Miki Kanai, Hirofumi Tsuru, Takanori Onuki, Shunsuke Nukaga, Shigehito Baba, Masanori Tsukada, Tadaaki Abe, Fujito Numano, Kei Murayama, Akihiko Saitoh
Saiseikai Niigata Kenoh Kikan Hospital. Niigata University School of Medicine. Juntendo University.
Japan
Internal Medicine
Intern Med 2026;
DOI: 10.2169/internalmedicine.7614-26
Abstract
Pulmonary hypertension (PH) is uncommon in myopathy, encephalopathy, lactic acidosis, and stroke-like (MELAS) and its hemodynamics are poorly described. A 2-year-old Japanese girl was referred to our hospital with PH diagnosed during pneumonia treatment. Cardiac catheterization revealed PH with a high cardiac output. The patient was diagnosed with MELAS based on multiorgan involvement and the detection of an m.3243A>G mutation. PH therapy was partially effective, and oxygen supplementation may help improve PH without worsening the high cardiac output.
Category
Class V. Pulmonary Hypertension Associated with Hematological, Systemic, Metabolic, Nutritional and Other Disorders
Medical Therapy. Adverse Effects or Lack of Adverse Effects
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