Isolated Vascular Rings Are Common Cardiovascular Malformations

Evans, William N.; Acherman, Ruben J.; Ciccolo, Michael L.; Lehoux, Juan; Berthoty, Dean; Montes, A. López; Mayman, G. A.; Restrepo, Humberto

doi:10.1177/21501351221122972

Cited by 7 publications

(5 citation statements)

References 14 publications

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“…Published data estimate the prevalence of a VR at about 7 per 10,000 live births (1). The incidence of the DAA in the literature is 1.5 per 10,000.…”

Section: Discussionmentioning

confidence: 98%

Case Report: An unusual case of a transposition of the great arteries with a double aortic arch: a highly complex fetal diagnosis with an unpredictable outcome

Masci,

Missineo,

Campanale

et al. 2024

Front. Cardiovasc. Med.

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Published data estimate the prevalence of the vascular ring at approximately 7 per 10,000 live births. The association of a double aortic arch with a D-transposition of the great arteries has been rarely described in the literature. In this study, we report the prenatal diagnosis of a 28-year-old woman. A fetal echocardiography at a gestational age of 24 weeks + 6 days showed a D-transposition of the great arteries and a double aortic arch with a ventricular septal defect and pulmonary stenosis. On the first night after birth, the baby experienced an increase in lactate levels, with the rate of oxygen saturation consistently below 80%. A few hours after birth, the patient underwent a Rashkind procedure. An echocardiography, CT chest x-ray, and CT angiogram confirmed a diagnosis with a severe reduction of the tracheal lumen (>85%) and bronchomalacia. Then, the patient underwent posterior tracheopexy and aortopexy and later an arterial switch operation, ventricular septal defect closure, and resection of a part of the infundibular septum, accepting the risk of potential neoaortic obstruction. The literature has reported only two cases of patients with a fetal echocardiogram diagnosis. Therefore, our patient is only the third one with a fetal diagnosis and the second one with a complex intracardiac anatomy, characterized not only by a ventricular septal defect but also by two separate components of the obstruction (a bicuspid valve and a dysplastic valve with a posterior deviation of the infundibular septum). In conclusion, a D-transposition of the great arteries with a double aortic arch remains an extremely unusual association. The clinical outcome of these patients presents a high degree of variability and is entirely unpredictable in prenatal life. Our greatest aim as fetal and perinatal cardiologists is to improve the management and outcome of these patients through a fetal diagnosis, recognizing types of congenital heart disease in newborns who require early neonatal invasive procedures.

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“…Published data estimate the prevalence of a VR at about 7 per 10,000 live births (1). The incidence of the DAA in the literature is 1.5 per 10,000.…”

Section: Discussionmentioning

confidence: 98%

Case Report: An unusual case of a transposition of the great arteries with a double aortic arch: a highly complex fetal diagnosis with an unpredictable outcome

Masci,

Missineo,

Campanale

et al. 2024

Front. Cardiovasc. Med.

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“…With the rich experience of prenatal diagnosis, the use of three-vessel tracheotomy for the diagnosis of vascular ring diseases has been widely recognized in recent years [ 14 ]. It has been reported that the prenatal detection rate of isolated vascular rings has increased from 2.4 to 10,000 to 7 per 10,000 [ 8 ]. But there are still misdiagnoses and missed diagnoses of vascular rings.…”

Section: Discussionmentioning

confidence: 99%

“…Early diagnosis of congenital vascular rings is intended to improve the symptoms of compression in patients with airway compression [ 19 ]. With the increase of prenatal detection rate of vascular rings, the incidence of rare diseases has been realized to be twice that of tetralogy of Fallot [ 8 ], and the evaluation of the relationship between vascular rings and airways is more important. However, there are few articles on the prenatal diagnosis of vascular ring diseases and with qualitative or quantitative assessments of airway prognosis in PubMed [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

“…Prenatal diagnosis of vascular ring is relatively difficult. In recent years, the value of the three-vessel tracheal view in fetal echocardiography in the diagnosis of vascular ring diseases has been recognized [ 6 – 8 ], but the guidelines state that only fetuses with high risk factors receive specialized fetal echocardiography. Most fetuses receive only a basic cardiac examination, and the success of detection depends on the operator’s experience and knowledge of the disease [ 9 ].…”

Section: Introductionmentioning

confidence: 99%

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Vascular ring: prenatal diagnosis and prognostic management based on sequential cross-sectional scanning by ultrasound

Zhou

et al. 2023

BMC Pregnancy Childbirth

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Background In terms of embryonic origin, vascular ring is a congenital anomaly in which the aortic arch and its branches completely or incompletely encircle and compress the trachea or esophagus. Early and accurate diagnosis of a vascular ring is the key to treatment. Prenatal diagnosis mainly relies on fetal echocardiography, but the rate of missed diagnosis and misdiagnosis is still very high, and the prognosis has not been evaluated. The aim of this study was to investigate the accuracy of prenatal diagnosis and to evaluate the prognosis semi-quantitatively according to the shape of the ring and the distance between the vessel and the trachea. Methods From 2019 to 2021, 37,875 fetuses underwent prenatal ultrasound examination in our center. All fetal cardiac examinations were performed using the fetal echocardiography method proposed by the American Institute of Ultrasound in Medicine (AIUM) combined with dynamic sequential cross-sectional observation (SCS). For SCS, the standard abdominal section was taken as the initial section, and the probe was moved cephalically along the long axis of the body until the superior mediastinum had disappeared. If a vascular ring was found, the shape of the ring and the distance of the branch to the airway were observed. The distance relationship with the airway was divided into three grades: I-III; the closer the distance, the lower the grade. The vascular rings were monitored every 4 weeks before birth. All were monitored before surgery or 1 year after birth. Results A total of 418 cases of vascular rings were detected. There was no missed diagnoses or misdiagnoses by SCS. The vessels formed different shaped rings according to their origin and route. Grade I, “入” and “O” rings have a poor prognosis and are associated with the highest risk of respiratory symptoms. Conclusions SCS can accurately diagnose vascular rings before delivery, evaluate the shape and size of the rings to conduct prenatal monitoring of children until birth, which plays a guiding role in airway compression after birth.

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“…Vascular rings present with a frequency as high as seven in 10,000 live births. 1 Contemporary fetal detection and use of computed tomography (CT) have led to increased early detection of these lesions and earlier repair in many centers. 2 Right aortic arch with aberrant left subclavian artery (RAA-ALSA) is a common form of vascular ring (15%-25%), and different surgical techniques have been proposed to repair the lesion.…”

Section: Introductionmentioning

confidence: 99%

Association and Repair of Right Aortic Arch With Aberrant Left Subclavian Artery With Subclavian Stenosis

Cheong,

Jhaveri,

Smerling

et al. 2023

World J Pediatr Congenit Heart Surg

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Surgical repair of right aortic arch and aberrant left subclavian artery has traditionally involved ligamentum division. Such patients can have stenosis at the origin of the aberrant subclavian artery either at the time of presentation or later. The more recently popularized repair involving resection of Kommerell diverticulum with transfer of the subclavian artery to the left carotid artery allows resection of the stenotic segment and serves as an effective treatment for subclavian stenosis as well. We present three cases of early repair of this arch anomaly with associated subclavian stenosis repaired successfully in that manner.

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Isolated Vascular Rings Are Common Cardiovascular Malformations

Cited by 7 publications

References 14 publications

Case Report: An unusual case of a transposition of the great arteries with a double aortic arch: a highly complex fetal diagnosis with an unpredictable outcome

Case Report: An unusual case of a transposition of the great arteries with a double aortic arch: a highly complex fetal diagnosis with an unpredictable outcome

Vascular ring: prenatal diagnosis and prognostic management based on sequential cross-sectional scanning by ultrasound

Association and Repair of Right Aortic Arch With Aberrant Left Subclavian Artery With Subclavian Stenosis

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