Hypertension in a Pediatric Patient With Repeat Aortic Coarctation Repair


Introduction

Coarctation of the aorta comprises approximately 5% to 8% of congenital heart defects and is often associated with valvular malformations. These defects are typically diagnosed early and are managed with surgical repair, balloon angioplasty, or endovascular stent placement. However, as the following case illustrates, complications can occur in this population despite early intervention.

Case Report

A 15-year-old male adolescent presented to the pediatric ED after repeated blood pressure (BP) checks by the school nurse revealed consistently elevated systolic and diastolic pressures. The patient’s hypertension was associated with symptoms of intermittent headache and light-headedness. His medical history was remarkable for a congenital aortic coarctation and a bicuspid aortic valve. The patient had undergone a subclavian flap repair prior to 1 month of age, followed by a balloon dilatation 1 year later for recurrent coarctation. The rest of the patient’s medical history was unremarkable, including normal renal function. He denied illicit drug or alcohol use, sexual activity, or trauma. On evaluation, the patient’s cardiac examination revealed a regular rate and rhythm with normally split S2; there were no rubs, murmurs, or gallops on auscultation. He had normal and equal pulses of the upper and lower extremities bilaterally. The patient presented without cyanosis. He was alert and oriented with normal upper and lower extremity reflexes. The neurological examination, including cranial nerve, strength, and gait testing, was unremarkable. The gastrointestinal examination showed a soft, nondistended abdomen, with no pulsatile masses. There was no abnormal swelling of his extremities.

Diagnosis

Although the physical examination findings were unremarkable, the patient’s vital signs were concerning as BP in his right upper extremity was as high as 208/110 mm Hg, while BP in his right leg was 130/68 mm Hg.

Treatment

The patient was given oral acetaminophen for his headache, after which there was significant improvement in his upper extremity BP. His chemistry panel, including creatinine levels, liver function, and urine electrolytes, were all within normal values. An electrocardiogram was significant for left ventricular hypertrophy without arrhythmia or conduction abnormalities. After discussion with the patient’s cardiologist, he was discharged home in the care of his parents, who were instructed to have close outpatient follow-up with the cardiologist and and undergo advanced imaging studies. The patient followed up with his cardiologist, who ordered cardiac magnetic resonance imaging (MRI). The MRI showed mild narrowing of the distal aortic arch with a minimal and clinically insignificant pressure gradient. Based on the MRI findings, the patient was referred to a pediatric nephrologist, who performed a 24-hour ambulatory BP evaluation. The results of this study showed the patient to have systolic hypertension at the 95th percentile for his age and height. Based on the patient’s athletic predilection, β-blockers were avoided, and he was instead started on the angiotensin-converting enzyme (ACE) inhibitor lisinopril, along with annual follow-up cardiac evaluation.

Discussion

The authors’ initial concern for this patient was the possibility of a recurrent coarctation causing a significant pressure gradient between the upper and lower extremities with associated symptoms. A review of the literature demonstrates such an occurrence is not uncommon in this patient population, especially in patients with a history of early intervention (ie, within the first year of life).

Keywords

Coarctation of aorta, hypertension

Author : Devin Smith, et al.