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Download Pulmonary Arterial Hypertension (PAH) Checklist
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Pulmonary Arterial Hypertension (PAH) Checklist: A Practical Guide for Clinicians Pulmonary arterial hypertension (PAH) is a rare, but serious, condition that leads to right heart failure if left untreated. While some biomarkers, drugs, and other disease conditions have been linked to PAH, the clinical signs and symptoms are non-specific and the disease remains difficult to recognize and diagnose. Based on newlyreleased guidelines, this checklist is designed as a practical guide to aid clinicians in the recognition of PAH risk factors and symptoms to allow early and accurate identification of patients for referral to pulmonary hypertension centers for specialized treatment of this complex disease. 1 of 3 © 2015 PRIME Education, Inc. All Rights Reserved. www.primeinc.org Clinical Symptoms1 Physical Signs1 Dyspnea Fatigue Weakness Chest pain/angina Syncope Dry cough Abdominal distention* Ankle swelling* Left parasternal lift Accentuated P2 Right ventricular third heart sound Pansystolic murmur of tricuspid regurgitation Diastolic murmur of pulmonary regurgitation Elevated jugular venous pressure* Hepatomegaly* Ascites* Peripheral edema* Cool extremities* Telangiectasia§ Sclerodactyly§ Inspiratory crackles¶ Spider nevi** Testicular atrophy** Palmar erythema** *Present in advanced disease. **Present in patients with concomitant liver disease. § Present in patients with scleroderma. ¶Present in patients with interstitial lung disease. Risk Factors and Associated Conditions1 Gender (F > M) Specific drugs/toxins, including: Family history (≥ 2 or more family members) Fenfluramine/dexfenfluramine Aminorex Toxic rapeseed oil Amphetamines/methamphetamines Dasatinib L-tryptophan Mutations associated with PAH, including: BMPR2 Activin receptor-like kinase 1 Endoglin BMPR1B SMAD9 CAV1 KCNK3 Associated conditions, including: Systemic sclerosis Sarcoidosis Systemic lupus erythematosus Primary Sjogren’s Syndrome HIV infection Portal hypertension Congenital heart disease Schistosomiasis Obesity and obstructive sleep apnea F = female; M = male; PAH = pulmonary arterial hypertension; BMPR2 = bone morphogenetic protein receptor 2; BMPR1B = bone morphogenetic protein receptor 1B; CAV1 = caveolin 1; KCNK3 = potassium channel subfamily K member 3; HIV = human immunodeficiency virus. 2 of 3 © 2015 PRIME Education, Inc. All Rights Reserved. www.primeinc.org Screening for PAH1 1 PH/PAH risk factors, signs, and/or symptoms present 5 Refer patient to PH specialty center for treatment 2 Echocardiogram suggests PH 6 Confirm PAH diagnosis with RHC Right atrial enlargement Pericardial effusion mPAP ≥ 25 mmHg PAWP ≤ 15 mmHg PVR > 3 Wood units Rule out left heart disease and lung disease 3 Chest x-ray ECG PFT + DLCO HRCT Arterial blood gases 4 Rule out chronic thromboembolic PH 7 Conduct specific diagnostic tests to determine PAH subtype CTD CHD Drugs/toxins Portopulmonary HIV Schistosomiasis Heritable (eg, BMPR2 mutation, etc.) V/Q Scan PAH = pulmonary arterial hypertension; PH = pulmonary hypertension; ECG = electrocardiogram; PFT = pulmonary function tests; DLCO = carbon monoxide diffusing capacity; HRCT = high resolution computed tomography; V/Q = ventilation/perfusion; RHC = right heart catheterization; mPAP = mean pulmonary arterial pressure; PAWP = pulmonary artery wedge pressure; PVR = pulmonary vascular resistance; CTD = connective tissue disease; CHD = congenital heart disease; HIV = human immunodeficiency virus; BMPR2 = bone morphogenetic protein receptor 2. Reference 1. Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). European Heart Journal. 2015; Epub ahead of print. There is no fee for this tool as it is sponsored by PRIME® through an educational grant from Gilead Sciences, Inc.
