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Abstrak :
Latar Belakang: Regurgitasi pulmoner berat pasca-bedah korektif TF berdampak sebagai beban berlebih pada ventrikel kanan dan akan mempengaruhi ukuran dan fungsinya. Tujuan: Mengetahui faktor yang berperan terhadap regurgitasi pulmoner berat pasca-bedah korektif TF dan dampaknya pada ventrikel kanan. Metode: Penelitian ini adalah studi potong lintang di Pelayanan Jantung Terpadu RSUPN Dr Cipto Mangunkusumo Jakarta pada pada April-Mei 2019. Kriteria inklusi adalah pasien TF yang menjalani koreksi TF dalam 5 tahun terakhir. Data demografi dan kuantitatif ekokardiografi diambil dengan pemeriksaan ekokardiografi. Analisis bivariat faktor risiko regurgitasi pulmoner berat yang bermakna dimasukkan ke dalam analisis regresi logistik multipel. Hasil analisis multivariat dilaporkan sebagai odds ratio (OR). Hasil: Terdapat 50 pasien yang sesuai kriteria inklusi. Sebanyak 22 pasien (44%) mengalami regurgitasi pulmoner berat dan 28 pasien (56%) mengalami regurgitasi pulmoner ringan sedang. Analisis multivariat regresi logistik menunjukkan indeks Nakata > 250 mm2/m2bermakna menimbulkan 15,1 kali risiko untuk menjadi regurgitasi pulmoner berat [OR 15,1 (IK 95% 3,1-72,6), p=0,001]. Analisis bivariat untuk ukuran dan fungsi ventrikel kanan tidak terdapat perbedaan yang bermakna pada kedua kelompok. Simpulan: Indeks Nakata > 250 mm2/m2berisiko terjadi regurgitasi pulmoner berat pada pasca- bedah korektif TF. Pada pemantauan 4 tahun, belum ada dampak dilatasi dan penurunan fungsi ventrikel kanan ......Background: Repaired tetralogy of Fallot (TF) result pulmonary regurgitation. Impact of severe pulmonary regurgitation were right ventricular (RV) volume overload predisposing dilatation and dysfunction of RV. Diameter pulmonary artery, McGoon ratio, Nakata index pre-operation, surgery technique can contribute to severe pulmonary regurgitation in the absence of an effective valve. Objective: The aim of this study was to identify predictors of severe pulmonary regurgitation and the impact to the RV. Methods: A cross sectional study of repaired TF in children at the integrated cardiovascular services (PJT) Dr. Cipto Mangunkusumo Jakarta from April-Mei 2019. The inclusion criteria included children underwent repaired TF in the last 5 years after minimal 6 months post repaired TF. Demographic data and echocardiography data were collected. Logistic regression analysis were used to identify the predictor for severe pulmonary regurgitation. Results: A total of 50 patients were enrolled to the study. There were 22 children (46%) with severe pulmonary regurgitation and 28 children (56%) with mild-moderate pulmonary regurgitation. Logistic regression analysis showed Nakata index showed Nakata index > 250 mm2/m215,1 times greater risk for severe pulmonary regurgitation [OR 15,1 (CI 95% 3,1-72,6), p=0,001]. Bivariate analysis for RV size and function showed no significant difference between the group. Conclusions: Nakata index > 250 mm2/m2was predictor for severe pulmonary regurgitation after TF repair. RV size and function showed no abnormality in 5 years follow up after TF repair.

Abstrak :
Latar Belakang: Tetralogi Fallot (TF) merupakan penyakit jantung bawaan (PJB) sianotik terbanyak. Terapi definitifnya berupa koreksi total melalui operasi jantung terbuka, namun usia terbaik koreksi masih menjadi perdebatan. Operasi saat usia < 3 tahun disebut koreksi dini. Angka kesintasan jangka panjang pasien TF pasca-operasi mencapai 90%. Masalah baru yang muncul adalah gangguan neurodevelopmental yang dapat mempengaruhi kualitas hidup pasien. Tujuan: Mengetahui sebaran pasien TF pasca-operasi jantung terbuka, serta membandingkan perkembangan, kognitif dan kualitas hidup pasien TF pasca-operasi jantung terbuka yang menjalani koreksi dini dibandingkan koreksi terlambat. Metode: 29 subjek kelompok koreksi dini dan 34 subjek kelompok koreksi terlambat dipilih secara konsekutif. Penilaian perkembangan menggunakan Denver II. Tingkat kognitif dinilai dengan the Capute scales dan uji intelegensi Wechsler. Kualitas hidup dinilai dengan laporan PedsQLTM. Perbedaan antar kedua kelompok subjek dianalisis dengan menggunakan uji Kai kuadrat, uji Fischer, dan uji t tidak berpasangan. Hasil: Median usia operasi kelompok koreksi dini adalah 1,8 tahun dan kelompok koreksi terlambat adalah 5,3 tahun. Sebesar 54% subjek menjalani koreksi terlambat. Mikrosefal terjadi pada 15% keseluruhan subjek. Pada kedua kelompok subjek ditemukan masalah perkembangan. Sebesar 75% subjek kelompok koreksi dini memiliki developmental quotient normal. Kelompok koreksi dini memiliki nilai verbal intelligence quotient (IQ) (p 0,002; IK 95% 5,8-24,6) dan full-scale IQ (p0,003; IK 95% 4,7-21,3) yang lebih tinggi dibandingkan kelompok koreksi terlambat. Laporan PedsQLTM anak menunjukkan rendahnya kualitas hidup pada fungsi emosi (p=0,02) dan sekolah (p=0,03) pada kelompok koreksi terlambat. Simpulan: Pasien TF yang menjalani koreksi dini memiliki dan kualitas hidup yang lebih tinggi dibandingkan kelompok koreksi terlambat, sehingga diperlukan sosialisasi usia operasi koreksi dini. ......Background: Tetralogy of Fallot (TF) is the most common cyanotic congenital heart disease. The definitive treatment is complete repair thru open heart surgery. At present, the most effective age category for repair is still being debated. Complete repair for children who are younger than 3 years is called early repair. Recent technological advancement has allowed the early repair to be performed earlier and improve the survival rate of the patients. However, these survivors risk having neurodevelopmental disorder which affect their health-related quality of life. Objective: To describe the characteristics of post open heart surgery TF patients and compare the TF patients who undergo early correction to ones who undergo late correction within the aspects of development, cognitive outcomes, and health-related quality of life. Design : Twenty nine subjects from early correction group and 34 subjects from late correction group were compared in development (Denver development screening II), cognitive outcomes (The Capute scales and Wechsler test), and health-related quality of life (PedsQLTM). Result : Median age of the subjects in early correction group is 1,8 years and in late correction group is 5,3 years. Fifty five percent undergo late correction. The prevalence of microcephaly is 15%. Developmental delay is found in both group. Seventy five percent of subject who undergo early correction have normal developmental quotient. Early correction group have higher verbal intelligence quotient (IQ) (p=0.002; CI 95% 5.8-24.6) and full scale IQ (p=0.003; CI 95% 4.7-21.3). Child report PedsQLTM showed lower quality of life in late correction group. Conclusions : Tetralogy of Fallot patients who undergo early correction have higher IQ and better health-related quality of life compared to late correction group. The age of early complete repair (< 3 years) needs to be disseminated.

Abstrak :
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. ......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.

Abstrak :
[ABSTRAK
Hipertrofi ventrikel kanan (HVKa) pada tetralogy fallot (TF) merupakan suatu respon adaptif akibat dari peningkatan tekanan di ventrikel kanan (VKa) dan hipoksia. HVKa yang berat vektor jantung akan mengarah ke kanan-posterior dapat menyebabkan gelombang S yang dalam di sadapan V6. Sementara itu pasien TF yang lama tidak dikoreksi akan mengalami paparan tekanan berlebih dan sianosis yang lebih lama juga, yang dapat menyebabkan perubahan-perubahan di tingkat seluler kardiomiosit yang pada akhirnya menyebabkan disfungsi VKa, dan sindrom curah jantung rendah (SCJR). Walaupun angka kesintasan pasca operasi baik, tapi perburukan SCJR dapat mengakibatkan kematian. Saat ini belum jelas bagaimana hubungan antara gelombang S di V6 dengan luaran total koreksi TF khususnya kejadian SCJR. Metode Penelitian dengan metode potong lintang. Subyek penelitian adalah TF yang menjalani total koreksi selama tahun 2013 sebanyak 150 pasien, 35 diantaranya dikeluarkan dari penelitian karena tidak memenuhi kriteri inklusi. Subyek dibagi menjadi 2 kelompok yaitu kelompok subyek dengan temuan kriteria S di V6 dan subyek yang untuk melihat hubungan temuan kriteria tersebut dengan variabel dasar. Kemudian dilakukan analisis bifariat terhadap kejadian SCJR, variabel dengan nilai p < 0.25 di masukkan dalam analisa multivariat. Nilai p< 0.05 dianggap bermakna. Hasil Usia yang lebih muda, saturasi dan hematokrit yang lebih tinggi ditemukan pada kelompok subyek memenuhi kriteria gelombang S di V6. Kemudian, usia yag lebih muda, saturasi yang tinggi, kriteria gelombang R di aVR, kriteria gelombang S di I dan kriteria gelombang S di V6 berhubungan dengan kejadian SCJR. Analisis multivariat kriteria gelombang S di V6 berhubungan dengan kejadian SCJR dengan OR 3.2, interval kepercayaan 95% 1.2 - 8.5 dan nilai p=0.02 Kesimpulan Kriteria EKG gelombang S di sadapan V6 untuk diagnosis HVKa berhubungan dengan kejadian SCJR pasca total koreksi pasien TF.

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ABSTRACT
Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease. Right ventricular hypertrophy (RVH) is an adaptive response due to pressure overload and hypoxia in right ventricle (RV); it can be manifested as tall R wave in right precordial leads. This is due to changing direction of cardiac-vector to right In severe RVH, the cardiac vector rotated to right posterior causing deep S wave in V6. Uncorrected TF will expossed to prolong pressure overload and hypoxia, it can caused changes in cardiomyocite that can leads to RV dysfunction, low cardiac output syndrom (LCOS), and arrhythmias. Although the post operation survival rate was quite good, but worsening LCOS could increase mortality. In present time, the association between S wave in V6 and postoperative TOF outcomes, especially LCOS, has not been explained. Methods This is a cross sectional study. 150 TOF patients underwent total correction in 2013 included in this study. 35 patients who didn?t meet the inclusion criteria were excluded. Subjects divided in 2 groups: (1) patients who meets S in V6 criteria, and (2) control subjects as baseline characteristic. Bivariate analysis was done for incidence of LCOS, the variable with P<0.25 included in multivariate analysis. The significant value was p<0.5. Results Multivariate analysis showed S wave in V6 correlated with the incidence of LCOS with odds ratio 3.2, CI 95% (1.2-8.5), p=0.02. Conclusion The ECG findings S wave in V6 leads to diagnose RVH correlated with incidence of LCOS in post total correction TOF. An S wave criterion in V6 of RVH patients? OR was 3.2 to predicts LCOS;Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease. Right ventricular hypertrophy (RVH) is an adaptive response due to pressure overload and hypoxia in right ventricle (RV); it can be manifested as tall R wave in right precordial leads. This is due to changing direction of cardiac-vector to right In severe RVH, the cardiac vector rotated to right posterior causing deep S wave in V6. Uncorrected TF will expossed to prolong pressure overload and hypoxia, it can caused changes in cardiomyocite that can leads to RV dysfunction, low cardiac output syndrom (LCOS), and arrhythmias. Although the post operation survival rate was quite good, but worsening LCOS could increase mortality. In present time, the association between S wave in V6 and postoperative TOF outcomes, especially LCOS, has not been explained. Methods This is a cross sectional study. 150 TOF patients underwent total correction in 2013 included in this study. 35 patients who didn’t meet the inclusion criteria were excluded. Subjects divided in 2 groups: (1) patients who meets S in V6 criteria, and (2) control subjects as baseline characteristic. Bivariate analysis was done for incidence of LCOS, the variable with P<0.25 included in multivariate analysis. The significant value was p<0.5. Results Multivariate analysis showed S wave in V6 correlated with the incidence of LCOS with odds ratio 3.2, CI 95% (1.2-8.5), p=0.02. Conclusion The ECG findings S wave in V6 leads to diagnose RVH correlated with incidence of LCOS in post total correction TOF. An S wave criterion in V6 of RVH patients’ OR was 3.2 to predicts LCOS;Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease. Right ventricular hypertrophy (RVH) is an adaptive response due to pressure overload and hypoxia in right ventricle (RV); it can be manifested as tall R wave in right precordial leads. This is due to changing direction of cardiac-vector to right In severe RVH, the cardiac vector rotated to right posterior causing deep S wave in V6. Uncorrected TF will expossed to prolong pressure overload and hypoxia, it can caused changes in cardiomyocite that can leads to RV dysfunction, low cardiac output syndrom (LCOS), and arrhythmias. Although the post operation survival rate was quite good, but worsening LCOS could increase mortality. In present time, the association between S wave in V6 and postoperative TOF outcomes, especially LCOS, has not been explained. Methods This is a cross sectional study. 150 TOF patients underwent total correction in 2013 included in this study. 35 patients who didn’t meet the inclusion criteria were excluded. Subjects divided in 2 groups: (1) patients who meets S in V6 criteria, and (2) control subjects as baseline characteristic. Bivariate analysis was done for incidence of LCOS, the variable with P<0.25 included in multivariate analysis. The significant value was p<0.5. Results Multivariate analysis showed S wave in V6 correlated with the incidence of LCOS with odds ratio 3.2, CI 95% (1.2-8.5), p=0.02. Conclusion The ECG findings S wave in V6 leads to diagnose RVH correlated with incidence of LCOS in post total correction TOF. An S wave criterion in V6 of RVH patients’ OR was 3.2 to predicts LCOS;Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease. Right ventricular hypertrophy (RVH) is an adaptive response due to pressure overload and hypoxia in right ventricle (RV); it can be manifested as tall R wave in right precordial leads. This is due to changing direction of cardiac-vector to right In severe RVH, the cardiac vector rotated to right posterior causing deep S wave in V6. Uncorrected TF will expossed to prolong pressure overload and hypoxia, it can caused changes in cardiomyocite that can leads to RV dysfunction, low cardiac output syndrom (LCOS), and arrhythmias. Although the post operation survival rate was quite good, but worsening LCOS could increase mortality. In present time, the association between S wave in V6 and postoperative TOF outcomes, especially LCOS, has not been explained. Methods This is a cross sectional study. 150 TOF patients underwent total correction in 2013 included in this study. 35 patients who didn’t meet the inclusion criteria were excluded. Subjects divided in 2 groups: (1) patients who meets S in V6 criteria, and (2) control subjects as baseline characteristic. Bivariate analysis was done for incidence of LCOS, the variable with P<0.25 included in multivariate analysis. The significant value was p<0.5. Results Multivariate analysis showed S wave in V6 correlated with the incidence of LCOS with odds ratio 3.2, CI 95% (1.2-8.5), p=0.02. Conclusion The ECG findings S wave in V6 leads to diagnose RVH correlated with incidence of LCOS in post total correction TOF. An S wave criterion in V6 of RVH patients’ OR was 3.2 to predicts LCOS, Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease. Right ventricular hypertrophy (RVH) is an adaptive response due to pressure overload and hypoxia in right ventricle (RV); it can be manifested as tall R wave in right precordial leads. This is due to changing direction of cardiac-vector to right In severe RVH, the cardiac vector rotated to right posterior causing deep S wave in V6. Uncorrected TF will expossed to prolong pressure overload and hypoxia, it can caused changes in cardiomyocite that can leads to RV dysfunction, low cardiac output syndrom (LCOS), and arrhythmias. Although the post operation survival rate was quite good, but worsening LCOS could increase mortality. In present time, the association between S wave in V6 and postoperative TOF outcomes, especially LCOS, has not been explained. Methods This is a cross sectional study. 150 TOF patients underwent total correction in 2013 included in this study. 35 patients who didn’t meet the inclusion criteria were excluded. Subjects divided in 2 groups: (1) patients who meets S in V6 criteria, and (2) control subjects as baseline characteristic. Bivariate analysis was done for incidence of LCOS, the variable with P<0.25 included in multivariate analysis. The significant value was p<0.5. Results Multivariate analysis showed S wave in V6 correlated with the incidence of LCOS with odds ratio 3.2, CI 95% (1.2-8.5), p=0.02. Conclusion The ECG findings S wave in V6 leads to diagnose RVH correlated with incidence of LCOS in post total correction TOF. An S wave criterion in V6 of RVH patients’ OR was 3.2 to predicts LCOS]

Abstrak :
Penyakit Jantung Bawaaan (PJB) Tetralogy of Fallot (TOF) merupakan PJB sianotik (biru) terbanyak. Tatalaksana definitif operasi sedini mungkin. Hampir semua pembiayaan menggunakan BPJS Kesehatan berdasarkan tarif INA-CBG. Adanya selisih negatif tarif INA-CBG tahun 2016 dengan tagihan perawatan operasi TOF menyebabkan banyak rumah sakit tidak mengoperasi dan merujuk ke RSJPD Harapan Kita. Dengan adanya tarif INA-CBG terbaru tahun 2023 yang mengalami kenaikan, diperlukan penelitian apakah terdapat selisih negatif. Perlu dianalisa faktor yang berhubungan dengan tagihan perawatan serta kepatuhan Clinical Pathway (CP) TOF. Penelitian observasional deskriptif cross sectional mengambil sampel pasien perawatan operasi total koreksi TOF tahun 2022 sejumlah 82 pasien. Hasil penelitian didapatkan sebagian besar usia ≥1 tahun, perempuan, status gizi normal, ada diagnosa sekunder, severitas PPK I (ringan) dan INA-CBG II (sedang), tidak ada komplikasi, kelas III, median lama CPB 103,5 menit dan AoX 55 menit, lama rawat post operasi 6 hari dan total 8 hari. Faktor yang berhubungan dengan tagihan perawatan adalah komplikasi, kelas rawat dan lama rawat CP. Hal yang belum sesuai dengan CP TOF yaitu lama rawat, pemeriksaan laboratorium, radiologi, pemakaian obat, alkes dan BMHP. Masih terdapat selisih negatif antara total tagihan perawatan pasien operasi total koreksi TOF tahun 2022 dengan total tarif INA-CBG terbaru tahun 2023. ......Tetralogy of Fallot (TOF) is the most common cyanotic Congenital Heart Disease (CHD). Definitive management is surgery earliest as possible. Almost all financing uses BPJS Kesehatan based on INA-CBG rate. Negative difference between the 2016 INA-CBG rates and TOF surgery treatment bill make many hospitals not operating and refering to Harapan Kita Hospital. With increasing in the latest 2023 INA-CBG rates, research for negative difference is needed. Analyzing factors related to care bills and adherence to TOF Clinical Pathway (CP) is studied too. This cross-sectional descriptive observational study took a sample of 82 patients undergoing TOF total  correction in 2022. Results showed that most were aged ≥1 year, female, normal nutritional status, had secondary diagnoses, severity of PPK I (mild) and INA-CBG II (moderate), no complications, class III, median duration of CPB 103.5 minutes and AoX 55 minutes, length of postoperative care 6 days and total of 8 days. Factors related to treatment bills are complications, class of care and CP length of care. Things not in accordance with the TOF CP are length of stay, laboratory, radiology, drug, medical equipment and BMHP use. There is still negative difference between the 2022 TOF total correction treatment patient bills and the latest 2023 INA-CBG rate.

Abstrak :
Latar Belakang: Low cardiac output syndrome (LCOS) pada operasi koreksi Tetralogy Fallot (TF) adalah komplikasi yang dapat meningkatkan angka kematian akibat cedera iskemia reperfusi. Terapi definitif cedera iskemia reperfusi belum ditemukan karena masalah translasi dari penelitian pada sel dan hewan coba ke penelitian pada manusia. Hatter Cardiovascular Institute merekomendasikan multitargeted therapy yang mengatasi obstruksi pembuluh darah (OPM), proteksi kardiomiosit, dan antiinflamasi. Penelitian ini menggunakan kombinasi siklosporin dan remote ischemic preconditioning (RIPC) untuk mencapai tujuan tersebut. Telah dilakukan pemeriksaan MDA dan edema mitokondria untuk membuktikan manfaat dari kombinasi siklosporin dan RIPC. Metodologi: Penelitian ini adalah uji acak tersamar ganda label terbuka yang dilakukan di RSCM dan JHC antara 2021 hingga 2022. Pasien TF usia 1-6 tahun diacak ke dalam kelompok kontrol yang mendapat terapi standar dan perlakuan yang mendapat siklosporin oral 3-5 mg/kgBB 2 jam sebelum operasi, dan RIPC sesaat setelah induksi. Limbah jaringan otot infundibulum dan sampel darah diambil di 3 fase: praiskemia, iskemia dan pascareperfusi. Ketiga jaringan yang diperoleh dilakukan isolasi mitokondria. Pemeriksaan JC-1 dan succinate dehydrogenase (SDH) dilakukan untuk mengukur kualitas isolat mitokondria. Pemeriksaan spektrofotometri terhadap isolat mitokondria dilakukan untuk mengukur edema mitokondria. Pemeriksaan MDA dilakukan untuk menilai stres oksidatif. Hasil: Terdapat 42 subyek yang mengikuti penelitian. Walaupun secara statistik tidak ada perbedaan bermakna kadar MDA, uji membran potensial, uji aktivitas enzim spesifik dan derajat edema mitokondria pada kelompok perlakuan dibandingkan kontrol namun terdapat kecenderungan penurunan MDA dan penurunan derajat edema mitokondria. Simpulan: Kombinasi siklosporin dan RIPC tidak bermakna secara statistik menurunkan kadar MDA dan derajat edema mitokondria pada pasien TF yang menjalani operasi koreksi. Terdapat kecenderungan penurunan MDA dan derajat edema mitokondria pada pasien TF yang mendapatkan siklosporin dan RIPC. ......Background: Low cardiac output syndrome on Tetrallogy Fallot correction surgery is a complication caused by ischemic reperfusion Injury (IRI), which increases mortality rate. Definitive treatment of IRI has not been found until now. A multitargeted treatment that attenuates microvascular obstruction, cardiomyocyte protection, and antiinflammation in human is proposed by Hatter Cardiovascular Institute. Based on this recommendation, this study used combination of cyclosporine as an antiinflammation and treatment for microvascular obstruction and remote ischemic preconditioning (RIPC) as a cardiomyocyte protection measure. Outcome of this treatment will be analyzed mostly by evaluation of mitochondrial edema. Methods: This is an open label randomized controlled trial on patients with Tetralogy of Fallot (TF) underwent surgery in RSCM and JHC on 2021 until 2022. We recruited 1-6 year-old TF patient planned for surgical correction. Cyclosporine and RIPC were given as treatment. Chopped infundibular muscle prior to aortic cross clamping defined as preischemic sample, after aortic cross clamping defined as postischemic sample, and after aortic cross clamping off defined as postreperfusion sample. We performed isolation of mitochondria of each sample. JC-1 and succinate dehydrogenase (SDH) assays were performed to measure quality of mitochondrial isolation. Results: Forty two subjects were recruited in this research. Although there was no significant difference in MDA level and the severity of mitochondrial edema between control and treatment group, we found lower MDA post- reperfusion and lower trend of mitochondrial edema in treatment group. Conclusion: Treatment of TF patient with Cyclosporine-RIPC combination therapy tends to reduce MDA level and mitochondrial edema significantly.

Abstrak :
Tetralogy of fallot is the most common form of cyanotic congenital heart disease, and one of the first to be successfully repaired by congenital heart surgeons. Although “fixed”, patients born with tetralogy of fallot cannot be considered “cured”. Improving survival and quality of life for this ever-increasing adult population will continue to challenge the current and future generations of cardiologists. Adult patients with tetralogy of fallot should be seen by a cardiologist specializing in the care of adults with congenital heart disease, to be monitored for late complications. They need to be checked regularly for any subsequent complications or disturbances of heart rhythm. This monograph is intended as both an introduction to the subject and a timely, comprehensive review, and will be welcomed by adult cardiologists, pediatric cardiologists, internists, surgeons, obstetricians, and intensivists who wish to learn about the most recent discoveries and advances concerning tetralogy of Fallot in adults. It will also be of interest to advanced undergraduates wanting to learn more about the subject.