Michielon Guido, Parisi Francesco, Squitieri Cosimo, Carotti Adriano, Gagliardi Giulia, Pasquini Luciano, Di Donato Roberto M
Dipartimento Medico-Chirurgico di Cardiochirurgia e Cardiologia Pediatrica, DMCCP, Ospedale Pediatrico Bambino Gesù, Roma, Italy.
Circulation. 2003 Sep 9;108 Suppl 1:II140-9. doi: 10.1161/01.cir.0000087442.82569.51.
Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for congenital heart disease.
Between 1988 and 2002, 43 patients (mean age 9.1+/-7.2 years) underwent 44 OHT for complex TGA (6), DORV (4), single ventricle (21), and other end-stage structural heart disease (11). Two discernible ventricular chambers were present in 18 pts (41.8%). Previous reconstructive or palliative procedures had been previously accomplished in 35 pts (83.3%), including atrial switch (5), systemic-to-pulmonary shunts (10), cavopulmonary anastomosis (9), Fontan completion (6), and others (5).
30-day survival for the 2-ventricle subgroup was 94.4+/-5.4% compared with 67.2+/-9.5% for the single ventricle subgroup (P=0.04) (overall 78.6%+/-3.3%). OHT following single ventricle staging to bi-directional cavopulmonary anastomosis exhibited 100% early survival, as opposed to 62.5+/-17.1% for OHT after systemic-to-pulmonary shunts, and 33.3+/-19.2% for OHT following failing Fontan (P=0.010). HLHS diagnosis (0.0085) and failing Fontan (P=0.003) were identified as independent predictors of early mortality by regression logistic modeling, while Fontan stage represented the only predictor of overall mortality by Cox proportional hazard. Overall 10-year survival was 54.3+/-11%.
OHT for structural congenital heart disease with single ventricle physiology entails substantial early mortality and bi-directional cavopulmonary anastomosis enables the best transition to heart transplant. OHT should be considered in the decision making process as an alternative to Fontan completion in high-risk candidates, since rescue-OHT after failing Fontan seems unwarranted.
评估患有先天性心脏病接受原位心脏移植（OHT）的儿童早期死亡的递增风险因素。
1988年至2002年间，43例患者（平均年龄9.1±7.2岁）因复杂型大动脉转位（6例）、右室双出口（4例）、单心室（21例）及其他终末期结构性心脏病（11例）接受了44次原位心脏移植。18例患者（41.8%）存在两个可辨认的心室腔。35例患者（83.3%）曾接受过先前的重建或姑息手术，包括心房调转术（5例）、体肺分流术（10例）、腔肺吻合术（9例）、Fontan手术完成（6例）及其他（5例）。
双心室亚组的30天生存率为94.4±5.4%，而单心室亚组为67.2±9.5%（P=0.04）（总体为78.6%±3.3%）。单心室分期至双向腔肺吻合术后的原位心脏移植早期生存率为100%，相比之下，体肺分流术后的原位心脏移植为62.5±17.1%，Fontan手术失败后的原位心脏移植为33.3±19.2%（P=0.010）。左心发育不全综合征诊断（0.0085）和Fontan手术失败（P=0.003）通过回归逻辑模型被确定为早期死亡的独立预测因素，而Fontan分期是Cox比例风险模型中总体死亡的唯一预测因素。总体10年生存率为54.3±11%。
具有单心室生理的先天性结构性心脏病的原位心脏移植存在显著的早期死亡率，双向腔肺吻合术能实现向心脏移植的最佳过渡。在决策过程中，对于高危候选者，原位心脏移植应被视为Fontan手术完成的替代方案，因为Fontan手术失败后进行挽救性原位心脏移植似乎没有必要。
