², E/e′ of 14, right ventricular dilatation and an estimated pulmonary arterial systolic pressure of 58 mmHg. She has mild smoking-related airflow obstruction: post-bronchodilator FEV1 is 82% predicted and TLCO is 68% predicted. Resting oxygen saturation is 96% on air. CT shows minor emphysema without interstitial lung disease, and ventilation–perfusion scintigraphy is normal. Right heart catheterisation, performed while clinically euvolaemic on furosemide, shows a mean pulmonary arterial pressure of 31 mmHg, pulmonary arterial wedge pressure of 14 mmHg, cardiac output of 4.5 L/min and pulmonary vascular resistance of 3.8 Wood units. After 500 mL intravenous saline over 8 minutes, the wedge pressure rises to 23 mmHg; mean pulmonary arterial pressure is 37 mmHg and cardiac output is 5.0 L/min. Her eGFR is 58 mL/min/1.73 m² and serum potassium is 4.2 mmol/L. Which management plan is most appropriate?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: A — Titrate loop diuresis, add an MRA and an SGLT2 inhibitor with monitoring, and withhold PAH-targeted therapy

Explanation lettering: D = shown as B · E = shown as C · B = shown as D · C = shown as E

The apparently pre-capillary resting profile is misleading because catheterisation was performed after diuresis. She has a strong HFpEF phenotype: advanced age, obesity, hypertension, diabetes, atrial fibrillation, orthopnoea, congestion, left atrial enlargement and concentric hypertrophy. With a borderline resting PAWP of 14 mmHg, saline challenge is appropriate; the rise to 23 mmHg exceeds the ≥18 mmHg threshold used to uncover abnormal left ventricular filling pressure. The pulmonary hypertension should therefore be managed as occult HFpEF-associated pulmonary hypertension rather than idiopathic PAH. NICE recommends considering an MRA and an SGLT2 inhibitor in HFpEF, with diuretics titrated to congestion. B is attractive because resting PVR is elevated, but the abnormal fluid response and cardiac phenotype argue against PAH; PAH drugs are not recommended routinely in PH due to left-heart disease. C is inappropriate because the lung disease is mild, oxygenation is preserved and PVR is below the >5 Wood unit definition of severe lung-disease-associated pulmonary hypertension. D reflects the elevated PVR and possible pre-capillary component, but evidence is insufficient to support routine sildenafil in HFpEF-associated combined physiology, and established HFpEF treatment should not be deferred. E is unnecessary: provocative haemodynamics have already exposed elevated filling pressure, while deliberately withdrawing diuresis risks recurrent congestion without resolving the management question.

Reference: 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension (2023) — https://publications.ersnet.org/content/erj/61/1/2200879 Chronic heart failure in adults: diagnosis and management (NG106) — Recommendations (2025) — https://www.nice.org.uk/guidance/ng106/chapter/recommendations