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"ABSTRAKTujuan : Koreksi transatrial-transpulmonary tanpa transannular patch (TA-TP tanpaTAP) memiliki keuntungan berupa preservasi annulus katup pulmonal dan fungsiventrikel kanan, Namun sering terjadi gradien RV-PA dan pRV/LV ratio yang masihtinggi sehingga terjadi low cardiac output syndrome (LCOS). Penelitian ini bertujuanuntuk mencari batasan gradien RV-PA dan pRV/LV ratio yang merupakan nilai prediktifterbaik terhadap kejadian LCOS pascakoreksi tetralogi Fallot TA-TP tanpa TAP.Metode : Pada bulan Oktober 2012 sampai Maret 2013, sebanyak 30 pasien TF menjalanikoreksi TF TA-TP tanpa TAP (mean usia 8,37±7,90 tahun). Dilakukan pengukurangradien RV-PA dan pRV/LV ratio intraoperatif dan postoperatif di ICU. Evaluasi kejadianLCOS dilakukan selama perawatan di ICU. Sebelum pasien pulang, dilakukanpemeriksaan ekokardiografi untuk menilai gradien RV-PA, fungsi ventrikel kanan, defekseptum ventrikel residual, derajat regurgitasi katup pulmonal dan katup trikuspid.Hasil : Sebanyak 30 (100%) subjek penelitian memiliki z-value ≥ -1, menjalani koreksiTF TA-TP tanpa TAP. Mean gradien RV-PA intraoperatif adalah 21,13±10,60 mm Hgdan mean pRV/LV ratio intraoperatif adalah 0,53±0,14. Mean gradien RV-PA di ICUadalah 20,83±7,10 mmHg dan mean pRV/LV ratio di ICU adalah 0,49±0,10. Tidak terjadiLCOS pada 30 (100%) subjek penelitian sehingga tidak dapat dilakukan analisis untukmencari batasan nilai gradien RV-PA dan pRV/LV ratio sebagai nilai prediktif terbaikterhadap kejadian LCOS pascakoreksi TF TA-TP tanpa TAP. Mean gradien RV-PAsebelum subjek penelitian rawat jalan adalah 23,47±6,95 mmHg. Regurgitasi katuppulmonal ringan pada 15 (50%) subjek penelitian dan regurgitasi katup trikuspid trivialmildpada 16 (53%) subjek penelitian. Disfungsi ventrikel kanan ringan 3 (10%), sedang20 (67%) dan berat pada 7 (23%) subjek penelitian. Mean TAPSE postoperatif adalah1,03±0,19. DSV residual tidak dijumpai, aritmia tidak dijumpai, reoperasi dan mortalitastidak ada.Simpulan : Koreksi TF TA-TP tanpa TAP memberikan hasil operasi dini yang baik padapasien TF dengan z-value katup pulmonal ≥ -1, pRV/LV ratio < 0,5 dan gradien RV-PA <25 mmHg pascakoreksi.

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ABSTRACTObjective : The benefits of the transatrial-transpulmonary (TA-TP) without transannularpatch (TAP) correction of tetralogy of Fallot (TOF) are preservation of pulmonary valveannulus and right ventricular function. However, TA-TP without TAP correction of TOFhad a higher incidence of low cardiac output syndrome (LCOS) because of the high rightventricle and pulmonary artery (RV-PA) pressure gradient and right ventricle and leftventricle pressure (pRV/LV) ratio. The purpose of this study were to analyze the cut offvalue of RV-PA pressure gradient dan pRV/LV ratio as the best predictor value forpostoperative LCOS in TA-TP without TAP correction of TOF.Methods : Between Oktober 2012 and Maret 2013, 30 patients with TOF underwent TATPwithout TAP correction (mean age 8,37±7,90 years, range 1-27 years). At the end ofcorrection, all patients underwent intraoperative direct measurement of RV-PA pressuregradient and pRV/LV ratio. The patients were evaluated for postoperative LCOS at theIntensive Care Unit (ICU). All the patients underwent echocardiographic examinationbefore hospital discharge. This included investigation of the presence RV-PA pressuregradien, RV function, residual VSD, pulmonary and tricuspid valve insufficiency.Results : Thirty patients with pulmonary valve annulus z-value ≥ -1, underwent TA-TPwithout TAP correction of TOF. Mean intraoperative RV-PA pressure gradient was21,13±10,60 mmHg and mean intraoperative pRV/LV ratio was 0,53±0,14. Mean RV-PApressure gradient measured 24 hours after correction at the ICU was 20,83±7,10 mmHgand mean pRV/LV ratio measured at 24 hours after correction at the ICU was 0,49±0,10.No patient had LCOS, we could not analyze the cut off value of RV-PA pressure gradientand pRV/LV ratio as the best predictor value for postoperative LCOS in this study. Nopatient had residual VSD. Mean RV-PA pressure gradient before hospital discharge was23,47±6,95 mmHg. Fifteen (50%) patients had mild pulmonary valve insufficiency and16 (53%) patients had trivial-mild tricuspid valve insufficiency. Three (10%) patients hadmild RV dysfunction. Postoperative mean TAPSE was 1,03±0,19. No patient hadarrhythmia, reoperation and mortality in this study.Conclusions : The TA-TP without TAP correction of TOF was applied successfully in 30patients with pulmonary valve annulus z-value ≥ -1, post-correction RV-PA pressuregradient < 25 mmHg and pRV/LV ratio < 0,5."

"Latar belakang: Pada Tetralogy of Fallot (TOF) dilakukan pembedahan dengan prosedur transannular patch (TAP) untuk mengatasi obstruksi ventrikel kanan. Namun, prosedur ini diketahui menyebabkan mortalitas dan morbiditas yang tinggi akibat regurgitasi pulmonal pasca operasi. Beberapa studi melaporkan Valve sparing repair (VSR) sebagai prosedur pembedahan TOF dengan angka mortalitas dan morbiditas yang lebih rendah. Namun, saat ini belum terdapat cukup data untuk membandingkan keluaran di antara kedua prosedur tersebut. Tujuan: Mengetahui prosedur pembedahan terbaik dalam tata laksana TOF. Metode: Penelitian ini merupakan tinjauan sistematis dengan meta analisis yang dibuat berdasarkan Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Meta analisis dilakukan untuk membandingkan luaran mortalitas dan morbiditas antara prosedur TAP dan VSR. Studi ini menggunakan artikel yang terbit dari database Cochrane, MEDLINE, EBSCOhost (CINAHL dan Academic Search Complete), dan Scopus. Analisis statistik dilakukan dengan software Review Manager v5.4 dan p-value <0,05 dianggap bermakna secara statistik. Hasil: Dari pencarian literatur didapatkan 144 studi dengan 14 studi yang memenuhi kriteria. Jumlah total subjek sebanyak 3.280 orang, 50,8% menjalani prosedur VSR. Kejadian mortalias perioperatif, mortalitas pascaoperasi, regurgitasi pulmonal pascaoperasi, regurgitasi pulmonal saat follow-up, dan operasi ulang pada kelompok VSR dan TAP berturut-turut adalah 0,14% vs 0,27% , 0,21% vs 0,28%, 2,87% vs 19,02%, 7,25% vs 20,13%, dan1,95% vs 3,23%. Tidak terdapat perbedaan risiko kematian perioperatif dan pasca operasi pada pasien yang menjalani prosedur VSR dan TAP. Pasien yang menjalani prosedur VSR memiliki risiko 0,08 kali lebih kecil mengalami regurgitasi pulmonal pasca operasi, 0,11 kali lebih kecil mengalami regurgitasi pulmonal saat follow-up, dan 0,43 kali lebih kecil menjalani operasi ulang dibandingkan pasien yang menjalani prosedur TAP. Kesimpulan: Prosedur VSR merupakan prosedur pembedahan terbaik dalam tata laksana TOF. Prosedur VSR memiliki risiko regurgitasi pulmonal dan operasi ulang yang lebih rendah dibandingkan TAP.

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Background: Transannular patch (TAP) is one of surgical procedures performed to treat right ventricular obstruction in patient with Tetralogy of Fallot (TOF). This procedure caused higher mortality and morbidity due to post operation pulmonary regurgitation. A few studies reported promising result from valve sparing repair (VSR) procedure with lower mortality and morbidity. Currently, there is still not enough data to compare outcomes between the two procedures. Aim: To know the best surgical procedure to treat TOF. Method: This study is a systematic review with metaanalysis written based on Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Metaanalysis was performed to compare mortality and morbidity outcomes between TAP and VSR procedures. Articles used for this study were obtained from Cochrane, MEDLINE, EBSCOhost (CINAHL and Academic Search Complete), and Scopus databases. Statistic analysis was performed using Review Manager v5.4 software with p-value <0,05 considered significance. Result: We obtained 144 studies from literature searching, 14 of which were included in this study. Total subject was 3.280 people, 50,8% of which underwent VSR procedure. Perioperative mortality, postoperative mortality, postoperative pulmonary regurgitation, pulmonary regurgitation at follow-up, and reoperation on VSR and TAP groups were respectively, 0,14% vs 0,27% , 0,21% vs 0,28%, 2,87% vs 19,02%, 7,25% vs 20,13%, and 1,95% vs 3,23%. There is no significant difference on perioperative and postoperative mortality between VSR and TAP group. Patient in VSR group has 0.08 times lower risk to have postoperative pulmonary regurgitation, 0.11 times lower risk to have pulmonary regurgitation at follow up, and 0.43 times lower risk to have reoperation compared to TAP group. Conclusion: VSR procedure is the best surgical procedure to treat TOF. This procedure has lower risk for pulmonary regurgitation and reoperation compared to TAP."