Contemporary surgical outcome and symptomatic relief following vascular ring surgery in children: effect of prenatal diagnosis.

OBJECTIVES: Our goal was to describe postoperative complications and outcomes in a large contemporary cohort of children with an isolated double aortic arch (DAA) or a right aortic arch (RAA) with left arterial ligament and to assess the impact of foetal diagnosis on outcomes. METHODS: We performed a retrospective analysis of all patients who underwent surgery for DAA or RAA with left arterial ligament between 2005 and 2019. RESULTS: A total of 132 children were operated on for a DAA (n = 77) or a RAA (n = 55). Prenatal diagnosis was made in 100/132 (75.8%). Median age at surgery for DAA was 5.0 (1.7-13.3) months and for RAA was 13.9 (6.4-20.1) months. There was no difference in the age at surgery between the prenatal and postnatal cases (8.6 [4.0-15.6] vs 5.4 months [1.8-17.7]; P = 0.37). No surgical deaths occurred. Vocal cord palsy was the most common complication, occurring in 12/132 (9%): of these, 11 resolved spontaneously and 1 required a temporary tracheostomy. Logistic regression demonstrated that older age at operation was the only predictor for a postoperative complication (P = 0.02). Overall, 21/67 (31%) of prenatally detected, symptomatic cases reported residual symptoms/signs 1 year after surgery compared to 18/28 (64%) of postnatally detected cases. Postnatal diagnosis was associated with persistent postoperative symptoms/signs [P = 0.006, odds ratio = 3.9 (95% confidence interval 1.5-9.4)]. CONCLUSIONS: Surgery to relieve a vascular ring resolves trache-oesophageal compressive symptoms in most cases, but parents/patients should be aware that symptoms/signs may persist in the first postoperative year despite effective release of the vascular ring. Earlier surgery and prenatal diagnosis may improve outcomes.

The Differential Diagnosis of Double Aortic Arch and Right Aortic Arch with Mirror-Image Branches in the Fetus: A Potential Novel Method.

The objective of this study was to explore a new method for the differential diagnosis between fetal double aortic arch (DAA) and right aortic arch with mirror-image branches (RAA-MB). Clinical data and prenatal echocardiographic features of the DAA (n = 22) and RAA-MB (n = 65) confirmed by postnatal or autopsy findings were analyzed retrospectively. The angles between the two aortic arches in the DAA group and between the right aortic arch and the mirror branch were measured. The differences between the two groups and differential diagnosis value of the angles were compared and analyzed based on the receiver operating characteristic curve. The proportion of left-sided ductal arteriosus (100%) was higher in the DAA group than that (32.3%) in the RAA-MB group, (P < 0.05). The proportion of conotruncal anomalies is higher in the RAA-MB group (64.6%) than in the DAA group (18.2%) (P < 0.05). There was a significant difference in the angles between the groups (DAA: 50.3° ± 8.3° vs. RAA-MB: 82.9° ± 13.8°) (P < 0.01). When the cutoff value was 62.8°, the sensitivity and specificity of the differential diagnosis were 95.5% and 96.9%, respectively. Distinguishing the angle measurement between DAA and RAA-MB is helpful in prenatal prognosis. We recommend a cutoff value of 62.8°.

Critical left coronary main trunk stenosis, chronic occluded right coronary artery, left subclavian artery occlusion, severe aortic regurgitation and porcelain aorta in a patient with aortitis.

BACKGROUND: Coronary involvement is rare but can be critical in patients with aortitis. Although cardiac ischemia can be resolved by coronary artery bypass grafting (CABG), patients complicated with cardiac ischemia, calcified aorta, and valve insufficiency pose difficult problems for surgeons. CASE PRESENTATION: A 71-year-old woman was referred to our institution because of unstable angina. She had been previously diagnosed with aortitis and left subclavian artery occlusion. Contrast-enhanced computed tomography revealed severe left coronary main trunk stenosis, right coronary artery occlusion, and porcelain aorta. Ultrasonic echocardiogram showed severe aortic regurgitation. We performed emergent coronary artery bypass grafting, aortic valve replacement and ascending aorta replacement under hypothermic circulatory arrest. CONCLUSIONS: The technique of circumferential calcified intimal removal and reinforcement with felt strips was effective for secure anastomosis. Unilateral cerebral perfusion from the right subclavian artery enabled good visualization and sufficient time to perform distal anastomosis.

Swing-back and trap-door technique repair for interrupted aortic arch with right-sided descending aorta.

A right-sided descending aorta with a left aortic arch is a rare congenital anomaly in which the aortic arch crosses the midline from the left side of the trachea coursing to the descending aorta in the right thoracic cavity. The surgical repair of an interrupted aortic arch with a right-sided descending aorta carries great risks of bronchial and oesophageal obstruction. Herein, we describe a case of successful surgical repair of an interrupted aortic arch with a right-sided descending aorta using the swing-back and trap-door techniques.

The python aortic arch.

Aortic arch anomalies are rare congenital cardiovascular malformations. We present a case of a 9-year-old asymptomatic boy with an unusual, unknown arch anomaly.

Antenatal Detection of Treatable Critical Congenital Heart Disease Is Associated with Lower Morbidity and Mortality.

OBJECTIVE: To establish the impact that timing of diagnosis and place of birth have on neonatal outcomes in those with readily treatable critical congenital heart disease. STUDY DESIGN: This was a population-based study with a complete national cohort of live-born infants with transposition of the great arteries and aortic arch obstruction in New Zealand between 2006 and 2014. Timing of diagnosis, place of birth, survival to surgery, in-hospital events, and neonatal mortality were reviewed. Live births with a gestation of ≥35 weeks and without associated major extracardiac anomalies were included for analysis. RESULTS: A total of 166 live-born infants with transposition of the great arteries and 87 with aortic arch obstruction were included. Antenatal detection increased from 32% in the first 3 years to 47% in the last 3 years (P = .05). During the same period, neonatal mortality decreased from 9% to 1% (P = .02). No deaths occurred after surgical intervention. An antenatal diagnosis was associated with decreased mortality (1/97 [1%] vs 11/156 [7%]; P = .03) and birth outside the surgical center was associated with increased risk of mortality (11/147 [7%] vs 1/106 [1%]; P = .02). Those with an antenatal diagnosis required fewer hours of mechanical ventilation (P = .02) and had shorter durations of hospital stay (P = .05) compared with those diagnosed >48 hours after birth. CONCLUSIONS: The mortality risk for transposition of the great arteries and critical aortic arch obstruction is greatest before cardiac surgery. Improved antenatal detection allowing delivery at a surgical center is associated with reduced mortality.

Cervical aortic arch: an unusual cause of a pulsatile neck mass.

A 72-year-old man presented with epistaxis on two occasions requiring admission. Prior to performing a bilateral sphenopalatine artery ligation diathermy, anaesthetic concern was raised regarding what the patient described as a 'congenital aortic aneurysm', with an overlying scar secondary to explorative surgery as a child. The abnormality was a cervical aortic arch (CAA). CAA is a rare vascular anomaly, which most commonly manifests as a pulsatile neck mass. In this case, we discuss the differential diagnosis for a pulsatile neck mass and considerations to be made in the workup. We also highlight the importance of cardiovascular risk factor management in patients with CAA.

An unusual case of interrupted cervical aortic arch associated with long segment coarctation of the descending thoracic aorta.

Interrupted aortic arch is a rare congenital anomaly in newborns and infants and is commonly associated with other cardiovascular anomalies. Here, we report an unusual case of type A interrupted cervical aortic arch associated with long segment coarctation of the descending thoracic aorta. Patent ductus arteriosus reconstituted the descending thoracic aorta. Proximal segments of the left common carotid and left subclavian arteries were atretic. Echocardiography-gated multidetector CT angiography not only identified the type of aortic arch interruption in the neonate but also delineated the exact anatomical details.

Correlation of Symptoms with Bronchoscopic Findings in Children with a Prenatal Diagnosis of a Right Aortic Arch and Left Arterial Duct.

A right aortic arch (RAA) with a left arterial duct (LAD) together encircle the trachea and have the potential to cause tracheobronchial compression and published guidelines recommend bronchoscopy in symptomatic patients. The aim of the study was to describe the incidence of tracheal compression in a cohort of prenatally diagnosed RAA and LAD. Retrospective review of clinical course and imaging of prenatal cases of RAA and LAD assessed with flexible bronchoscopy over an 11-year period. 34 cases of prenatally diagnosed RAA with LAD underwent bronchoscopy at median age of 9 months (range 0.4-123) of whom 11 had respiratory symptoms and 23 were asymptomatic. In the neonatal period, three cases demonstrated respiratory symptoms. An aberrant left subclavian artery (ALSA) was identified in 29 cases. Pulsatile tracheal compression was identified in 32/34 (94%) cases and two cases showed normal tracheal appearances. Significant tracheal compression (> 70% occlusion) was present in 25/34 (74%) cases of which 16 were asymptomatic. Significant carinal compression (> 70% occlusion) was identified in 14/34 (42%) cases, an ALSA was observed in 13/14. Surgical relief of a vascular ring has been performed in 27 (79%) cases at a median age of 15 months (range 0.6-128 months). At surgery, a fibrous remnant of an atretic left aortic arch was identified in 11/27 (41%) cases. Significant tracheal compression may be present in infants even without symptoms. If early relief of airway compression is to be achieved to promote normal development of tracheal cartilage, early bronchoscopy should be considered.

Surgical Relief of Left Ventricular Outflow Tract Obstruction in Adults With Congenital Aortic Stenosis and Associated Aortic Annulus Hypoplasia and/or Subaortic Obstruction.

BACKGROUND: In children and adolescents, a Ross/Konno operation is commonly done to both enlarge the aortic root and provide a competent aortic valve with relief of left ventricular outflow tract obstruction (LVOTO). Optimum management is not so straightforward in adults. METHODS: Between 1995 and 2014, 16 patients of mean age 39.4 years (18-57 years) with hypoplastic aortic annulus (AA) measuring 20mm and less, and mean aortic valve/LVOT gradient of 61mmHg (30-70mmHg) presented for surgery. RESULTS: Eight patients with mean LVOT/AA diameter 19.6mm (18-20mm) underwent an "inclusion-cylinder" type Ross procedure (RP). Eight patients with more severe LVOT/AA obstruction, with mean diameter of 17.4mm (16-19mm) underwent mechanical aortic valve replacement (AVR) with standard Konno-type aortoventriculoplasty. There was zero early and late mortality; with mean follow-up of 11.6 years (3-21 years) in the Ross group and 6 years (2-10 years) in the Konno-AVR group. One patient in the Konno-AVR group had reoperation after 2 years for RVOT obstruction. The postoperative echocardiograms of these patients at last follow-up show residual mean gradient across LVOT/AA of 4.4mmHg (2-6mmHg) after RP, and 11.9mmHg (8-17mmHg) after Konno-AVR. CONCLUSIONS: In adults, the "inclusion-cylinder" Ross-procedure is a good alternative for mild to moderate aortic root hypoplasia. However, for cases with severe LVOT obstruction, a Ross-Konno is not possible with the same method of autologous support used in a non-Konno RP, and this could be expected to have an impact on late durability and the need for further intervention, in a group that has already undergone multiple procedures in childhood. Both methods of RP and Konno-AVR lead to excellent early and late results.

Interrupted aortic arch complicated with takotsubo cardiomyopathy mimicking aortic dissection.

A 50-year-old man presented to the emergency department with interscapular pain, diaphoresis and restlessness. Initial examination raised the possibility of aortic dissection; however, the CT scan did not concur with the diagnosis. An ECG showed ST segment elevation in leads V1-V6 and echocardiography showed severe left ventricular systolic dysfunction. Coronary angiography through the right femoral artery was attempted but the diagnostic catheter could not be advanced to the ascending aorta. Radiocontrast injection showed complete obstruction of the descending aorta. Coronary angiography through right radial approach showed mild left anterior descending disease. The aortogram showed complete interruption of the ascending aorta with extensive collateral network. Left ventricle gram was consistent with stress-induced cardiomyopathy. We noticed intermittent confusion and agitation. MRI of the brain showed areas of deep white matter ischaemia as well as microhaemorrhages, suggesting posterior reversible leucoencephalopathy syndrome. He unfortunately went into cardiac arrest and could not be revived.

Surgical Repair of Cervical Aortic Arch With Brain Circulation Anomaly Through Clamshell Incision.

We report the successful surgical repair of a cervical aortic arch and diverticulum with a brain circulation anomaly through a clamshell incision. Because of the reliability of selective antegrade cerebral perfusion and superior exposure, we chose an approach through a clamshell incision. We describe the utility of this approach for treating a cervical aortic arch with a diverticulum.

Extra-anatomical bypass in complex and recurrent aortic coarctation and hypoplastic arch.

OBJECTIVES: Our goal was to report the selection schemes, technical variations and long-term outcome of extra-anatomical bypass to correct complex, recurrent aortic coarctation and hypoplastic aortic arch. METHODS: Between 1989 and 2012, 53 patients (mean age 13.2 ± 4.3, median 11.6, range 9-23 years) with complex aortic coarctation (n = 33; long-segment hypoplastic aortic arch in 15), recurrent coarctation (n = 20; anastomosic pseudoaneurysm in 10), underwent correction using extra-anatomical bypass, either with (n = 18: femoral bypass = 13, left heart bypass = 5) or without (n = 35) extracorporeal circulation via a left lateral thoracotomy (n= 48) and combined median sternotomy and median laparotomy (n = 5). The decision to use extracorporeal circulation was based on the anatomical location of the coarctation, the length of the hypoplasia and a history of previous repair. Preoperatively, mean systolic blood pressure was 130 ± 30 mmHg at rest and 180 ± 40 mmHg during exercise, with a mean pressure gradient of 80 ± 11.6 (range 40-120) mmHg. RESULTS: Various extra-anatomical bypass strategies included left subclavian artery to descending aorta (n = 38), ascending aorta to left subclavian artery (n = 3), ascending aorta to descending aorta (n = 4), aortic arch to descending aorta (n = 3) and ascending aorta to abdominal aorta (n = 5). Graft size (median 18, range 10-26, mm) was chosen according to the diameter of the vessel proximal and distal to the planned graft. No operative deaths, paraplegia or abdominal malperfusion occurred. The mean reduction in systolic blood pressure was 60 ± 25 mmHg without pressure gradients. During a mean follow-up of 18.3 ± 3.7 years, there were no reoperations, graft complications or pseudoaneurysm formation on anastomotic sites. Seven (11.6%) patients are on antihypertensive medications. No patient presented with claudication nor did anyone experience orthostatic problems from the steal phenomenon. CONCLUSIONS: Extra-anatomical bypass is safe, an effective technique, and achieves satisfactory long-term results.

A novel procedure for reconstructing an extensive hypoplastic aortic arch in older children.

Aortic arch reconstruction is the key to successfully repairing an interrupted aortic arch (IAA) with tubular hypoplasia of the aortic arch (THAA), especially in older children. We report a novel reconstruction technique involving aortapulmonary fusion that was used to treat THAA in a 9 year-old patient with IAA. In this procedure, the underside of the aortic arch and the upside of the main pulmonary artery were fused to reconstruct the aortic arch. The short-term outcome of the procedure has been promising. This procedure may represent an alternative for repairing extensive THAA in older children.

Palliation Outcomes of Neonates Born With Single-Ventricle Anomalies Associated With Aortic Arch Obstruction.

BACKGROUND: The two most common surgical strategies for the treatment of neonates born with single-ventricle anomalies associated with aortic arch obstruction are the Norwood operation and pulmonary artery banding plus coarctation repair (PAB+COA). We reviewed characteristics and outcomes of neonates who underwent those two surgical strategies at our institution. METHODS: Between 2002 and 2012, 94 neonates with a single ventricle and aortic arch obstruction (excluding hypoplastic left heart syndrome) underwent Norwood (n = 65) or PAB+COA (n = 29). Outcomes were parametrically modeled, and risk factors associated with early and late death were analyzed. RESULTS: Competing-risks analysis showed that, at 2 years after the operation, 24% of patients had died or received transplantation and 75% had undergone a Glenn shunt. At 5 years after the Glenn shunt, 10% of patients had died or received transplantation, 62% had undergone Fontan, and 28% were alive awaiting Fontan. Overall 8-year survival was 70%. Outcomes after Norwood included extracorporeal membrane oxygenation use in 9 (14%), unplanned reoperation in 13 (20%), hospital death in 10 (15%), and interstage death in 8 (12%), with 8-year survival of 66%. Outcomes after PAB+COA included extracorporeal membrane oxygenation use in 1 (3%), unplanned reoperation in 9 (30%), hospital death in 1 (3%), and interstage death in 3 (10%), with 8-year survival of 76%. There was an association trend between underlying anatomy and survival (hazard ratio [HR], 2.1; 95% confidence interval [CI], 0.9 to 4.7; p = 0.087). On multivariable analysis, factors associated with death were extracorporeal membrane oxygenation use (HR, 5.5; 95% CI, 1.9 to 15.9; p = 0.002), genetic syndromes/extracardiac anomalies (HR, 3.5; 95% CI, 1.5 to 8.2; p = 0.003), and weight of 2.5 kg or less (HR, 3.0; 95% CI, 1.3 to 7.2; p = 0.012). CONCLUSIONS: Anatomic and patient characteristics influence palliation outcomes in neonates born with single-ventricle anomalies associated with aortic arch obstruction. Although the Norwood operation is applicable in most of these patients, the PAB+COA strategy is a valid alternative in well-selected patients.

Three-dimensional printed prototypes refine the anatomy of post-modified Norwood-1 complex aortic arch obstruction and allow presurgical simulation of the repair.

Three-dimensional (3D) printed prototypes of malformed hearts have been used for education, communication, presurgical planning and simulation. We present a case of a 5-month old infant with complex obstruction at the neoaortic to transverse arch and descending aortic junction following the neonatal modified Norwood-1 procedure for hypoplastic left heart syndrome. Digital 3D models were created from a routine 64-slice CT dataset; then life-size solid and magnified hollow models were printed with a 3D printer. The solid model provided further insights into details of the anatomy, whereas the surgical approach and steps of the operation were simulated on the hollow model. Intraoperative assessment confirmed the anatomical accuracy of the 3D models. The operation was performed in accordance with preoperative simulation: sliding autologous flaps achieved relief of the obstruction without additional patching. Knowledge gained from the models fundamentally contributed to successful outcome and improved patient safety. This case study presents an effective use of 3D models in exploring complex spatial relationship at the aortic arch and in simulation-based planning of the operative procedure.