日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Pediatric Cardiology and Cardiac Surgery 36(2): 159-165 (2020)
doi:10.9794/jspccs.36.159

症例報告Case Report

心室中隔欠損を合併し急速に心不全が進行したMarfan症候群の乳児例An Infant with Marfan Syndrome and Ventricular Septal Defect and Progressive Heart Failure

1新潟大学医歯学総合病院小児科Department of Pediatrics, Niigata University Medical and Dental Hospital ◇ Niigata, Japan

2新潟大学医歯学総合病院心臓血管外科Department of Cardiovascular Surgery, Niigata University Medical and Dental Hospital ◇ Niigata, Japan

受付日:2019年6月20日Received: June 20, 2019
受理日:2019年11月20日Accepted: November 20, 2019
発行日:2020年6月1日Published: June 1, 2020
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Marfan症候群(MFS)に心室中隔欠損(VSD)を合併し,急速に心不全が進行し,手術前後で心機能が低下した乳児例を経験した.症例は2か月の女児で,VSDによる高肺血流性心不全と診断され,当院を紹介となった.家族歴,身体所見からMFSが疑われた.高肺血流に心機能低下も伴うため,段階的に修復術を施行された.肺動脈絞扼術直後と心内修復術直後にそれぞれ心機能は低下したが,徐々に回復した.3歳時に水晶体脱臼を認め,父がMFSであることから改訂Ghent基準を満たした.MFSは潜在的な心機能障害を有する可能性があり,治療介入が必要な先天性心疾患を合併する例では,術前の心不全の進行や術後管理に注意し,慎重な経過観察や治療戦略を考慮すべきである.

We describe the case of an infant with Marfan syndrome (MFS) complicated by ventricular septal defect (VSD), who developed rapidly progressive heart failure and reduced cardiac function after surgery. The patient was a 2-month-old girl who was diagnosed with heart failure caused by excessive pulmonary blood flow due to VSD and referred to our hospital. MFS was suspected based on family history and physical findings. Since excessive pulmonary blood flow was accompanied by a decrease in cardiac function, a staged repair was performed. Although the cardiac function decreased immediately after pulmonary artery banding and intracardiac repair, it gradually recovered. At the age of 3 years, lens luxation was found. Since the patient’s father had MFS, she met the revised Ghent criteria. MFS may induce cardiac dysfunction. If a patient with congenital heart disease requires therapeutic intervention, attention should be paid to the progression of preoperative heart failure and postoperative management, and follow-up and treatment strategies should be carefully considered.

Key words: Marfan syndrome; ventricular septal defect; dilated cardiomyopathy; acute heart failure

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This page was last modified on 2020-06-18T11:26:49.000+09:00


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