Preoperative in-hospital mortality in neonates with critical CHD

Objective: Data regarding preoperative mortality in neonates with critical CHD are sparse and would aid patient care and family counselling. The objective of this study was to utilise a multicentre administrative dataset to report the rate of and identify risk factors for preoperative in-hospital mortality in neonates with critical CHD across US centres. Study design: The Pediatric Health Information System database was utilised to search for newborns ≤30 days old, born 1 January 2009 to 30 June 2018, with an ICD-9/10 code for d-transposition of the great arteries, truncus arteriosus, interrupted aortic arch, or hypoplastic left heart syndrome. Preoperative in-hospital mortality was defined as patients who died prior to discharge without an ICD code for cardiac surgery or interventional catheterisation. Results: Overall preoperative mortality rate was at least 5.4% (690/12,739) and varied across diagnoses (d-TGA 2.9%, TA 8.3%, IAA 5.5%, and HLHS 7.3%) and centres (0–20.5%). In multivariable analysis, risk factors associated with preoperative mortality included preterm delivery (<37 weeks) (OR 2.3, 95% CI: 1.8–2.9; p < 0.01), low birth weight (<2.5 kg) (OR 3.8, 95% CI: 3.0–4.7; p < 0.01), and genetic abnormality (OR 1.6, 95% CI: 1.2–2.2; p < 0.01). Centre average surgical volume was not a significant risk factor. Conclusion: Approximately 1 in 20 neonates with critical CHD suffered preoperative in-hospital mortality, and rates varied across diagnoses and centres. Better understanding of the factors that drive the variation (e.g. patient factors, preoperative care models, surgical timing) could help identify patient care improvement opportunities and inform conversations with families.

Surgical Management for Late Presentation of Interrupted Aortic Arch with Aortopulmonary Window

We report a case of advanced age at presentation of interrupted aortic arch with aortopulmonary window. Although the association between these two defects is well established, their overall prevalence is extremely low. This case presents us with an excellent opportunity to discuss decision making regarding aortic arch repair techniques according to age at which primary surgical correction is intended.

Management of Interrupted Aortic Arch With Associated Anomalies: A Single-Center Experience

Objectives Interrupted aortic arch (IAA) includes a broad spectrum of associated anomalies. In this study, we present our surgical management and patient-specific decisions regarding IAA anomalies with early- and mid-term outcomes. Methods The medical records of 25 patients undergoing IAA repair between 2014 and 2019 were retrospectively reviewed. Sixteen patients had type B (64%) interruptions, 7 had type A (28%) interruptions, and 2 had type C (8%) interruptions. Fourteen patients had an isolated ventricular septal defect, and 3 of them had associated left ventricular outflow tract obstruction. Other associated anomalies were functional single ventricle (n = 5), Taussig–Bing anomaly (n = 3), aortopulmonary window (n = 1), multiple ventricular septal defects (n = 1), and truncus arteriosus with dextrocardia (n = 1). The initial operation age was 17.2 ± 14 (range: 1 - 60) days. Results Single-stage total repair was performed for 15 patients. Six patients underwent aortic arch repair and pulmonary artery banding. Four patients with left ventricular outflow tract obstruction or who were premature underwent the hybrid procedure. The aortic arch repair was performed in 16 cases (64%) by the anterior patch augmentation technique, in 3 cases (12%) by the reverse left subclavian artery flap technique, and in 3 cases (12%) by direct end-to-end anastomosis. Postoperative early mortality occurred in 4 (16%) patients, and sternal closure was delayed in 13 (52%) patients. Three patients who underwent a hybrid procedure due to left ventricular outflow tract obstruction underwent biventricular repair 8 to 13 months later. Eight patients (38%) required reintervention due to arch restenosis during the follow-up period. The mean follow-up was 37.1 ± 21.7 months. Conclusion Planning surgical treatment according to the characteristics of the patients and accompanying anomalies may improve the results.

Aortic valve replacement using Konno procedure for prior Yasui procedure

A 2-year-old girl underwent conversion to the Konno procedure by removing the Damus–Kaye–Stansel anastomosis after the neonatal Yasui procedure for an interrupted aortic arch with left ventricular outflow tract stenosis. Her postoperative course was uneventful. However, left ventricular outflow tract restenosis occurred due to narrowed ventricular septal defect and moderate neoaortic regurgitation from the old pulmonary valve. The Konno procedure was performed by removing the Damus–Kaye–Stansel anastomosis for left ventricular outflow tract restenosis and neoaortic regurgitation and performing right ventricular outflow tract reconstruction and ventricular septal defect closure. Left ventricular outflow tract restenosis was not observed.

Anterior Translocation of the Right Pulmonary Artery to Relieve Bronchial Compression in Truncus Arteriosus With Interrupted Aortic Arch

We report an anterior translocation of the right pulmonary artery procedure to relieve severe left bronchial obstruction that was caused by right pulmonary artery stent placement in a 1-year-old patient with truncus arteriosus and interrupted aortic arch. After neonatal repair, the patient re-presented with severe truncal valve regurgitation, right pulmonary artery stenosis, and severe biventricular dysfunction, which was treated with truncal valve repair and right pulmonary artery plasty. The patient suffered from left bronchial compression from right pulmonary artery stent placement, which was successfully treated by the translocation procedure. Bronchial stenosis was successfully relieved by the translocation procedure. Indications, advantages, and disadvantages of this procedure are discussed.

Kết quả phẫu thuật điều trị bệnh lý gián đoạn quai động mạch chủ tại Bệnh viện Nhi Trung ương

TÓM TẮT Mục tiêu: Đánh giá kết quả tạo hình quai động mạch chủ trong phẫu thuật một thì điều trị bệnh lý GĐQĐMC tại Bệnh Viện Nhi Trung Ương từ năm 2012 đến 2019. Phương pháp: Nghiên cứu hồi cứu 53 bệnh nhân được phẫu thuật sửa chữa một thì bệnh tim bẩm sinh GĐQĐMC từ tháng 01 năm 2012 đến tháng 12 năm 2019. Kết quả: Bệnh nhân gồm 32 nam và 21 nữ. Tuổi trung bình là 43 ± 53 ngày, cân nặng trung bình khi phẫu thuật 3.4 ± 0.7 kg. Trung bình thời gian chạy máy tim phổi nhân tạo (TPNT) là 128 ± 28 phút, thời gian cặp chủ 92 ± 18 phút và thời gian tưới máu não chọn lọc là 34 ± 11 phút. Tử vong sớm sau mổ có 9 bệnh nhân (16.9%). Thời gian theo dõi trung bình 37 ± 21 [8-95] tháng. Tỷ lệ sống sót chung sau phẫu thuật là 83% tại thời điểm 8 năm. Tỷ lệ sống sót không phải can thiệp lại do tái hẹp quai ĐMC là 97.6% tại thời điểm 8 năm, không có bệnh nhân tử vong trong quá trình theo dõi. Kết luận: Phẫu thuật một thì sửa chữa bệnh lý GĐQĐMC ở Bệnh viện Nhi Trung ương là hoàn toàn khả thi và có thể thực hiện được ở lứa tuổi sơ sinh và nhũ nhi với tỷ lệ tử vong thấp. Từ khóa: Gián đoạn quai động mạch chủ, thông liên thất, hẹp đường ra thất trái, cửa sổ chủ phế. ABSTRACT OUTCOMEOF SURGICAL REPAIR FOR INTERRUPTED AORTIC ARCH IN NATIONAL CHILDREN’S HOSPITAL, HANOI Objective: The objective was to determine outcomes of aortic arch reconstruction in one-stage repair of interrupted aortic arch in Nation Hospital of Pediatrics from 2012 to 2019. Methods: Records of 53 consecutive patients undergoing interrupted aortic arch repair between January 2012 to December 2019 were reviewed. Single-stage repair was performed in all of the patients. Results: The patients consisted of 32 males and 21 females. Mean age of the patients was 43 ± 53 days and mean weight was 3.4 ± 0.7 kg. The average cardiopulmonary bypass time was 128 ± 28 minutes, the aortic cross-clamp time was 92 ± 18 minutes and the selective cerebral perfusion time was 34 ± 11 minutes. Early mortality was 16.9%. Mean follow-up was 37 ± 21 [8-95] months. Actuarial survival including early death was 83% at 8 years. The freedom from reintervention of recurrent aortic arch obstruction was 97.6% at 8 years. No late death occurred. Conclusions: Single-stage repair of interrupted aortic arch with intracardiac defects is safe and feasible with newborn and infant in National Hospital of Pediatrics. Keyword: Interrupted aortic arch, ventricular septal defect, left ventricular outflow tract obstruction, aortopulmonary window