Abstract
Following advances in medical care, the rate of survivors with congenital heart disease has increased, including those who were previously considered inoperable due to frailty. Today, various treatment strategies, including surgery and medical therapy are available depending on the underlying condition. We report a case of unrepaired congenital heart disease associated with chromosomal abnormalities that required intensive care due to worsening pulmonary hypertension during bronchoscopy. Similar cases are expected to be more common in the future. A 2-year-old boy with trisomy18 and unrepaired double outlet right ventricle, who had undergone tracheostomy and ventilator management, was being followed as an outpatient. His respiratory and circulatory status remained stable despite the gradual worsening progression of pulmonary hypertension. The patient presented with bloody sputum due to frequent suctioning and underwent bronchoscopy in the otolaryngology department. The patient developed profound cyanosis during the procedure of bronchoscopy and was admitted to the intensive care unit for the management of a pulmonary hypertension crisis. He received intensive care, including deep sedation and nitric oxide inhalation. He was transferred to the general ward on day 16 and discharged on day 31. Invasive procedures can exacerbate pulmonary hypertension and lead to hemodynamic compromise in patients with unrepaired congenital heart disease. Therefore, close collaboration among pediatricians, pediatric cardiologists, and physicians in other medical departments is essential to prepare and collaborate for this specific pathophysiology.
