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Interruption of coronary artery — Clinical Guidelines

Overview

Interruption of the aortic arch (IAA) is a congenital anomaly characterized by an abnormal termination of the aortic arch, often associated with other congenital heart defects and chromosomal abnormalities, particularly deletions in chromosome 22q11. 1

Diagnosis

Prenatal Diagnosis: Feasible via fetal echocardiography, distinguishing between type A and type B IAA. 1

Echocardiography: Essential for postnatal confirmation and differentiation of IAA types. 1

Genetic Testing: Fluorescent in situ hybridization (FISH) for 22q11 microdeletion recommended, especially in type B IAA cases. 1

Neuroradiological Imaging: Useful in assessing associated neurological complications, such as myelopathy and aneurysms. 2

Management

Palliative Surgery: Establishment of continuity between the pulmonary artery and descending thoracic aorta using a prosthetic graft, with pulmonary artery banding and ductus arteriosus ligation. 3

Hemodynamic Correction: Surgical intervention aimed at correcting hemodynamic abnormalities, such as bypass surgery from ascending aorta to abdominal aorta. 2

Timing of Total Correction: Initial palliative procedures in neonates with planned definitive correction at a later date to reduce morbidity and mortality. 3

Special Populations

Pregnancy: Prenatal detection and genetic counseling for 22q11 microdeletions are crucial. 1

Pediatrics: Early surgical intervention is critical, with a focus on palliative measures followed by definitive correction. 2 3

Comorbidities: Neurological complications require multidisciplinary management, including surgical correction of hemodynamic abnormalities. 2

Key Recommendations

Perform prenatal echocardiography and genetic testing (FISH for 22q11 microdeletion) for accurate diagnosis and risk stratification. (Evidence: Moderate) 1

Initiate palliative surgical interventions in neonates with IAA to establish hemodynamic stability, planning for definitive correction later. (Evidence: Weak) 3

Address associated neurological complications through comprehensive neuroradiological assessment and surgical correction of hemodynamic abnormalities when indicated. (Evidence: Weak) 2

References

1 Volpe P, Marasini M, Caruso G, Gentile M. Prenatal diagnosis of interruption of the aortic arch and its association with deletion of chromosome 22q11. Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology 2002. link 2 Ling F, Bao YH. Myelopathy and multiple aneurysms associated with aortic arch interruption: case report. Neurosurgery 1994. link 3 Jones EL, Plauth WH, Hatcher CR. A palliative operation for all types of aortic arch interruption in the neonate. The Journal of thoracic and cardiovascular surgery 1975. link

Interruption of coronary artery