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 33(2): 156-162 (2017)


房室弁疾患の外科治療Surgery for Congenital Atrioventricular Valve Disease

富山大学大学院医学薬学研究部外科学(呼吸・循環・総合外科)講座Graduate School of Medicine and Pharmaceutical Sciences, Department of Cardiothoracic Surgery, University of Toyama ◇ Toyama, Japan

発行日:2017年3月1日Published: March 1, 2017





【Ebstein病】 2004年,da Silvaらにより報告されたCone reconstructionは術直後から遠隔期まで,三尖弁逆流が良好に制御され,今後,本疾患に対する標準術式となることが期待される.

Surgery for atrioventricular valve disease in infants and children has been a major therapeutic challenge for many years.

[Congenital mitral valve disease] It poses special clinical and technical difficulties that include a wide spectrum of morphologic abnormalities, high prevalence of associated cardiac anomalies, and small experience at each institute. Although conservative procedures may obviously be preferred procedures of choice, mitral valve replacement should be considered the procedure of choice in patients with severe deformities wherein conservative surgical treatment is unfeasible. Mitral valve replacement carries the disadvantage of requiring repeat valve replacement for relative prosthetic valve obstruction because of somatic growth.

[Atrioventricular septal defect] It is important to maintain or create two-competent, nonstenotic atrioventricular valves. Effective atrioventricular valve repair depends on adequate septation.

[Atrioventricular valve disease in patients with functional single ventricle] Surgery for patients with a functional single ventricle who have atrioventricular valve regurgitation remains a clinical challenge. Neonates or early infants who require surgical treatment of atrioventricular regurgitation still have poor outcomes. Although valve repair is preferable, prosthetic valve replacement should be considered when valve repair is questionable.

[Ebstein’s anomaly] Cone reconstruction of the tricuspid valve results in central blood flow through the tricuspid orifice and full coaptation of the leaflets. This technique can be performed with low mortality and morbidity. Early and long-term outcomes are favorable.

Key words: congenital mitral valve disease; atrioventricular septal defect; univentricular heart; Ebstein’s anomaly

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