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BACKGROUND: Children undergoing orthotopic heart transplant (OHT) may require complex reconstruction of superior vena cava (SVC) anomalies. SVC anatomy and mode of reconstruction are potential risk factors for SVC obstruction. METHODS: A retrospective single-center review was conducted of patients undergoing initial OHT between January 1, 1990, and July 1, 2021. Simple SVC anatomy included a single right SVC to the right atrium or bilateral SVCs with a left SVC to an intact coronary sinus, without prior superior cavopulmonary connection. Presence of anomalous SVC anatomy, superior cavopulmonary connection, or previous atrial switch operation defined complex anatomy. Reconstructive strategies included atrial anastomosis; direct SVC-to-SVC anastomosis; and augmented SVC anastomosis using innominate vein, patch, cavopulmonary connection, or interposition graft. The primary outcome was reintervention for SVC obstruction. RESULTS: Of 288 patients, pretransplant diagnoses included congenital heart disease (n = 155 [54%]), cardiomyopathy (n = 125 [43%]), and other (n = 8 [3%]). Most (n = 208 [72%]) had simple SVC anatomy compared with complex SVC anatomy (80 [28%]). Reintervention for SVC obstruction occurred in 15 of 80 (19%) with complex anatomy and 1 of 208 (0.5%) with simple anatomy (P = .0001). Reintervention was more common when innominate vein or a patch was used (9/25 [36%]) compared with an interposition graft (1/7 [14%]) or direct anastomosis (6/82 [7%]; χ2 = 13.1; P = .001). Most reinterventions occurred within 30 days of OHT (14/16 [88%]). CONCLUSIONS: Patients with complex SVC anatomy have a higher rate of reintervention for SVC obstruction after OHT compared with those with simple SVC anatomy. In cases of complex SVC anatomy, interposition grafts may be associated with less reintervention compared with complex reconstructions using donor tissue.
RESUMO
OBJECTIVE: Neonatal interventional strategies for pulmonary atresia with intact ventricular septum are based on tricuspid valve hypoplasia and right ventricle-dependent coronary circulation. We sought to evaluate long-term outcomes comparing biventricular (BiV) versus single-ventricle (SV) strategies. METHODS: Retrospective review was performed of 119 patients diagnosed with pulmonary atresia with intact ventricular septum from 1995 to 2018. Descriptive statistics summarized patient characteristics and a multivariable Cox survival model was used to compare treatment strategies. RESULTS: Of 119 patients, 62 (52.1%) were male and 13 (10.9%) had a chromosomal abnormality. BiV was pursued in 53.8% (64 out of 119) and SV in 46.2% (55 out of 119) with median tricuspid valve z scores of -1.59 (interquartile range, -3.03 to 0.21) and -5.12 (interquartile range, -5.60 to -4.06), respectively. The median follow-up was 6 years (interquartile range, 2-15 years). Overall survival at 1, 3, and 10 years was 82.4% (98 out of 119), 80.6% (96 out of 119) and 79.8% (95 out of 119), respectively. End states include 36 (30.3%) BiV, 33 (27.7%) SV, 22 (18.5%) alive without definitive end state, 21 (17.6%) death before end state, 4 (3.4%) 1-and-a-half ventricle, and 3 (2.5%) transplants. No SV were converted to BiV, whereas 4 out of 64 (6.3%) BiV were converted to SV. After adjusting for gender, chromosomal abnormalities, gestational age, and birth weight, SV patients had a significantly higher hazard of mortality (hazard ratio, 9.0; 95% CI, 2.65-30.69; P < .001). Mortality was higher in those with right ventricle-dependent coronary circulation (41.9% [13 out of 31]) compared with those without right ventricle-dependent coronary circulation (7.3% [6 out of 82]) (P < .001). CONCLUSIONS: Pulmonary atresia with intact ventricular septum remains a challenging lesion for those patients on the SV pathway, particularly with right ventricle-dependent coronary circulation.
