- 著者
- Seigo Okada Jun Muneuchi Yusaku Nagatomo Kaori Nonaka Chiaki Iida Hiromitsu Shirouzu Ryohei Matsuoka Mamie Watanabe Kunitaka Joo
- 出版者
- International Heart Journal Association
- 雑誌
- International Heart Journal (ISSN:13492365)
- 巻号頁・発行日
- vol.59, no.4, pp.873-876, 2018-07-31 (Released:2018-07-31)
- 参考文献数
- 11
- 被引用文献数
- 6 8
We present a 16-year-old male patient with hypoplastic left heart syndrome who developed protein-losing enteropathy (PLE) and plastic bronchitis (PB) after a Fontan operation. He received medical therapies, including albumin infusion, unfractionated heparin, and high-dose anti-aldosterone therapy but could not obtain clinical relief. Biphasic cuirass ventilation (BCV) led to expectoration of bronchial casts and prompt resolution of PB. Notably, clinical symptoms related to PLE were dramatically improved after starting BCV. A brief period of BCV increased stroke volume from 26±1.4 to 39±4.0 mL. This case suggests that BCV could be an effective treatment for PLE in patients with failing Fontan circulation.
- 著者
- Takahiro Motonaga Yuji Ohnishi Seigo Okada Yasuo Suzuki Takashi Furuta Mai Kawamura Naoko Okayama Yutaka Suehiro Shunji Hasegawa
- 出版者
- International Heart Journal Association
- 雑誌
- International Heart Journal (ISSN:13492365)
- 巻号頁・発行日
- pp.21-821, (Released:2022-07-14)
- 参考文献数
- 25
- 被引用文献数
- 2
Marfan syndrome is an autosomal dominant genetic disorder of the fibrous connective tissue caused by pathogenic mutations in the fibrillin-1 gene. Neonatal Marfan syndrome is a rare type of Marfan syndrome that is genotypically and phenotypically different from classical Marfan syndrome and has a poor prognosis. Most patients with neonatal Marfan syndrome die during infancy due to severe and rapidly progressive cardiovascular disorders. Here, we present a case of an 11-year-old girl with neonatal Marfan syndrome due to a novel missense mutation in exon 27 of the fibrillin-1 gene. Her condition was critical due to progressive mitral and tricuspid regurgitation. Mitral valve replacement, performed at the age of 6 months, improved her critical condition. Our case suggests that early mitral valve replacement may lead to better outcomes in patients with neonatal Marfan syndrome.