Case Report

A Case of Right Isomerism Showing Long Survival Without Surgery

Authors: Keiko Matsuura, MD, PhD, Shinichiro Akizuki, MD, PhD, Natsuki Nakamura, MD, PhD, Hatsue Ishibashi-Ueda, MD, PhD, Masatsugu Moriyama, MD, PhD

Abstract

We report an autopsy case of right isomerism in a 42-year-old Japanese man. Although he had not undergone any surgical intervention, he was healthy and did not exhibit any symptoms of heart failure. The cardiac findings indicated right isomerism with complex and severe cardiac malformations, including a morphologically common atrium, common atrioventricular valve, double inlet right ventricle, total anomalous pulmonary venous drainage, pulmonary outflow obstruction, pulmonary atresia, and major aortopulmonary collaterals from the descending aorta. He had no spleen, and exhibited some gastrointestinal malformations. The absence of risk factors for mortality from right isomerism, such as pulmonary venous obstruction, AV valve regurgitation, sepsis or severe arrhythmia, might have contributed to the longevity of this patient. The presence of pulmonary outflow obstruction that is reported to confer a better prognosis might also have contributed to his longevity.


Key Points


* Right isomerism is defined as a cardiac anomaly with bilateral, morphologically identical right atrial appendages, often associated with complex and severe cardiac anomalies.


* The long-term outcome of patients with right isomerism is poor. Therefore, adult cases of right isomerism with complex cardiac anomalies untreated by surgery are extremely rare.


* It is considered that the absence of pulmonary venous obstruction and significant atrioventricular valve regurgitation and the presence of pulmonary outflow obstruction might have contributed to the present patient's longevity.

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