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(Circulation. 1999;99:455-456.)
© 1999 American Heart Association, Inc.

Images in Cardiovascular Medicine

Isolated Congenital Absence of the Pulmonary Valve

Fause Attie, MD; Maria Rijlaarsdam, MD; Eduardo Chuquiure, MD

From the Instituto Nacional de Cardiologia Ignacio Chavez, México DF, Mexico.

Correspondence to Fause Attie, MD, Instituto Nacional de Cardiologia Ignacio Chavez, Juan Badiano 1, 14080, Tlalpan, México DF, Mexico.

A66-year-old man was admitted for evaluation of a heart murmur, atypical thoracic pain, and exertional dyspnea. Physical examination disclosed a harsh systolic ejection murmur and a single second heart sound followed by an early diastolic murmur best heard at the left second and third intercostal space. A chest radiograph showed moderate cardiomegaly and massively dilated main pulmonary trunk (Figure 1). The ECG showed atrial fibrillation and right bundle-branch block.

View larger version (131K): [in this window] [in a new window]   Figure 1. Posteroanterior chest x-ray. Heart is enlarged. Observe bulging of main pulmonary artery.

A transthoracic echocardiogram revealed dilated right chambers with aneurysmal dilation of the pulmonary trunk and branches. Pulmonary valve leaflets were not visualized. Doppler examination at the pulmonary annulus demonstrated back-and-forth flow, with a mild systolic gradient of 15 mm Hg (Figure 2). Color Doppler showed moderate pulmonary regurgitation. MRI depicted an enlarged main pulmonary artery and the absence of pulmonary valve leaflets (Figure 3). Cardiac catheterization ruled out the presence of intracardiac shunts. The right ventricular systolic pressure was 42 mm Hg, and pulmonary arterial systolic pressure was 28 mm Hg. Coronary arteriography was normal. Considering the echocardiographic and MRI findings, a right ventriculogram was not performed. Cardiac surgery was not considered because the patient improved with digitalis and diuretic therapy and is in NYHA functional class I.

View larger version (49K): [in this window] [in a new window]   Figure 2. Parasternal short-axis echocardiogram of pulmonary artery. Left, Observe grossly dilated pulmonary trunk (PT) and branches. Right, Continuous Doppler recording at pulmonary annulus demonstrates a small systolic gradient and diastolic regurgitation. AA indicates ascending aorta; . . . [Full Text of this Article]