Bioprosthetic Aortic Valve Replacement in a Donor Heart before Orthotopic Heart Transplantation.

Current criteria for donor hearts limit the number of hearts available for transplantation, despite an increasing number of recipients on waiting lists. We report the case of a patient with ischemic cardiomyopathy and refractory ventricular tachycardia who underwent successful orthotopic heart transplantation and concurrent aortic valve replacement with a donor heart that had displayed moderate aortic valve regurgitation. The patient was a 71-year-old man with a history of advanced heart failure, 5-vessel coronary artery bypass grafting, and paroxysmal ventricular tachycardia. He was not a candidate for repeat revascularization or myocardial ablation, so he was placed on the heart-transplant list as status 1A. On intra-aortic balloon pump support, the patient waited 51 days for a donor match to be identified. Despite the donor heart's having moderate aortic valve regurgitation, the decision was made to use that heart. We performed a back-table aortic valve replacement with a 23-mm St. Jude Epic bioprosthesis, and then performed the orthotopic heart transplantation. The patient did well and was discharged from the hospital on postoperative day 11. This case indicates that expanding donor criteria to include otherwise healthy hearts with certain aortic valve defects is feasible, if surgical experience and expertise permit.

Ventricular arrhythmias and acute left ventricular dysfunction as a primary and life-threatening manifestation of a mitochondrial crisis: A novel management strategy.

Mitochondrial disorders are genetic diseases that result in a deficiency of energy metabolism (ATP production). A "mitochondrial crisis" can occur in the setting of infection, dehydration, or physiologic stress. The hallmark of a mitochondrial crisis is failure of multiple individual organ systems. The mortality of mitochondrial crisis is high and therapy is supportive but involves a specific strategy of hydration with dextrose-containing IV fluids, avoidance of many medications known to worsen mitochondrial function, and limitations of oxygenation as this can promote free radical production. We report a case of a patient with known mitochondrial disease that presented with a mitochondrial crisis with prominent and life-threatening cardiac manifestations including long QT, ventricular arrhythmias, and acute left ventricular systolic dysfunction in addition to rhabdomyolysis, lactic acidosis, and an acute kidney injury. This patient was managed successfully with a specifically tailored supportive strategy, a high-dose metabolic cocktail, permissive hypoxia, and low-protein diet. At 10 weeks post discharge all electrocardiographic abnormalities resolved and ventricular recovery has been observed. Given the increased survival of this population of patients into adulthood it is important that these adjunctive therapeutic strategies require consideration by clinicians treating this group of patients.