Key result
Right isomerism allows 42-year survival without surgery, aided by absent pulmonary venous and present outflow obstruction.
Why the study?
Right isomerism typically has a poor prognosis, but cases of long survival without surgery are rarely reported and not well understood.
Case Report (n=1)
No
Long-term survival in right isomerism without surgery is possible in the absence of specific risk factors like pulmonary venous obstruction and AV valve regurgitation.
Answers are grounded in this paper and cite their sources.
Rare survival to adulthood without surgery in right isomerism with protective anatomy; leaves open whether such features predict outcomes in larger cohorts.
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.
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Matsuura et al. (2007) conducted a case report in Right isomerism (n=1). Natural history without surgical intervention was evaluated on Survival and autopsy findings. A 42-year-old man with right isomerism and severe cardiac malformations survived without surgery, likely aided by the absence of pulmonary venous obstruction and presence of pulmonary outflow obstruction.
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