Page 24 - Delaware Medical Journal - September 2017
P. 24
Refractory Dyspnea in an Adolescent Treated for Asthma
Abigail Strang, MD; Anne Marie Daly, DO; Michael McCulloch, MD
CASE REPORT
A 17-year-old female presented to a local ED following an eight-month history of dyspnea. Her symptoms prompted visits to her primary care doctor on multiple occasions, and she was treated unsuccessfully with asthma and allergy medications, including inhaled albuterol and beclamethasone, cetirizine, and montelukast. Her other current long-term medications included
oral contraceptives for dysmenorrhea and methylphenidate for ADHD. The patient had a remote history of wheezing with viral illnesses as a young child with minimal respiratory symptoms in the few years prior to the onset of dyspnea.
with albuterol, there was no change in symptoms with other medication trials. Her dyspnea was worse with activity and had
progressed to the point that she was unable
elevator at school. Although she had never participated in competitive athletics, prior to the onset of symptoms she was able to participate marginally in regular gym class and routine cardiovascular activities for age. Prior to this ED visit, no outpatient work-up was performed for these symptoms.
In the ED, the patient had normal room-air oxygen saturations and clear lungs. There was no clubbing or lower extremity swelling noted. An EKG showed a right QRS-axis deviation and right ventricular hypertrophy with a strain pattern, prompting further evaluation (Image 1). Echocardiogram revealed an intact atrial septum, right atrial and ventricular dilation with decreased right ventricular function, tricuspid regurgitation, and suprasystemic right ventricular
systolic pressures. She was admitted to a
pediatric hospital for further evaluation, including a cardiac catheterization,
which showed severely diminished right ventricular systolic and diastolic function and severely elevated pulmonary vascular resistance with minimal vasoreactivity
to oxygen and nitric oxide. Pulmonary function testing was normal and did not suggest underlying persistent asthma.
Chest CT (Image 2) and lung perfusion testing did not show signs of pulmonary emboli or chronic interstitial lung disease. Laboratory studies were negative for evidence of infection, hypercoaguable state, and collagen-vascular diseases. Polysmonography did not show evidence of sleep apnea or hypoventilation, although oxygen saturations were low-normal
while sleeping. Genetic testing revealed a heterozygous mutation in the BMPR2 gene (R213X) consistent with familial pulmonary arterial hypertension.
IMAGE 1
Electrocardiogram showing right-axis QRS deviation, right ventricular hypertrophy, and a right ventricular strain pattern.
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Del Med J | September 2017 | Vol. 89 | No. 9
