Ruptured sinus of Valsalva (RSOV) is a rare disease with a wide range of clinical manifestations, from asymptomatic murmur to cardiogenic shock and death. We present the case of a young woman known with dextrocardia and pulmonary agenesia; she was diagnosed with RSOV and during follow-up, with endocarditis. Her evolution was good despite the association of severe heart conditions and the refusal of surgical intervention.
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