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.

Genetic Etiology of Left-Sided Obstructive Heart Lesions: A Story in Development.

Congenital heart disease is the most common congenital defect observed in newborns. Within the spectrum of congenital heart disease are left-sided obstructive lesions (LSOLs), which include hypoplastic left heart syndrome, aortic stenosis, bicuspid aortic valve, coarctation of the aorta, and interrupted aortic arch. These defects can arise in isolation or as a component of a defined syndrome; however, nonsyndromic defects are often observed in multiple family members and associated with high sibling recurrence risk. This clear evidence for a heritable basis has driven a lengthy search for disease-causing variants that has uncovered both rare and common variants in genes that, when perturbed in cardiac development, can result in LSOLs. Despite advancements in genetic sequencing platforms and broadening use of exome sequencing, the currently accepted LSOL-associated genes explain only 10% to 20% of patients. Further, the combinatorial effects of common and rare variants as a cause of LSOLs are emerging. In this review, we highlight the genes and variants associated with the different LSOLs and discuss the strengths and weaknesses of the present genetic associations. Furthermore, we discuss the research avenues needed to bridge the gaps in our current understanding of the genetic basis of nonsyndromic congenital heart disease.

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.

Fetal congenital midaortic syndrome with unilateral renal artery stenosis prenatally presenting with polyhydramnios and postpartum as hyponatremic hypertensive syndrome.

The midaortic syndrome (MAS) is a rare anomaly, characterised by narrowing of the distal aorta and its major branches. The most common symptom is severe arterial hypertension. The combination of hyponatremia, polyuria and renovascular hypertension caused by a unilateral renal artery stenosis is described as hyponatremic hypertensive syndrome. We report a case of MAS with unilateral renal artery stenosis in a preterm female neonate. A pregnant woman at 34 weeks of gestation was referred with fast growing abdominal circumference and pain. The ultrasound revealed severe polyhydramnios and fetal myocardial hypertrophy. Within the first 48 hours of the neonatal period, the diagnosis of MAS was made. We conclude that symptomatic MAS, caused by unilateral renal artery stenosis, resulting in increased renin-angiotensin-aldosterone system activity and subsequent polyuria of the non-stenotic kidney, lead to clinically significant polyhydramnios.

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.

A review of aortic disease research in Malaysia.

INTRODUCTION: Aortic disease includes conditions such as chronic aortic aneurysms, acute aortic syndromes and congenital aortic abnormalities, amongst others. This paper reviews all research on aortic disease performed in Malaysia and published between 2000-2016. METHODS: A literature search was conducted in PubMed, Scopus, MyJurnal and the UKM Journal Repository. The search process was based on a previously published methodology. The medical subject headings (MeSH) search terms used were "aortic", "aorta" and "Malaysia". RESULTS: Two-hundred-thirteen papers were identified, of which 60 papers were selected and reviewed on the basis of their relevance. The epidemiology, pathophysiology, clinical presentations, case reports, investigations, treatment and outcomes of aortic disease in Malaysia were reviewed and summarised. The clinical relevance of the studies performed are discussed. CONCLUSION: The review provided an insight into the pathophysiology, prevalence and epidemiology of aortic diseases in Malaysia, how the condition is managed, and the outcomes of treatment. Limitations of the research performed in Malaysia to date were identified and recommendations for further research and improvement in clinical practice were recommended.

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.

Congenital Thoracic Aortic Disease.

Congenital abnormalities of the thoracic aorta encompass a variety of disorders with variable clinical manifestations ranging from asymptomatic to life threatening. A variety of imaging modalities are available for the evaluation of these anomalies with computed tomography (CT) commonly preferred due to its excellent spatial resolution and rapid acquisitions, avoiding the need of general anesthesia or even sedation. We review the embryology, imaging findings, and associations of multiple congenital thoracic aorta malformations with emphasis in the role of CT angiography in the evaluation of these pathologies.

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.

Congenital aortocaval fistula between right aortic sinus of Valsalva and superior vena cava: A rare case report.

Thoracic aortocaval fistula is a very rare cause of left to right shunt. Drainage of fistula into the superior vena cava (SVC) is very uncommon. Clinical symptoms depend on the size of the shunt. We report a rare case of an asymptomatic 27-year-old woman with congenital aortocaval fistula to the SVC with a small amount of left to right shunt that was considered for serial medical follow-up.

Persistent fifth aortic arch: the "great pretender" in clinical practice.

Persistence of the embryonic "fifth aortic arch" in postnatal life is a rare, enigmatic - and at times controversial - condition, with variable anatomical forms and physiological consequences. First described in humans over 40 years ago by Van Praagh, the condition was labelled the "great pretender" by Gerlis 25 years later, because of its apparent propensity to mimic anatomically similar structures. Despite many subsequent case reports citing the condition, the true developmental origin of these structures remains unresolved, and has been the subject of debate among embryologists for more than a century. A persistent fifth aortic arch has been defined as an extrapericardial structure, arising from the ascending aorta opposite or proximal to the brachiocephalic artery, and terminating in the dorsal aorta or pulmonary arteries via a persistently patent arterial duct. This description may therefore encompass various anatomical forms, such as a unilateral double-lumen aortic arch, an unrestrictive aortopulmonary shunt, or a critical vascular channel for either the systemic or pulmonary circulation. The physiological properties of these vessels, such as their response to prostaglandins, may also be unpredictable. In this article, we demonstrate a number of cases that fulfil the contemporary definition of "persistent fifth aortic arch" while acknowledging the embryological controversies associated with this term. We also outline the key diagnostic features, particularly with respect to the use of new cross-sectional imaging techniques.

Repair of Circumflex Aortic Arch in an Adult.

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