(Circulation. 1999;99:455-456.)
© 1999 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Isolated Congenital Absence of the Pulmonary Valve
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.
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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.
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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.
Acknowledgments
We would like to thank ABC Hospital for the magnetic resonance imaging performed in this case.
Footnotes
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.
Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke's Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
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