AL amyloidosis is a rare condition characterized by the overproduction of an unstable free light chain, protein misfolding and aggregation, and extracellular deposition that can progress to multiorgan involvement and failure. To our knowledge, this is the first worldwide report to describe triple organ transplantation for AL amyloidosis and triple organ transplantation using thoracoabdominal normothermic regional perfusion recovery with a donation from a circulatory death (DCD) donor. The recipient was a 40-year-old man with multiorgan AL amyloidosis with a terminal prognosis without multiorgan transplantation. An appropriate DCD donor was selected for sequential heart, liver, and kidney transplants via our center's thoracoabdominal normothermic regional perfusion pathway. The liver was additionally placed on an ex vivo normothermic machine perfusion, and the kidney was maintained on hypothermic machine perfusion while awaiting implantation. The heart transplant was completed first (cold ischemic time [CIT]: 131 minutes), followed by the liver transplant (CIT: 87 minutes, normothermic machine perfusion: 301 minutes). Kidney transplantation was performed the following day (CIT: 1833 minutes). He is 8 months posttransplant without evidence of heart, liver, or kidney graft dysfunction or rejection. This case highlights the feasibility of normothermic recovery and storage modalities for DCD donors, which can expand transplant opportunities for allografts previously not considered for multiorgan transplantations.

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.

Purpose Chronotropic incompetence (CI) in patients with heart failure is common and associated with impaired exercise intolerance and adverse outcomes. This study sought to determine the effects of closed loop stimulation (CLS) rate-adaptive pacing on functional capacity in patients with heart failure with reduced ejection fraction (HFrEF) and CI implanted with cardiac resynchronization therapy (CRT) devices. Methods A randomized, blinded, cross-over designed trial enrolled patients with HFrEF and CI implanted with a Biotronik CRT-D to complete a quality of life questionnaire, 6-min walk distance (6MWD), and cardiopulmonary exercise testing after two programmed periods: 1-week period of CLS and 1-week period of standard accelerometer (DDDR). Results Nine patients (6 males, mean age 71.4 years, 7 with New York Heart Association Class III, mean ejection fraction 39 ± 8%) were enrolled. Quality of life trended higher in CLS as compared to DDDR (550.8 ± 123.9 vs 489.3 ± 164.9, p = 0.06). There were no differences between CLS and DDDR in 6MWD (293.1 ± 90.2 m vs 315.1 ± 95.5 m, p = 0.52), peak heart rate (HR) 110.7 ± 14.7 bpm vs 109.7 bpm ± 14.1, p = 0.67), or peak VO2 (12.3 ± 4.9 ml/kg/min vs 12.9 ± 5.9, p = 0.47). As tests were submaximal as indicated by low respiratory exchange ratios (0.98 ± 0.11 vs 1.0 ± 0.8, p = 0.35), VE/VCO₂ slope also showed no difference between CLS and DDDR (35.8 ± 5.6 vs 35.4 ± 5.7, p = 0.65). Five patients (56%) preferred CLS programming (p = 1.0). Conclusions In patients with HFrEF and CI implanted with a CRT-D, peak HR, peak VO2, and 6MWD were equivalent, while there was a trend toward improved quality of life in CLS as compared to DDDR. Clinical trial registration URL: https://www.clinicaltrials.gov. Unique identifier: NCT02693262.

Heart transplantation (HTx) remains the definitive therapy for select patients with Stage D heart failure. Donation after circulatory death (DCD) has emerged as a promising strategy to expand the donor pool, with studies showing comparable survival to donation after brain death (DBD). However, little is known about how DCD impacts post-transplant quality of life. Days alive and out of the hospital (DAOH) at 1 year is a validated, patient-centered metric reflecting both post-transplant morbidity and survival. We conducted a single-center, retrospective cohort study of 226 adult patients who underwent HTx between January 2021 and June 2023 (117 DBD and 109 DCD recipients). The primary outcome was DAOH at 1 year. Secondary outcomes included post-transplant graft function, inotrope and intensive care unit (ICU) duration, readmissions, and mortality. Baseline characteristics and outcomes were compared using appropriate statistical methods, and quantile regression was used to adjust for clinical confounders. Baseline characteristics were largely similar, although DBD recipients had higher pre-operative acuity. Median DAOH at 1 year did not differ significantly between DBD and DCD recipients (344 vs 343 days; p = 0.896). Adjusted analyses confirmed no clinically meaningful difference. Secondary outcomes, including 1-year mortality, ICU stay, and readmission rates, were also comparable. In this cohort, DCD recipients achieved similar DAOH at 1 year compared to DBD recipients, supporting the use of DCD as a viable strategy in HTx. This is one of the first studies to compare post-transplant quality of life in DBD and DCD HTx recipients.

Transthyretin (TTR) amyloidosis (ATTR) is a progressive, systemic disease caused by misfolded TTR (variant [hereditary] or wild type) that aggregates into amyloid fibrils, which then deposit into o...