Background: Patients with single-ventricle physiology represent a high-risk group for heart transplantation. Due to complex anatomical and physiological challenges, including multiple prior sternotomies, pulmonary artery abnormalities, and systemic consequences of altered circulation, they represent both a surgical and a clinical challenge. We aimed to analyze perioperative challenges, as well as early and long-term complications, in this specific group of patients. Methods: We performed a retrospective data analysis of a high-volume heart transplant center, focusing on patients with single-ventricle physiology who were scheduled for heart transplantation due to end-stage heart failure. We retrospectively analyzed the period from the beginning of the transplant program in November 1985 to the end of November 2024. Results: Among 1553 transplanted patients (adults and children), 29 were transplanted due to congenital heart disease (congenital valvular disease not included). In this group, nine patients were transplanted due to end-stage heart failure in the course of single-ventricle physiology. Age at transplantation ranged from 7 to 31 years (median, 17 years), and body weight ranged from 15 to 69 kg (median, 47.9 kg). All nine patients referred for heart transplantation presented with single-ventricle physiology. Their underlying congenital heart defects were heterogeneous and included hypoplastic left heart syndrome (HLHS), double-outlet left ventricle (DOLV), transposition of the great arteries (TGA) with associated ventricular septal defects (VSDs), atrial septal defects (ASDs), valvular abnormalities such as tricuspid and or pulmonary valve atresia or stenosis, systemic or atrioventricular valve regurgitation, and vascular abnormalities, including right-sided aortic arch, aortic coarctation, and pulmonary artery hypoplasia, stenosis, or occlusion, as well as associated pulmonary vascular abnormalities such as left pulmonary artery stenosis and MAPCAs. All patients had previously undergone staged palliative procedures, including Norwood, Hemi-Fontan, Fontan, bidirectional Glenn, modified Blalock-Taussig shunts, Bjork-Fontan, or pulmonary artery banding, often with repeated interventions such as balloon angioplasty, stent placement, or MAPCA closure. Extracardiac comorbidities were common and included coagulopathies, protein-losing enteropathy, hepatic dysfunction, and chronic venous insufficiency. Preoperative functional status was markedly impaired in all patients (NYHA III-IV, INTERMACS 3-4), with severely reduced exercise capacity and thrombotic events in several individuals. Perioperative transplant surgical strategies included femoral cannulation in four cases and standard aortic and caval cannulation in five cases. Pulmonary artery reconstruction was required in all patients. Extended donor pulmonary arteries were applied in eight cases, while a bifurcated Dacron prosthesis was utilized in one patient. Perioperative mortality was 33%, with three deaths attributed to bleeding and hemodynamic instability, while overall mortality was 44% including one late death unrelated to transplantation. Protein-losing enteropathy, although persistent in the immediate postoperative period, resolved in all surviving patients, underscoring the transformative impact of transplantation. Conclusions: These findings emphasize the importance of individualized surgical planning, extended donor pulmonary artery harvesting, and careful preoperative coordination. Heart transplantation remains a viable and life-extending option for selected single-ventricle patients, despite the significant technical and clinical challenges involved.

Background: This study aimed to compare the long-term outcomes of a modified réparation à l'étage ventriculaire (REV) and the Rastelli repair for ventricular septal defect (VSD) and pulmonary outflow tract obstruction without ventriculoarterial concordance. Methods: The study included 100 consecutive patients who underwent a modified REV (n = 50) or Rastelli repair (n = 50) for transposition of the great arteries, double outlet right ventricle, or double outlet left ventricle with VSD and pulmonary outflow tract obstruction. The mean ages of the patients who underwent the modified REV and Rastelli repair were 2.50 years (range: 0.30-12.48) and 5.91 years (range: 0.36-46.15), respectively (P < .05). Results: Actuarial survival and arrhythmia-free survival rates were 83.4% ± 4.0% (P > .05) and 72.4% ± 5.0%, respectively at 37.25 years (P > .05). The reoperation-free survival was 18.7% ± 4.6% at 31.82 years (P < .05). The freedom from the first, second, third, and fourth reoperations after the modified REV versus the Rastelli repair was 35.9% ± 8.3% at 29.8 years versus 6.7% ± 4.3% at 31.8 years (P < .05); 83.2%±6.3% at 29.8 years versus 21.2% ± 15.6% at 35.8 years (P < .05); 94.1% ± 4.1% at 29.8 years versus 56.1% ± 10.9% at 37.3 years (P < .05); and 97.0% ± 3.0% at 29.8 years versus 74.3% ± 10.2% at 37.3 years (P < .05), respectively. The most common causes of reoperation after the Rastelli repair were pulmonary stenosis, left ventricular outflow tract obstruction, and arrhythmia. However, the most common cause of reoperation after the modified REV was pulmonary regurgitation. Conclusion: Modified REV and Rastelli repair have shown satisfactory results in long-term follow-up. However, the Rastelli repair could not avoid repeated reoperations, especially for biventricular outflow tract obstruction and arrhythmia.

Double outlet left ventricle (DOLV) is a rare congenital cardiac anomaly in which both great arteries originate entirely or predominantly from the morphologic left ventricle. The aim of this study is to explore its clinical presentations and compare the diagnostic accuracy of transthoracic echocardiography (TTE) with computed tomographic angiography (CTA) on DOLV. We took TTE as a first-line examination modality, considering CTA and surgical results as the gold standard. Ten suspected patients with DOLV were identified at the Children's Hospital of Chongqing Medical University from March 2009 to November 2023. The clinical presentations, TTE, CTA, and follow-up data of the children were analyzed retrospectively. All 10 cases (100%) underwent TTE examination, and 7 patients (70%) received CTA examination. Six patients (60%) ultimately underwent surgery and were confirmed to have DOLV. DOLV were initially diagnosed using TTE with a diagnostic accuracy rate of 85.71% after comparisons with CTA and surgery results. Ventricular septal defect (100%) and atrial septal defect (80%) were the common associated abnormality. The clinical manifestation of DOLV was atypical, and TTE has a relatively high diagnostic accuracy for DOLV in pediatrics, making it a very valuable tool for early detection.

Among the conotruncal abnormalities, double-outlet left ventricle (DOLV) has been considered an embryologic conundrum, and no more than 200 cases have been described since 1975. DOLV refers to anomalous ventriculoarterial connections with the great arteries arising entirely or predominantly from the left ventricle. It represents a heterogenous group of heart defects considering embryology, morphology, and hemodynamics. DOLV can be detected prenatally or postnatally by transthoracic echocardiography. This study presents a case series of 3 newborns with DOLV and a leftward anterior aorta. The neonates showed symptoms of hypoxia and cardiac compromise after birth and required emergency interventional and surgical procedures. The presented cases contribute interesting insights into the variability of DOLV, and they show the importance of multimodality imaging with echocardiography, computed tomography, and cardiac catheterization. Complex heart defects coexisting with DOLV require precise visualization before surgical or interventional procedures (eg, a single ventricular vs a biventricular solution).

Double outlet left ventricle (DOLV) is a rare congenital heart anomaly, and cases of DOLV with an intact ventricular septum are uncommon. To date, only four such cases have been reported in the medical literature. This report presents a case of prenatally diagnosed DOLV. A fetal echocardiogram at 21 weeks of gestation demonstrated both great arteries, aorta and pulmonary artery, arising from the left ventricle with severely dysplastic tricuspid valve and severe hypoplasia of the right ventricle. Subsequent echocardiograms demonstrated no ventricular septal defect. The patient required balloon atrial septostomy in the first week of life, underwent pulmonary artery banding at 5 weeks of life, and is currently status post-bidirectional Glenn, and is awaiting final Fontan palliation. Prenatal diagnosis aided in predicting and guiding postnatal management.