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Idiopathic pulmonary arterial hypertension

Last reviewed: 14 Sep 2025
Last updated: 24 Oct 2024

Summary

Definição

História e exame físico

Principais fatores diagnósticos

  • age 20-50 years
  • female sex
  • dyspnea
  • accentuated pulmonic component (P2) to the second heart sound
  • tricuspid regurgitation murmur
  • family history
Detalhes completos

Outros fatores diagnósticos

  • fatigue
  • peripheral edema
  • cyanosis
  • stimulant use
  • syncope
  • chest pain
  • near syncope
  • early diastolic, high-pitched murmur in the pulmonary area
  • jugular vein distension
Detalhes completos

Fatores de risco

  • family history
  • female sex
  • bone morphogenetic protein receptor type 2 (BMPR2) mutations
  • appetite suppressants
  • drugs and toxins
Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

  • chest radiography
  • ECG
  • transthoracic Doppler echocardiography
  • right heart catheterization
  • antinuclear antibodies (ANA)
  • pulmonary function tests
  • arterial blood gas (ABG)
  • nocturnal oximetry
  • ventilation-perfusion (V/Q) scintigraphy
  • 6-minute walk test
  • B-type natriuretic peptide (BNP) or N-terminal proBNP (NT-proBNP)
  • CBC
  • LFTs
  • thyroid function tests
  • HIV serology
Detalhes completos

Investigações a serem consideradas

  • high-resolution chest CT scan ± CT pulmonary angiography
  • cardiac MRI
  • vasodilator testing with inhaled nitric oxide, inhaled iloprost, or intravenous epoprostenol
Detalhes completos

Algoritmo de tratamento

CONTÍNUA

positive response to acute vasoreactivity testing with no contraindication to calcium-channel blockers

negative response to acute vasoreactivity testing or contraindication to calcium-channel blockers: without cardiopulmonary comorbidity

Colaboradores

Autores

Muhammad Sameed, MD

Director Advance Lung Disease Program

Department of Pulmonary & Critical Care Medicine

Assistant Professor of Medicine

Thomas Jefferson University Hospital - Einstein Health

Philadelphia

PA

Declarações

MS declares that he has no competing interests.

Gustavo A. Heresi, MD, MS
Gustavo A. Heresi

Director, Pulmonary Vascular and CTEPH Program

Department of Pulmonary and Critical Care Medicine

Respiratory Institute

Cleveland Clinic

Cleveland

OH

Declarações

GAH has received speaking and advisory board fees from Bayer Healthcare, the manufacturer of riociguat.

Agradecimentos

Dr Muhammad Sameed and Dr Gustavo A. Heresi would like to gratefully acknowledge Dr Raed A. Dweik, a previous contributor to this topic.

Declarações

RAD declares that he has no competing interests.

Revisores

Michael J. Krowka, MD

Division of Pulmonary and Critical Care Medicine

Mayo Clinic College of Medicine

Rochester

MN

Declarações

MJK declares that he has no competing interests.

Charlie Elliot, MB ChB, MRCP

Consultant Physician in Respiratory and General Internal Medicine

Sheffield Pulmonary Vascular Disease Unit

Royal Hallamshire Hospital

Sheffield

UK

Declarações

CE has received reimbursement for attending several conferences as well as lecture fees from Actelion Pharmaceuticals, GSK, and Bayer.

Maria Giovanna Triveri, MD, PhD

​Assistant Professor of Cardiology,

Medical Director of the Pulmonary Hypertension Program

Icahn School of Medicine at Mount Sinai Hospital

New York

NY

Declarações

MGT has participated on Advisory Boards of Bayer and Actelion and received financial compensation for attendance.

Créditos aos pareceristas

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Referências

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Principais artigos

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.Texto completo  Resumo

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.]Texto completo  Resumo

Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987 Aug;107(2):216-23. Resumo

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.] Resumo

Frost A, Badesch D, Gibbs JSR, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019 Jan;53(1):1801904.Texto completo  Resumo

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.Texto completo  Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.
  • Idiopathic pulmonary arterial hypertension images
  • Diagnósticos diferenciais

    • Pulmonary arterial hypertension (PAH) associated with left-sided heart disease (pulmonary venous hypertension)
    • PAH associated with respiratory diseases and/or hypoxia
    • PAH due to chronic thrombotic and/or embolic disease
    Mais Diagnósticos diferenciais
  • Diretrizes

    • Pulmonary rehabilitation for adults with chronic respiratory disease
    • 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension
    Mais Diretrizes
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