Highlights & Basics
- Idiopathic pulmonary arterial hypertension (IPAH) is a rare, frequently fatal disease.
- Presenting symptoms and signs are nonspecific and include dyspnea on exertion, fatigue, and a loud pulmonary component of the second heart sound.
- Diagnosis of exclusion. Transthoracic Doppler echocardiography and right heart catheterization can diagnose pulmonary hypertension. Laboratory tests and imaging studies can rule out known causes of pulmonary hypertension.
- Treatment options include calcium-channel blockers, prostanoids, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, the soluble guanylate cyclase stimulator riociguat, the selective prostacyclin IP receptor agonist selexipag, the activin signaling inhibitor sotatercept, and lung transplantation as appropriate.
- Prognosis is not good, as most patients eventually develop right ventricular failure. It is best managed in centers with expertise in pulmonary hypertension.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Clinical classification and prevalence of pulmonary hypertension. CTEPH, chronic thromboembolic pulmonary hypertension; CpCPH, combined post- and pre-capillary pulmonary hypertension; IpcPH, isolated post-capillary pulmonary hypertension; mPAP, mean pulmonary arterial pressure; PH, pulmonary hypertension; PVR, pulmonary vascular resistance.
ECG showing a tall R wave and small S wave (R/S ratio >1) in lead V1, qR complex in V1, right axis deviation, and right atrial enlargement (P wave ≥2.5 mm in lead II)
Transthoracic echocardiogram: apical 4-chamber view showing significant right atrial and right ventricular dilation
Comprehensive risk assessment in pulmonary arterial hypertension (three-strata model). 6MWD, 6-minute walking distance; BNP, brain natriuretic peptide; CI, cardiac index; cMRI, cardiac magnetic resonance imaging; CPET, cardiopulmonary exercise testing; HF, heart failure; NT-proBNP, N-terminal pro-brain natriuretic peptide; PAH, pulmonary arterial hypertension; pred., predicted; RA, right atrium; RAP, right atrial pressure; sPAP, systolic pulmonary arterial pressure; SvO2, mixed venous oxygen saturation; RVESVI, right ventricular end-systolic volume index; RVEF, right ventricular ejection fraction; SVI, stroke volume index; TAPSE, tricuspid annular plane systolic excursion; VE/VCO2, ventilatory equivalents for carbon dioxide; VO2, oxygen uptake; WHO-FC, World Health Organization functional class. a: Occasional syncope during heavy exercise or occasional orthostatic syncope in a stable patient. b: Repeated episodes of syncope even with little or regular physical activity. c: Observe that 6MWD is dependent upon age, height, and burden of comorbidities.
Variables used to calculate the simplified four-strata risk-assessment tool
Citations
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Klinger JR, Elliott CG, Levine DJ, et al. Therapy for pulmonary arterial hypertension in adults: update of the CHEST guideline and expert panel report. Chest. 2019 Mar;155(3):565-86. [Erratum in: Chest. 2021 Jan;159(1):457.][Abstract]
Frost A, Badesch D, Gibbs JSR, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019 Jan;53(1):1801904.[Abstract][Full Text]
Galiè N, Channick RN, Frantz RP, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J. 2019 Jan;53(1):1801889.[Abstract][Full Text]
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