Aortic Valve Replacement in an Adult 20 Years after a Single-Stage Extra-Anatomic Repair of an Interrupted Aortic Arch

Introduction

Interrupted aortic arch is a rare congenital malformation that occurs in 3 cases per million [[1]]. This anomaly is defined as a loss of luminal continuity between the ascending and the descending aorta, and there is usually a considerable distance between the ascending and descending parts of the aorta [[2]]. A classification system, based on the location of the interrupted segment, was introduced by Celoria and Patton in 1959. In type A (30 - 40 %), the interruption lies distal to the left subclavian artery. Type B (53 %) is the most common type with an interruption between the left subclavian artery and the left common carotid artery. In rare type C (4 %), the interruption is proximal to the left common carotid artery. The natural history is very poor, 80 % of children die within the first month if untreated [[3]].

We describe a single-stage, extra-anatomic repair in an adolescent patient with a type C interrupted aortic arch and his subsequent aortic valve replacement 19 years later.

References

• 1 Conova C R, Carrel T, Dubach P, Turina M, Reinhart W H. Interrupted aortic arch: fortuitous diagnosis in a 72-year-old female patient with severe aortic insufficiency [in German].  Schweiz Med Wochenschr. 1995;  125 26-30
• 2 Backer C L, Mavroudis C. Congenital Heart Surgery Nomenclature and Database Project: patent ductus arteriosus, coarctation of the aorta, interrupted aortic arch.  Ann Thorac Surg. 2000;  69 (Suppl 4) S298-S307
• 3 Celoria G C, Patton R B. Congenital absence of the aortic arch.  Am Heart J. 1959;  58 407-413
• 4 Goldmuntz E, Clark B J, Mitchel L E, Jawad A F, Cuneo B F, Reed L. Frequency of 22q11 deletions in patients with conotruncal defects.  J Am Coll Cardiol. 1998;  32 492-498