Kana Inoue, Taku Ishii, Yohei Yamaguchi, Satoshi Nagahara, Makito Sakurai, Susumu Hosokawa, Shozaburo Doi
Institute of Science Tokyo. Chiba Kaihin Municipal Hospital. Musashino Red Cross Hospital. Tokyo Healthcare University.
Japan
European Heart Journal Case Reports
Eur Heart J Case Rep 2025; 9:
DOI: 10.1093/ehjcr/ytaf357
Abstract
Background: Initial treatment based on acute vasoreactivity test results is essential for idiopathic or hereditary pulmonary arterial hypertension. In paediatric patients who are usually examined under sedation, accurately identifying those who are positive in the acute vasoreactivity test is often difficult.
Case summary: Patient 1: A 10-year-old boy was diagnosed with heritable pulmonary arterial hypertension at the age of 5 years, following symptoms of exertional dyspnoea and fatigue. During the initial right heart catheterization, his mean pulmonary artery pressure was 21 mmHg under sedation. After pulmonary hypertension-targeted therapy was initiated, symptoms improved. However, during a follow-up examination at 10 years of age, the patient was awake, causing the pulmonary artery pressure to rise from 33/10 (21) to 99/55 (70) mmHg. Nonetheless, under nitric oxide inhalation, the rise in pulmonary artery pressure was minimal, even when the patient was awake. Subsequently, he responded well to calcium channel blockers. Patient 2: A 5-year-old girl experienced syncope and exertional cyanosis. Under sedation, initial right heart catheterization showed a pulmonary artery pressure of 44/15 (27) mmHg, which increased to 82/49 (57) mmHg upon awakening and reduced to 38/12 (22) mmHg after nitric oxide inhalation. Diagnosed with idiopathic pulmonary arterial hypertension positive for the acute vasoreactivity test, she responded well to calcium channel blockers.
Discussion: Acute vasoreactivity test may be potentially underestimated during sedation in paediatric patients with pulmonary arterial hypertension. Thus, evaluating pulmonary artery pressure by reducing anaesthesia depth or stimulating wakefulness can lead to an appropriate diagnosis and treatment.
Category
Class I. Pulmonary hypertension Associated with a Favorable Response to Nitric Oxide and Calcium Channel Blockers
Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes