Isolated Congenital Absence of the Pulmonary Valve
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.
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.
Congenital absence of the pulmonary valve is rarely found as an isolated lesion. Usually it is associated with a ventricular septal defect. The critical period for survival is during infancy. Most symptoms are caused by respiratory distress or infection secondary to airway obstruction produced by the dilated pulmonary artery compressing the bronchus or by intractable cardiac failure. The long-term survival of this patient can be explained by the absence of significant obstruction at the level of the pulmonary ring due to the degree of pulmonary regurgitation and by the absence of associated defects.
We would like to thank ABC Hospital for the magnetic resonance imaging performed in this case.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
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