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
Pediatric Cardiology and Cardiac Surgery 38(3): 198-203 (2022)

症例報告Case Report

胎児期に診断し,無治療で経過観察できている先天性右心耳瘤の一例A Case of Congenital Right Atrial Appendage Aneurysm Diagnosed Prenatally and Followed-Up Without Treatment

1旭川医科大学小児科Department of Pediatrics, Asahikawa Medical University ◇ Hokkaido, Japan

2網走厚生病院小児科Department of Pediatrics, Abashiri-Kosei General Hospital ◇ Hokkaido, Japan

受付日:2022年5月12日Received: May 12, 2022
受理日:2022年7月12日Accepted: July 12, 2022
発行日:2022年8月1日Published: August 1, 2022

先天性右心耳瘤は1968年のMorrowらによる報告以来,新生児から成人を含め国内外での症例が数十例しかいない稀な心内構造異常である.合併症として不整脈や血栓塞栓症,動悸や息切れなどが問題になる場合があり,適切な診断および治療が重要であるものの,その指針は定まっていない.症例は在胎29週の男児で,前医で右房拡大を指摘され,当院に紹介された.胎児心臓超音波検査では,14.2×7.9 mm(実測値:0.7 cm2)の瘤を認め,先天性右心耳瘤と診断した.診断時の瘤のサイズ(<20×13 mm,実測値:<2 cm2)を参考に無治療で経過観察できると判断し,胎児期は合併症なく経過し出生した.生後の瘤のサイズは22×11 mm(実測値:1.7 cm2)であり,右房とのサイズの比率が胎児期から変化がなかったため,引き続き経過観察をする方針にした.現在1歳になったが,合併症は起こさずに経過している.先天性右心耳瘤のフォローアップには,瘤のサイズが一つの目安になると考える.

Congenital right atrial appendage aneurysm is a rare heart disease, with only a few dozen cases worldwide since the report by Morrow et al. in 1968. Complications include arrhythmia, thromboembolism, palpitation, and dyspnea. Appropriate diagnosis and treatment are important, but guidelines are not yet established. The patient’s mother was referred to our hospital at 29 weeks of gestation for enlarged right atrium. Fetal echocardiography revealed a 14.2×7.9 mm (0.7 cm2) aneurysm, which was diagnosed as congenital right atrial aneurysm. Based on the aneurysm size at diagnosis (<20×13 mm, <2 cm2), it was decided that the patient could be followed-up without treatment, and the baby was born without complications. At birth, the aneurysm size was 22×11 mm (1.7 cm2), and the ratio of the aneurysm size to the right atrium was unchanged from the fetal period. The patient is now nearing 1 year old without any complications. We think that the aneurysm size can guide the follow-up of congenital right atrial appendage aneurysms.

Key words: congenital right atrial appendage aneurysm; right atrial enlargement; fetal echocardiography; supraventricular arrhythmia; thromboembolism

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