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Pulmonary Arterial Hypertension

Valerie L Ward, MD
Robert D Pugatch, MD

June 10, 1997

Presentation

A 40-year-old man presented for routine chest radiographs.

Imaging Findings

PA chest radiograph
Lateral chest radiograph

A posterior-anterior (PA) chest radiograph demonstrates enlargement of the main pulmonary artery (long black arrow) and right pulmonary artery (short black arrow). The peripheral pulmonary arteries are reduced in caliber (white arrow). The lung volumes are normal without evidence of underlying pulmonary parenchymal disease.

A lateral radiograph also demonstrates enlargement of both the right (short black arrow) and left (long black arrows) pulmonary arteries. Cardiomegaly, with predominant right ventricular enlargement (white arrow), is present.

Differential Diagnosis

The above described findings may be seen in primary pulmonary hypertension and recurrent pulmonary thromboemboli. Many other disorders, however, can include these imaging findings and also have additional, more specific features. Such disorders include mitral valve stenosis (includes left atrial enlargement), emphysema (includes hyperexpansion and bullae), and Takayasu's arteritis (includes irregular dilatation of the aorta and its main branches).

Diagnosis

Primary pulmonary hypertension

Discussion

This patient did not have a known cause for the chest radiograph findings. Therefore, primary pulmonary hypertension (PPH) was diagnosed. PPH is an uncommon disorder that usually occurs after puberty. It can be familial and is more common in females. Symptoms include dyspnea, chest pain, hemoptysis, and murmurs. On the PA chest radiograph, a transverse diameter of the right descending pulmonary artery of 17 mm or greater is a reliable indicator of PPH. Cardiac catheterization in these patients shows a pulmonary artery systolic pressure greater than or equal to 30 mmHg.

Histologic changes in PPH include initial vasoconstriction of pulmonary vascular smooth muscle. Eventually, intimal fibrosis and smooth muscle hypertrophy occur. These latter findings contribute to the irreversibility of the hypertension. Irreversible morphologic changes in the pulmonary vascular bed lead to progressive clinical decompensation including cor pulmonale and cardiac failure. Bilateral lung transplantation was the definitive therapy in this patient.

References

1. Armstrong P, Wilson AG, Dee P, Hansell DM. Imaging of diseases of the chest. 2nd ed. St Louis: Mosby, 1995

2. Fraser RS, Pare JAP, Fraser RG, Pare PD. Synopsis of disease of the chest. 2nd ed. Philadelphia: Saunders, 1994.

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