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Congenital atresia of aortic valve — Clinical Guidelines

Overview

Congenital aortic valve atresia involves the absence of the aortic valve, often associated with hypoplastic left heart syndrome and other cardiac anomalies such as ventricular septal defects and coarctation of the aorta. 2 3 4

Diagnosis

Key Anatomic Features: Absence of aortic valve, often with hypoplastic left ventricle, and frequently associated with ventricular septal defects (VSD) in some cases 3.

Associated Anomalies: Coarctation of the aorta present in 75% of cases, typically proximal to the ductus arteriosus 4.

Imaging Techniques: Angiography reliably identifies coarctation associated with aortic atresia 4.

Cardiac Catheterization: Essential for detailed anatomic assessment and planning surgical interventions 2.

Management

Surgical Repair: Primary surgical palliation focusing on reconstructing the aortic arch and addressing associated defects like VSD and coarctation 2 3.

Right Ventricular Function: Selection of patients with thicker right ventricular walls for surgery may improve outcomes 2.

Specific Approaches: Tailored surgical techniques based on morphologic features, such as subpulmonary or perimembranous VSDs, to enhance surgical correction feasibility 3.

Special Populations

Turner Syndrome: Patients with Turner syndrome may present with additional coronary anomalies like fistulas, requiring careful intraoperative assessment 1.

Pediatric Considerations: Management heavily relies on pediatric cardiac surgical expertise due to the complexity and variability of associated anomalies 2 3 4.

Key Recommendations

Assess Right Ventricular Wall Thickness: Prioritize surgical intervention in infants with thicker right ventricular walls to improve short-term and long-term outcomes (Evidence: Moderate 2).

Evaluate for Coarctation of the Aorta: Routinely investigate for coarctation using angiography to guide surgical reconstruction extent (Evidence: Moderate 4).

Consider Morphologic Variants: Tailor surgical approaches based on specific anatomic features, such as VSD location, to optimize surgical correction feasibility (Evidence: Weak 3).

References

1 Horne D, Morris SA, Colquitt JL, Molossi SM, McKenzie ED. Incidental Finding of Right Coronary Artery to Pulmonary Artery Fistula During Surgical Repair of Aortic Arch Atresia in Turner Syndrome. World journal for pediatric & congenital heart surgery 2017. link 2 Hawkins JA, Doty DB. Aortic atresia: morphologic characteristics affecting survival and operative palliation. The Journal of thoracic and cardiovascular surgery 1984. link 3 Thiene G, Gallucci V, Macartney FJ, Del Torso S, Pellegrino PA, Anderson RH. Anatomy of aortic atresia. Cases presenting with a ventricular septal defect. Circulation 1979. link 4 Von Rueden TJ, Knight L, Moller JH, Ewards JE. Coarctation of the aorta associated with aortic valvular atresia. Circulation 1975. link

Congenital atresia of aortic valve