Comprehensive Diagnosis and Treatment on Pulmonary Arterial Hypertension (PAH)

Definition and Prevalence of Pulmonary Arterial Hypertension
Pulmonary Arterial Hypertension (PAH) is a rare condition defined as an artery wedge pressure (PAWP) ≤15 mmHg and PVR 3 WU in the absence of other causes of precapillary pulmonary hypertension such as lung disease, CTEPH, and etc. The prevalence is about 15 for every 1 million population. Some literature reported that PAH has a female predominance of 65-80%. Interestingly, some studies suggest the mean age of diagnosis is around the age of thirties, while some other data suggests the mean age of the diagnosis is around the age of fifties. Regarding race, PAH can affect all races. 1,2,3

Pulmonary Arterial Hypertension Diagnosis
A detailed history taking, physical examination, and most importantly high index of suspicion are essential to diagnose PAH. Early phase of PAH can be asymptomatic, thus obtaining a careful history may help diagnose PAH. History findings may reveal history of alcohol consumption and obesity. Working diagnosis of PAH should be considered in patients who show minimal signs of specific heart or lung disease. Physical examination on patients with PAH may reveal right parasternal lift, accentuated pulmonary component of second heart sound, pansystolic murmur (indicating tricuspid regurgitation), third heart sound, diastolic murmur (indicating pulmonary valve insufficiency), and pedal edema. Diagnosis of PAH is often delayed because its nonspecific symptoms that can be found in many other conditions. 3

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Picture 1. Gross pathology on patient who died of severe pulmonary arterial hypertension secondary to persistent patent ductus arteriosus 3

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Picture 2. Chest radiograph of patient with non-idiopathic pulmonary hypertension shows enlarged pulmonary arteries. This patient had atrial septal defect 3

Supporting examination such as electrocardiography (ECG) may show right ventricular hypertrophy, right axis deviation, right atrial enlargement, RV strain, right bundle branch block, and QT prolongation. Chest radiography is abnormal in 90% of patients with PAH and usually shows right ventricular enlargement, prominent central pulmonary artery, and decreased pulmonary markings. Right heart catheterization is recommended to confirm the diagnosis of PAH and to evaluate the severity of hemodynamic dysfunction while 6-minute walk test (6MWT) is recommended to stratify the severity and estimate the prognosis of PAH.1,3

Pulmonary Arterial Hypertension Treatment Recommendations
PAH management must be multifaceted. It consists of supportive therapy such as oxygen therapy, diuretics, digoxin, and anticoagulation agents. Effective therapy should be given in early stages, before irreversible changes in pulmonary vasculature occur. Guidelines recommend that management of PAH be carried out in specialized centers by providers experienced in treating PAH. Currently, research is still on its way to invent certain medication that may reverse the disease progression.3

  1. Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016; 37: 67-119. doi:10.1093/eurheartj/ehv317
  2. Yaghi S, Novikov A, Trandafirescu T. Clinical update on pulmonary hypertension. J Investig Med. 2020; 68: 821-27. doi:10.1136/jim-2020-001291
  3. Schwab KE. Pulmonary arterial hypertension treatment & management [Internet]. Los Angeles: Emedicine Medscape Journal; Aug 6th 2021. Available at:

Written by: Nada Nafisha Humaera
The writer is currently on her clerkship as a final year of co-assistant in West Nusa Tenggara Central Hospital, Universitas Mataram, West Nusa Tenggara. She has been participating in journalism activities such as an exchequer of BAPIN (2018-2019), a member of team redaction at Indonesian Medical Student Scientific Journals BAPIN (2017-2018), and also as the head of Scientific Department of AVICENNA Universitas Mataram (2017-2018).

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Comprehensive Diagnosis and Treatment on Pulmonary Arterial Hypertension (PAH)

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