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"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."

"[ABSTRAKHipertrofi 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.MetodePenelitian 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.HasilUsia 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=0.02KesimpulanKriteria EKG gelombang S di sadapan V6 untuk diagnosis HVKa berhubungan dengan kejadian SCJR pasca total koreksi pasien TF.

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ABSTRACTTetralogy 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.MethodsThis 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.ResultsMultivariate 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.ConclusionThe 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.MethodsThis 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.ResultsMultivariate 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.ConclusionThe 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.MethodsThis 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.ResultsMultivariate 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.ConclusionThe 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.MethodsThis 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.ResultsMultivariate 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.ConclusionThe 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.MethodsThis 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.ResultsMultivariate 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.ConclusionThe 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]"

"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

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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."

"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."

"Tetralogy of fallot (ToF) is the most common form form of cyanotic congenital heart disease after 1 year of age, with overall incidence approaching 10 % of all congenital heart disease...."

"Latar belakang. Evaluasi fungsi ventrikel kanan (right ventricle = RV) pada pasien dengan tetralogy of fallot (TOF) penting dilakukan, karena gangguan fungsi RV akan mempengaruhi hasil keluaran dan prognosis setelah operasi. Hipoksia serta beban tekanan pada RV yang kronis pada anak TOF, terutama pada usia lebih besar akan menyebabkan penurunan fungsi RV yang dapat dinilai melalui beberapa parameter ekokardiografi. Tujuan dari penelitian ini adalah rnengetahui fungsi RV pada pasien TOF yang belum menjalani operasi dan subjek normal, fungsi RV antara kelornpok usia < 4 tahun dan 4 tahun keatas, serta mengetahui korelasinya dengan usia, saturasi dan hematokrit sebelum operasi, serta right ventricular end diastolic pressure (RVEDP). Metode Penelitian. Penelitian ini adalah studi potong lintang. Subjek penelitian adalah pasien TOF yang belum menjalani operasi dan subjek normal tanpa kelainan struktural jantung. Penelitian ini dilakukan di Pusat Jantung Nasional Harapan Kita, bulan Juli sampai November 2011. Penilaian fungsi RV dilakukan menggunakan ekokardiografi melalui beberapa parameter yaitu TAPSE, MPI, S, E, A velocity; IV A dan strain speckle tracking 2D. Diambil juga data usia, saturasi perifer, kadar hematokrit dan RVEDP. Data kemudian diolah dengan menggunakan SPSS 11. Hasil. Pada kelompok TOF ditemukan penurunan fungsi RV hila dibandingkan dengan subjek normal, yang dapat dilihat dari nilai TAPSE (1,6 ± 0,24 ern vs 1,87 ± 0,23 em, p = 0,000 ); RV MPI (0,37 ± 0,11 ms vs 0.26 ± 0,05 rns, p = 0,000); S, E, A velocity (12.6 ± 2.2 ern/s vs 13.9 ± 2.02 ern/s, p 0,000; 13.2 ± 4.1 ern/s vs 17.76 ± 2.9 ern/s, p = 0,000; 14.56 ± 4.9 ern/s vs 10.64 ± 2.8 ern/s , p = 0,001) ; IV A (3.87 ± 1.4 rn/s2 vs 4.57 ± 1.6 rn/s2 , p = 0.050) dan strain global speckle traking 2D (-24.78 % ± 5,80 vs -33.29 % ± 5,82 , p = 0,000). Tidak diternukan perbedaan fungsi RV pada kelompok usia< 4 tahun dan 4 tahun keatas keeuali untuk nilai T APSE (p = 0.03). Dari multivariat analisis didapatkan usia dan saturasi oksigen perifer berkorelasi sedang dengan perbedaan nilai TAPSE pada kelornpok TOF (r = 0,4, p = 0.023, dan r = 0,4 , p = 0.037). Kesimpulan. Pada pasien TOF terjadi penurunan fungsi RV jika dibandingkan dengan subjek normal. Tidak terlihat adanya perbedaan fungsi RV antara kelompok umur pada pasien TOF keeuali pada parameter T APSE. Usia dan saturasi oksigen perifer berkorelasi sedang dengan perbedaan nilai TAPSE pada kelornpok TOF.

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Background. Evaluation of righfve~nction (RV =right ventricle) in tetralogy of fallot (TOF) patient is important because -of RV dysfunction would influence the outcome and prognosis after surgery. Chronic hypoxia and RV pressure overload in TOF patient, especially at older age, would decrease RV function, which can be assessed through multiple echocardiography parameters. The purpose of this study was to determine RV function in preoperative TOF patients and normal subject, between age group of <4 years old a'1d ~4 years old, and its correlation with age, preoperative peripheral saturation and hematocrit and right ventricular end diastolic pressure (RVEOP). Methods. This study is the cross sectional. The study subject is preoperative TOF patients and normal subject , performed at National Cardiovascular Center Harapan Kita, from July to November 2011. Assessment of RV function performed through several echocardiographic parameters: T APSE, MPI, S, E, A velocity; IV A and Speckle tracking 2D strain. We also collect data about age, preoperative peripheral saturation and hematocrit levels, and RVEOP. The data were analized using SPSS 11. Results. In TOF group there were decreased RV function when compared with normal subject group, which can be seen from TAPSE values (1.6 ± 0.24 em vs. 1.87 ± 0.23 em, p = 0.000); RV MPI (0.37 ± 0:26 ms vs. 0.11 ± 0.05 ms, p = 0.000); S, E, A velocity (12.6 ± 2.2 cm/s vs. 13.9 ± 2:02 cm/s, p 0.000; 13.2 ± 4.1 cm/s vs. 17.76 ± 2.9 cm/s, p = 0.000; 14:56 ± 4.9 cm/s vs. 10.64 ± 2.8 cm/s, p = 0.001); IV A (3.87 ± 1.4 m/s2 vs. 4:57 ± 1.6 m/s2 , p = 0050) and global strain speckle traking 20 (-24.7 ± 5,80 % vs. -33.29 ± 5.82%, p = 0.000). There were no difference found in RV function in the age group of <4 years old and ~4 years old except for the T APSE value (p = 0.03). From multivariate analysis, age and peripheral oxygen saturation moderately correlate with differences in TAPSE value in TOF group (r = 0,4, p = 0.023, dan r = 0,4 , p = 0.037). Conclusions. In TOF patients there were decreased RV function when compared with normal subject. There were no differences in RV function between age group in TOF patients except in T APSE value. Age and peripheral oxygen saturation moderately correlate with the difference in TAPSE value in the TOF group."

"TOF dengan kriteria risiko tinggi pembedahan membutuhkan tindakan paliatif sebelum menjalani operasi korektif yaitu TOF repair. Pemasangan stent Alur Keluar Ventrikel Kanan (Stenting AKVK) yang merupakan terapi paliatif transkateter invasif beberapa tahun terakhir muncul sebagai alternatif terhadap terapi paliatif pembedahan modified-Blalock-Thomas Taussig Shunt (mBTTS). Akan tetapi, studi mengenai pola pertumbuhan arteri pulmonal pasca stenting AKVK dan mBTTS di Indonesia belum ada. Membandingkan kecepatan pertumbuhan arteri pulmonal pasca stenting AKVK dan mBTTS pada Fisiologi TOF. Studi merupakan uji klinis kohort retrospektif pada pasien penyakit jantung bawaan dengan fisiologi TOF yang menjalani terapi paliatif di Rumah Sakit Jantung dan Pembuluh Darah Harapan Kita pada Desember 2019-Desember 2023. Pertumbuhan LPA dan RPA pasca terapi paliatif dinilai dengan serial ekokardiogram. Luaran primer yaitu pertumbuhan cabang arteri pulmonal harian menggunakan z-score per hari yang dianalisis dengan mixed-model. Sebanyak total 139 pasien [pasca stenting AKVK (n=35) dan pasca mBTTS(n=104)] dengan total 492 ekokardiogram serial didapatkan median z-score RPA/hari pasca stenting AKVK (2,5 x 10-3) lebih tinggi dibandingkan mBTTS (0,9 x 10-3) bermakna secara statistik (p<0,001). Median z-score LPA/hari pasca stenting AKVK (1,7 x 10-3) lebih tinggi dibandingkan mBTTS (0,7 x 10-3), bermakna secara statistik (p<0,001). Stenting AKVK memiliki rasio distribusi pertumbuhan LPA terhadap RPA secara bermakna yang lebih baik (0,93 (IQR 0,87-1,01) dibandingkan mBTTS [0,91(IQR 0,83-0,98),p<0,01]. Pertumbuhan cabang arteri pulmonal kanan dan kiri secara bermakna lebih cepat pasca stenting AKVK dibandingkan dengan mBTTS serta menghasilkan distribusi pertumbuhan cabang arteri pulmonal yang lebih simetris dibandingkan mBTTS pada pasien dengan TOF Fisiologi.

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Right Ventricular Outflow Tract Stenting (RVOT stenting) has emerged as an invasive transcatheter palliative therapy alternative to modified-Blalock-Thomas Taussig Shunt (mBTTS). However, pulmonary artery growth rate comparison after both option is limited. This study aimed to compare pulmonary artery growth rate after RVOT stenting and mBTTS on TOF physiology. The study was a consecutive retrospective cohort of TOF physiology who underwent palliative therapy at the Cardiovascular Center Harapan Kita between December 2019-2023. RPA and LPA growth post palliative therapy assessed by serial echocardiogram. Primary outcome was daily pulmonary artery growth represented by z-score per day and analysed using mixed-model. Total 139 patients [RVOT stenting (n=35); mBTTS (n=104)] were obtained. Results of analysis of a total 492 echocardiograms, median RPA z-score/day after RVOT stenting were higher significantly [(2.5 x 10-3 ) vs (0.9 x 10-3), p<0.001)]. Median LPA z- score/day after RVOT stenting were higher significantly [(1.7 x 10-3) vs (0.7 x 10-3), p<0.001)]. RVOT stenting had better LPA to RPA growth distribution ratio significantly [0.93 (IQR 0.87-1.01) vs 0.91(IQR 0.83-0.98),(p<0.01)]. RVOT stenting significantly promotes faster growth of pulmonary artery branches resulting in a more symmetric distribution of growth than mBTTS in TOF physiology."

"Latar belakang: Peningkatan pada hemoglobin (Hb) dan hematokrit (Ht) pada anak dengan penyakit jantung bawaan sianosis. Kondisi ini menyebabkan perubahan hemodinamik dan koagulasi yang dapat meningkatkan mortalitas dan morbiditas pasien. Operasi reparasi Tetralogy of Fallot (TOF) di Indonesia seringkali terlambat sehingga pasien menderita hipoksemia kronis. Penelitian ini bertujuan untuk mengetahui hubungan kadar Hb dan Ht pada luaran hasil operasi reparasi TOF serta mengetahui titik potong Hb dan Ht yang optimal untuk menghindari morbiditas dan mortalitas pascaoperasi. Metode: Dilakukan suatu studi retrospektif kohort pada pasien yang menjalani operasi reparasi TOF di Pusat Jantung Nasional Harapan Kita dalam periode 1 Januari 2015 hingga 30 Juni 2020. Analisis statistik dilakukan pada kadar Hb dan Ht praoperasi TOF dengan luaran mortalitas, kejadian perdarahan, transfusi darah, reoperasi, dan defisit neurologis pascaoperasi untuk menilai titik potong Hb dan Ht optimal serta pengaruh pada masing-masing luaran operasi tersebut.Hasil: Sebanyak 806 pasien dilibatkan sebagai sampel penelitian dengan median usia 53 bulan dan SpO2 80%. Terdapat pengaruh bermakna usia, berat badan, dan SpO2 terhadap Hb dan Ht (p<0,05). Terjadinya peningkatan risiko perawatan >72 jam sebesar 1,5 kali lipat pada Hb yang tinggi serta risiko sebesar 1,6 kali lipat pada Ht yang tinggi. Terdapat peningkatan risiko perdarahan pascaoperasi >10mL/Kg sebesar 4,6 kali lipat pada Hb yang tinggi serta peningkatan risiko sebesar 5,4 kali lipat pada Ht yang tinggi. Tidak terdapat pengaruh bermakna Hb dan Ht terhadap kematian intraperawatan, kejadian reoperasi, dan defisit neurologis pascaoperasi. Diperoleh titik potong (nilai optimal) Hb 16,75 gr/dL dan Ht 51,20% dengan kemampuan prediktor yang cukup baik (AUC Hb = 0,71; AUC Ht = 0,72).Simpulan: Peningkatan hemoglobin dan hematokrit secara bermakna mempengaruhi durasi ICU, perdarahan pascaoperasi, dan banyaknya transfusi pascaoperasi. Untuk praoperasi TOF, diperoleh kadar optimal Hb di bawah 16,75gr/dL dan Ht di bawah 51,20%.

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Introduction: Increase of hemoglobin (Hb) and hematocrit (Ht) occurs in children with cyanotic heart disease. These conditions will lead some hemodynamic and coagulation changes that can increase patient mortality and morbidity. Tetralogy of Fallot (TOF) repair surgery in Indonesia mostly in late condition, where the patient suffers from chronic hypoxemia. Aim of this study are to determine the impact of high Hb and Ht on TOF repair surgery outcome as well as to determine the optimal value of Hb and Ht to avoid postoperative morbidity and mortality.

Method: A retrospective cohort study was conducted on patients undergoing TOF repair surgery Pusat Jantung Nasional Harapan Kita from January 1, 2015 until June 30, 2020. Statistical analysis was carried out on the preoperative TOF Hb and Ht levels with mortality, bleeding incidence, blood transfusion, reoperation, and postoperative neurological deficits to find the optimal Hb and Ht cutoff point and the effect on each of these operative outcomes.

Results: A total of 806 patients were included in the study sample with median age of 53 months and an SpO2 of 80%. There was a significant effect of age, body weight, and SpO2 on Hb and Ht (p <0.05). There was an increased risk of treatment > 72 hours by 1.5 times for high Hb and a risk of 1.6 times for high Ht. There an increased risk of postoperative bleeding> 10mL / Kg by 4.6 times in high Hb and an increased risk of 5.4 times in high Ht. Transfusions> 15mL / Kg were found to increase by 1.5 times at high Hb levels and 1.7 times at high Ht levels. There was no significant effect of Hb and Ht on inhospital mortality, reoperative incidence, and postoperative neurological deficits. The cut points obtained in this study were Hb 16.75 gr / dL and Ht 51.20% with a fairly good predictor ability (AUC Hb = 0.71; AUC Ht = 0.72) on postoperative bleeding.

Conclusion: High hemoglobin and hematocrit is significantly affected the ICU duration, postoperative bleeding, and the number of transfusions. The cut-off point taken from the relationship between hemoglobin and hematocrit on postoperative bleeding has a fairly good predictor ability. Optimal hemoglobin is below 16.75 gr/dL and optimal hematocrit is below 51.20%."

"ABSTRAKLatar belakang: Keberhasilan operasi TF usia terlambat memiliki kegagalan karena risiko sindrom curah jantung rendah akibat disfungsi ventrikel sebelumnya. Tujuan: mengetahui efek disfungsi ventrikel hasil kateterisasi terhadap luaran jangka pendek sindrom curah jantung rendah dan mortalitas pascakoreksi TF. Metode: penelitian kohort retrospektif data rekam medis. Pasien TF pascakateterisasi diagnostik periode 1 Januari 2015-31 Desember 2016, diikuti dan dievaluasi luaran pascaopersinya berupa mortalitas, sindrom curah jantung rendah dan sindrom respon inflamasi sistemik SRIS . Hasil: terdapat 186 pasien kateterisasi, memiliki 114 data hemodinamik lengkap, 92 menjadi subjek penelitian. Rentang usia 6 bulan-23 tahun 6 bulan, laki-laki lebih banyak daripada perempuan 49 53,3 banding 43 46,7 . Status gizi kurang 64 69,9 , abses serebri 6 6,5 , riwayat spell 64 69,6 , mengalami hemokonsentrasi 56 60,9 , tekanan akhir diastolik ventrikel kanan TDAVKa >12 mmHg 44 47,8 dan ventrikel kiri TDAVKi >12 mmHg 46 50 . Karakteristik pascabedah adalah SRIS 42 45,7 , mortalitas 16 17,4 dan sindrom curah jantung rendah 64 69,6 . Analisis bivariat terhadap mortalitas bermakna untuk saturasi udara ruangan [OR 0,94 IK 0,88-1,003; p=0,037 ], bermakna terhadap sindrom curah jantung rendah adalah peningkatan TDAVKa p=0,017 dan TDAVKi p=0,024 , spell berulang p=0,03 dan SRIS p

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ABSTRACTBackground successful TF correction at late age has failure because low cardiac output syndrome risk due to known ventricular dysfunction before. Objective to determine the effect of ventricular dysfunction catheterization measurement on short term outcome, comprises of low cardiac output syndrome LCOS and mortality. Methods a retrospective cohort study of medical record. TF child performed diagnostic catheterization from 1 January 2015 31 December 2016, followed and evaluated surgery outcome. Results 186 TF catheterization patients that period, with 114 had complete hemodynamic data, only 92 as research subjects. The age range was 6 months 23 years 6 months, boys were more than girls 58 55.2 vs 46 43.8 . Undernourish was 64 69.9 , 6 6.5 cerebral abscesses, 64 69.6 had spell history, hemoconcentration 56 60.9 , right ventricular end diastolic pressure RVEDP 12 mmHg 44 47.8 and left ventricular end diastolic pressure LVEDP 12 mmHg 46 50 . Outcome for SIRS 42 45.7 , mortality 16 17.4 and LCOS 64 69.6 . Bivariate analysis of predictor variables on mortality was significant for room air saturation, OR 0.94 IK 0.88 1.003, p 0.037 , significant for LCOS was elevated RVEDP variable p 0,017 and LVEDP p 0,024 , recurrent spell p 0,03 and SIRS p"

"Penelitian dengan menggunakan pendekatan kualitatif ini mengkaji ketidaktepatan pengisian catatan rekam medis dan ketidaktepatan melakukan koding dalam INA-CBG yang menyebabkan pelayanan menanggung risiko financial pada kasus Tetralogy of Fallot di unit Pediatrik Kardiologi dan Penyakit Jantung Bawaan RS Harapan Kita periode Januari-September 2013. Dengan hasil 21,4% kasus dengan diagnosis sekunder yang tidak lengkap dan selisih klaim Rp 251.273.615,00 (4%). Faktor yang menyebabkan ketidaklengkapan isian rekam medik adalah: tanggung jawab, sarana, standar pelayanan operasional, pembinaan, pemantauan, dan sosialisasi. Saran untuk dilakukan peningkatan sarana dan prasarana fisik serta pengelolaan kebijakan seperti adanya SPO pengisian rekam medis, sosialisasi, pembinaan staf dan pemantauan secara berkala."