Criteria

New York Heart Association (NYHA)/World Health Organization (WHO) classification of functional status of patients with pulmonary hypertension[53]

  • Functional class I: patients with pulmonary hypertension in whom there is no limitation of usual physical activity; ordinary physical activity does not cause increased dyspnea, fatigue, chest pain, or presyncope.

  • Functional class II: patients with pulmonary hypertension who have mild limitation of physical activity. There is no discomfort at rest, but normal physical activity causes increased dyspnea, fatigue, chest pain, or presyncope.

  • Functional class III: patients with pulmonary hypertension who have a marked limitation of physical activity. There is no discomfort at rest, but less than ordinary activity causes increased dyspnea, fatigue, chest pain, or presyncope.

  • Functional class IV: patients with pulmonary hypertension who are unable to perform any physical activity and who may have signs of right ventricular failure at rest. Dyspnea and/or fatigue may be present at rest, and symptoms are increased by almost any physical activity.

Risk assessment in pulmonary arterial hypertension[3]

The European Society of Cardiology(ESC)/European Respiratory Society(ERS) guidelines highlight a combination of clinical, functional, and hemodynamic measures as a tool to stratify risk of mortality at 1 year.[3]

The ESC/ERS guidelines recommend a three-strata model for risk assessment at the time of diagnosis, using categories of low, intermediate, and high risk of mortality at 1 year. This is used to assess survival and guide management.[3][54]

[Figure caption and citation for the preceding image starts]: 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.​European Heart Journal. 2022 Oct 7;43(38):3618-731; used with permission [Citation ends].com.bmj.content.model.Caption@46a48414

During monitoring and follow-up after initial therapy, the ESC/ERS guidelines recommend a four-strata model, which uses categories of low, intermediate-low, intermediate-high, and high risk to guide treatment decisions.[3] 

[Figure caption and citation for the preceding image starts]: Variables used to calculate the simplified four-strata risk-assessment tool​European Heart Journal. 2022 Oct 7;43(38):3618-731; used with permission [Citation ends].com.bmj.content.model.Caption@9258720

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