Hybrid repair of aberrant right subclavian artery with aortic dissection caused by Kommerell diverticulum.

BACKGROUND: Aberrant right subclavian artery (ARSA) with associated Kommerell diverticulum (KD) is a rare congenital aortic disease. KD patients have a high risk of rupture, dissection, and compression of adjacent structures. Although several treatment options have been proposed (traditional surgery, hybrid operation, and endovascular intervention), a consensus regarding optimal surgical management has not yet been established. CASE PRESENTATION: A case of successful hybrid repair of distal aortic arch dissection aneurysm by dissecting KD and ARSA with debranching of right and left common carotid arteries, left subclavian artery, and stent grafting was presented. CONCLUSIONS: The hybrid operation is suitable for elderly patients or those with high risks. Along with intervention, the hybrid operation needs to be developed as a minimally invasive method.

Selective Lesser Curvature Augmentation With Geometric Study for Repair of Aortic Arch Obstruction.

BACKGROUND: We repaired aortic coarctation and interrupted aortic arch with extended end-to-end anastomosis (EAA) through median sternotomy and performed lesser curvature augmentation with a pulmonary autograft patch (PAP) in selected patients with a long gap between anastomotic sites. We reviewed these outcomes and geometric implications. METHODS: All neonates and infants with biventricular morphology who underwent aortic arch reconstruction through median sternotomy between 2005 and 2019 were evaluated. Aortic arch geometry was analyzed with computed tomography routinely performed before and after surgery from 2009 on. RESULTS: There were 91 consecutive patients (median age, 1.2 months). Ten patients received PAP. One early death and no late deaths were noted. Overall survival was 98.9% at 10 years. Two left bronchomalacia and 1 recoarctation occurred in patients with EAA. Freedom from recoarctation was 97.4% at 10 years. We examined 68 patients with computed tomography. We used PAP in patients with a significantly longer gap between anastomotic sites indexed by the square root of the body surface area; its cutoff value was 29.0 mm/m (area under the curve, 0.86 mm/m). The PAP created a significantly greater arch angle (median, 91° versus 83°) and arch/descending diameter ratio (median, 1.2 versus 1.0) and preserved the arch width indexed by the square root of the body surface area (median, before surgery: 35.7 versus 34.4 mm/m; after surgery: 36.5 versus 29.9mm/m), compared with EAA. CONCLUSIONS: Aortic arch reconstruction with the current combined strategy provides satisfactory outcomes. Guided by geometric analysis, lesser curvature augmentation can be applied to patients who might experience recoarctation or airway compression with a directly anastomosed aortic arch.

Double aortic arch with atretic left arch distal to the origin of left subclavian artery accompanied by dyspnea: A case report and literature review.

Double aortic arch with atretic left arch distal to the origin of left subclavian artery is a rare type of vascular ring, and it can be easily confused with the right aortic arch with mirror branching. We provided a rare case of a 10-month-old infant with dyspnea. Echocardiography showed a suspicious double aortic arch with atretic left arch distal to the origin of left subclavian artery, which was confirmed intra-operatively. We summarize ultrasonic image characteristics of the disease and combine it with computed tomography angiography, bronchoscopy, and clinical symptoms in order to improve the detection rate and treatment strategy.

Aortic atresia and interrupted aortic arch communicating through external carotid anastomosis.

We describe the case of a newborn girl who displayed association of aortic atresia and interrupted aortic arch, with retrograde flow in ascending aorta, through extracranial anastomoses between vertebral arteries (arisen from descending aorta) and external carotids.

Autologous Tissue Reconstruction of Interrupted Aortic Arch Using Aberrant Subclavian Artery.

Reconstruction of the aortic arch in type B interruption requires extensive mobilization of the descending aorta and the proximal branches of the arch to perform a tension-free anastomosis. The association of a coexistent type II aortopulmonary window and an aberrant subclavian artery reduces the degree of mobility that can be achieved by dissection alone, and it usually entails sacrifice of the aberrant artery to achieve satisfactory mobilization. We report a novel technique to use the aberrant subclavian artery as autologous tissue in the reconstruction of the aortic arch for repair of type B interruption associated with type II aortopulmonary window.

How Could Aortic Atresia With Interrupted Aortic Arch Survive? About a Neonatal Repair on Two Ventricles.

Aortic atresia with interrupted aortic arch (IAA) is an exceptional condition. In absence of associated lesion, the flow to the brain and the retrograde flow to the coronary arteries can only come from vessels on the descending aorta. This flow needs to be large enough to supply the brain and the myocardium. The only IAA type compatible with survival is type C where the flow from descending aorta could be insured by the left carotid and the left vertebral artery, branch of the left subclavian artery. Only one such a case was described in the literature. All the other surviving cases had an associated lesion including: a double aortic arch, an aorto-pulmonary window (AP window), an aberrant right subclavian artery or a bilateral patent ductus arteriosus (PDA). We report a case of aortic atresia with IAA type B associated with a double aortic arch that underwent a successful bi-ventricular one-stage neonatal Norwood-Rastelli repair and is doing well after 18 months.

Lobectomy with ECMO Support in an Infant Who Developed Pulmonary Interstitial Emphysema Following Repair of Hypoplastic Aortic Arch.

Pulmonary interstitial emphysema (PIE) is a common problem in premature neonates with respiratory distress syndrome. This condition is often related to barotrauma caused by mechanical ventilation or continuous positive airway pressure applied to low birth weight neonates. The clinical diagnosis can be challenging. However, after proper diagnosis, several interventions are available for successful management. We describe an infant who developed severe PIE with recurrent pneumothoraces and development of a persistent bronchopleural fistula shortly after repair of a hypoplastic aortic arch and description of successful lobectomy with the assistance of extracorporeal support (ECMO).

Lobectomy with ecmo support in an infant who developed pulmonary interstitial emphysema following repair of hypoplastic aortic arch

Abstract Pulmonary interstitial emphysema (PIE) is a common problem in premature neonates with respiratory distress syndrome. This condition is often related to barotrauma caused by mechanical ventilation or continuous positive airway pressure applied to low birth weight neonates. The clinical diagnosis can be challenging. However, after proper diagnosis, several interventions are available for successful management. We describe an infant who developed severe PIE with recurrent pneumothoraces and development of a persistent bronchopleural fistula shortly after repair of a hypoplastic aortic arch and description of successful lobectomy with the assistance of extracorporeal support (ECMO).

Histopathological evaluation of aortic dissection: a comparison of congenital versus acquired aortic wall weakness.

OBJECTIVES: The aim of this study was to identify pathological changes of aortic dissection based on histopathological evaluation of aortic wall weakness by comparing patients with and without congenital abnormalities. METHODS: We reviewed records of patients who underwent repair for dissection-related aortic disease between 2008 and 2015. Fifty patients (20 men and 30 women; mean age 66.9 ± 14.0 years) who underwent surgery with subsequent histopathological examination of the aortic wall were divided into 2 groups. Group 1 had congenital abnormalities, including Marfan syndrome and bicuspid aortic valve (n = 5), and Group 2 had no congenital abnormalities (n = 45). We compared the histopathological characteristics of the aortic wall in these patients. RESULTS: There were significant differences in age and body surface area between the 2 groups. Although 80% of Group 1 patients developed dissection at the middle of the media, all Group 2 patients developed dissection at the outer one-third of the media, which is along the pathway of the vasa vasorum of the aortic wall. Both groups showed the same extent of degeneration of the vasa vasorum. Group 1 showed a severe score of mucoid extracellular matrix accumulation in the aortic media. CONCLUSIONS: Although it may be multifactorial, congenital maldevelopment of the media tends to result in dissection of the centre of the media, and acquired aortic wall weakness is concentrated in the outer third of the media. Degeneration of the vasa vasorum may be an important emerging substrate for developing aortic dissection.

Repair of Circumflex Aortic Arch in an Adult.

We describe the diagnoses and repair of a symptomatic circumflex aortic arch in an adult.

Modeling Outcomes: Modified Aortic Arch Advancement for Neonatal Hypoplastic Arch.

OBJECTIVE: Numerous surgical approaches regarding aortic arch advancement for neonatal arch hypoplasia have been described. These repairs can be classified into two categories: those that incorporate a patch and those that do not. The decision between repairs remains largely experiential, rather than empirical, because of the limited number of reported outcomes. We report early outcomes from neonates undergoing modified aortic arch advancement with an anterior patch and our experience using computational fluid dynamic modeling to better understand the hemodynamic consequences associated with this repair. METHODS: A retrospective review of neonates undergoing aortic arch advancement with anterior patch in 2014 at a single institution was performed. Anatomical, perioperative, and follow-up data were collected. Three-dimensional cardiac magnetic resonance images were used to generate computational fluid dynamic models of the modified anterior patch and direct end-to-side repairs. Cardiac waveform inputs were simulated and hemodynamic analyzed. RESULTS: Ten neonates underwent modified aortic arch advancement. No hemodynamically significant gradients were observed at a median follow-up of 0.77 (0.30-1.2) years. Asymmetrical flow was observed in the end-to-side repair, whereas more concentric laminar flow was observed throughout the modified model. Spatial variations in velocities immediately distal to the anastomosis were greater in the end-to-side model (0.35 vs 0.17 m/s, P < 0.001). Time-averaged variations in wall shear stress during systole were greater in the end-to-side model at the same location (3.44 vs 1.98 dynes/cm, P < 0.001). CONCLUSIONS: Early outcomes after the use of an anterior patch for neonatal hypoplastic aortic arch repair show favorable hemodynamic outcomes.

Unlikely culprit: congenital middle aortic syndrome diagnosed in the sixth decade of life.

A 58-year-old woman was admitted with acute heart failure. She had a long history of resistant hypertension, with an unremarkable work up for secondary causes in the past. Her brachial blood pressure was 210/70 mm Hg, with ankle blood pressure of 100/70 mm Hg. CT angiogram revealed marked narrowing of the descending thoracic aorta between the left subclavian artery and the diaphragm, consistent with middle aortic syndrome (MAS). She was initially managed with diuretics and antihypertensives. Subsequently thoracotomy revealed a severely hypoplastic segment of the descending aorta. The diseased segment was resected and aortic reconstruction performed. Histopathology showed fragmentation of the medial elastic fibres and fibrosis of the medial and intimal layers. These findings along with gross aortic hypoplasia and absence of features of Takayasu's arteritis, suggest that our patient had congenital MAS. The patient has done well since her surgery. We believe this is the first case of congenital MAS reported in the sixth decade of life.

Single-Stage Total Arch Replacement Including Resection of Kommerell Diverticulum in a Patient With Loeys-Dietz Syndrome.

Loeys-Dietz syndrome (LDS) is an autosomal dominant genetic connective tissue disorder associated with aortic aneurysmal disease. Kommerell diverticulum (KD) is a rare aortic diverticulum, for which the indication for surgery and the surgical techniques remain subjects of debate. We describe our experience with a successful total aortic arch replacement including KD resection through a median sternotomy for a pediatric patient with LDS.

Vascular rings.

The term vascular ring refers to congenital vascular anomalies of the aortic arch system that compress the esophagus and trachea, causing symptoms related to those two structures. The most common vascular rings are double aortic arch and right aortic arch with left ligamentum. Pulmonary artery sling is rare and these patients need to be carefully evaluated for frequently associated tracheal stenosis. Another cause of tracheal compression occurring only in infants is the innominate artery compression syndrome. In the current era, the diagnosis of a vascular ring is best established by CT imaging that can accurately delineate the anatomy of the vascular ring and associated tracheal pathology. For patients with a right aortic arch there recently has been an increased recognition of a structure called a Kommerell diverticulum which may require resection and transfer of the left subclavian artery to the left carotid artery. A very rare vascular ring is the circumflex aorta that is now treated with the aortic uncrossing operation. Patients with vascular rings should all have an echocardiogram because of the incidence of associated congenital heart disease. We also recommend bronchoscopy to assess for additional tracheal pathology and provide an assessment of the degree of tracheomalacia and bronchomalacia. The outcomes of surgical intervention are excellent and most patients have complete resolution of symptoms over a period of time.

Split-graft technique in neonatal heart transplant for aortic atresia.

We describe a neonate with aortic atresia and hypoplastic aorta, listed for heart transplant after extracorporeal membrane oxygenation resuscitation and ductal stenting. The donor aorta was detached from the graft, after an isolated arch reconstruction prior to the transplant itself in a routine fashion. To the best of our knowledge, this is the first reported case of neonatal arch reconstruction before transplantation performed with grafts from the same donor in a split-way strategy.

Contemporary surgical approaches and outcomes in adults with Kommerell diverticulum.

BACKGROUND: Surgery in patients with Kommerell diverticulum (KD) is controversial. Although the presence of symptoms is an accepted indication, the KD itself may be a risk factor for dissection and rupture, although size criteria for operation are undefined. METHODS: A retrospective review of 19 adult patients who underwent surgical treatment of KD between March 2004 and June 2013 was performed. Mean age was 48 years (range, 32 to 68 years). Fifteen patients were female, 15 were symptomatic, and 13 had a right aortic arch. Sixteen patients with aberrant subclavian artery underwent a two-stage procedure involving subclavian-common carotid artery transposition or bypass followed by aortic resection, including origin of the KD, with interposition graft reconstruction. Aortic resection was performed with left heart bypass (n = 10) or deep hypothermic circulatory arrest (n = 9). RESULTS: There were no deaths or strokes. Complications after aberrant subclavian artery revascularization were transient ptosis (n = 3), graft occlusion (n = 1), recurrent laryngeal nerve injury (n = 1),phrenic nerve injury (n = 1), and reintubation (n = 1). Complications after aortic resection were intraoperative type A dissection (n = 1), phrenic nerve injury (n = 1), chylothorax (n = 1), and transient neurologic dysfunction (n = 1). Mean hospital stay after aberrant subclavian artery revascularization was 2 ± 2 days and after aortic resection, 6.4 ± 2.4 days. Of 18 available pathology specimens, all 18 showed medial degeneration. Mean follow-up was 3.3 years. CONCLUSIONS: This is the largest reported single-center experience with the surgical management of KD in adults, verifying its safety and efficacy. The high percentage of KD with medial degeneration suggests asymptomatic patients with an enlarged KD also may benefit from resection.

Reoperation after vascular ring repair.

The majority of patients having surgical intervention for a vascular ring have resolution of their symptoms. However, 5% to 10% of these patients develop recurrent symptoms related either to airway or esophageal compression and may require reoperation. In our series of 300 patients with vascular rings, we performed a reoperation on 26 patients, not all of whom were originally operated on at our institution. The four primary indications for reoperation were Kommerell diverticulum (n = 18), circumflex aorta (n = 2), residual scarring (n = 2), and tracheobronchomalacia requiring aortopexy (n = 4). All patients undergoing reoperation have had preoperative evaluation with bronchoscopy and computed tomographic scanning (CT) with 3-dimensional reconstruction. Patients with dysphagia have had a barium esophagram and esophagoscopy. Patients with a Kommerell diverticulum have undergone resection of the diverticulum and transfer of the left subclavian artery to the left carotid artery. The aortic uncrossing procedure has been used in patients with a circumflex aorta. Aortopexy has been used to treat anterior compression of the trachea by the aorta. Results of these reinterventions have been successful in nearly all cases. Lessons learned from these reoperations can be applied to prevent the need for reoperation by properly selecting the correct initial operation. A dedicated team caring for these children consisting of medical imaging, otolaryngology, cardiovascular-thoracic surgery, and critical care is imperative.

Persistent right aortic arch and associated axial skeletal malformations in cats.

Persistent right aortic arch (PRAA) in cats is an uncommon vascular anomaly with clinical signs referable to oesophageal obstruction. To our knowledge no reports of axial skeletal malformations concomitant to PRAA have been reported in cats. The aim of this study is to depict a new clinical feature in cats affected by PRAA. In the study six cats with a diagnosis of vascular ring anomaly were enrolled. A complete physical examination, a neurological examination and a total body radiograph were performed on each animal. Four of the six cats showed contemporary PRAA and skeletal malformations. Additionally, for the first time, a genetic test was performed on one subject to detect DNA alterations in the homologous DiGeorge region of cat. The percentage of skeletal malformations reported in the normal population was compared with animals with PRAA and showed a higher frequency. Genetic testing failed to demonstrate a correlation between PRAA and DiGeorge genomic deletion. A review of veterinary and human diseases that presented both conditions was assessed. The few animals enrolled do not allow definitive conclusions. Further studies are required to corroborate the correlation between PRAA and axial skeletal malformations in cats.