An Orange a Day Keeps the Doctor Away: Scurvy-Induced Pulmonary Hypertension

Abstract INTRODUCTION: Severe vitamin C deficiency has been reported rarely in the literature as a cause of a reversible form of pulmonary hypertension (PH). We review its presenting symptoms, evaluation, and treatment through two pediatric cases with a novel discussion regarding the timeline of recovery and potential benefits of screening in an at-risk population. DESCRIPTION: Case 1: A 4-year-old male with autism presented with a new cough and dyspnea in the setting of a 1-month refusal to bear weight. He was admitted for hypoxia requiring support via high-flow nasal cannula. Initial work-up demonstrated B-type natriuretic peptide (BNP) 2,109 pg/mL and troponin 1.36 ng/mL. Radiographic findings were notable for osteopenia of the hips and spine. Transthoracic echocardiogram (TTE) demonstrated elevated peak tricuspid valve regurgitation (TR) gradient and severely elevated right ventricular systolic pressure (RVSP) estimate. Inhaled nitric oxide (iNO) and milrinone infusion were initiated. After extensive work-up, the patient's limited diet of chicken nuggets and protein bars prompted consideration of nutrition-related etiologies, and empiric IV ascorbic acid was started. Case 2: A 4-year-old male with non-verbal autism and a 3-week history of refusal to bear weight was admitted following hemodynamic decompensation requiring intubation during sedation for a pelvic MRI. Physical exam was notable for swollen, erythematous gums and lower extremity petechiae. Workup was notable for maximum BNP 1,599 pg/mL and troponin 0.12 ng/mL. Radiographic findings demonstrated mild, diffuse osteopenia of bilateral lower extremities. TTE was significant for elevated peak TR gradient and moderately elevated RVSP estimate. He was started on iNO and milrinone infusion for PH management. His diet was noted to be limited to graham crackers and plain yogurt, so he was empirically started on IV ascorbic acid. Each case was found to have severe vitamin C deficiency with levels <5mcmol/L. Within two days of ascorbic acid supplementation, both cases had normalization of their TTEs and BNP levels off all PH-specific medication. They were both discharged on enteral vitamin C supplementation without the need for PH-specific therapy. DISCUSSION: When patients present with new-onset and severe PH, vitamin C level is not readily obtained. These two cases demonstrate the role of considering vitamin C deficiency when assessing patients with PH, since supplementation in both cases led to rapid reversal of PH with normalization of echocardiography within two days. Given these cases, we postulate that regular vitamin C screening in diet-restrictive individuals could decrease morbidity and ultimately be lifesaving.