Early Experience With Reverse Double Switch Operation for the Borderline Left Heart.

This study reviews our early experience with the "reverse" double switch operation (R-DSO) for borderline left hearts. A retrospective review of children with borderline left hearts who underwent R-DSO between 2017 and 2021 was conducted. Patient characteristics and early hemodynamic and clinical outcomes were collected. R-DSO was performed in 8 patients with no operative or postoperative deaths; 5 underwent decompressing bidirectional Glenn. Left ventricular (LV) poor-compliance was the dominant pathophysiology. Four patients had undergone staged LV recruitment but were not candidates for anatomical biventricular circulation due to LV hypoplasia and/or diastolic dysfunction. 7/8 patients had risk factors for Fontan circulation including pulmonary vein stenosis, pulmonary hypertension, and pulmonary artery stenosis. Median age at R-DSO was 3.7 years (19 months-12 years). All patients were in sinus rhythm at discharge. At median follow-up of 15 months (57 days-4.1 years) no mortalities, reoperations or heart transplants had occurred. All patients had normal morphologic LV systolic function. In one patient, pre-existing pulmonary hypertension (HTN) resolved after R-DSO. Reinterventions included transcatheter mitral valve replacement for residual mitral stenosis and neo-pulmonary balloon valvuloplasty. In 4 patients follow-up catheterization done at a median of 519 days (320 days-4 years) demonstrated median cardiac index of 3.2 L/min/m2 (2.2-4); median sub-pulmonary left ventricular end diastolic pressure was 9 mm Hg (7-15); median inferior vena cava/baffle pressure was 8 mm Hg (7-13). R-DSO is an alternative to anatomical biventricular repair or single ventricle palliation in patients with borderline left hearts and can result in low inferior vena cava pressures and favorable early results. This approach can also relieve pulmonary HTN and allow future transplant candidacy.

Epstein-Barr Virus Predicts Malignancy After Pediatric Heart Transplant, Induction Therapy and Tacrolimus Don't.

BACKGROUND: Patients after heart transplantation are at increased risk for malignancy secondary to immunosuppression and oncogenic viral infections. Most common among children is posttransplant lymphoproliferative disorder (PTLD), occurring in 5% to 10% of patients. We used a national database to examine the incidence and risk factors for posttransplant malignancy. METHODS: The United Network for Organ Sharing database was queried for pediatric (<18 years) heart transplant recipients from October 1987 through November 2019. Freedom from malignancy after transplant was assessed with Kaplan-Meier analysis. Cox regression was performed to generate hazard ratios (HRs) and 95% CIs for risk of malignancy development. RESULTS: Of 8581 pediatric heart transplant recipients, malignancy developed in 8.1% over median follow-up time of 6.3 years, with PTLD compromising 86.4% of the diagnosed cancers. The incidence of PTLD development was 1.3% at 1 year and 4.5% at 5 years. Older age at the time of transplant was protective against the development of malignancy (HR, 0.98; 95% CI, 0.96-0.99; P < .001), whereas a history of previous malignancy (HR, 1.9; 95% CI, 1.2-3.0; P = .007) and Ebstein-Barr virus (EBV) recipient-donor mismatch (HR, 1.7; 95% CI, 1.3-2.2; P < .001) increased the risk. Induction therapy, used in 78.9% of the cohort, did not increase malignancy risk (P = .355) nor did use of maintenance tacrolimus (P = .912). CONCLUSIONS: PTLD occurred after 7% of pediatric heart transplants, with risk increased by younger age and EBV mismatch, highlighting the importance of PTLD monitoring in EBV-seronegative recipients. Induction therapy, used in most of the pediatric heart transplants, does not seem to increase posttransplant malignancy nor does tacrolimus, the most commonly used calcineurin inhibitor.

Palliative care in lung transplantation.

The role of palliative care in lung transplantation has grown exponentially in the past two decades. From assisting with evaluating and optimizing candidates for transplant, to playing a crucial role in patients requiring extracorporeal cardiopulmonary life support (ECLS) as a bridge to lung transplant, perioperatively, or even during their first year post-transplant, palliative care has been shown to be an effective if underutilized tool in the armamentarium used to care for lung transplant patients. For patients being considered for primary transplantation and for lung transplant recipients, palliative care can decrease symptom burden and help to lessen the psychological distress experienced by patients and family members. For older patients listed for transplant, palliative care can help address cognitive impairment, depression, and frailty. Patients listed for lung re-transplant also benefit from palliative care involvement to address frequent symptom exacerbations, hospitalizations, and higher morbidity and mortality. Even for organ donors and their families, palliative care can facilitate communication and provide support to the family. While palliative care use in lung transplantation may be gradually increasing, further work is necessary to optimally integrate palliative care into lung transplantation. Barriers to lung transplant patients receiving palliative care are multifactorial and include physician, patient, and institutional factors. The potential role of palliative care in every aspect of lung transplantation has made a knowledge of palliative care principles crucial for the lung transplant practitioner. In this review, we will clearly delineate the potential benefit of palliative care for the perioperative lung transplant patient and make an argument for its increased use in this patient population.

A Small-Town Heart.

Melanie presented at twenty weeks of gestation to an obstetrics clinic in a critical access hospital in rural Vermont. She was excited to undergo routine fetal ultrasonography, but her obstetrician gave her grave news: the ultrasound revealed hypoplastic left heart syndrome, a devastating congenital heart defect. Initially, Melanie agreed in general to pursue surgical care for her fetus-a three-stage process that has somewhat uncertain results and could only be done in tertiary care facilities far from her home in Vermont. A week later, while the maternal fetal medicine and pediatric cardiology units made arrangements with colleagues in Boston, Melanie began having second thoughts. An ethics meeting was called to discuss conflicting clinician reactions to Melanie's dilemma. Most of the clinicians were stunned that the patient would change her mind. What advice should the ethics consultant offer the team about caring for Melanie?

Current expectations for surgical repair of isolated ventricular septal defects.

BACKGROUND: Ventricular septal defect (VSD) is the most commonly recognized congenital heart defect. With the development of device closure for intracardiac defects, we sought to evaluate current expectations for surgical closure of isolated VSD. METHODS: Between January 1, 2000, and December 31, 2006, 215 patients underwent isolated VSD repair at a median age of 10 months (range, 20 days to 18 years) and a median weight of 7 kg (range, 2 to 66 kg). The following VSD types were found: 172 perimembranous (80%), 28 supracristal (13%), 6 inlet (3%), and 9 muscular (4%). One hundred eight patients (50%) had evidence of congestive heart failure or failure to thrive preoperatively. Thirty-one patients (14%) had aortic valve cusp prolapse, and 63 (29%) had genetic abnormalities. RESULTS: Incidence of significant postoperative complications was extremely low. No patient underwent reoperation for a residual VSD. None had complete heart block. One operative mortality (0.5%) and 2 late deaths (0.9%) occurred. Median postoperative hospital length of stay was 5 days (range, 2 to 187 days). In the immediate postoperative period, 6 patients (2.8%) required reoperation. No patients were discharged on antiarrhythmic agents, had complete heart block, or required permanent pacing. At mean follow-up of 2.1 +/- 2.0 years, 99.5% (211 of 212) of patients were asymptomatic from a cardiac standpoint. None exhibited greater than mild new-onset tricuspid valve regurgitation. No aortic valve injuries occurred. CONCLUSIONS: Surgical closure of isolated VSD is a safe, effective therapy. Risk of death, complete heart block, and reoperation is minimal. As new technologies for VSD closure evolve, results such as these should be considered when evaluating patients, choosing therapeutic options, and counseling families.