10:30 AM - 12:00 PM
[II-S11-02] 境界型左心系構造を有する複雑心奇形に対する、2心室修復を目指した新生児期hybrid stage I palliationの効果
[Abstract]
Background: Instead of primary definitive surgery, “hybrid stage 1 palliation” (HS1P) consisting of bilateral pulmonary artery banding (bPAB) and ductal stenting (DS) has been initially performed to decide the indication of biventricular repair or its procedure for the neonates with borderline left heart structures since 2010.
Objective: To evaluate the benefit of this strategy.
Methods: Since 2010, 5 patients have undergone HS1P. The main diagnoses were (1) Critical aortic stenosis (cAS) with endocardial fibroelastosis (EFE) (n= 2), (2) Borderline left ventricle (LV), parachute mitral valve (MV) with mitral stenosis (MS) and coarctation of e aorta (CoA) (n= 1), (3) Interrupted aortic arch (IAA) with hypoplastic aortic valve (AV) (n= 2). The median age at bPAB and DS were 7 days (range: 6-14 days) and 9 days (2-27) respectively.
Results: (cAS) Case1: With subsequent balloon aortic valvotomy, surgical aortic valvloplasty and balloon dilatation of banded pulmonary arteries (PTBD), left ventricle end diastolic volume (LVEDV) increased from 61 to 108% of normal size and mitral regurgitation improved from severe to mild, so Ross-Konno procedure was performed at the age of 9 months successfully. Case 2: Although LVEF improved from 20% to 54%, severe EFE remained and LVEDV decreased from 151% to 59% of normal size. So extra-cardiac total cavo-pulmonary connection was performed at the age of 40 months following Norwood type arch reconstruction concomitant with bidirectional Glenn at the age of 23 months. (MS) Case3: For gradual increasing of LV volume preload, PTBD and surgical PA debanding were repeated and LVEDV increased from 70% to 144% of normal size and MV diameter increased from 55% to 84% normal size. So at the age of 13 months, Arch reconstruction, MV repair, and bilateral PA plasty was performed successfully. (IAA) Case 4: AV diameter increased from 80% to 111% of normal size, so Arch reconstruction, VSD closure, and bilateral PA plasty was performed at the age of 18months. Case5: AV diameter was still 49% of normal size after HS1P, then Yasui operation was selected at 18month. No post-operative death is experienced during the median follow-up period of 4 years (range: 1.5-5.5 years).
Summary: Neonatal HS1P would be a beneficial palliation as a bridge to decision therapy for biventricular repair or its procedure. In addition, subsequent gradual volume loading for development of left heart structures sometimes made biventricular repair possible.
Background: Instead of primary definitive surgery, “hybrid stage 1 palliation” (HS1P) consisting of bilateral pulmonary artery banding (bPAB) and ductal stenting (DS) has been initially performed to decide the indication of biventricular repair or its procedure for the neonates with borderline left heart structures since 2010.
Objective: To evaluate the benefit of this strategy.
Methods: Since 2010, 5 patients have undergone HS1P. The main diagnoses were (1) Critical aortic stenosis (cAS) with endocardial fibroelastosis (EFE) (n= 2), (2) Borderline left ventricle (LV), parachute mitral valve (MV) with mitral stenosis (MS) and coarctation of e aorta (CoA) (n= 1), (3) Interrupted aortic arch (IAA) with hypoplastic aortic valve (AV) (n= 2). The median age at bPAB and DS were 7 days (range: 6-14 days) and 9 days (2-27) respectively.
Results: (cAS) Case1: With subsequent balloon aortic valvotomy, surgical aortic valvloplasty and balloon dilatation of banded pulmonary arteries (PTBD), left ventricle end diastolic volume (LVEDV) increased from 61 to 108% of normal size and mitral regurgitation improved from severe to mild, so Ross-Konno procedure was performed at the age of 9 months successfully. Case 2: Although LVEF improved from 20% to 54%, severe EFE remained and LVEDV decreased from 151% to 59% of normal size. So extra-cardiac total cavo-pulmonary connection was performed at the age of 40 months following Norwood type arch reconstruction concomitant with bidirectional Glenn at the age of 23 months. (MS) Case3: For gradual increasing of LV volume preload, PTBD and surgical PA debanding were repeated and LVEDV increased from 70% to 144% of normal size and MV diameter increased from 55% to 84% normal size. So at the age of 13 months, Arch reconstruction, MV repair, and bilateral PA plasty was performed successfully. (IAA) Case 4: AV diameter increased from 80% to 111% of normal size, so Arch reconstruction, VSD closure, and bilateral PA plasty was performed at the age of 18months. Case5: AV diameter was still 49% of normal size after HS1P, then Yasui operation was selected at 18month. No post-operative death is experienced during the median follow-up period of 4 years (range: 1.5-5.5 years).
Summary: Neonatal HS1P would be a beneficial palliation as a bridge to decision therapy for biventricular repair or its procedure. In addition, subsequent gradual volume loading for development of left heart structures sometimes made biventricular repair possible.