両大血管右室起始症

OBJECTIVES: To characterize hospital survival and risk factors after aortic root replacement performed by congenital units in adult patients, and to compare risk profiles of repaired conotruncal defects to other underlying diagnoses. METHODS: Adult patients after aortic root replacement in a congenital cardiac unit were identified in the European Congenital Heart Surgeons Association (ECHSA) database. Underlying pathology, prior surgeries, and types of index aortic root surgery were characterized. Primary endpoints were hospital and 30-day survival. Secondary endpoints included circulatory arrest, length of intensive care unit and hospital stays, and discharge to chronic care facility. Multiple logistic regression analyses were performed to test for independent risk factors. RESULTS: N=2544 patients after full aortic root replacement, performed in 101 different hospitals (1999 to 2024), were identified. N=249 patients (10%) with primary conotruncal defects included double-outlet-right ventricle (n=14, 6%), pulmonary atresia with VSD (n=42, 17%), transposition of great arteries (n=71, 29%), Tetralogy of Fallot (n=88, 35%), Truncus arteriosus communis (n=34, 14%). Mechanical Bentall was performed in n=163 cases (65%), valve-sparing in n=43 cases (17%), biological Bentall in n=20 (8%), homograft replacement in n=23 (9%). Conotruncal diagnosis was linked to longer CPB times (p<0.001), longer aortic cross-clamp times (p<0.001), longer ICU stay (p<0.001) and longer hospital stay (p<0.001). Multiple logistic regression revealed duration of CPB (p<0.001) and conotruncal lesion (OR 2.1, p=0.037) as independent risk factors for hospital mortality. CONCLUSIONS: Among adult congenital heart defect patients undergoing aortic root replacement, those with conotruncal defects represent a distinct subgroup characterized by a high-risk operative profile.

BACKGROUND: Uncorrected adults congenital heart disease (ACHD) may present with profound hypoxia and dual risks of thrombosis and bleeding, posing major challenges for definitive surgical repair. In such settings, right ventricular outflow tract (RVOT) stenting may provide a life-saving palliative option. CASE PRESENTATION: We report a 30-year-old woman with double-outlet right ventricle, subaortic ventricular septal defect, and severe infundibular pulmonary stenosis who suffered cardiac arrest due to profound hypoxia. Her clinical profile was complicated by polycythemia, upper extremity deep vein thrombosis, and active gastrointestinal bleeding. In light of prohibitive surgical risk, urgent RVOT stenting was undertaken, requiring multiple technical adaptations due to severe obstruction. Post-procedure, oxygen saturation improved to 92%, gastrointestinal bleeding ceased, and deep vein thrombosis regressed without anticoagulation. She subsequently completed phase II cardiac rehabilitation with improved functional capacity. CONCLUSION: Urgent RVOT stenting can serve as a feasible therapeutic option in high-risk ACHD patients, effectively restoring pulmonary blood flow while simultaneously stabilizing thrombotic and bleeding complications.

Conotruncal anomalies comprise a heterogeneous group of congenital heart defects arising from abnormal embryologic development of the cardiac outflow tract and the great arteries. Aortic root dilatation represents a frequent yet comparatively underexplored finding in this population, and clear guidelines regarding surveillance and surgical treatment remain limited. This article reviews mechanisms of aortic root dilatation, diagnostic evaluation, and surgical indications and principles for aortic root surgery in adults with conotruncal anomalies, including lesion-specific considerations for Tetralogy of Fallot, d-transposition of the great arteries, truncus arteriosus, and double outlet right ventricle.

Background Hearts suitable for Fontan palliation include those with an anatomically single ventricle (absence of a second ventricular mass), those with a single atrioventricular connection, and those with two ventricles in whom biventricular repair is not feasible due to anatomical or physiological complexity (functionally univentricular physiology). We describe the anatomical spectrum of such hearts over a decade at a single tertiary center. Methodology We encountered 420 patients undergoing the Fontan operation over a decade, between 2010 and 2019. Their detailed medical records were reviewed in this retrospective study, and the information gathered is presented in this article. The cardiac anatomy is presented depending on the information gathered from the medical records. Results A total of 420 patients underwent the Fontan operation during the study period. Only 30 (7.1%) had a true anatomically single ventricle. The remaining 390 had two ventricular chambers, including those with a hypoplastic ventricle. The most common anatomical diagnosis was double-outlet right ventricle (DORV) in 143 (34%) patients, followed by tricuspid atresia in 118 (28%) patients. Major aortopulmonary collateral arteries were present in 250 (59.5%) patients, and left superior vena cava in 61 (14.5%) patients. Completion Fontan operation after bidirectional Glenn shunt was the most common surgical pathway (n = 254, 60.5%), though primary Fontan (n = 15, 3.6%) and other staged approaches were also performed. Conclusions The anatomical spectrum of patients undergoing Fontan operation is wide and includes true anatomically single ventricle, single atrioventricular connection, and functionally univentricular physiology with two ventricular chambers. DORV was the most common diagnosis, followed by tricuspid atresia. The decision for Fontan operation was driven by a combination of anatomical and physiological factors. Early and long-term outcomes were not analyzed in this study.