Pulmonary Arterial Hypertension Clinical Presentation

Updated: Feb 06, 2023
  • Author: Kristin E Schwab, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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The clinical manifestations of pulmonary arterial hypertension (PAH) are frequently masked by the underlying disease entities. Obtaining a careful history may help differentiate PAH from groups 2-5 pulmonary hypertension. Important clues to a specific cause include the following:

  • History of heart murmur
  • Deep venous thrombosis (DVT) or pulmonary embolism (PE)
  • Arthritis or arthralgias
  • Rash
  • Heavy alcohol consumption
  • Hepatitis
  • Heavy snoring
  • Daytime hypersomnolence
  • Morning headaches
  • Morbid obesity
  • Family history of pulmonary hypertension
  • Drug use, in particularly diet drugs and illicit drugs
  • Medications

Patients with PAH may also have nonspecific symptoms secondary to pulmonary hypertension. These may include the following:

  • Dyspnea upon exertion
  • Fatigue
  • Lethargy
  • Syncope with exertion
  • Chest pain
  • Anorexia
  • Right upper quadrant pain

Less common symptoms include the following:

  • Cough
  • Hemoptysis
  • Hoarseness (due to compression of the recurrent laryngeal nerve by the distended pulmonary artery)

Physical Examination

The intensity of the pulmonic component of the second heart sound (P2) may be increased and the P2 may demonstrate fixed or paradoxic splitting. A systolic ejection murmur may be heard over the left sternal border. The murmur may be augmented by inspiration. A right ventricular heave may be palpated.

A prominent A wave may be observed in the jugular venous pulse. A right-sided fourth heart sound (S4) with a left parasternal heave may be auscultated.

Right ventricular failure leads to systemic venous hypertension and cor pulmonale. The signs of right ventricular failure include a high-pitched systolic murmur of tricuspid regurgitation, hepatomegaly, a pulsatile liver, ascites, and peripheral edema. In this scenario, a right ventricular third heart (S3) sound is also heard.

Signs of underlying cardiac, pulmonary, hepatic, or collagen-vascular disease are often present.

Patients with pulmonary arterial hypertension (PAH) often develop cor pulmonale, which further worsens hypoxemia and perpetuates pulmonary hypertension.



The most common—and feared—complication from pulmonary hypertension is right-sided heart failure. Progression to right-sided heart failure is part of the natural history of pulmonary arterial hypertension (PAH), and it is often present to some degree at the time of diagnosis. Registry and institutional data further implicate this as the most common cause of death in PAH patients, with data suggesting that 44-73% of PAH patients who die do so because of right-sided heart failure or sudden cardiac death. [18, 19]

In addition to right-sided heart failure, other causes of death include complications that arise because of dilatation of the pulmonary artery. These include pulmonary artery dissection and rupture, massive hemoptysis, and left main compression syndrome, where the left main coronary artery is compressed by the pulmonary artery trunk. [20] Hemoptysis is often secondary to a bronchial arterial source, as hypoxic vasoconstriction in the pulmonary arteries leads to collateralization and proliferation of the bronchial arteries. Supraventricular and, less commonly ventricular, arrhythmias may also occur, presumably triggered by right-sided heart disease.