Surgical Management of a Neonate with Congenitally Corrected Transposition of the Great Vessels, Hypoplastic Right Aortic Arch, and Ebstein Anomaly

Filippelli, Sergio; Perri, Gianluigi; Kirk, Richard; Hasan, Asif; Griselli, Massimo

doi:10.1111/jocs.12200

Cited by 2 publications

(2 citation statements)

References 7 publications

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“…The modified Blalock Taussig shunt is sometimes used as a part of surgical treatment in severely symptomatic neonates with Ebstein anomaly [43]. In cases where aortic arch courses on the right, the construction of the modified Blalock Taussig shunt may be difficult and thus application of further modifications or even alternative surgical methods may be needed [20,44].…”

Section: Discussionmentioning

confidence: 99%

“…Associated cardiac anomalies include pulmonary stenosis, pulmonary atresia, ventricular septal defect, cardiomyopathies, coarctation of aorta, bicuspid aortic valve, mitral valve prolapse, and other rarer abnormalities [2,13,[15][16][17][18][19]. Among associated cardiac anomalies, right aortic arch is extremely rare and only few such cases have been described in the literature so far [1,[20][21][22]. Non-cardiac malformations or genetic syndromes are found in about 19-22% of patients with Ebstein anomaly.…”

Section: Introductionmentioning

confidence: 99%

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Ebstein Anomaly and Right Aortic Arch in Patient with Charge Syndrome

Lubaua

Teraudkalna

2021

Medicina

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Ebstein anomaly is a rare congenital heart disease characterized by a varying degree of anatomical and functional abnormalities of tricuspid valve and right ventricle. It often coexists with other congenital cardiac malformations. Up to 79–89% of patients with Ebstein anomaly have interatrial communication in the form of patent oval foramen or atrial septal defect and more than one-third has other types of cardiac malformations. Association between Ebstein anomaly and right aortic arch is extremely rare and only few cases have been described in the literature so far. Much rarer than with other cardiac malformations, Ebstein anomaly is associated with non-cardiac malformations or genetic syndromes. Several cases of association between Ebstein anomaly and Charge syndrome have been reported, nevertheless, Ebstein anomaly accounts for less than 1% of cardiac defects seen in patients with Charge syndrome. In this case report, we present a unique case of a patient with Charge syndrome where both Ebstein anomaly and right aortic arch are present. The diagnosis of Ebstein anomaly and right aortic arch was established prenatally. In the first years of life, the patient did not exhibit any remarkable symptoms. However, over time, deterioration of right ventricle function and increased tricuspid regurgitation were observed, requiring consideration of surgical treatment at the age of five. In addition, delay in physical, motor, and mental development was observed and thus, at the age of five, the patient was consulted by a medical geneticist and a gene panel to test for structural heart defects was ordered. The test showed a mutation in chromodomain helicase DNA binding protein 7 (CHD7) gene, which, along with clinical features, allowed to establish a diagnosis of Charge syndrome. To the best of the authors’ knowledge, this is the first case report of a patient with Charge syndrome, Ebstein anomaly, and right aortic arch that has been described in the literature.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Ebstein Anomaly and Right Aortic Arch in Patient with Charge Syndrome

Lubaua

Teraudkalna

2021

Medicina

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Combined Norwood and Modified Left-Sided Starnes Procedures for Congenitally Corrected Transposition of the Great Arteries

Vaidya,

Karim,

Miyairi

et al. 2024

World J Pediatr Congenit Heart Surg

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The combination of congenitally corrected transposition of the great arteries (ccTGA), Ebstein anomaly of the systemic atrioventricular valve, and critical systemic ventricular outflow tract obstruction is extremely rare. Management of a neonate with these lesions in cardiogenic shock is quite challenging. Tricuspid valve exclusion has been increasingly and successfully used to manage neonatal Ebstein anomaly patients. We extended this idea of tricuspid valve exclusion (modified Starnes) combined with a Norwood Stage 1 operation with a modified systemic to pulmonary artery shunt to successfully manage a neonate with ccTGA, severe systemic atrioventricular valve regurgitation, and critical ventricular outflow tract obstruction.

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Surgical Management of a Neonate with Congenitally Corrected Transposition of the Great Vessels, Hypoplastic Right Aortic Arch, and Ebstein Anomaly

Cited by 2 publications

References 7 publications

Ebstein Anomaly and Right Aortic Arch in Patient with Charge Syndrome

Ebstein Anomaly and Right Aortic Arch in Patient with Charge Syndrome

Combined Norwood and Modified Left-Sided Starnes Procedures for Congenitally Corrected Transposition of the Great Arteries

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