Divisions of Cardiology and Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Can J Cardiol. 2019 Apr;35(4):446-452. doi: 10.1016/j.cjca.2018.12.006. Epub 2018 Dec 11.
Common arterial trunk (CAT) is a rare anomaly with a spectrum of pathology. We sought to identify current trends and factors associated with postnatal outcomes.
This was a single-centre review including 153 live births with planned surgery. Patients were analyzed as 2 cohorts based on era of CAT diagnosis (1990 to 1999 vs 2000 to 2014) and complexity of disease (simple vs complex). "Complex" required the association with significant aortic arch obstruction, truncal valve (TV) stenosis/regurgitation, and/or branch pulmonary artery (PA) hypoplasia, respectively.
Sixteen (10%) died preoperatively, and this outcome was associated with significant TV stenosis (odds ratio [OR] 4.55; P = 0.01) and regurgitation (OR 3.17; P = 0.04); 130 (95%) of 137 operated infants underwent primary complete repair. Their survival rates to 1 year improved from 54% to 85% after 2000, although this outcome remained substantially lower for cases with a complex vs simple CAT repair (76% vs 95%; OR 6.46; P = 0.006). Other risk factors associated with decreased 1-year survival included diagnosis before 2000 (OR 4.48; P = 0.038) and a lower birth weight (OR 8.0 per kg weight; P = 0.001). Finally, of 93 survivors beyond year 1 of life, 76 (82%) had undergone a total of 224 reinterventions. Only 15 (16%) were alive without any surgical or catheter-based reintervention at study end.
Despite recent surgical improvements, postnatal mortality continues to be substantial if CAT is complicated by significant pathology of the TV, aortic arch, or branch PAs. Reoperations and catheter interventions are eventualities for most patients during childhood.
共同动脉干(CAT)是一种罕见的异常,具有多种病理学表现。我们试图确定与出生后结局相关的当前趋势和因素。
这是一项单中心回顾性研究,纳入了 153 例计划手术的活产儿。根据 CAT 诊断的时代(1990 年至 1999 年与 2000 年至 2014 年)和疾病的复杂性(简单与复杂)将患者分为 2 个队列进行分析。“复杂”分别需要与严重主动脉弓阻塞、干瓣(TV)狭窄/反流和/或分支肺动脉(PA)发育不良相关。
16 例(10%)术前死亡,该结局与严重 TV 狭窄(优势比[OR] 4.55;P = 0.01)和反流(OR 3.17;P = 0.04)相关;137 例手术婴儿中有 130 例(95%)接受了原发性完全修复。2000 年后,其 1 年生存率从 54%提高到 85%,但复杂型 CAT 修复与简单型 CAT 修复相比,生存率仍然明显较低(76%比 95%;OR 6.46;P = 0.006)。与 1 年生存率降低相关的其他危险因素包括 2000 年前诊断(OR 4.48;P = 0.038)和出生体重较低(OR 每公斤体重 8.0;P = 0.001)。最后,在 1 岁以上的 93 例幸存者中,有 76 例(82%)共接受了 224 次再介入治疗。研究结束时,仅有 15 例(16%)无任何手术或导管介入治疗仍存活。
尽管最近的手术有所改善,但如果 CAT 伴有 TV、主动脉弓或分支 PA 的严重病变,出生后死亡率仍然很高。大多数患者在儿童期都需要进行再次手术和导管介入治疗。