Complications of Surgical Treatment of Aortic Arch Hypoplasia in Infants

Aortic arch surgery in neonatal patients remains problematic despite the constant evolution and improvement of treatment methods. Even after successful correction, complications associated with aortic arch reconstruction are more common in young children. This is especially true for newborns and infants with concomitant complex congenital intracardiac abnormalities. Despite the risk of postoperative complications, the risk of surgical treatment of aortic arch hypoplasia is lower than the cumulative hazards associated with the natural course of this defect. That is why pediatric cardiologists are obliged to constantly monitor the operated patients in order to timely identify and treat complications. The aim. To analyze the complications in the early and remote postoperative periods after the reconstruction of the aortic arch in the neonatal period. Materials and methods. The work is based on a study of 445 patients under 1 year of age, who underwent surgical treatment of aortic arch hypoplasia from 2010 to 2019. The criterion for inclusion in the study group was the presence of isolated hypoplasia of the aortic arch and combination with other defects, which were corrected by two-ventricular repair. The majority of the treated patients were male (284 [63.8% of the total number of patients]). The median age of the patients was 0.7 months (0.3; 2.7). The median body weight of the patients was 3.7 kg (3.25; 4.59). Results. At the hospital stage, 12 patients died, which accounted for 3.1% of the entire cohort of operated patients. Complications occurring at the hospital stage were recorded in 75 (16.8%) patients. In the long term, a complicated course was observed in 72 (16.6%) children. Among them, the most frequent complications were: respiratory failure requiring prolonged mechanical ventilation (36.9%), dilated sternum due to severe heart failure (17.4%), diaphragmatic paresis (8.7%), chylothorax (5.4%). In the long term, 47 (10.5%) patients developed aortic arch restenosis, which required reinterventions. The proportion of patients without reoperations in the follow-up period according to the KaplanMeier analysis was 93.4% after 1 year, 91.2% after 4 years, and 76.5% after 9 years. Residual hypertension requiring medical treatment was reported in 59 (13.2%) patients. Conclusions. Aortic arch hypoplasia is a complex congenital heart disease; its surgical treatment is accompanied by the development of complications both in the early and in the long-term period. The main complications at the hospital stage were respiratory and heart failures which were associated with the presence and correction of concomitant congenital heart defects. The main complication of the follow-up period was reobstruction at the level of the aortic arch. Anatomical correction of reobstruction is safe with both endovascular and surgical treatments.

Reconstruction of the Aortic Arch in Newborns and Infants Using an Extended End-to-End Anastomosis

The problem of the effectiveness of obstruction at the level of the aortic arch is still a matter of discus-sion in the modern literature. Traditionally, by excision of the coarctation part, in the presence of hypoplasia, the incision is extended to a narrowed area and a modification of the classical end-to-end anastomosis is applied in the form of an elongated or expanded variant. Recently, when proximal part is involved in the pathological process, cardiac surgeons have been more likely to use median sternotomy using other types of plastic surgery, including dilation of the narrowed area with a pericardial patch, or pulmonary artery tissue. Accordingly, the analysis of the results of the use of end-to-end anastomosis in young children with aortic arch hypoplasia, especially in view of long-term survival and the level of reoperation, is an important issue of neonatal cardiac surgery. The aim. To evaluate the effectiveness of the use of an extended end-to-end anastomosis after reconstruction of the aortic arch in children under 1 year of age. Materials and methods. The study material included 348 infants who underwent surgical correction of aortic arch hypoplasia through the method of extended end-to-end anastomosis from 2010 to 2020. The operations were performed at the National Amosov Institute of Cardiovascular Surgery of the NAMS of Ukraine and the Ukrainian Children’s Cardiac Center. The study group included only patients with two-ventricular physiology. There were 233 male patients (67%) and 115 female patients (33%). The mean age was 1.07 (0.20; 2.30) months, the mean weight was 3.89 (3.30; 4.90) kg, the mean body surface area was 0.23 (0.20; 0.28) m2. Diagnosis of aortic arch hypoplasia was based on two-dimensional echocardiography. Results. According to echocardiography, after surgery there was a significant decrease in the pressure gradient in the aortic arch from 48.3 ± 20.3 to 16 ± 6.9 (p<0.05), left ventricular PV increased significantly from 61.6 ± 12% to 66.3 ± 6.4% (p> 0.05). The hospital mortality was 1.7% (n = 6). The causes of mortality were not related to the end-to-end aortic arch technique. The duration of follow-up period ranged from 1 month to 9.3 years. Two deaths occurred in the follow-up period. Thirty-two (9.1%) patients developed aortic arch restenosis in the postoperative period. Balloon dilatation of restenosis was performed in 21 patients. Eleven patients underwent repeated aortic arch repair surgery through the median sternotomy. There were no central nervous system complications in the follow-up period. Conclusions. The use of an extended end-to-end anastomosis in the surgical treatment of aortic arch hypoplasia demon strates low hospital mortality and high long-term survival. Indications for the effective use of this type of reconstruction are hypoplasia of the isthmus and distal aortic arch.

Contemporary outcomes of aortic arch hypoplasia and coarctation repair in a tertiary paediatric cardiac surgery centre

Objectives: There are several studies reporting the outcomes of hypoplastic aortic arch and aortic coarctation repair with combination of techniques. However, only few studies reported of aortic arch and coarctation repair using a homograft patch through sternotomy and circulatory arrest with retrograde cerebral perfusion. We report our experience and outcomes of this cohort of neonates and infants. Methods: We performed retrospective data collection for all neonates and infants who underwent aortic arch reconstruction between 2015 and 2020 at our institute. Data are presented as median and inter-quartile range (IQR). Results: The cohort included 76 patients: 49 were males (64.5%). Median age at operation was 16 days (IQR 9–43.25 days). Median weight was 3.5 kg (IQR 3.10–4 kg). There was no 30 days mortality. Three patients died in hospital after 30 days (3.95%), neurological adverse events occurred in only one patient (1.32%) and recurrent laryngeal nerve injury was noted in four patients (5.26%). Only three patients required the support of extracorporeal membrane oxygenation (ECMO) with a median ECMO run of 4 days. Median follow-up was 35 months (IQR 18.9–46.4 months); 5 years survival was 93.42% (n = 71). The rate of re-intervention on the aortic arch was 9.21% (n = 7). Conclusion: Our experience shows excellent outcomes in repairing aortic arch hypoplasia with homograft patch under moderate to deep hypothermia with low in-hospital and 5 years mortality rates.

Kết quả trung hạn và yếu tố nguy cơ phẫu thuật chuyển gốc động mạch kèm theo sửa chữa quai động mạch chủ một thì

TÓM TẮT Mục tiêu: Nghiên cứu này nhằm đánh giá kết quả trung hạn và các yếu tố nguy cơ đối với phẫu thuật chuyển gốc kèm theo sửa chữa quai động mạch chủ một thì tại Bệnh viện Nhi Trung Ương. Đối tượng và phương pháp: Nghiên cứu liên tiếp 31 bệnh nhân từ tháng 2 năm 2010 đến tháng 12 năm 2016. Bao gồm 9 bệnh nhân chẩn đoán chuyển gốc động mạch, 22 bệnh nhân chẩn đoán bất thường Taussig-Bing, kết hợp với tổn thương thiểu sản quai, hẹp eo động mạch chủ hoặc gián đoạn quai động mạch chủ. Bệnh nhân được tiến hành sửa chữa toàn bộ một thì: tái tạo quai động mạch chủ sử dụng kĩ thuật tưới máu não trọn lọc trước khi tiến hành phẫu thuật chuyển gốc và sửa các thương tổn trong tim khác. Nhóm chuyển gốc động mạch, có 1 bệnh nhân chuyển gốc động mạch lành vách liên thất kèm theo hẹp eo động mạch chủ, 1 bệnh nhân chuyển gốc động mạch kèm theo thông liên thất hẹp eo và thiểu sản quai động mạch chủ, 6 bệnh nhân chuyển gốc động mạch kèm theo thông liên thất và hẹp eo động mạch chủ, 1 bệnh nhân chuyển gốc động mạch kèm theo thông liên thất và gián đoạn quai động mạch chủ. Nhóm bất thường Taussig-Bing, có 12 bệnh nhân kèm theo thiểu sản quai và hẹp eo động mạch chủ, 8 bệnh nhân kèm theo hẹp eo động mạch chủ, 2 bệnh nhân kèm theo gián đoạn quai động mạch chủ. Thời gian cặp động mạch chủ trung bình: 172,32 ± 31,36 phút, thời gian tưới máu não chọn lọc trung bình: 38,76 ± 12,30 phút Kết quả: Có 6 bệnh nhân (19,4%) tử vong tại viện, không có bệnh nhân tử vong muộn. Có 2 bệnh nhân (6,5%) phải mổ lại do hẹp đường ra thất phải, không có bệnh nhân nào phải can thiệp lại quai động mạch chủ. Nhiễm trùng bệnh viện là yếu tố nguy cơ chính duy nhất gây tử vong tại viện (p=0,036). Cấu trúc động mạch vành, hẹp eo động mạch chủ và những tổn thương giải phẫu khác không phải là yếu tố nguy cơ gây tử vong trong phân tích đa biến Kết luận: Kết quả trung hạn của phẫu thuật sửa chữa 1 thì đối với bệnh lý chuyển gốc động mạch, bất thường Taussig - Bing kèm theo bệnh lý quai động mạch chủ tại Bệnh viện Nhi Trung Ương là khả quan. Từ khóa: Chuyển gốc động mach, hẹp eo/thiểu sản quai động mạch chủ, thông liên thất, phẫu thuật một thì ABSTRACT MIDTERM OUTCOMES AND RISK FACTORS FOR SINGLE STAGE REPAIR OF ARTERIAL SWITCH OPERATION COMBINED WITH AORTIC ARCH RECONSTRUCTION Objectives: The mid-term outcome and risk factor of single stage repair for TGA or Taussig-Bing anomaly combined with arch artery anomaly was evaluated in this study in National Hospital of Pediatrics Methods: From February 2010 to December 2016, a consecutive 31 patients diagnosed with TGA (9 patients) or Taussig-Bing anomaly (22 patients) associated with aortic arch hypoplasia, coarctation of the aorta or interrupted aortic arch (IAA) underwent single stage repair. The aortic arch reconstruction was perform using autologous tissueand regional cerebral perfusion before arterial switch operation. In TGA group, there were 1 patient with intact ventricular septum (IVS) and CoA, 1 patient with ventricular septal defect (VSD) with CoA and AAH, 6 patients with VSD and CoA, and 1 patient with IAA. Taussig-Bing anomaly group showed 12 patients with CoA and AAH, 8 patients with discrete CoA, and two patients with IAA. Aortic cross clamp time was 172,32 ± 31,36 min and regional cerebral perfusion time was 38,76 ± 12,30 min. Results: There were 6 (19,4%) hospital deaths and no late deaths. Two patients (6,5%) required reoperation due to right ventricle outflow tract obstruction, and no patient required re-intervention for re-coarctation. Nosocomial infection is the only one significant risk factor for hospital mortality (p=0,036). Coronary artery pattern, CoA and others anatomic lesions is not a risk factor for death by multivariable analysis. Conclusions: The mid-term outcome of single stage repair for TGA or Taussig-Bing anomaly combined with aortic arch anomaly can be performed with good results Keywords: Transposition of the great arteries, coarctation/aortic arch hypoplasia, ventricular septal defect, single stage repair.

Off-Pump Aortic Bypass for Hypoplastic Arch and Aortic Coarctation Via Anterolateral Thoracotomy

We report the case of a 21-year-old woman who was referred with uncontrolled hypertension. Computed tomography angiography revealed aortic arch hypoplasia and severe aortic coarctation. An off-pump ascending-to-descending aortic bypass surgery using synthetic graft was performed via left anterolateral thoracotomy. The patient recovered well and was discharged home uneventfully after five days. This procedure was performed without touching the head vessels or any collateral vessels. We consider this a safe and less invasive alternative technique for adult coarctation patients who have aortic hypoplasia or interrupted aorta.

Two-Stage Repair of Aortic Arch Hypoplasia Associated with Other Congenital Heart Defects in Newborns and Infants

Background. The optimal tactics of surgical treatment of aortic arch hypoplasia combined with other congenital heart defects remain controversial. In recent years, the leading clinics of the world prefer one-stage repair of such a combination, however, two-stage treatment is still used by cardiac surgeons and remains one of the variants of surgical tactics. The aim. To analyze immediate and long-term results of a two-stage repair of aortic arch hypoplasia with other con-genital heart defects in newborns and infants. Materials and methods. The study included 138 infants who were selected for a two-stage repair of aortic arch hy-poplasia with other congenital heart defects. There were 79 (57.2%) male and 59 (42.8%) female patients. The mean age of the patients was 1.4 ± 0.7 months, mean body weight was 4.1 ± 2.0 kg. The main method of the defect diagnosis and evaluation of immediate and long-term results was echocardiographic examination. The aortic arch segment was consid-ered hypoplastic if the Z-score deviation was less than –2.0. Results. The hospital mortality rate was 5.8% (n = 8). Two patients (1.4%) had delayed sternal closure in the early postoperative period. As a result of severe myocardial failure, two patients underwent extracorporeal membrane oxy-genation (ECMO). According to echocardiography before discharge, the mean pressure gradient at the site of aortic arch repair was 16 ± 6 mmHg. There was one death in the follow-up period. In 16 (11.5%) patients, aortic arch restenosis developed in the postoperative period. In total, 20 re-interventions were performed: 11 endovascular balloon dilatations and 9 surgical re-interventions. In the early postoperative period, aortopexia was performed in two patients (1.4%) due to compression of the left main bronchus. Conclusions. Two-stage repair of aortic arch hypoplasia with other congenital heart defects in newborns and infants is effective and safe treatment with good immediate and long-term results. This study shows that this surgical strategy can be effectively used in this complex pathology.

Reconstruction of the Aortic Arc through Left Thoracotomy in Patients with Isolated Coarctation and Aortic Arch Hypoplasia

Background. The choice of surgical approach for the reconstruction of aortic arch hypoplasia is a rather controversial issue. Traditionally, left lateral thoracotomy is used to correct aortic coarctation with hypoplasia of one or more arc seg-ments. However, in recent years, more and more clinics have begun widespread use of median sternotomy for the surgical treatment of aortic arch hypoplasia. The purpose of the research was to analyze the immediate and long-term results of surgical treatment of isolated hypoplasia of the aortic arch in infants through left thoracotomy. Materials and methods. From 2010 to 2019, 159 infants with coarctation and hypoplasia of one or more segments of the aortic arch underwent reconstruction of the aortic arch at the National Amosov Institute of Cardiovascular Surgery of the NAMS of Ukraine and Ukrainian Children’s Cardiac Center. The study included only patients with biventricular physiology and subsequent biventricular correction. There were 108 male patients (68%) and 51 female patients (32%). The mean age of the patients was 2.4 ± 0.9 months, the mean body weight was 4.7 ± 2.0 kg. The main method of diagnosis, i.e. determining the defect and assessing the immediate and long-term results, was echocardiographic examination. The study group consisted of 155 patients (97.5%) in whom the reconstruction of the aortic arch was performed through left thoracotomy, in the remaining 4 patients (2.5%) the intervention was performed through median sternotomy. Results. In-hospital mortality was 0.6% (n = 1). The average time of the operation was 126 ± 40 minutes, the aortic cross-clamp time was 24.7 ± 5.1 minutes. The mean duration of mechanical ventilation after the surgery was 41 ± 15.9 hours. The mean follow-up period ranged from 1 month to 13 years. There were no deaths in the long-term period. Ten (6.4%) patients developed aortic arch restenosis in the postoperative period. In 8 patients, the narrowing was treated by endovascular dilatation. Two patients underwent repeated aortic arch repair using median sternotomy. There were no cases of compression of the trachea, main bronchi, neurological complications in the follow-up period. Conclusions. Reconstruction of the aortic arch hypoplasia through left thoracotomy is an effective and safe treatment option in infants associated with good immediate and long-term results. The study indicates that this surgical strategy can be used in most patients with hypoplasia of the isthmus and distal aortic arch.