Department of Cardiology, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK.
Department of Cardiac Surgery, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK.
Cardiol Young. 2023 Jul;33(7):1097-1101. doi: 10.1017/S1047951122002128. Epub 2022 Jul 11.
The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the literature.
The hospital records, echocardiographic and other imaging modality data, outpatients' records, operation notes, and other electronic data were reviewed. Patients were reviewed, and the final outcomes of surgery were observed.
Six cases (two males) with common arterial trunk presented over a 30-year period. Five had a complete unbalanced atrioventricular septal defect (83%) and one (17%) had tricuspid atresia associated with common arterial trunk. All had antenatal diagnosis. Two cases (33%) were excluded from initial surgical palliation due to Trisomy 21 in one and severe truncal valve regurgitation in one. Initial surgical palliation was performed in four cases (67%) at median age of 31 days (2-60) and consisted of disconnection and reconstruction of the pulmonary arteries and establishing controlled pulmonary blood flow. There were no early deaths. Conversion to cavopulmonary shunt was not possible in two due to severe airway problems in one and pulmonary arteries anatomy in one. They died at 11 and 16 months, respectively. Two patients (33%) underwent cavopulmonary shunt with 1 (17%) being alive at 18 months - 12 months after cavopulmonary shunt. The second patient proceeded to Fontan completion at 19 months but required catheter takedown 3 months later and died 3.5 years later.
Univentricular hearts with common arterial trunk carry extremely poor short- to medium-term outcomes. This should inform antenatal and postnatal counselling and decision-making.
单心室心脏缺陷伴共同动脉干极为罕见。文献中缺乏基于人群的结局研究。
回顾了医院记录、超声心动图和其他成像方式数据、门诊记录、手术记录和其他电子数据。对患者进行了复查，并观察了手术的最终结果。
30 年间共有 6 例（2 例为男性）共同动脉干患者。5 例存在完全性非均衡性房室间隔缺损（83%），1 例（17%）存在三尖瓣闭锁伴共同动脉干。所有患者均有产前诊断。2 例（33%）因 1 例 21 三体和 1 例严重的干瓣反流而被排除在初始外科姑息治疗之外。4 例（67%）在中位年龄 31 天（2-60 天）时进行了初始外科姑息治疗，包括肺动脉的切断和重建以及建立控制性肺血流。没有早期死亡。2 例（33%）因严重气道问题和肺动脉解剖问题无法转为腔静脉肺动脉分流术。他们分别在 11 个月和 16 个月时死亡。2 例（33%）患者行腔静脉肺动脉分流术，其中 1 例（17%）在分流术后 12 个月存活 18 个月。第 2 例患者在 19 个月时行 Fontan 完成术，但 3 个月后需要导管拆除，并在 3.5 年后死亡。
单心室伴共同动脉干的患儿短期至中期预后极差。这应该为产前和产后咨询和决策提供信息。
