A 77-year-old American national was admitted with history of palpitation, chest pain and shortness of breath. He was very fragile and weighed only 48 kg. He was diagnosed to have severe rheumatic mitral regurgitation, severe tricuspid regurgitation, atrial fibrillation, and left ventricular failure. He had an attempted mitral clip procedure in another hospital, which failed. He was a known case of stage 4 chronic renal failure, sickle cell anemia, thrombocytopenia, diabetes mellitus and visual defects as tubular vision. The patient had multiple history of sickle cell crisis. Two years back he was admitted with septicemia, comatosed, ventilated and had tracheostomy in a hospital in the USA. This case was discussed in our heart failure clinic and accepted for the high risk procedure.

Surgical technique

All precautions were taken to deal with problems of sickle cell disease on cardiopulmonary bypass. The patient had exchanged transfusion before instituting cardiopulmonary bypass. He was hydrated well and 2.5 L of blood was removed at the beginning of CPB to a separate cardiotomy reservoir and discarded. CPB was started with blood prime to replace the lost volume and kept on normo-thermic. Operating room temperature was kept at 25 degrees C. Ultra-filtration was also used. The mitral valve was replaced with 27 mm bioprosthetic valve and tricuspid valve was repaired with 30 mm MC3 annuloplasty ring. Transatrial closure of the left atrial appendage was done using two-layer suture. The patient came off bypass with stable hemodynamics on minimal inotropic support. In the postop period, all precautions were taken to avoid sickle cell crisis. He had a smooth outcome and was discharged on 15th post-op day. He is being followed up in our clinic and is doing well.

Discussion

Patient with pre-op high morbidity underwent a very high risk procedure. This case was initially managed in another cardiac institute and decided not to subject him for open heart surgery due to his comorbidities. Hence mitral clip procedure was attempted, but failed. Following this, he had multiple admissions with pulmonary edema. The family support and the patient’s motivation was great and encouraged us to take him up for surgery. This helped to achieve a good result.

Mitral valve replacement and tricuspid valve repair in a patient with sickle cell disease