The prevalence of adult congenital heart disease (ACHD) is increasing, with heart failure being the leading cause of death. For many ACHD patients, heart transplantation is the only treatment option for advanced heart failure, though significant extracardiac involvement may require multi-organ transplantation. Despite the rising number of ACHD transplants, multi-organ transplants in this population remain challenging, and a substantial gap remains between those in need and those who receive a transplant. While short-term outcomes may be worse for ACHD patients, long-term outcomes are comparable and even superior to other cardiomyopathies. Extracardiac organ dysfunction is common in ACHD patients, often precluding heart-alone transplantation. Fontan-associated liver disease, pulmonary vascular and restrictive lung disease, and renal dysfunction frequently necessitate multi-organ transplantation. ACHD patients have a unique immunological and sensitization profile, increasing their risk for infection, rejection, and malignancies, requiring specialized pretransplant desensitization and post-transplant immunosuppression strategies. ACHD transplantation presents unique surgical challenges, including chest reentry, vascular access issues, bleeding risks, extensive anatomical reconstruction, the need for longer vascular segments from donors, and prolonged ischemic times. Decisions regarding heart-alone versus heart-liver, heart-lung, or heart-kidney transplantation demand careful evaluation. These complex surgical plans require extensive multimodal imaging and collaboration with ACHD cardiac imaging specialists and abdominal transplant teams. Comprehensive coordination and psychosocial support are crucial for ACHD patients throughout the transplant process. A dedicated multidisciplinary team and an established and separate pathway for pre-, peri-, and postoperative care in centers with ACHD and multi-organ transplant expertise are essential. There is need for a revised organ allocation system to ensure timely access to transplantation for ACHD patients.

Patients with heart transplants who present with severe aortic stenosis may be deemed high-risk surgical candidates due to immunosuppression and multiple comorbid conditions. Appropriately selected patients may be successfully treated with transcatheter aortic valve replacement. (Level of Difficulty: Advanced.)

The preCARDIA system is a device combining a balloon-mounted catheter and an extracorporeal system designed for intermittent occlusion of the superior vena cava. Studies have established safety and efficacy in acute decompensated heart failure. We present a single-center experience detailing 90 days outcomes and procedural insights. A 24 hours therapy session demonstrated reduced pulmonary wedge pressures and increased urine output, with cardiac output remaining unchanged. There was one readmission and no heart failure-related readmissions at 90 days. The preCARDIA device appears to be a safe mechanical diuretic strategy to manage patients with acute decompensated heart failure beyond current therapeutic strategies.

Cardiac amyloidosis is a less common indication for orthotopic heart transplantation (OHT). Light chain (AL) amyloidosis specifically poses challenges. While previous small studies have established the validity of OHT for AL amyloidosis, we present experience from a large contemporary series at a single center. This was a retrospective single-center study of patients receiving OHT between 2009 and 2023 at our institution. A data warehouse was curated from our electronic medical records and United Network for Organ Sharing databases. Univariable and multivariable analyses of donor and recipient characteristics were performed. Patients who received OHT after 2016 were active in our electronic medical record and hence AL-OHT patients after 2016 had granular details regarding immunosuppression, chemotherapy regimens, amyloidosis, and post-transplant-related outcomes within their first-year post-OHT. Thirty-one patients received an OHT for cardiac AL while 595 received OHT for non-amyloid indication during the study period. One-, three-, five-, and eight-year survival (87%, 83%, 73%, and 67%, respectively) was not statistically different compared to non-AL OHT patients (92%, 85%, 79%, and 68%, respectively). In a subset of 18 patients with access to electronic medical record data, all patients remained on tacrolimus and prednisone through 1-year post-OHT, but nearly half were off mycophenolate. Select AL patients with advanced cardiac involvement can gain survival advantage with OHT similar to other advanced heart failure patients.