Borsheim Kirsten E, Woods Ronald K, Kuhn Evelyn M, Frommelt Peter C
Division of Cardiology, Department of Pediatrics, Herma Heart Institute, Medical College of Wisconsin, Children's Wisconsin, 9000 W Wisconsin, Mail station 713, Milwaukee, WI, 53226, USA.
Division of Pediatric Cardiothoracic Surgery, Department of Surgery, Herma Heart Institute, Medical College of Wisconsin, Children's Wisconsin, Milwaukee, WI, USA.
Pediatr Cardiol. 2023 Mar;44(3):587-598. doi: 10.1007/s00246-022-02962-5. Epub 2022 Jul 19.
We sought to characterize the clinical course and outcomes of intervention for Tetralogy of Fallot (TOF) with associated conal septal hypoplasia (CSH) compared to those with identifiable conal septum on initial newborn echocardiogram. We performed a retrospective, 1:2 case-control study of children with TOF anatomy, 33 with CSH and 66 with typical TOF, who underwent surgical repair from 1991-2019 at Children's Wisconsin. Data on echocardiographic anatomic features, systemic oxygen saturations, medical therapies, admissions, palliative interventions, operative strategies, and long-term follow-up were compared. The CSH group had fewer hypercyanotic spells (6% vs 42%, p < 0.001), beta-blockers prescribed (12% vs 41%, p = 0.005), and hospital admissions for cyanosis (12% vs 44%; p = 0.001) prior to any intervention. Of 14 who required palliative intervention, 8 had balloon pulmonary valvuloplasty (BPV) (7 from the CSH group and 1 from the control group), and 6 had systemic-to-pulmonary artery shunts (all from the control group). Definitive repair was performed at a significantly older age in the CSH group (10.2 ± 10 vs 5.6 ± 5.9 months, p = 0.011), with less subpulmonary muscle resection (57.6% in vs 92.4%, p < 0.001) and higher use of a transannular patch (84.8% vs 65.2%, p = 0.040). The average time to surgical reintervention was similar in both groups (9.7 ± 5.9 vs 8.6 ± 6.4 years in controls). We conclude that infants with TOF and CSH have a milder preoperative clinical course with fewer hypercyanotic spells or need for medical therapy. They also respond well to palliative BPV and can safely undergo later definitive repair compared to typical TOF with a well-developed conal septum.
我们试图描述与初始新生儿超声心动图上可识别圆锥间隔的法洛四联症（TOF）相比，合并圆锥间隔发育不全（CSH）的TOF干预的临床过程和结果。我们对威斯康星儿童医院1991年至2019年接受手术修复的TOF解剖结构患儿进行了一项回顾性1:2病例对照研究，其中33例患有CSH，66例为典型TOF。比较了超声心动图解剖特征、体循环血氧饱和度、药物治疗、住院情况、姑息性干预、手术策略和长期随访的数据。CSH组在任何干预前的重度发绀发作较少（6%对42%，p<0.001），开具β受体阻滞剂的比例较低（12%对41%，p=0.005），因发绀住院的比例较低（12%对44%；p=0.001）。在14例需要姑息性干预的患儿中，8例行球囊肺动脉瓣成形术（BPV）（CSH组7例，对照组1例），6例行体肺分流术（均来自对照组）。CSH组进行确定性修复的年龄显著更大（10.2±10个月对5.6±5.9个月，p=0.011），肺下肌切除术较少（57.6%对92.4%，p<0.001），使用跨环补片的比例较高（84.8%对65.2%，p=0.040）。两组手术再次干预的平均时间相似（对照组为9.7±5.9年对8.6±6.4年）。我们得出结论，患有TOF和CSH的婴儿术前临床过程较轻，重度发绀发作较少或药物治疗需求较少。与圆锥间隔发育良好的典型TOF相比，他们对姑息性BPV反应良好，并且可以安全地接受后期确定性修复。
