Case report: Pulmonary hypertensive crisis leading to cardiac arrest during endoscopic evaluation in a 6-year-old boy with autism, severe malnutrition, and undiagnosed scurvy

Abstract

Pediatric gastroenterologists are often responsible for the evaluation of malnutrition in the setting of selective eating. Endoscopic evaluation for conditions including eosinophilic esophagitis and celiac disease can help to identify and treat mucosal disease contributing to food selectivity. However, undiagnosed micronutrient deficiencies can cause cardiovascular derangements that significantly increase a patient's anesthetic risk. Vitamin C deficiency in particular, alone or in combination with severe malnutrition, is associated with a severe but reversible form of pulmonary arterial hypertension that, while life threatening in the acute phase, may significantly improve within days of starting ascorbic acid replacement therapy. Here we present a case of a 6-year-old boy with autism spectrum disorder (ASD), severe malnutrition, and undiagnosed chronic vitamin C deficiency who developed a pulmonary hypertensive crisis after induction of general anesthesia leading to cardiac arrest during endoscopic evaluation. While the association between food selectivity among youth with neurodevelopmental differences and vitamin C deficiency is well-described, and pulmonary hypertension is a recognized rare complication of scurvy, extant literature has not addressed next steps to improve patient outcomes. Using this case report as a foundation, we discuss specific patient populations to screen and treat for micronutrient deficiencies prior to anesthesia and propose a novel clinical algorithm for pre-anesthesia risk stratification and mitigation in patients specifically at risk for scurvy and associated pulmonary hypertension.

Keywords: ARFID; autism; malnutrition; micronutrient deficiencies; pulmonary hypertension (PAH); risk stratifcation; scurvy; vitamin C deficiency.

Figure 1

Representative images from echocardiograms obtained during the patient's course. (A) Immediately after cardiac arrest while on ECMO. Left panel: Apical 4-chamber view demonstrates right ventricular dilation (double headed arrows) and hypertrophy (single headed arrows). Right panel: Increased peak velocity of the tricuspid valve regurgitation jet indicates elevated right ventricular pressure. (B) Four weeks after cardiac arrest – on sildenafil. Left Panel: Parasternal short axis image demonstrates normal end-systolic septal position (indicated by circle), normal right ventricular size, and mild right ventricular hypertrophy. Right Panel: Normal peak velocity of the tricuspid valve regurgitation jet indicates normal right ventricular pressure. (C) Nine months after cardiac arrest – off sildenafil. Left Panel: Parasternal short axis image demonstrates normal end-systolic septal position (indicated by circle), normal right ventricular size, and no right ventricular hypertrophy. Right Panel: Normal peak velocity of the tricuspid valve regurgitation jet indicates normal right ventricular pressure.

Figure 2

Timeline of case.

Figure 3

Approach to pre-anesthesia evaluation in patients at risk for vitamin C deficiency.