[III-JCKAP6-2-3] Anatomical reconstruction of giant right atrial aneurysm in Ebstein anomaly: a case report
キーワード：Ebstein anomaly, Cone repair, Giant right atrium
Objective
We present a case report detailing the successful surgical repair of a giant right atrial aneurysm in a patient with Ebstein anomaly. A 12-year-old girl was incidentally found cardiomegaly on a chest radiography. Further evaluation by echocardiography revealed Ebstein anomaly, carpentier type B. Which presented with giant right atrial aneurysm and downward displacement of the septal and posterior leaflet in the tricuspid valve, with severe tricuspid regurgitation.
Methods
The patient underwent anatomical cone repair including vertical plication of atrialized right ventricle, detaching and reattaching tricuspid valve to true annulus, tricuspid valve repair with papillary muscle splitting, and surgical reduction of a giant right atrium. After resection of the right atrial aneurysm, a meticulous anatomical reduction-plasty of the right atrium was performed.
Results
The morphologic features of the resected atrial tissue showed paper-thin aneurysmal sac at antero-inferior side of the right atrium. Pathological evaluation revealed endocardial tissue with interstitial edema and mild fibrosis. The patient's postoperative course was stable, and follow-up chest radiography showed reduction in cardiomegaly and echocardiography presented no significant tricuspid regurgitation. The patient was discharged from the hospital after 15 days of surgery.
Conclusion
Following resection of the giant right atrial aneurysmal sac, meticulous anatomical reduction-plasty of the right atrium is imperative for successful anatomical cone repair and optimal postoperative outcomes.
We present a case report detailing the successful surgical repair of a giant right atrial aneurysm in a patient with Ebstein anomaly. A 12-year-old girl was incidentally found cardiomegaly on a chest radiography. Further evaluation by echocardiography revealed Ebstein anomaly, carpentier type B. Which presented with giant right atrial aneurysm and downward displacement of the septal and posterior leaflet in the tricuspid valve, with severe tricuspid regurgitation.
Methods
The patient underwent anatomical cone repair including vertical plication of atrialized right ventricle, detaching and reattaching tricuspid valve to true annulus, tricuspid valve repair with papillary muscle splitting, and surgical reduction of a giant right atrium. After resection of the right atrial aneurysm, a meticulous anatomical reduction-plasty of the right atrium was performed.
Results
The morphologic features of the resected atrial tissue showed paper-thin aneurysmal sac at antero-inferior side of the right atrium. Pathological evaluation revealed endocardial tissue with interstitial edema and mild fibrosis. The patient's postoperative course was stable, and follow-up chest radiography showed reduction in cardiomegaly and echocardiography presented no significant tricuspid regurgitation. The patient was discharged from the hospital after 15 days of surgery.
Conclusion
Following resection of the giant right atrial aneurysmal sac, meticulous anatomical reduction-plasty of the right atrium is imperative for successful anatomical cone repair and optimal postoperative outcomes.