Subclavian Flap Research Articles

Impact of surgical strategy and postrepair transverse aortic arch size on late hypertension after coarctation repair during infancy

BackgroundLate hypertension (HTN) after coarctation of the aorta (CoA) repair contributes to higher morbidity and mortality. An association between transverse aortic arch (TAA) hypoplasia and HTN has been found, but its relationship with surgical strategy is unclear. We studied the association between late HTN and initial surgical strategy pertaining to the TAA. MethodsWe retrospectively reviewed patients who underwent surgical repair of CoA during infancy with at least 10 years of follow-up, excluding those with atypical coarctation, major associated heart defects, and residual isthmic narrowing. TAA diameter z-score immediately postrepair was measured as a marker of surgical strategy. Systemic HTN at latest follow-up was assessed using standard criteria. ResultsA total of 130 patients underwent surgical repair of CoA (76% via thoracotomy, 24% via sternotomy) with resection and end-to-end anastomosis (62%), extended end-to-end anastomosis (30%), subclavian flap (5%), or arch repair with patch (4%), at a median age of 14 days (interquartile range [IQR], 7-62 days). The median postrepair TAA diameter z-score was −2.04 (IQR, −2.69 to 1.24). At a mean follow-up of 17.3 years, 43 of the 130 patients (33%) developed HTN. After controlling for age at repair, sex, and presence of a genetic syndrome, HTN was not associated with immediate postrepair TAA diameter z-score (P = .41), type of surgical incision (P = .99), or type of surgical repair (P = .66). ConclusionsIn patients undergoing surgical repair of CoA during infancy, late HTN was not associated with immediate postrepair TAA size or surgical strategy pertaining to the TAA. These results suggest that factors other than surgical strategy, such as differential growth of the TAA during childhood, may be important.

The role of 3D technology in the practical education of congenital coarctation and its treatment—a feasibility pilot study

BackgroundCoarctation of the aorta (CoA) is a congenital disease with an incidence of 4 out of 10,000 live births, therefore proper education of its treatment is essential. Understanding the disease and the wide array of treatment options is often difficult. Additive manufacturing technology can be used to produce 3D printed hands-on surgical training tools (HOSTT), which can be used for the education and practical training of CoA. This study aimed to investigate the effectiveness of a 3D printable HOSTT for the simulation of coarctation surgery, and it’ possible role in practical education.MethodsParticipants were medical students of Semmelweis University between the second and sixth academic year. A virtual 3D model of an aorta with CoA was generated from a computed tomography angiography scan. Each participant received a 3D-printed aorta phantom and performed either one of four surgical treatment modalities. The simulated surgeries included end-to-end anastomosis, end-to-side anastomosis, prosthetic patch, and subclavian flap aortoplasty. Participants provided feedback, evaluating their understanding of the disease and its treatment by the four surgical reconstruction modalities on a seven-point Likert scale before and after the sessions.Results21 medical students participated in this study. Participants’ average rating of their understanding of CoA disease and it treatment options before practical training was 4.62 ± 1.07. After training, their average rating increased to 6.19 ± 1.08, showing statistically significant difference.ConclusionsWithin this study’s limitations, the applied HOSTT, manufactured using 3D printing, was effective for the practical training of CoA’s surgical treatment methods for medical students.

Aortic Coarctation Associated with Distal Aortic Arch Hypoplasia in Neonates Can Be Effectively Repaired through Left Thoracotomy

Objectives. Aortic coarctation in neonates remains a clinical challenge. Low weight, arch hypoplasia and ductal dependence have been identified as risk factors for recurrent coarctation. We hypothesized that a tailored surgical technique may prevent recurrence. Methods. Retrospective evaluation of neonates treated for coarctation through thoracotomy was done. No primary percutaneous procedure was performed and repairs through sternotomy were excluded. Aortic hypoplasia was defined as a ratio arch diameter (mm)/patient’s weight (kg) < 1. Extended end-to-end anastomosis (EEEA), subclavian flap (Waldhausen) and Amato aortoplasty were performed. Mortality and recurrent obstruction requiring re-intervention were assessed. Results. Records of 340 consecutive patients (2003–2019) were analyzed. Preoperative median age and weight were, respectively, 10 days (1–30) and 3080 grams (1400–5180). Arch hypoplasia was documented in 31 patients (9.1%). Prostaglandin was infused in 220 (65.3%). Critical preoperative status was documented in 35 (10.8%). EEEA repair was performed in 273 (80.3%), Waldhausen was performed in 42 (12.4%), and Amato was performed in 25 (7.4%). The last two were more likely to be performed in the presence of arch hypoplasia ( p < 0.0001). Hospital mortality occurred in 2 patients (0.6%). Thirty-six procedures (31 percutaneous/5 surgical) were performed for recurrent arch obstruction in 33 patients. Three late deaths occurred. Low-weight, hypoplastic arch, and ductal dependency did not influence the outcome. All survivors were free from residual coarctation at a mean follow-up of 3.6 ± 3.4 years postoperatively. Conclusions. Surgical repair remains the procedure of choice for neonatal coarctation. A tailored approach using alternative techniques seemed to offer comparable results even in presence of associated risk factors.

From Crafoord’s End-to-End Anastomosis Approach to Percutaneous Interventions: Coarctation of the Aorta Management Strategies and Reinterventions

First described in 1760 by the anatomist Morgagni, coarctation of the aorta (CoA) is a congenital heart defect characterized by narrowing the aorta, typically distal to the left subclavian artery. It accounts for approximately 5–8% of all congenital heart diseases, with an incidence estimated at 4 per 10,000 live births. In 1944, the Swedish surgeon Clarence Crafoord achieved the first successful surgical CoA repair by performing an aortic end-to-end anastomosis on two patients aged 12 and 27 years old. Presently, the most prevalent techniques for surgical repair, particularly in infants and neonates with isolated coarctation, involve resection with end-to-end anastomosis (EEA) and the modified Crafoord technique (extended resection with end-to-end anastomosis (EEEA)). Subclavian flap aortoplasty (SCAP) is an alternative surgical option for CoA repair in patients under two years of age. In cases where the stenosis extends beyond resection and end-to-end anastomosis feasibility, patch aortoplasty (PP) employing a prosthetic patch can augment the stenotic region, especially for older patients. Despite advances in pediatric cardiology and cardiac surgery, recoarctation remains a significant concern after surgical or interventional repair. This comprehensive review aims to provide a thorough analysis of coarctation management, covering the pioneering techniques introduced by Crafoord using end-to-end anastomosis and now extending to the contemporary era marked by percutaneous interventions as well as the recoarctation rate associated with each type.

Coarctation Repair in an Adult Funambulist (Dombari)

Five to seven percent of all congenital cardiac disorders are caused by aortic coarctation. Funambulism or tight rope walking is a part of street circus acts earning livelihood for Dombari community, which requires utmost upper and lower limb strength and balance. Coarctation can be repaired surgically or with interventional approach. End-to-end anastomosis, subclavian flap aortoplasty, and extra-anatomic bypass grafts are a few surgical fixes. However, these approaches are not without risks involving the subclavian artery and spinal arteries thus putting limbs in jeopardy. Interventional procedures include balloon angioplasty and use of bare metal or covered stents but are associated with complications like repeated interventions or surgery, migration of stents compromising branches of aorta, thereby causing limb paresis or plegia, and are not very cost effective. The authors hereby discuss a cost-effective surgical management and spinal protection strategy for coarctation in a funambulist patient.

ABNORMAL INTER-ARM SYATOLIC BLOOD PRESSURE DIFFERENCE IN ADULTS WITH CONGENITAL HEART DISEASE

Objective: Many papers have been reported that hypertension and cardiovascular disease are high frequency in adults with congenital heart disease. It is well known that significant inter-arm blood pressure difference can predict cardiovascular risk. We investigate the inter-arm blood pressure difference in adults with congenital heart disease. Design and method: We enrolled 100 consecutive subjects aged 20 years or more, who visited the Department of Adult Congenital Heart Disease, Chiba Cardiovascular Center. The exclusion criteria were history of arrhythmia, which makes accurate pulse wave analysis impossible and history of cardiac operation within 6 months. The patients were classified into 3 groups. The patients in group A had undergone surgery, which sacrifice subclavian arteries (subclavian flap method and original Blalock-Taussig shunt). The patients in group B had undergone surgery to their subclavian arteries, which conserve their subclavian arteries (modified Blalock- Taussig shunt). The patients in group C had not undergone surgery to their subclavian arteries. The bilateral brachial blood pressures were simultaneously measured using form PWV/ABI (Colin, Komaki, Japan). The inter-arm systolic blood pressure difference greater than 10mmHg was defined as significant inter-arm systolic blood pressure difference. Results: Patients’ age was 36.6±14.9 years. Mean difference between bilateral brachial systolic blood pressures was 8.2±13.6 mmHg. Twenty-one patients demonstrated the inter-arm systolic blood pressure difference (29%). Nine patients were classified as group A and all patients in group A demonstrated inter-arm systolic blood pressure difference (29.1±35.6mmHg). Sixteen patients were classified as group B and 9 patients had undergone modified Blalock-Taussig shunt bilaterally. Four patients (44%) in group B demonstrated inter-arm systolic blood pressure difference (bilateral; 3, unilateral: 1). In 75 group C patients, 8 patients (11%) demonstrated inter-arm systolic blood pressure difference. There was no difference between group C patients in with and without the inter-arm systolic blood pressure difference in body mass index, hemoglobin A1 level, lipid profile. Conclusions: The significant inter-arm systolic blood pressure difference in adults with congenital heart disease is not uncommon. The intervention can strongly influence on their inter-arm systolic blood pressure difference.

Outcomes of surgery for coarctation of the aorta based on a new classification system.

To evaluate early- and long-term outcomes of the surgical treatment for coarctation of the aorta based on a new classification system. A retrospective clinical review of 111 patients with coarctation of the aorta who underwent surgery (March 2011 to August 2020) was performed. We categorised coarctation of the aorta into type I, with all three head vessels tightly packed; type II, with the left subclavian artery separated from the two other head vessels; and type III, with all three head vessels separated from one another. Each type included subtype a, with a short isthmic portion, and subtype b, with a long isthmic portion. The median patient age and weight at operation were 8 (range, 1-1490) days and 3.2 (range, 1.9-18.5) kg, respectively. Extended end-to-end anastomosis was performed via sternotomy in 54, via thoracotomy in 12, end-to-side anastomosis in 31, autologous main pulmonary artery patch augmentation in 12, and modified end-to-end anastomosis combined with subclavian artery flap aortoplasty in two patients. There was one (0.9%) case of early mortality and 12 (10.8%) cases of post-operative complications. Two (1.8%) late deaths occurred during follow-up. Five (4.5%) patients underwent balloon dilatation and three (2.7%) underwent reoperation for restenosis of coarctation of the aorta. All patients with type Ia (21 patients, 18.9%) underwent extended end-to-end anastomosis via sternotomy or thoracotomy. According to the early and late outcomes observed in this study, surgical treatment of coarctation of the aorta using the new classification system could be safe and low risk.

Surgical Experience With Nine Cases of Obstructed Right Aortic Arch.

Right-sided aortic arch obstruction is an extremely rare congenital anomaly. A variety of surgical approaches have been described. This study reviews our institutional experience over the last 30 years. Our surgical database at the University of Alabama at Birmingham and Children's Hospital of Alabama from 1992 to 2022 was reviewed to include all patients who underwent surgical repair for right-sided aortic arch obstruction. A total of nine patients underwent surgical repair for right-sided aortic arch obstruction. Surgical approach was via thoracotomy (n = 2, 22%), sternotomy (n = 5, 56%), or combined (n = 2, 22%). Primary extended end-to-end anastomosis was utilized for patients with discrete coarctation (n = 1, 11%), reverse subclavian flap for coarctation with associated distal arch hypoplasia (n = 2, 22%), GORE-TEX® tube graft for circumflex aorta (n = 1, 11%), and aortic arch advancement (n = 5, 56%) with or without patch augmentation for those with an interrupted or severely hypoplastic aortic arch. Reintervention was required in one patient (11%) for recoarctation. All patients were discharged in good condition. There was no hospital mortality and at 10.5 years (mean) follow-up there was one late death. Right aortic arch obstruction is a rare entity. Surgical approach should be tailored to the anatomy and associated intracardiac defects. Preoperative imaging with a CT angiogram is useful for operative planning. Sternotomy with single-stage primary repair is safe, effective, and our preferred surgical approach for patients with right aortic arch obstruction and associated intracardiac pathology.

Abstract 15015: Long Term Outcomes After Interrupted Aortic Arch Repair: A Study From the Pediatric Cardiac Care Consortium

Introduction: Interrupted aortic arch (IAA) is a congenital heart disease that can be successfully repaired. Children are at risk of multiple procedures and residual abnormalities. We used the Pediatric Cardiac Care Consortium (PCCC) to describe post-repair survival of patients with IAA. Methods: We queried the PCCC for patients receiving IAA repair 1982-2003. Death was ascertained from the PCCC and US National Death Index (NDI) to December 2019. Kaplan Meier (KM) plots and multivariable analysis assessed overall long-term survival and baseline characteristics associated with increased hazard of death post-repair. Results: We identified 380 patients (male 200; female 180) with IAA repair (excluding truncus arteriosus). Most had end-to-end anastomosis (n=234, 83.6%), 87 (31.1%) by interposition graft, and 47 (16.78%) by subclavian or carotid flap. There was no data for 11 patients. A total of 280 survived to discharge. Over a median of 22.9 years (IQR 19.78-27.52 years), 39 died. The 25-year survival post-discharge after repair was 86% (Figure 1). Type B2 IAA (aberrant right subclavian artery) was associated with increased death (aHR=1.814; 95% CI=0.891-3.694) versus non-type B IAA and cases with normal subclavians (A1+B1)) after adjusting for era, sex, presence of chromosomal anomalies, and type of surgery. Interposition graft was associated with increased hazard versus end-to end anastomosis (aHR=1.942; 95% CI=0.946-3.988) or vascular flaps (aHR=1.144; 95% CI=0.456-2.870). Other risk factors were female sex and chromosomal anomalies (DiGeorge syndrome primarily) (aHR = 1.948; 95% CI=0.946-4.011 and aHR=1.090; 95% CI=0.429-2.770). Conclusions: Patients with repaired IAA have decreased long-term survival. Female sex, chromosomal anomalies, type B IAA, aberrant right subclavian artery, and interposition graft were risk factors for poor long-term outcomes. Additional analysis of risk factors for long-term outcomes and causes of death is underway.

Late Aortic Aneurysm Following Subclavian Flap Aortoplasty for Coarctation of the Aorta: A Report of Two Adult Cases

Late Aortic Aneurysm Following Subclavian Flap Aortoplasty for Coarctation of the Aorta: A Report of Two Adult Cases

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

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. 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. 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. Planning surgical treatment according to the characteristics of the patients and accompanying anomalies may improve the results.

Tailored surgical technique for neonatal coarctation repair through thoracotomy

Aortic coarctation in neonate continues to pose a clinical challenge. Low weight, arch hypoplasia and ductal dependence have been identified as risk factors for recurrent coarctation. We hypothesized that a tailored surgical technique may prevent this complication. Retrospective evaluation of neonates who presented for coarctation repaired through thoracotomy was conducted. No primary percutaneous procedure was performed and sternotomy repairs were excluded. Hypoplasia was defined as a ratio of echocardiographic and perioperative distal arch diameter (mm)/weight (kg) < 1. Traditional extended end-to-end anastomosis (EEE), subclavian flap (Waldhausen) and double flap distal arch enlargement by Amato aortoplasty were employed. Mortality and recoarctation requiring reintervention in follow-up were assessed. Records of 340 patients (2003–2019) were analyzed. Median age and weight at repair were 10 [2–30] days and 3080 [1100–5200] g, respectively. Arch hypoplasia was documented in 31 patients (9.1%). Prostaglandin was infused in 220 (65.3%), preoperative mechanical ventilation and inotropic support was documented in 35 (10.8%). EEE repair was performed in 273 (80.3%). Waldhausen in 42 (12.4%) and Amato in 25 (7.4%) were more likely to be performed in presence of arch hypoplasia ( P < 0.0001). Concomitant PAB for associated VSD was necessary in 72 (21.2%). Hospital mortality occurred in 2 (0.58%). Thirty-six procedures (31 percutaneous, 5 surgical) for recoarctation were performed in 33 patients in a median delay of 111 [26–3050] days. One late death occurred. Both low weight ( P = 0.14), hypoplastic arch ( P = 0.65) and ductal dependency ( P = 0.34) did not influence outcome. At 3.6 ± 3.4 years postoperatively, all survivors presented with no residual coarctation. Primary surgical repair for neonatal coarctation still appears to be the gold standard. Even in presence of associated risk factors, a tailored approach using alternative techniques seems to offer comparable results in neonatal coarctation repair.

Clinical Outcomes of Extrapleural Closure of a Patent Ductus Arteriosus Concomitant with Aortic Coarctation Repair.

The aim of this study was to present an extrapleural approach for the closure of patent ductus arteriosus (PDA), with the repair of aortic coarctation (CoA) in the same session, in critically ill newborns and infants as an alternative to the transpleural surgical technique. Between December 2007 and November 2010, 44 critically ill patients with PDA and coarctation of the aorta were operated on during the same session with the extrapleural approach. The diagnoses of the patients were made by transthoracic echocardiography (TTE). We investigated the aortic arch, the length of the coarctation segment, peak-to-peak gradients, the aortic valve, and intracardiac defects prior to the surgery using TTE. Cardiac angiography was performed to determine whether the patients were suitable for an interventional approach in hemodynamically stable patients. Twenty-eight patients had congestive heart failure with mild to moderate pulmonary and systemic hypertension. The median gestational age and weight of neonates were 2.1 kg (range: 1.4 to 2.9 kg), and 31.4 weeks (range, 28.6 to 37 weeks), respectively. During the operations, PDA was closed using double clips. Resection of coarctation with an extended end-to-end anastomosis was performed in 27 patients. Subclavian flap angioplasty was performed in four patients, and an aortic patch repair was performed in two infants. Postoperative PDA flow and residual aortic gradient were evaluated using echocardiography prior to discharge from the hospital and during the follow-up period. There were three in-hospital deaths (6.8%). During the follow-up period, two patients died (4.8%). The mean follow-up period was 48.3±21.5 months (range: 29-56 months). Patent foramen ovale, atrial septal defect, and ventricular septal defect were the additional cardiac pathologies. These were hemodynamically insignificant. We detected that the intracardiac defects closed spontaneously. During the follow-up period, recoarctation developed in six patients (20%). We found that the risk factors for recoarctation in patients were to have a gradient from coarctation area, which was higher than ≥ 50 mmHg, and the length of coarctation segment that was longer than 1 cm in their first operation (P = 0.033). The median time from the first surgery to recoarctation was 25.4±13.2 months (range: 16-36 months). Balloon dilatation was performed in four patients. We performed redo-surgery in the remaining two patients with recoarctation. The mean intubation time was 9.1±13.4 hours (range: 5.8-19.8 hours). Transthoracic echocardiography showed normal left ventricular dimensions and systolic function in 34 patients during follow up (87.1%). Our experiences show that surgical repair of aortic coarctation and PDA closure at the same session may be performed safely and with acceptable mortality and morbidity via an extrapleural approach. Interventional approach as a less invasive method may be used in patients who have developed recoarctation.

Does age at aortic coarctation repair have an impact on left ventricle size and function?

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Adults with repaired aortic coarctation (CoA) require lifelong follow-up due to late complications, including left ventricular (LV) myocardial dysfunction. Age at the time of CoA repair is an important prognostic factor in these patients (pts). Purpose To evaluate LV size, ejection fraction (EF) and global longitudinal strain (GLS) values using 2D speckle tracking echocardiography (STE) in a population of adult pts with repaired CoA and to assess the relationship between these echocardiographic parameters and age at the time of CoA repair. Methods Retrospective analysis of adult pts with repaired CoA, followed in a Grown Up Congenital Heart Disease Centre. Pts with hemodynamically significant concomitant cardiac lesions were ruled out. Epidemiologic and clinical data were obtained from clinical records. Transthoracic echocardiograms were reviewed in order to assess GLS using 2DSTE (Echopac Software, GE). Results The study population consisted of 63 pts (61.9% male), with a mean age of 35.3 years at the time of the echocardiographic evaluation. The mean age at the time of the CoA repair was 117 months (95% CI 89.8-144.1 months). Surgical repair was performed in 46 pts (73%): resection with subclavian artery flap aortoplasty (n = 21); patch aortoplasty (n = 15) and head-to-head anastomosis (n = 10). In 10 pts there was no data regarding the type of surgical repair. Seven pts (11.1%) were submitted to percutaneous intervention (6 with aortic stent implantation and 1 with balloon aortic angioplasty). Mean LVEF was 63.4% (CI 95% 55.6 – 71.2%) and mean LV end-diastolic diameter (LVEDD) was 50mm (CI 95% 43-57mm). Mean GLS was - 17.3 (CI 95% 14.8- 19.8), which is inferior to the mean normal values reported for the software used. Age at the time of CoA repair had a statistically significant positive linear relationship with LVEDD (r= 0.282; p= 0.026) and a linear negative relationship with both GLS (r= -0,29; p= 0.022) and LVEF (r= -0.33; p= 0.05). Conclusion Older age at the time of CoA repair was associated with increased LVEDD and decreased GLS and LVEF. Also, GLS may be an important tool for the identification of subclinical LV dysfunction in adult pts with repaired CoA.

Isthmus Flap Aortoplasty for Repair of Long-segment Coarctation of the Aorta

Although resection with end-to-end anastomosis is currently the standard approach in the patients with coarctation of the aorta, repair for a long and narrow isthmus has been a surgical challenge. Herein, we describe a novel surgical technique including aortic isthmus reconstruction that allows for direct anastomosis without the use of foreign materials or tension-relieving procedures such as subclavian flap aortoplasty. Although resection with end-to-end anastomosis is currently the standard approach in the patients with coarctation of the aorta, repair for a long and narrow isthmus has been a surgical challenge. Herein, we describe a novel surgical technique including aortic isthmus reconstruction that allows for direct anastomosis without the use of foreign materials or tension-relieving procedures such as subclavian flap aortoplasty. Commentary: Isthmus Flapping – Another Tool in the Box for CoarctationOperative Techniques in Thoracic and Cardiovascular SurgeryVol. 26Issue 3PreviewMiyahara et al. provide a clear description of a novel all native tissue solution to the problem of coarctation associated with long segment isthmus hypoplasia that preserves the left subclavian artery.1 I won't comment further on the issue of on-pump via sternotomy versus an off-pump left chest approach with a suitable ductus arteriosus. The flap technique is a useful to addition to the many tools and tricks we already employ for various coarctation morphologies and demands no complicated technical maneuvers. Full-Text PDF

Post-operative musculoskeletal outcomes in patients with coarctation of the aorta following different surgical approaches

BackgroundThe aim of this study was to examine range of motion and muscle function in the upper extremity and spine in patients with coarctation of the aorta (CoA) comparing different surgical approaches. MethodsFrom October 2017 to February 2019, 150 patients were assessed for inclusion. A total of 99 patients (n = 75 CoA, n = 24 control), were included and assessed regarding muscle function, arm length and circumference, and spinal and thoracic mobility. ResultsThere were significant differences between the right and left arm in patients with CoA, operated with the subclavian flap technique compared to controls in regards to shoulder flexion (p < 0.001), elbow flexion (p = 0.001), shoulder abduction (p = 0.02), handgrip strength (p = 0.01), length of upper arm (p < 0.001), lower arm (p < 0.001), and of whole arm (p < 0.001), circumference regarding upper arm (p = 0.001), lower arm (p < 0.001), and wrist (p < 0.001). Structural scoliosis was more frequent in patients who had undergone thoracotomy (25.4%) than patients who had not undergone a thoracotomy (5.9%, p = 0.04), and were often located in the thoracic part of the spine. ConclusionPatients with CoA operated on using the subclavian flap technique have impaired muscle function as well as reduced arm length and circumference. An increased rate of structural scoliosis was found in patients who underwent thoracotomy, in comparison with patients who had not undergone a thoracotomy. Further research is needed to determine whether muscle function impaired by surgical procedures can be improved with exercise.

Left arm structure and function late after subclavian flap repair of aortic coarctation in childhood.

Concerns exist over the long-term consequences of subclavian artery ligation in subclavian flap repair for coarctation of the aorta. We sought to analyse upper limb structural and functional performance in adults who have had surgery in childhood for coarctation of the aorta, using either subclavian flap repair or end to end aortic anastomosis. Two-group observational design using anatomical and upper limb functional performance measures. Purposive sampling from our specialist adult congenital heart disease database of patients who received subclavian flap repair or end to end anastomosis for coarctation of the aorta as children. Upper limb measurements were completed using MRI and blood flow velocity with ultrasound imaging. Bilateral standardised upper limb functional testing of assessment of strength, dexterity and a standardised self-report of upper limb disability was completed. Eighteen right-handed patients, 9 with subclavian repair, (38 ± 12 years, 78% males) were studied. Age at repair was 4.7 ± 5.9 years; mean time from initial repair 32 ± 9 years. The subclavian group had a larger difference between right and left when compared the end to end anastomosis group in: lower arm muscle mass (94.5 ± 42.3 mls versus 37.8 ± 94.5 mls, p = 0.008), lower arm maximal cross-sectional area, (5.9 ± 2.8 cm2 versus 2.9 ± 2.6 cm2, p = 0.038) and grip strength (14.7 ± 8.3 lbs versus 5.9 ± 5.3 lbs, p = 0.016) There were no significant functional differences between groups. In adults with repaired coarctation of the aorta, those with subclavian flap repair had a greater right to left arm muscle mass and grip strength differential when compared to those with end to end anastomosis repair.

Long-term outcomes following repair of truncus arteriosus and interrupted aortic arch.

We aim to evaluate the long-term outcomes following repair of truncus arteriosus with an interrupted aortic arch. We reviewed all children (n = 24) who underwent repair of truncus arteriosus and an interrupted aortic arch between 1979 and 2018 in a single institution. The morphology of the interrupted aortic arch was type A in 5, type B in 18 and type C in 1. The median age at repair was 10 days and the median weight was 3.1 kg. Direct end-to-side anastomosis of the ascending and descending aorta was performed in 16 patients (67%, 16/24), patch augmentation in 5 patients (21%, 5/24) and direct anastomosis with the use of an interposition graft to the descending aorta in 2 patients (8%, 2/24). One patient, the first in the series, underwent interrupted aortic arch repair via subclavian flap aortoplasty prior to truncus repair. A period of deep hypothermic circulatory arrest was used in 16 patients, and isolated cerebral perfusion was used in 8 patients. The early mortality rate was 17% (4 out of 24 patients). There were no late deaths and overall survival was 83 ± 8% [95% confidence interval (CI) 61-93] at 20 years. Freedom from any reoperation was 33 ± 11% (95% CI 14-54) at 5 years and 13 ± 9% (95% CI 2-34) at 10 years. Six patients underwent 10 aortic reoperations. Freedom from aortic arch reoperation was 69 ± 11% (95% CI 42-85) at 10 and 20 years. Follow-up was 95% complete (19/20), with a median follow-up time of 20 years. At last follow-up, no clinically significant aortic arch obstruction was identified in any patient, and all patients were in New York Heart Association Class I/II. Repair of truncus arteriosus with an interrupted aortic arch with direct end-to-side anastomosis results in good survival beyond hospital discharge. Although the long-term functional state of patients is good, reoperation rates are high.

Increased aortic wall stiffness is predictive of aortic dilation in adult patients following coarctation of the aorta repair

Patients after repaired coarctation of the aorta are at risk for dilation and dissection of both the ascending and descending aorta. Histologic abnormalities seen are indistinguishable from patterns seen with Marfan syndrome, and lead to a stiff aortic wall, characterized by decreased distensibility, strain, and increased B-stiffness. We sought to analyze the relationship of aortic wall stiffness measured by cardiac MRI to aortic dilation following coarctation of the aorta repair. Fifty-two adult patients (26 females) with history of previous coarctation of the aorta repair who had undergone a recent cardiac MRI were identified. Studies and charts were reviewed retrospectively. Aortic strain, distensibility, and B-stiffness values were measured at level of ascending aorta and descending aorta. Relationships between measurements of aortic stiffness were compared to aortic dimensions. Median age at coarctation repair was 0.2 years (range 0.1–36 years). Types of repair included subclavian artery flap (44%), end-to-end anastomosis (33%), and patch angioplasty (15%). Time from repair to cardiac MRI was 25 years (range 1–42). By cardiac MRI, 38% had a dilated ascending aorta defined as a Z-score of >2.5. On average the ascending aorta was stiffer than the descending aorta for all three parameters. For both the ascending and descending aorta the 3 parameters of aortic stiffness were predictive of increasing aortic dimensions (P value < 0.01). By univariate analysis, risk factors for a dilated ascending aorta defined as a Z-score of >2.5 included older age at the time of initial repair, presence of a bicuspid aortic valve, and worse parameters of ascending aorta stiffness: % strain, distensibility, and B-stiffness, and a lower descending aorta % strain. By multivariate analysis, older age at initial repair and both a lower % strain at both the ascending and descending aorta remained statistically significant.

Short and midterm results of surgical treatment of interrupted aortic arch

Objective: To analyze the early and midterm results of surgical treatment of interrupted aortic arch (IAA) with double-ventricular procedure. Methods: The data of the 68 cases with the main diagnosis of IAA with biventricular structure from June 2009 to June 2017 at Department of Cardiac Surgery, Guangdong General Hospital was collected, including 46 cases of type A, 22 cases of type B. There was no type C case. Except for 5 cases without patent ductus arteriosus or other intracardiac malformations, the remaining 63 cases were combined with intracardiac deformity. The age of operation was 8 days to 18 years, including 28 cases(41.2%) of newborns, 32 cases(47.1%) within 1 year old, 6 cases(8.8%) from 1 to 15 years old, and 2 cases(2.9%) above 15 years old. Of the 5 patients with no intracardiac malformations, 4 patients were treated with a left-posterior thoracic lateral incision for primary surgical correction with end-to-side anastomosis, and 1 adult patient was treated with a median sternotomy incision to complete the anastomosis with the assist of extracorporeal circulation. Of the 63 patients with intracardiac malformation, 2 underwent reconstruction of the aortic arch via posterolateral left thoracic incision merely; 2 patients underwent staged surgery to correction the IAA and intracardiac malformation; the remaining 59 patients underwent the one-stage procedure of IAA and intracardiac malformation correction with deep hypothermic circulatory arrest and 43 cases of selective cerebral perfusion. A total of 55 cases of end-to-end or end-to-side anastomosis were performed in all aortic arch reconstruction. There were 6 cases of connection the arch to descending aorta with autogenous pulmonary artery conduit, and 1 case with homemade bovine pericardial conduit. And there were 5 cases of anastomosis enlargement with autogenous pulmonary artery patch, and 1 case with left subclavian artery flap patch. There were 1 case performed with D. K.S. operation for the severe hypogenetic aortic valve and ascending aorta, and 1 case with left ventricular double outlet channel operation for the severe subvalvular aortic stenosis. Results: Follow-up to 9 years, 4 deaths (5.9%) related to circulatory system diseases: 3 cases died within 30 days after surgery (1 case was neonatal, 2 cases were 1 to 12 months old); one died 8 months after surgery for low cardiac output syndrome. In the early postoperative period, 11 cases (16.2%) of residual aortic arch obstruction (pressure gradient >30 mmHg) were observed, of which 3 cases were moderate (4.4%); follow-up to 5 years after operation was residual obstruction in 3 cases and moderate in 2 cases. There were 3 cases with aortic valve and subvalvular obstruction (differential pressure >30 mmHg), 3 cases were followed up to 5 years after aortic valve and subvalvular obstruction, and 2 cases were moderate or above. To date, 5 patients have undergone 6 reoperations of the cardiovascular system: 3 cases because of pulmonary artery stenosis, 2 cases because of severe aortic arch stenosis, and 1 case because of aortic subvalvular obstruction. Conclusions: One-stage surgical repair of IAA is safe and clinically effective in the neonatal and infant. But some patients still requires reoperation for re-obstruction from subvalvular or aorta arch. For some patients, the choice of aortic arch reconstruction depends on the specific situation.