先天性房室弁疾患の外科治療には未解決な問題が多く，challengingな領域である．
【先天性僧帽弁膜症】形態異常が多岐にわたること，ほかの心疾患を合併する頻度が高いことから，治療に難渋する疾患群である．弁形成術が第一選択であるが，形成が不可能な高度の病変に対しては人工弁置換術が選択される．弁置換術後は，身体の発育に応じて人工弁のサイズアップが必要となる．
【房室中隔欠損症】左側房室弁狭窄を招来することなくいかに逆流を防止するかが問題となる．房室中隔欠損の弁形成の成否は房室弁を左右に正しく分割できるか否かにかかっており，正しい分割が行われないと，弁形成は決して成功しない．
【単心室症例に合併する房室弁疾患】房室弁逆流の存在は，Fontan型手術を目指す単心室症例の予後を著しく悪化させる．特に新生児や乳児早期から手術介入を要する高度の房室弁病変の治療は非常に困難である．弁形成術の成績は不良で，症例によっては人工弁置換を選択したほうが良好な結果が得られることもある．
【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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