5 wk old IVF twin born at 34 1/2 wks gestation weighing 2 pounds, 2 ounces. Pt not gaining weight and is only 5 pounds. Pt also has stridor since birth.

Abnormal development of complex vascular remodeling during embryogenesis results in malformations and vascular rings Vascular rings Complete Double aortic arch R aortic arch with aberrant L subclavian a. and L ductus arteriosus Incomplete Aortic arch anomalies account for 1-3% of congenital heart disease Males have x greater risk Double aortic Arch – Vascular Ring

Symptoms Complete vascular rings are more often associated with significant symptoms Signs of increased respiratory effort (nasal flaring, retractions, tachypnea, intermittent cyanosis) Some infants will lie with their back arched and neck extended to minimize tracheal compression Stridor, wheezing, course upper airway sounds Recurrent respiratory infections Esophageal compression symptoms (dysphagia, feeding difficulty, vomiting)

Associated Anomalies (up to 50%) 2 nd cardiac lesion (VSD, tetralogy, coarcs, PDA) TE fistula, cleft palate, subglottic stenosis Genetic and malformation syndromes DiGeorge, Down, CHARGE

Diagnosis X-ray Echocardiogram to evaluate for other cardiac abnormalities MRI/MRA to define the vascular anatomy Rx Surgical division (low risk) Thoracotomy Division point made in smaller of 2 arches, usually the L Video-assisted thoracoscopic Endoscopic robotic assisted techniques

Pt had a vascular ring correction and is now doing well at 6 months.