Adult Congenital Heart DiseaseImportance of the Right Ventricle
Carole A. Warnes, MD*
Divisions of Cardiovascular Diseases and Pediatric Cardiology and Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota
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Figure 1 Pathologic Specimens
(A) Pathologic specimen of the right ventricle with the free wall removed to demonstrate the 3 anatomic regions. (B) Pathologic specimen of the heart cut transversely demonstrating the crescent shape of the right ventricle. Photos courtesy of Dr. W. D. Edwards, consultant in pathology, Mayo Clinic.
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Figure 2 Chest Radiograph of a Patient With Severe Valvular Pulmonary Stenosis
The heart size is normal even though the right ventricular pressure in this patient is 150 mm Hg. Note the characteristic post-stenotic dilation of the pulmonary artery.
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Figure 3 Pathologic Specimen Cut in the 4-Chamber Plane From a Patient With Ebstein Anomaly
The tricuspid valve is displaced markedly inferiorly, and the right ventricular wall is extremely thin. Photo courtesy of Dr. W. D. Edwards, consultant in pathology, Mayo Clinic.
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Figure 4 Chest Radiograph of a 32-Year-Old Man With Severe Ebstein Anomaly
The patient was referred late with severe right ventricular (RV) enlargement, severe RV dysfunction, and severe tricuspid regurgitation. He was cyanotic secondary to a small atrial septal defect and could walk only a few yards.
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Figure 5 Congenitally Corrected Transposition
Schematic diagram of a normal heart (left) and congenitally corrected transposition (right) in which there is both atrioventricular and ventriculoarterial discordance. Reproduced with permission from Warnes (31). Ao = aorta; LA = left atrium; LV = morphologic left ventricle; PT = pulmonary trunk; RA = right atrium; RV = morphologic right ventricle.
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Figure 6 Chest Radiographs of a Patient With Congenitally Corrected Transposition Before and After a Mechanical Systemic AV Valve Replacement
At age 71 years (left), the patient had severe atrioventricular (AV) valve regurgitation, but the function of her systemic ventricle was preserved with an ejection fraction (EF) of 50%. Now at age 85 years (right), the EF of her systemic ventricle is still 50%, and her exercise capacity exceeds 100% of predicted.
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