Miki Kanai, Hirofumi Tsuru, Takanori Onuki, Shunsuke Nukaga, Shigehito Baba, Masanori Tsukada, Tadaaki Abe, Fujito Numano, Kei Murayama, Akihiko Saitoh
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.