Cardiovascular Disease |

An Unusual Presentation of Aortic Coarctation in a 33-Year-Old Navajo Sheep Herder FREE TO VIEW

Drew Harris, MD; Salerno Amy, MD
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Yale University, New Haven, CT

Chest. 2014;146(4_MeetingAbstracts):106A. doi:10.1378/chest.1991716
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SESSION TITLE: Cardiovascular Global Case Reports

SESSION TYPE: Global Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Aortic coarctation (CoA) is the fifth most common congenital heart defect with an estimated incidence of 1 in 2500 births. CoA is typically diagnosed and corrected in early childhood. Unrepaired or undiagnosed CoA presenting during adult life is rare and the mean life expectancy of patients with CoA is 31 years.1 Most patients who survive into adulthood present with cardiovascular complications. We report a case of a 33 year old Navajo male presenting in severe sepsis from a lower extremity abscess who was subsequently diagnosed with CoA.

CASE PRESENTATION: A 33 year old male with a childhood diagnosis of bicuspid aortic valve (BAV) and untreated Diabetes Mellitus (A1C 11%) was admitted to an Indian Health Service hospital with an infected ulcer on his right lower extremity (RLE). He presented to the hospital with progressive erythema, swelling and pain of his RLE after 3 days on his feet herding sheep. He was febrile to 38.6C and had equal blood pressures in both arms (135/60 on the right, 138/65 on the Left). He had a 3cm fluctuant ulcer on the lateral aspect of his foot with blanching erythema and tenderness. Pedal pulses were not palpable nor audible with doppler. Subsequently a right thigh blood pressure was noted to be 115/55 and he had a significant radio-femoral pulse delay. He was admitted to the intensive care unit with severe sepsis and acute kidney injury. After undergoing an operative debridement, his sepsis resolved with intravenous fluid and broad spectrum antibiotics. MRA of his thorax revealed coarctation of the proximal descending thoracic aorta which resulted in focal stenosis of 70%. An echocardiogram confirmed a bicuspid aortic valve without stenosis or regurgitation. He was referred to a tertiary center for repair of his CoA.

DISCUSSION: CoA is a congenital lesion usually diagnosed in childhood and often coexisting with concomitant congenital heart defects, most commonly BAV. Most infants born with CoA develop heart failure following closure of the ductus arteriosus and require intervention for survival. Survival past infancy without intervention is due to less severe narrowing or the development of collateral circulation that bypasses the coarctation. The natural history of unrepaired CoA is dismal with the mean age of death 31 years.1 Mortality is usually from hypertensive related cardiovascular disease including heart failure, cerebral hemorrhage or aortic rupture. Less commonly, patients present with claudication.2 We present a case of adult onset CoA with an unusual preceding diagnosis: sepsis from an infected lower extremity ulcer. It is likely that in addition to uncontrolled diabetes, impaired blood flow distal to the coarctation promoted the development of infection in this patient. Although the presentation of a diabetic foot infection would not typically warrant a CoA workup, this patient’s exam findings and history of BAV increased the index of suspicion. Given that 25-75% of patients with a BAV have coexisting CoA,3 clinicians should maintain a high index of suspicion for CoA and consider routine screening with aortic imaging in patients with presumptive isolated BAV.

CONCLUSIONS: Adult patients with uncorrected CoA typically present with cardiovascular disease complications, however atypical presentations such as an infected lower extremitiy ulcer can occur. Clinicians should maintain a high index of suspicion for CoA and consider routine screening with aortic imaging in patients with presumptive isolated BAV regardless of the patient’s initial presentation.

Reference #1: Kenny D and Hijazi Z. Coarctation of the aorta: From fetal life to adulthood. Cardiology Journal 2011, vol18, No 5, 487-495.

Reference #2: Fitzpatrick CM, Clouse WD, Eliason JL, Gage K, Podberesky DJ, Bush DM. Infrarenal aortic coarctation in a 15-yr-old with claudication. J Vasc Surg 2006;44:1117.

Reference #3: Tanous D, Benson LN and Horlick EM. Coarctation of the aorta: evaluation and management. Current Opinion in Cardiology 2009, 24: 509-515.

DISCLOSURE: The following authors have nothing to disclose: Drew Harris, Salerno Amy

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