Idiopathic pulmonary arterial hypertension - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

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.

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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

Key Articles

Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019 Jan;53(1):1801913.[Abstract][Full Text]
Humbert M, Kovacs G, Hoeper MM, et al; ESC/ERS Scientific Document Group. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022 Oct 11;43(38):3618-731. [Erratum in: Eur Heart J. 2023 Feb 23:ehad005.][Abstract][Full Text]
Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987 Aug;107(2):216-23.[Abstract]
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]

Referenced Articles

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