Introduction Cancer survivors and patients with active malignancies are considered high risk for solid organ transplantation, as there is concern for recurrence and their concurrent co-morbidities. We present a case of combined kidney and repeat heart transplant (HT) for a patient with cardiac allograft vasculopathy (CAV) found to have pheochromocytoma and monoclonal gammopathy of unknown significance (MGUS) on pre-transplant evaluation. Case Report A 47-year-old male with a history of orthotopic HT 20 years prior due to viral myocarditis with post-transplant course complicated by CAV, chronic kidney disease stage 4 (presumed due to tacrolimus), MGUS, and atrial fibrillation presented with acute decompensated heart failure requiring inotropes. Echocardiogram revealed EF of 35%, global hypokinesis; RHC showed RA 19, RV 37/2, PA 38/17/25, PW 27, CI 1.6. LHC showed ISHLT CAV3: 70% mLAD lesion and diffuse 50-80% disease in distal LAD, Cx, and RCA. An IABP was placed and the patient was managed in the cardiac intensive care unit. Imaging revealed a right adrenal mass. He underwent an IR guided adrenal biopsy that demonstrated a pheochromocytoma, with biochemical markers of active tumor. The patient received a laparoscopic adrenalectomy and was listed for dual organ transplant. With worsening hemodynamics, he required peripheral VA-ECMO and received combined heart and renal transplant a month later. His post-operative course was complicated by RV dysfunction requiring central VA-ECMO, transplant pyelonephritis, and delayed renal allograft function requiring transient hemodialysis. Cardiac function recovered in 2 weeks post-transplant. Two and a half months post-transplant, a renal biopsy was performed for worsening acute kidney injury and demonstrated kappa light chain proximal tubulopathy. Given known MGUS, patient was diagnosed with monoclonal gammopathy of renal significance and started on started on Ixazomib-Cytoxan-Dexamethasone. He has shown improvement clinically and is now over one year post-transplant. Summary Combined heart kidney transplantation may be an option for selected patients with active malignancies like localized pheochromocytoma with MGUS. Long term follow up outcomes are unknown in this patient population. Cancer survivors and patients with active malignancies are considered high risk for solid organ transplantation, as there is concern for recurrence and their concurrent co-morbidities. We present a case of combined kidney and repeat heart transplant (HT) for a patient with cardiac allograft vasculopathy (CAV) found to have pheochromocytoma and monoclonal gammopathy of unknown significance (MGUS) on pre-transplant evaluation. A 47-year-old male with a history of orthotopic HT 20 years prior due to viral myocarditis with post-transplant course complicated by CAV, chronic kidney disease stage 4 (presumed due to tacrolimus), MGUS, and atrial fibrillation presented with acute decompensated heart failure requiring inotropes. Echocardiogram revealed EF of 35%, global hypokinesis; RHC showed RA 19, RV 37/2, PA 38/17/25, PW 27, CI 1.6. LHC showed ISHLT CAV3: 70% mLAD lesion and diffuse 50-80% disease in distal LAD, Cx, and RCA. An IABP was placed and the patient was managed in the cardiac intensive care unit. Imaging revealed a right adrenal mass. He underwent an IR guided adrenal biopsy that demonstrated a pheochromocytoma, with biochemical markers of active tumor. The patient received a laparoscopic adrenalectomy and was listed for dual organ transplant. With worsening hemodynamics, he required peripheral VA-ECMO and received combined heart and renal transplant a month later. His post-operative course was complicated by RV dysfunction requiring central VA-ECMO, transplant pyelonephritis, and delayed renal allograft function requiring transient hemodialysis. Cardiac function recovered in 2 weeks post-transplant. Two and a half months post-transplant, a renal biopsy was performed for worsening acute kidney injury and demonstrated kappa light chain proximal tubulopathy. Given known MGUS, patient was diagnosed with monoclonal gammopathy of renal significance and started on started on Ixazomib-Cytoxan-Dexamethasone. He has shown improvement clinically and is now over one year post-transplant. Combined heart kidney transplantation may be an option for selected patients with active malignancies like localized pheochromocytoma with MGUS. Long term follow up outcomes are unknown in this patient population.
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