Variations in the Evaluation and Management of Vascular Rings: A Survey of American Clinicians.

Vascular rings are arterial malformations that lead to the compression of the trachea and/or esophagus. While "tight" rings often produce symptoms and require surgery, "loose" rings rarely produce symptoms. Given advances in fetal echocardiography, this diagnosis is now more often made prenatally. This poses a new conundrum in the management of asymptomatic patients, leading to practice variation and creating a target for clinical system improvement. Hence, we conducted this survey aiming to demonstrate the practice variation existing in current evaluation and management of these patients. An anonymous web-based survey was distributed to several listservs for pediatric cardiologists and pediatric cardiothoracic surgeons. Survey questions targeted respondent practice characteristics, testing obtained, and indications for testing or surgical referral. In total 61 responses were received, predominantly from pediatric cardiologists (95%) in the United States (97%). About 60% of clinicians reported frequently diagnosing patients with vascular rings by fetal echocardiogram, with only about 20% diagnosing them frequently on evaluation of symptoms. Computed tomography angiography and echocardiogram were the most common imaging modalities employed. Most clinicians obtained cross-sectional imaging at the time of diagnosis and referred to surgery once patients had at least occasional symptoms. Respondents demonstrated a low degree of agreement (Krippendorf's alpha 0.48). Few statistically significant patterns were identified between respondents based on their practice characteristics. This study identified significant variation between clinicians regarding the evaluation and management of vascular rings. Further research or expert opinions may help to standardize practice, saving costs and improving the quality of care for affected patients.

The Impact of Prenatal Diagnosis on Clinical Outcomes of Isolated Vascular Rings From a Statewide Paediatric Cardiology Tertiary Service.

BACKGROUND: Vascular rings, including right aortic arch with aberrant left subclavian artery (RAA-ALSCA), double aortic arch (DAA) and pulmonary artery sling (PAS), are congenital anomalies that may cause airway and oesophageal compression. As prenatal detection has improved, literature comparing clinical outcomes of antenatally versus postnatally diagnosed cases continues to emerge. The aim is to define a statewide tertiary paediatric institution's clinical profile and outcomes of prenatal versus postnatally diagnosed isolated vascular rings. METHOD: A retrospective single-centre review of isolated RAA-ALSCA, DAA and PAS between 1 January 1999 and 31 December 2020 was conducted. Clinical characteristics, surgical and follow-up information were collected. Antenatal and postnatally diagnosed groups were compared. RESULTS: Out of 123 cases diagnosed with isolated vascular rings, 98 (79.7%) cases had RAA-ALSCA, 21 (17.1%) with DAA, 4 (3.3%) with PAS. The antenatal detection rate was 73.6% in the past decade; 20.3% had a genetic disorder, of which 48% had 22q11.21 microdeletion. Of prenatally diagnosed cases, 31.3% developed symptoms, commonly stridor and dysphagia, at a median age of 2.0 months (IQR 0.0-3.0), compared to a median age of diagnosis for the postnatal cohort of 9 months (IQR 1.0-40.7). Postnatally diagnosed cases were more likely to present with symptoms, primarily respiratory distress, than prenatally diagnosed cases (p=0.006). Fifty-nine (59) cases (50% antenatally diagnosed) required vascular ring division; 6.8% had residual symptoms following surgery. DISCUSSION: Antenatal diagnosis has improved and leads to better parental awareness and more timely, appropriate intervention. Postnatally diagnosed patients were older, more likely to be symptomatic, underwent more investigations and were commenced on more medications for symptom management prior to diagnosis. One in five cases of isolated vascular ring anomalies carried a genetic diagnosis, which has important implications on prenatal counselling and genetic testing.

Subclavian Steal Phenomenon Associated With Vascular Ring in an Infant Who Had a Prenatally Diagnosed Right Aortic Arch With an Atretic Aberrant Left Subclavian Artery.

Vascular ring is a rare congenital anomaly in which the abnormal origin of the aorta or its branches and pulmonary arteries leads to encircling and compression of the trachea and esophagus. A right aortic arch (RAA) with an aberrant left subclavian artery is one of the most common forms of vascular ring. Here, we report a case of a prenatally diagnosed vascular ring resulting from an RAA with an aberrant left subclavian artery. When the infant was 7 months of age, the development of noisy breathing prompted further evaluation with cardiac magnetic resonance imaging that showed an atretic left subclavian artery associated with collateral retrograde flow from the left vertebral artery to the distal portion of the subclavian artery. Our findings indicate that an untreated RAA with an aberrant left subclavian artery may be associated with an increased risk of developing subclavian artery steal syndrome.

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.

Fetal echocardiographic markers to differentiate between a right and double aortic arch.

OBJECTIVES: To describe the fetal echocardiographic features of a double aortic arch (DAA) and secondly, to assess the performance of these features to differentiate between a right aortic arch with left duct (RAA-LD) in a blinded cohort of vascular rings. METHODS: Review of records to identify surgically confirmed cases of DAA diagnosed prenatally from 2014 to 2018 (cohort-A). Prenatal echocardiograms were reviewed and the segments of the aortic arches anterior and posterior to the trachea, aortic isthmuses and the presence/absence of the Z-sign were described. The utility of these markers were assessed in a separate cohort (B) of fetuses with surgically confirmed cases of DAA or RAA-LD. RESULTS: Cohort-A comprised 34 cases with DAA; there was a dominant RAA in 32/34 (94%) and balanced left aortic arch (LAA) and RAA in two cases. The proximal LAA was seen in 29/34 (85%), distal LAA in 15/34 (44%) and the LAA aortic isthmus in 4/34 (12%). The "Z" configuration was present in 29/34 (85%) cases. The most predictive marker for DAA in cohort-B was the Z-sign (sensitivity: 100%, specificity: 81%). CONCLUSION: The "Z" sign is a useful differentiator between RAA-LD and DAA. The absence of visualization of the left aortic isthmus does not preclude the presence of a DAA.

Influence of Fetal Diagnosis on Management of Vascular Rings.

BACKGROUND: Fetal diagnoses of vascular rings have been increasing. We compared management strategies and outcomes of infants with fetal diagnosis with those with postnatal diagnosis to inform recommendations regarding optimal management. METHODS: A retrospective review was performed of vascular ring operations from January 2000 to June 2019. Standard demographic data (preoperative clinical status, timing of diagnosis, cross-sectional imaging, operative and perioperative details, and clinical outcomes) were collected. Statistical analysis was performed to compare characteristics and outcomes of fetal versus postnatal diagnosis. RESULTS: Of 190 patients, 15% (n = 29) were diagnosed prenatally. Anatomic variants were double aortic arch (n = 66, 14 fetal diagnoses), right aortic arch, aberrant left subclavian artery (n = 94, 12 fetal diagnoses), circumflex aorta (n = 7, 1 fetal diagnosis), and pulmonary artery sling (n = 19, 2 fetal diagnoses). An increasing frequency of fetal diagnoses has been noted in the past 10 years. In 2012 1 of 9 patients (11%) had a fetal diagnosis, whereas in 2018 8 of 11 (72%) had a fetal diagnosis (P < .001). Patients with a fetal diagnosis were significantly younger at the time of surgery (13.1 months [interquartile range (IQR), 20.6] vs 24.0 months [IQR, 87.0], P = .029). There was no difference in postoperative complications or length of stay (3 days [IQR, 1] for fetal diagnoses vs 4 days [IQR, 3] for postnatal diagnoses, P = .50). CONCLUSIONS: Fetal diagnosis leads to the potential for expectant management of vascular ring patients. This has resulted in earlier time of intervention with no increase in postoperative morbidity. This may lead to improved long-term outcomes and potentially alter the natural history for these children.

Double aortic arch: an elusive diagnosis in a child with CHD.

An 18-month-old male with pulmonary atresia and ventricular septal defect presented with stridor after neonatal systemic-to-pulmonary artery shunt surgery, that persisted on follow-up. CT angiography revealed a vascular ring with balanced double aortic arch.

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°.

Double-lumen Aortic Arch: Persistence of the Fifth Aortic Arch?

Abstract Double-lumen aortic arch is a rare congenital anomaly related to persistence of the fifth aortic arch. It may be found alone or in association with other anatomical changes of the heart. We report a case of double-lumen aortic arch associated with coarctation of the aorta and patent ductus arteriosus in a child with a congenital malformation known as the VACTERL association (vertebral defects, imperforate anus, cardiopathy, tracheoesophageal fistula, renal abnormalities and limb anomalies).

Persistent wheezing along with apparent stridor caused by a rare anomaly: double aortic arch.

Vascular rings are rare anomalies that occur as a result of abnormal development of the aortic arch complex, often manifested by signs of tracheo-oesophageal compression during the infant period. A double aortic arch anomaly is the most common type of vascular ring pathology. Here we report a case presenting with wheezing and stridor in a 10-month-old child.

Postnatal impact of a prenatally diagnosed double aortic arch.

BACKGROUND: A double aortic arch (DAA) is increasingly identified before birth; however, there are no published data describing the postnatal outcome of a large prenatal cohort. OBJECTIVE: To describe the associations, symptoms and impact of prenatally diagnosed DAA. METHODS: Retrospective review of consecutive cases seen at two fetal cardiology units from 2014 to 2019. Clinical records including symptoms and assessment of tracheobronchial compression using flexible bronchoscopy were reviewed. Moderate-severe tracheal compression was defined as >75% occlusion of the lumen. RESULTS: There were 50 cases identified prenatally and 48 with postnatal follow-up. Array comparative genomic hybridisation (aCGH) was abnormal in 2/50 (4%), aCGH was normal in 33/50 (66%) and of those reviewed after birth, 13 were phenotypically normal. After birth, there was a complete DAA with patency of both arches in 8/48 (17%) and in 40/48 (83%) there was a segment of the left arch which was a non-patent, ligamentous connection.Stridor was present in 6/48 (13%) on the day of birth. Tracheo-oesophageal compressive symptoms/signs were present in 31/48 (65%) patients at median age of 59 days (IQR 9-182 days). Tracheal/carinal compression was present in 40/45 (88%) cases. Seven of 17 (41%) asymptomatic cases demonstrated moderate-severe tracheal compression. All morphologies of DAA caused symptoms and morphology type was not predictive of significant tracheal compression (p=0.3). CONCLUSIONS: Genetic testing should be offered following detection of double aortic arch. Early signs of tracheal compression are common and therefore delivery where onsite neonatal support is available is recommended. Significant tracheal compression may be present even in the absence of symptoms.

Fetal double aortic arch: prenatal sonographic and postnatal computed tomography angiography features, associated abnormalities and clinical outcomes.

BACKGROUND: Fetal double aortic arch (DAA) malformation is a rare congenital heart disease with few reported cases in the literature. We aimed to investigate the characteristics of prenatal ultrasound and postnatal computed tomography angiography (CTA) of DAA and to describe the associated anomalies and clinical outcomes to improve prenatal diagnosis and assist in perinatal management. METHODS: The obstetric ultrasound imaging databases of seven tertiary referral centers were reviewed retrospectively to identify fetuses with a prenatal diagnosis of DAA between January 2013 and December 2018. Ultrasonographic findings, associated anomalies, genetic abnormalities, postnatal CTA images, and long-term postnatal outcomes were evaluated. RESULTS: A total of 36 cases out of 40 prenatally diagnosed DAA fetuses were confirmed by postnatal diagnosis (fetal autopsy, CTA, and surgery). In this cohort of 36 confirmed cases, 24 (67%) were isolated anomalies, while 12 (33%) were associated with intracardiac or extracardiac anomalies, and 2 (6%) had a 22q11.2 chromosome deletion. Among nine cases of pregnancy termination with a fetal autopsy, 7 had other abnormalities. Among the remaining 27 live births, 16 (59%) were asymptomatic and 11 (41%) received surgical treatment due to tracheal or esophageal compression symptoms, all with satisfactory outcomes. Prenatal echocardiography showed that DAA was mainly characterized by a bifurcation of the ascending aorta into the right and left aortic arch and the formation of a complete O-shaped vascular ring around the trachea on the three-vessel tracheal view. A variant in the aortic arch branching pattern was found for the first time. The airway obstruction, branching pattern, and atretic arch of DAA were clearly shown by postnatal CTA. CONCLUSIONS: Fetal DAA has unique features on prenatal echocardiography and postnatal CTA, and systematic prenatal examination and timely postnatal CTA evaluation are required. A certain proportion of intracardiac and extracardiac abnormalities are associated with DAA, but the probability of chromosome abnormalities is low, especially for isolated DAA.The clinical outcomes of isolated DAA are favorable, even if surgery is performed due to symptoms. Determining whether other malformations or chromosomal anomalies exist is crucial for prognosis evaluation and prenatal counseling.

Truncus Arteriosus With Double Aortic Arch.

Truncus arteriosus (TA) or common arterial trunk is a congenital cardiac anomaly having high association with arch anomalies such as right aortic arch or aortic arch interruption. However, TA with double aortic arch (DAA) is a rare occurrence. We report a case of TA with DAA where the diagnosis of DAA was missed initially.

The usefulness of intraoperative neurological monitoring for esophageal cancer with double aortic arch; a case report.

BACKGROUND: Double aortic arch (DAA) is a congenital anomaly of the aorta. Esophageal cancer with DAA is rare, and consequently, the appropriate surgical approach has not been standardized. Herein, we report the utilization of intraoperative neurological monitoring (IONM) system to preserve the function of the recurrent laryngeal nerve. CASE PRESENTATION: A 79-year-old man with esophageal cancer was diagnosed with DAA incidentally. The descending aorta was located on the right side of the thoracic vertebrae. Safe dissection of the mediastinal lymph nodes was difficult using the right transthoracic approach because of the anatomical abnormalities. During surgery, we used cervical mediastinoscopy combined with the IONM system to preserve the bilateral recurrent laryngeal nerves. Severe complications, including recurrent nerve palsy, were not observed postoperatively. CONCLUSION: IONM may be useful for evaluation of the function of the recurrent laryngeal nerve, and it would be suitable for atypical cases of esophageal cancer.

Surgical Management of Right Aortic Arch Obstruction Associated With Rare Form of Vascular Ring.

This clinical case demonstrated surgical management for a rare case of vascular ring associated with an elongated and kinked aortic arch and a right descending aorta in a ten-year-old male using an extra-anatomic bypass grafting method and dividing the vascular ring. Computer tomography performed at six-month follow-up showed a favorable surgical outcome.