Idiopathic pulmonary arterial hypertension
Resumo
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
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
Fatores de risco
- family history
- female sex
- bone morphogenetic protein receptor type 2 (BMPR2) mutations
- appetite suppressants
- drugs and toxins
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
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
Algoritmo de tratamento
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
Director, Pulmonary Vascular and CTEPH Program
Department of Pulmonary and Critical Care Medicine
Respiratory Institute
Cleveland Clinic
Cleveland
OH
Divulgaciones
GAH has received speaking and advisory board fees from Bayer Healthcare, the manufacturer of riociguat.
Agradecimientos
Dr Muhammad Sameed and Dr Gustavo A. Heresi would like to gratefully acknowledge Dr Raed A. Dweik, a previous contributor to this topic.
Divulgaciones
RAD declares that he has no competing interests.
Revisores por pares
Michael J. Krowka, MD
Division of Pulmonary and Critical Care Medicine
Mayo Clinic College of Medicine
Rochester
MN
Divulgaciones
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
Divulgaciones
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
Divulgaciones
MGT has participated on Advisory Boards of Bayer and Actelion and received financial compensation for attendance.
Agradecimiento de los revisores por pares
Los temas de BMJ Best Practice se actualizan de forma continua de acuerdo con los desarrollos en la evidencia y en las guías. Los revisores por pares listados aquí han revisado el contenido al menos una vez durante la historia del tema.
Divulgaciones
Las afiliaciones y divulgaciones de los revisores por pares se refieren al momento de la revisión.
Referencias
Artículos principales
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 Resumen
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 Resumen
Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987 Aug;107(2):216-23. Resumen
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.] Resumen
Frost A, Badesch D, Gibbs JSR, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019 Jan;53(1):1801904.Texto completo Resumen
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 Resumen
Artículos de referencia
Una lista completa de las fuentes a las que se hace referencia en este tema está disponible para los usuarios con acceso a todo BMJ Best Practice.
Diferenciales
- 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
Más DiferencialesGuías de práctica clínica
- Pulmonary rehabilitation for adults with chronic respiratory disease
- 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension
Más Guías de práctica clínica
Inicie sesión o suscríbase para acceder a todo el BMJ Best Practice
El uso de este contenido está sujeto a nuestra cláusula de exención de responsabilidad