Natural History of Asymptomatic and Unrepaired Vascular Rings: Is Watchful Waiting a Viable Option? A New Case and Review of Previously Reported Cases

Abstract

Vascular rings are a rare form of congenital heart disease in which abnormal aortic arch anatomy leads to encircling of the esophagus and/or trachea by the aortic vasculature. Symptoms can develop from this and prompt the need for surgery. A natural history study has been done on mildly symptomatic patients but no such study has been done on asymptomatic patients. We present a case report of three children with asymptomatic vascular rings who continue to receive follow-up without intervention and review all published cases of asymptomatic or unrepaired vascular rings. Clinical observation of asymptomatic and mildly symptomatic vascular rings, regardless of aortic arch anatomy, seems to be a safe approach. Children with mild symptoms almost invariably seem to have resolution of their symptoms by four years of age.

Case of an octogenarian with an asymptomatic double aortic arch undergoing CABG

“…Surgical repair of vascular rings is not typically indicated unless the patient is symptomatic or imaging suggests aneurysmal changes [4] . Some sporadic cases in which surgical treatments were performed in symptomatic adult patients have been reported.…”

Hybrid repair of an adult with a double aortic arch, coarctation of the aorta, and left subclavian artery aneurysm

“…Compression and palsy of the recurrent laryngeal nerve, called Ortner syndrome, leads to hoarseness 2 . Surgical repair of vascular rings is not typically indicated unless the patient is symptomatic or imaging suggests aneurysmal changes 3 . Dysphagia was resolved after obliteration of the nondominant aortic arch.…”

Asymptomatic Balanced Double Aortic Arch Associated With Tetralogy of Fallot

“…resolve by the age of four years. 7 On the other hand, Pandit et al report that asymptomatic DAA with TOF is extremely rare and should be operated to prevent long-term respiratory or gastrointestinal complications. 8 In our case, a two-year-old child presented for TOF repair had asymptomatic balanced DAA with mild left bronchial compression diagnosed incidentally on imaging.…”