Arif Maqsood Ali, Azhar Mehmood Kayani, Muhammad Ali and Agha Babar Hussain
Background: Paravalvular leak (PVL) can complicate mitral and aortic valves replacement. Most PVLs are often clinically insignificant. However, large leaks can lead to heart failure and infective endocarditis. Intravascular hemolytic anemia is common in small PVLs. Reoperation for closure of PVL is associated with high mortality. Transcatheter closure is less invasive and can be used in high-risk patients.
Case summary: We present a case of a 38-year-old man with a history of Aortic Valve replacement (AVR) and Mitral valve replacement (MVR) who developed hemolytic anemia and haemoglobinurea. The patient was managed initially conservatively but later underwent redo valve surgery after exclusion of other causes of hemolytic anemia. Postoperatively, haemoglobinurea disappeared dramatically whereas anemia resolved gradually after surgery.
Discussion: Significant intravascular hemolysis is a rare but serious complication of PVL that poses diagnostic problem to cardiac surgeons, but also for cardiologists and internal medicine professionals especially when the prosthetic valve function is considered adequate. PVL is the flow of blood through a track between the native cardiac tissue and the implanted valve due to any compromise in closure between the two. PVL is also more frequently seen after mitral (up to 20%) valve replacement than aortic prosthetic valves. PVLs are more frequently diagnosed by Transesophageal echocardiography (TEE) than Transthoracic echocardiography (TTE) due to its ability to detect minute jets of regurgitated blood.
Conclusion: Either repair or re-replacement of prosthetic valves with PVLs is needed in about 1% to 5% of patients. The case study is presented to highlight PVL as a rare cause of haemoglobinurea and hemolytic anemia.
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