Congenital Aortic Anomalies Research Articles

A Rare Case of Right Aortic Arch, Evaluation Using CT

This case report discusses a 76-year-old male with a right aortic arch and an aberrant retroesophageal left subclavian artery, identified during a CT scan. The imaging revealed Kommerell's diverticulum without affecting the pulmonary trunk. Classified as type II, this variant emphasizes the role of CT in diagnosing congenital thoracic aortic anomalies, often missed by echocardiography.

A case of Kommerell diverticulum in adolescence presented with dysphagia

BackgroundKommerell diverticulum (KD) with right aortic arch and aberrant left subclavian artery (ALSCA) is a rare congenital aortic anomaly. To improve organ compression symptoms and avoid rupture of aneurysms in adulthood (19 years old–), surgical treatment is considered the only curative option. However, in childhood (–18 years old), several problems regarding approach and technique selection have been reported. Surgical treatment for KD in infancy (birth–2 years old) has been reported recently, but rarely in adolescence (13–19 years old). We herein report a case of KD in which the patient underwent graft replacement during adolescence. Case presentationA 13-year-old boy was admitted to our hospital presenting with dysphagia and body weight loss. Esophagography showed upper esophageal stenosis caused by extrinsic compression. Contrast-enhanced computer tomography showed saccular aneurysm formation of KD with right aortic arch (RAA) and ALSCA. Elective surgery including KD resection and graft replacement of the descending aorta was performed via right thoracotomy under partial extracorporeal circulation. The ALSCA was reconstructed by graft interposition. No postoperative complication was observed. Follow-up esophagography showed no residual stenosis.ConclusionWe experienced a case of KD with dysphagia and weight loss in adolescence, which was successfully treated with surgery. Graft replacement could be an effective treatment option, facilitating recovery even during the growth period.

Blood flow assessment technology in aortic surgery: a narrative review.

Blood flow assessment is an emerging technique that allows for assessment of hemodynamics in the heart and blood vessels. Recent advances in cardiovascular imaging technologies have made it possible for this technique to be more accessible to clinicians and researchers. Blood flow assessment typically refers to two techniques: measurement-based flow visualization using echocardiography or four-dimensional flow magnetic resonance imaging (4D flow MRI), and computer-based flow simulation based on computational fluid dynamics modeling. Using these methods, blood flow patterns can be visualized and quantitative measurements of mechanical stress on the walls of the ventricles and blood vessels, most notably the aorta, can be made. Thus, blood flow assessment has been enhancing the understanding of cardiac and aortic diseases; however, its introduction to clinical practice has been negligible yet. In this article, we aim to discuss the clinical applications and future directions of blood flow assessment in aortic surgery. We then provide our unique perspective on the technique's translational impact on the surgical management of aortic disease. Articles from the PubMed database and Google Scholar regarding blood flow assessment in aortic surgery were reviewed. For the initial search, articles published between 2013 and 2023 were prioritized, including original articles, clinical trials, case reports, and reviews. Following the initial search, additional articles were considered based on manual searches of the references from the retrieved literature. In aortic root pathology and ascending aortic aneurysms, blood flow assessment can elucidate postoperative hemodynamic changes after surgical reconfiguration of the aortic valve complex or ascending aorta. In cases of aortic dissection, analysis of blood flow can predict future aortic dilatation. For complicated congenital aortic anomalies, surgeons may use preoperative imaging to perform "virtual surgery", in which blood flow assessment can predict postoperative hemodynamics for different surgical reconstructions and assist in procedural planning even before entering the operating room. Blood flow assessment and computational modeling can evaluate hemodynamics and flow patterns by visualizing blood flow and calculating biomechanical forces in patients with aortic disease. We anticipate that blood flow assessment will become an essential tool in the treatment planning and understanding of the progression of aortic disease.

Beating heart thoracic aortic surgery under selective myocardial perfusion for patients with congenital aortic anomalies.

We evaluated beating heart thoracic aortic surgery (BHTAS) using selective myocardial perfusion (SMP) in patients with aortic anomalies with complex surgical needs. Between 2012 and 2018, 27 infants with aortic anomalies underwent BHTAS using SMP. Median body weight was 3.5kg (range 2.6-5.2). In total, 15 cases of aortic coarctation, 7 cases of hypoplastic left heart syndrome, and 5 cases of interrupted aortic arch were included. An extended aortic arch anastomosis maneuver was used in 7 cases and aortic arch reconstruction compensated with an autologous pericardium patch was used in 6 cases. A Norwood-type procedure was used in 11 cases. The median Aristotle comprehensive score was 13.9 (7.0-20.0). BHTAS cases were not inferior in postoperative CK-MB/CK ratio (12.4 ± 2.8 in BHTAS vs 13.9 ± 3.6 in CTAS, p = 0.09), and there were no instances of myocardial ischemia. Two late deaths occurred due to shunt obstruction (n = 1) and valve malfunction (n = 1). Freedom from aortic reintervention was not inferior to conventional thoracic aortic surgery. BHTAS cases exhibited shorter cardiac arrest time than that of conventional thoracic aortic surgery in similar surgical procedures [CoA/VSD cases: 48.0 ± 8.0min in the BHTAS cases and 65.7 ± 10.8min in the CTAS cases (p = 0.0122), simple CoA/re-CoA cases: 0min in the BHTAS cases and 20.1 ± 3.6min in the CTAS cases]. BHTAS reduced cardioplegic arrest time while maintaining postoperative CK-MB/CK ratio, mid-term death, and aortic reintervention rates.

Three-Dimensional Printing of a Complex Aortic Anomaly

Complex congenital aortic anomalies include diverse types of malformations that may be clinically asymptomatic or present with respiratory or esophageal symptoms. These anomalies may be associated with other congenital heart diseases. It is hard to identify the accurate anatomic vessel location from two-dimensional imaging data, such as computed tomography. As an additive manufacturing method, three-dimensional (3-D) printing can covert the acquired imaging data into 3-D physical models. This protocol describes the procedure for modeling the volumetric DICOM imaging into 3-D data and printing it as an anatomically realistic 3-D model. Using this model, surgeons can identify the vessel location of complex aortic anomalies, which is helpful for pre-operative planning and intra-operative guidance.

Clues and techniques of prenatal ultrasonographic diagnosis for fetal aortic arch anomalies

Objective To explore the ultrasonographic features, diagnostic clues and methods of fetal aortic arch anomalies, and to improve the diagnosis rate of prenatal ultrasonography for fetal aortic arch anomalies. Methods Fetuses with aortic arch anomalies diagnosed by prenatal ultrasound were selected as the study objects. The prenatal ultrasonographic features of the three views of the aortic arch were carefully observed. The three views included the followings: the transverse view(the view of three vessels and trachea), the sagittal view (the longitudinal view of the aortic arch), and the coronal view (the coronal view of the descending aorta). The prenatal diagnostic clues and diagnostic techniques of aortic arch anomalies were summarized and analyzed. Results Eighty-one cases with aortic arch anomalies were followed up. Fifteen cases were left-sided aortic arch and left-sided ductus arteriosus with aberrant right subclavian artery, 22 cases with right aortic arch and left-sided ductus arteriosus with aberrant left subclavian artery, 4 cases were mirror right aortic arch and left-sided ductus arteriosus, 2 cases were mirror right aortic arch and right-sided ductus arteriosus, 11 cases were double aortic arch, 23 cases were coarctation of the aorta, and 4 cases were interruption of aortic arch. Among them, 27 cases were complicated with other cardiac and extra-cardiac malformations and 13 cases with chromosomal abnormalities. Prenatal ultrasonographic clues for diagnosis of coarctation of the aorta included the three vessels and trachea view showing small diameter and disproportion to the diameter of the ductus arteriosus. And the longitudinal view of the aortic arch was helpful for showing the position and the extent of coarctation of the aorta. Prenatal ultrasonographic clues for diagnosis of interruption of aortic arch were that the three vessels and trachea view and the longitudinal view of the aortic arch showed the interruption between aortic arch and descending aorta. And the abnormal combination cases of the aortic arch segments also had their corresponding diagnostic clues. These clues included that the three vessels and trachea view showed abnormal position, arrangement and running of the branches of the aortic arch. And the coronal view of the descending aorta also showed abnormal changes in the branches of aortic arch. Conclusions Various types of congenital aortic arch anomalies have characteristic ultrasonography and their corresponding diagnostic clues and methods. The combined use of the three views of the aortic arch is effective in prenatal diagnosis for aortic arch anomalies. Key words: Ultrasonography, prenatal; Aortic arch anomalies; Diagnostic clues

Young adult cardiovascular diseases: a single center coronary computed tomography angiography study

Purpose and backgroundCardiovascular disease (CVD) is one of the leading causes of death. However, the prevalence of CVD in young adults (<40 years of age) has not been well documented. We conducted this study to determine the proportion of CVD in both symptomatic and asymptomatic young adults. Materials and methodsCoronary CT angiography images obtained from April 2015 to July 2017 in our institution was retrospectively reviewed. Young adults were separated into two groups according to whether they had experienced chest discomfort. The diagnosis was classified as normal, coronary artery disease (CAD), myocardial bridging (MB), congenital coronary anomaly, congenital cardiac anomaly, cardiomyopathy, and aortic anomaly. The proportion of different diagnoses in two groups and cardiovascular risk factors were analyzed. ResultsTotally 107 patients (mean age, 35.6 ± 3.55 years) were grouped into 36 cases of symptomatic group and 71 patients of asymptomatic group. Cardiovascular anomalies were found in 61 cases (41%). No significant difference in the occurrence rates of CAD (14% vs 11%, p = 0.53), MB (31% vs 42%, p = 0.51), and congenital coronary anomaly (7% vs 3%, p = 0.26) between groups. Mild arterial stenosis was implied in most CAD cases. Hypertension was the only risk factor significantly correlated with CAD. ConclusionsAlthough young adults are conventionally identified as low-risk, more than 60% of the cases in our cohort were proved to present cardiovascular anomalies, with no significant relation to cardiac symptoms. Early interventions should be conducted for aggressive CVD subtypes to prevent future acute events.

Evaluation of congenital aortic valve anomalies by cardiac MRI

Evaluation of congenital aortic valve anomalies by cardiac MRI

Congenital anomalies of the left brachiocephalic vein detected in adults on computed tomography.

Anomalous left brachiocephalic vein (BCV) is a rare and less known systemic venous anomaly. We evaluated congenital anomalies of the left BCV in adults detected during computed tomography (CT) examinations. This retrospective study included 81,425 patients without congenital heart disease who underwent chest CT. We reviewed the recorded reports and CT images for congenital anomalies of the left BCV including aberrant and supernumerary BCVs. The associated congenital aortic anomalies were assessed. Among 73,407 cases at a university hospital, 22 (16 males, 6 females; mean age, 59years) with aberrant left BCVs were found using keyword research on recorded reports (0.03%). Among 8018 cases at the branch hospital, 5 (4 males, 1 female; mean age, 67years) with aberrant left BCVs were found using CT image review (0.062%). There were no significant differences in incidences of aberrant left BCV between the two groups. Two cases had double left BCVs. Eleven cases showed high aortic arches. Two cases had the right aortic arch, one case had an incomplete double aortic arch, and one case was associated with coarctation. Aberrant left BCV on CT examination in adults was extremely rare. Some cases were associated with aortic arch anomalies.

Pregnancy outcomes following exposure to abatacept during pregnancy

ObjectiveTo characterize pregnancy outcomes following maternal and paternal exposure to abatacept, using clinical trial and post-marketing data available to the manufacturer. MethodsAll confirmed cases of pregnancy with outcome data reported to the manufacturer up to September 1, 2014 were included. Sources included clinical trials, spontaneously reported (unsolicited) post-marketing cases, and the Organization of Teratology Information Specialists registry. Details recorded included number of live births, spontaneous abortions and terminations, pregnancy complications, and congenital anomalies. ResultsA total of 161 pregnancies with known outcomes were identified between 1995 and September 2014: 151 were following maternal exposure to abatacept and 10 were following paternal exposure. Seven of 86 (8.1%) live births following maternal exposure had congenital anomalies (cleft lip/cleft palate, congenital aortic anomaly, meningocele, pyloric stenosis, skull malformation, ventricular septal defect/congenital arterial malformation, and Down׳s syndrome with premature rupture of membranes at 17 weeks that resulted in a live birth via cesarean section and subsequent infant death). In addition, 59 of the 151 (39.0%) cases with maternal exposure resulted in abortions (40 spontaneous and 19 elective). Of the 10 pregnancies with paternal exposure, there were nine live births and one elective abortion, with no congenital abnormalities identified and no fetal deaths. ConclusionsBased on these data, there does not appear to be a pattern of congenital anomalies following maternal or paternal exposure to abatacept. No cases of vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, or limb abnormalities (VACTERL) were noted. Spontaneous abortion rates were within expected range. Abatacept should be used during pregnancy only if the potential benefit to the mother justifies the potential risk to the fetus.

Role of Computed Tomography in Assessment of the Thoracic Aorta.

Thoracic aortic disease is increasing in prevalence and can result in serious morbidity and mortality. Computed tomography (CT) angiography is an important imaging modality for assessment of thoracic aortic pathology due to wide availability, rapid acquisition, reproducibility, superior spatial and temporal resolution, and capability for 3D image post-processing. CT is the preferred imaging modality in the acute setting to rapidly identify patients with acute aortic syndromes including dissection, intramural hematoma, and penetrating aortic ulcer. CT also plays an important role in post-procedural surveillance of the thoracic aorta for early and late complications from open or endovascular repair. Incidentally detected thoracic aortic aneurysms and congenital aortic anomalies such as coarctation can be thoroughly characterized and followed over time for potential elective intervention. Drawbacks of CT include exposure to radiation and iodinated contrast media; however, recent strategies for dose reduction and contrast optimization have significantly decreased these risks. Electrocardiogram (ECG)-gated CT angiography provides additional information about the aortic root, coronary arteries, and other cardiac structures without motion artifacts.

Unicuspid Aortic Stenosis in a Patient with Turner Syndrome: A Case Report.

Congenital aortic valve anomalies are the cause of premature aortic stenosis in pediatric and younger adult populations. Despite being very rare, unicuspid aortic valves account for approximately 5% of isolated aortic valve replacements. Patients with aortic stenosis, present with the same symptomatology independent of leaflet morphology. However, the presence of bicuspid and unicuspid aortic stenosis is associated with a higher incidence of aortopathy, especially in Turner syndrome patients. Turner syndrome, an X monosomy, is associated with aortic valve anomalies, aortopathy, and hypertension. These risk factors lead to a higher incidence of aortic dissection in this population. Patients with Turner syndrome and aortic stenosis that present for aortic valve replacement should therefore undergo extensive aortic imaging prior to surgery. Transthoracic echocardiography is the diagnostic tool of choice for valvular pathology, yet it can misdiagnose unicuspid aortic valves as bicuspid valves due to certain similarities on imaging. Transesophageal echocardiography is a better tool for distinguishing between the two valvular abnormalities, although diagnostic errors can still occur. We present a case of a 50-year-old female with history of Turner syndrome and bicuspid aortic stenosis presenting for aortic valve replacement and ascending aorta replacement. Intraoperative transesophageal echocardiography revealed a stenotic unicommissural unicuspid aortic valve with an eccentric orifice, which was missed on preoperative imaging. This case highlights the importance of intraoperative transesophageal echocardiography in confirming preoperative findings, diagnosing further cardiac pathology, and ensuring adequate surgical repair.

Surgical strategy for Kommerell's diverticulum with aberrant subclavian artery.

Kommerell's diverticulum is an extremely rare congenital aortic anomaly. Because of its rarity, the optimal surgical strategy for Kommerell's diverticulum has not been established. In this study, we reviewed our experience of surgical correction of this anomaly. Between 2007 and 2013, we managed 9 surgical cases of Kommerell's diverticulum; 7 had a right aortic arch with an aberrant left subclavian artery, and 2 had a left aortic arch with an aberrant right subclavian artery. None of these patients had any symptom resulting from structural compression between the aneurysm and the aberrant subclavian artery. All patients underwent surgical treatment to prevent aneurysmal rupture. Six patients had replacement of the thoracic descending aorta and in-situ reconstruction of the aberrant subclavian artery through a right thoracotomy, and 3 underwent the same procedures through a left thoracotomy. Three different methods of extracorporeal circulation were applied, according to the anatomical features of each case. There was one hospital death. This patient developed severe cerebral infarction and died of multiple organ failure on the 65th postoperative day. There were no other major complications nor any need for rehospitalization. Kommerell's diverticulum should be treated using an optimal strategy based on each patient's anatomical features and other characteristics.

Bronşiyal Astım Tanısıyla İzlenirken Tekrarlayan Akciğer Enfeksiyonları Ve Disfaji Yakınması İle Başvuran Bir Hastada Vasküler Ring Anomalisi

Vascular ring is a congenital aortic anomaly causes various findings depending on the compression of trachea and esophagus. A 8 year-old girl with a diagnosis of bronchial asthma, consulted to our pediatric cardiology clinic due to recurrent pulmonary infections and dysphagia. In our patient, vascular ring anomaly with a predominance of right aortic arch was diagnosed and referred for surgical correction. This report is presented in order to emphasize the importance of detailed echocardiographic examination in patient with asthma, recurrent pulmonary infections, and especially in children with swallowing disorders.

Acute Superior Vena Cava Syndrome After Rupture of Kommerell's Diverticulum: A Case Report

Kommerell's diverticulum, a rare congenital aortic anomaly, is dilatation at the region in which an aberrant subclavian artery branches from either a left-sided or right-sided thoracic aorta. We report a rare case of acute superior vena cava syndrome that developed in a young healthy male patient who presented to the emergency department in imminent respiratory arrest after rupture of this diverticulum.

MRT zur Therapiekontrolle bei Patienten mit Aortenisthmusstenose

Aortic isthmus stenosis is the most common congenital aortic anomaly and is often a problem for therapy surveillance. In addition to possible comorbidities (e.g. bicuspid aortic valve) it is accompanied by various middle and long-term complications depending on the primary choice of the therapeutic procedure. Magnetic resonance imaging (MRI) plays an important role for the mostly young patients in the control of the aortic isthmus stenosis and therapy because it is non-invasive and there is no X-ray exposure. Radiologists should be well-informed on the principles of the therapeutic procedure in order to be competent in the interpretation of MRI findings. Due to the continuous development of MRI technology, techniques for functional evaluation (e.g. dynamic MRA, 4D PC flow measurement) are increasingly becoming available in addition to high-resolution MR angiography (MRA), which could predict the risk of possible complications, such as aneurysms. However, in this aspect further studies are necessary. Interventional therapy with stents and stent grafts is often employed for the therapy of possible complications following an operation (aneurysms, restenosis) but because of massive metal artefacts the use of MRI is often sometimes severely limited.

Open Thoracic Aortic Repair for Dysphagia in Patients with Kommerell's Diverticulum and Right-Sided Aortic Arch with Aberrant Left Subclavian Artery

Kommerell's diverticulum of an aberrant left subclavian artery associated with a right-sided aortic arch is a rare congenital aortic anomaly. This communication reports two cases of this congenital anomaly leading to severe dysphagia. Both cases were successfully repaired with a staged open approach rather than an endovascular approach, which would not have addressed the presenting symptom of dysphagia.

Left-sided Cervical Aortic Arch Associated With Pseudocoarctation, Aneurysm Formation, and Anomalous Left Brachiocephalic Vein

Cervical aortic arch (CAA) is a rare congenital aortic anomaly. It can be associated with several cardiovascular abnormalities including aortic aneurysm, valvular disease, and pseudocoarctation. In this report, we present a case of CAA with combined association of both aneurysm formation and pseudocoarctation. This combination of anomalies is extremely uncommon, described in only 4 cases in the literature. In addition, our patient had an anomalous subaortic left brachiocephalic vein that, to our knowledge, was not previously described in the literature to be associated with CAA. The radiographic, multidetector computed tomography scan, and magnetic resonance angiography imaging findings are presented.

Unusual Congenital Aortic Anomaly with Rare Common Celiamesenteric Trunk Variation: MR Angiography and Digital Substraction Angiography Findings

Magnetic resonance angiography and digital substraction angiography (DSA) findings in a case with a rare congenital thoracoabdominal aortic hypoplasia and common celiamesenteric trunk variation with occlusion of infrarenal abdominal aorta are described here. To our knowledge, this aortic anomaly has not been previously described in the English literature. DSA is the optimum imaging modality for determination of aortic hypoplasia, associated vascular malformations, collateral vessels, and direction of flow within vessels.

Vascular anomalies causing tracheoesophageal compression: a 20-year experience in diagnosis and management.

Vascular rings and other congenital aortic arch anomalies may be major causes of tracheoesophageal obstruction in children. In this report, our diagnostic approach, surgical treatment, and early and late results for 30 patients are reported. During a 20-year period (1982-2002), 30 children underwent surgery for tracheoesophageal compression caused by aortic arch anomalies. The median age at operation was 8 months (range, 36 days to 94 months), and the median patient weight was 8 kg (range, 2.4-16 kg). At 53.3% of cases, double aortic arch was by far the most common encountered cause of compression. Patients were admitted with respiratory distress, stridor, apnea, dysphagia, or recurrent respiratory tract infections. Diagnosis was established by barium esophagogram, computed tomography, magnetic resonance imaging, and angiography. The operative approaches were through a left thoracotomy or a median sternotomy. Operative mortality rate was 3.3%. Follow-up data from 2 months to 10 years (mean follow-up, 34 weeks) were available for all 30 patients. Twenty-six patients (86.7%) were essentially free of symptoms, 3 patients (10%) had residual respiratory problems, and 1 patient (3.3 %) had a gastroesophageal reflux problem. These results suggest that surgical correction of symptomatic vascular rings can be performed with low mortality and morbidity rates.