第55回日本小児循環器学会総会・学術集会

講演情報

標本展示講演

標本展示講演(II-TISL)

2019年6月28日(金) 15:00 〜 16:00 第5会場 (中ホールB)

座長:稲井 慶(東京女子医科大学 循環器小児・成人先天性心疾患科)

[II-TISL-01] 修正大血管転位症の解剖学

森田 紀代造 (東京慈恵会医科大学)

キーワード:Congenitally Corrected Transposition of The Great Arteries, discordant atrioventricular and ventriculo-arterial connections, Morphology

CCTGA is a complex congenital anomaly with a wide spectrum of morphological features and clinical profiles. The underlyingmalformation is the combination of discordant atrioventricular and ventriculo-arterial connections (“duplicated discordance”), results in normal physiology despite the presence of the transposition of the great arteries. The most common anatomical arrangement is situs solitus with L-looping of the ventricles and the aorta anterior and leftward of the pulmonary artery (S,L,L) in 95 percent of caseswhile situs inversus with D-looping of the ventricles and the aorta anterior and rightward (I,D,D) is also seen in the other 5% of ccTGA cases.  The majority of cases have any associated anatomical abnormalities (ie VSD in 60 to 80 % of patients, LVOTO or PS/PA in 40 to 50%, and abnormalities of the tricuspid valve in up to 90% with Ebstein’s anomaly in 50%. The isolated ccTGA without  malformation is seen  1% of cases. Due to the malalignment of the atrial and inlet ventricular septum with the reversed off-setting of the attachments of the leaflets of the atrioventricular valves to the septum, the regular atrioventricular node is anatomically impossible to connect to the atrioventricular bundle in ccTGA with situs soitus {SLL} arrangement andthe anterior AV node is located above the lateral margin of the area of pulmonary to mitral fibrous continuity which gives rise to a penetrating bundle (PB) and the non-branching bundle (NBB) encircles pulmonary valve anteriorly and descends onto the anterior part of the trabecular septum on the right side (morphological LV) surface of the septum.