Research Day

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Introduction: Pulmonary arterial hypertension (PAH) is characterized as average pulmonary artery pressure of greater than 25mm Hg at rest. Childhood idiopathic pulmonary hypertensions (CIPH) is distinguishable from PAH by the absence of family history or underlying disease. Symptoms of CIPH include dyspnea, exercise limitation, and syncope. Common organ-specific changes include right ventricular hypertrophy and right ventricular strain. We present the case of a 6-year-old girl presenting with idiopathic pulmonary arterial hypertension aggravated by esophageal intubation. Methods: This case is from the files of one of the authors, a forensic pathologist. Case Report and Discussion: Diagnosed with pulmonary arterial hypertension when she was six years old, this patient had a history of syncope on exertion. She was readmitted to the hospital shortly afterward with seizures secondary to pulmonary hypertensive crisis and hypoxia. Two months later, after another seizing episode, the patient’s mother called EMS, who began chest compressions at the scene. While under EMS care, the patient was intubated and subsequently became pulseless and asystolic. She was pronounced dead in the Emergency Department. The case was then referred to the coroner. Autopsy findings include marked right ventricular dilation and hypertrophy and lung microscopic features consistent with CIPH. The endotracheal tube was found with its tip in the distal esophagus with the cuff inflated and surrounded by blanched underlying mucosa, all indicative of esophageal intubation, which was considered a contributory cause of death. The case serves as an excellent example of CIPH. In addition, the esophageal intubation allows for discussion of complications related to medical procedures.



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