Group 4 Pulmonary Hypertension Clinical Presentation

Updated: Mar 16, 2020
  • Author: Paresh Chandra Giri, MD, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Patients usually present with exercise intolerance, fatigue, and progressive dyspnea. Because these symptoms are nonspecific, they are typically attributed to more common diseases such as obstructive lung disease, cardiac abnormalities, obesity, and deconditioning. When pulmonary pressures continue to increase and right ventricular failure develops, chest discomfort, syncope, hemoptysis, light-headedness, and peripheral edema can occur. [29]

The symptoms arise from limitations in cardiac output caused by an increased pulmonary vascular resistance (PVR) and increased minute ventilation requirements secondary to increased dead space ventilation. It is essential that clinicians take a careful history in order to recognize previous events consistent with venous thromboembolism and other risk factors (see Risk Factors). Although most patients with chronic thromboembolic pulmonary hypertension (CTEPH) have a history of at least one previous acute thromboembolic event, approximately 30-40% have no such history, thereby making the diagnosis of CTEPH more challenging. [13]


Physical Examination

Findings of CTEPH on physical examination may include a diminished or fixed splitting of the second heart sound (S2), accentuation of the pulmonic closure sound (P2), a palpable right ventricular heave, jugular venous distention, a right-side third heart sound (S3), tricuspid regurgitation, hepatomegaly, ascites, and peripheral edema. Bruits may be heard on auscultation over the peripheral lung fields; they rise from turbulent flow through partially obstructed pulmonary arteries. Cyanosis may be a sign of right-to-left shunting through a patent foramen ovale.



If not appropriately diagnosed and treated, CTEPH results in right ventricular failure, severe hypoxemic respiratory failure with need for mechanical ventilation, cardiogenic shock, and death. If CTEPH is diagnosed in a timely manner, treatment options can also carry potential complications. Different medical and surgical therapies for CTEPH and complications thereof are discussed in Complications in the Treatment section.