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"Co-occurrence of cyanotic congenital heart disease (CCHD) and phaeochromocytoma (PCC) and paraganglioma (PGL) are rare, although some cases have been reported. We report a case of left paraganglioma in a 20-year-old lady with an underlying CCHD who underwent palliative Glenn shunt, subsequently developed polycythaemia and cavernous sinus thrombosis presented with palpitation, sweating, headache and hypertension of 3-months duration at the age of 17. The abdominal CT scan revealed an enhancing left paraaortic mass measuring 5.2 cm x 4.4 cm x 3.8 cm. A 24-hour urine catecholamine demonstrated raised noradrenaline level to six times upper limit of normal and hence diagnosis of left sympathetic (sPGL) was made. In view of the delayed diagnosis and significant morbidity associated with her condition, surgical treatment is no longer an option. Therefore, vigilant screening and early treatment of PCC-PGL in patients with CCHD are crucial in order to avoid significant morbidity and ensure a good quality of life."

"Penyakit Jantung Bawaan (PJB) sering dikaitkan dengan malnutrisi yang dipengaruhi oleh berbagai faktor yang mengakibatkan peningkatan morbiditas dan mortalitas, penatalaksana yang tepat dapat menurunkan infeksi, lama rawat, bahkan kematian. Penelitian ini dilakukan untuk mengidentifikasi faktor-faktor yang berhubungan dengan status gizi kurang pada anak dengan PJB. Penelitian ini menggunakan observasional analitik dengan rancangan case control.  Sampel penelitian berjumlah 114 anak PJB di Rumah Sakit Jantung Jakarta periode Juli 2020 hingga Juni 2023. Uji statistik menunjukkan tidak ada hubungan antara usia, jenis kelamin, riwayat BBLR, pemberian ASI eksklusif, jenis PJB dan penyakit penyerta terhadap status gizi kurang pada anak PJB, terdapat hubungan antara kelengkapan imunisasi dengan status gizi kurang pada anak PJB (p value <0,05). Simpulan: dari penelitian ini yaitu faktor nutrisi dan organik tidak berhubungan dengan status gizi kurang anak PJB. Oleh karena itu pelayanan perlu memberikan perhatian terkait status nutrisi dan imunisasi disamping masalah jantung.

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Congenital Heart Disease (CHD) is often associated with malnutrition which is influenced by various factors resulting in increased morbidity and mortality, appropriate management can reduce infection, length of stay, and even death. This research was conducted to identify factors associated with malnutrition status in children with CHD. This study used an analytical observational with a case control design. The research sample consisted of 114 CHD children at the Jakarta Heart Hospital for the period July 2020 to June 2023. The result of this study showed that there was no relationship between age, gender, history of LBW, exclusive breastfeeding, type of CHD and comorbidities on malnutrition status in CHD children, there is a relationship between complete immunization and malnutrition status in CHD children (p value <0.05). Conclusion from this research, nutritional and organic factors are not related to the malnutrition status of CHD children. Therefore, services need to pay attention to nutritional status and immunization in addition to heart problems."

"B-type natriuretic peptide(BNP), merupakan suatu bagian dari keluarga peptida natriuretik yang diproduksi oleh kardiak miosit dan disekreksi ke sirkulasi dalam merespon adanya peningkatan volume intrakardiakyangmenyebabkan diuresis, natriuresis dan vasodilatasi, juga menghambat sistem renin-angiotensin- aldosteron dan aktivitas simpatik. Anak dengan kelainan jantung bawaan akan terjadi peningkatan volumedan tekanan intrakardiaak yang signifikan sehingga diikuti peningkatan NT-proBNPdi sirkulasi.NilaiNT-proBNP akan meningkat pada awal kehidupan dan mulai turun secara drastissetelahnya.Malnutrisi merupakan penyebab morbiditas yang utama pada anak dengan penyakit jantung bawaan. Penelitian cross sectional observasional dengan jumlah 80 subjek yang memenuhi kriteria inklusi dan eksklusi yang akan menjalani operasi bedah jantung terbuka di Rumah Sakit Jantung dan Pembuluh Darah Harapan Kita dan yang akan menjalani pemeriksaan darah rutin dan direncanakan operasidi Rumah Sakit Ibu dan Anak Harapan kita pada bulan Februari - Mei 2019. Data pasien berupa data karakteristik demografis (usia, jenis kelamin, berat badandan tinggi badan) dan diagnostik pasien dicatat. Kadar NT-proBNP akan diambil prabedah. Analisis data menggunakan uji Mann-Whitney. Data nilai NT-proBNP pada pasien tanpa penyakit jantung bawaan sesuai rentang usia > 1bulan - <1 tahun dengan nilai tengah 142,5 pg/mL ( 49-935 pg/mL), >1 tahun - £2 tahun dengan nilai tengah 142 pg/mL ( 44-545 pg/mL) dan >2 tahun - £7 dengan nilai tengah 70 pg/mL ( 14-1440 pg/mL). Sedangkan nilai NT-pro BNP pasien dengan penyakit jantung bawaan dengan nilai tengah 2558, nilai terendah 426 pg/mL dan nilai tertinggi 33166 pg/mL. Hasilnya terdapat perbedaan bermakna (p = 0,001) nilai NT-pro BNP pada pasien tanpa penyakit jantung bawaan dan pasien dengan penyakit jantung bawaan. Terjadi peningkatan nilai tengah kadar NT-pro BNP > 20 kali antara anak tanpa penyakit jantung bawaan dan anak dengan penyakit jantung bawaan.Terdapat perbedaan yang bermakna kadar NT-pro BNP pada subjek tanpa penyakit jantung bawaan dan dengan penyakit jantung bawaan. Terjadi peningkatan kadar NT- pro BNP pada bayi baru lahir dan akan menurun seiring bertambah nya usia. Proporsi gangguan status gizi pada pasien dengan penyakit jantung bawaan mengalami peningkatan.

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B-type natriuretic peptide (BNP), a member of the natriuretic peptide family, is produced in cardiac myocytes and secreted into circulation in response to cardiac volume load, that causing diuresis, natriuresis and vasodilatation, as well as inhibition of the renin-aldosteron system and sympathetic activity. Children with heart disease caused increase pressure and cardiac volume followed by elevated NT-proBNP in circulation. NT-proBNP levels were significantly elevated in the first days of life and gradually decresed into normal level in child without heart disease. Malnutrition was the cause of morbidity for child with congenital heart disease.

We conducted a cross-sectional trial on 80 subjects who are fulfilled our study criteria. The study was performed at the National Cardiovascular Centre Harapan Kita and Mother and Child Hospital Harapan Kita Indonesia, in the period of February-Mei 2019. Subjects demographic data (age, gender, weight and height) and diagnoastic data were recorded. Levels of NT-proBNP were acquired presurgically. We utilized Mann - Whitney test to analysed the data.

NT-ProBNP levels data was observed from child without heart disease based on interval of age> 1month -£1 year with median value 142,5 pg/mL ( 49-935 pg/mL), >1 month -£2 year with median value 142 pg/mL ( 44-545 pg/mL) and >2 year - £7 year with median value 70 pg/ml ( 14-1440 pg/mL). Whereas NT-proBNP levels from child with heart disease median value 2558 pg/mL, minimum levels 426 pg/mL and maximum levels 33166 pg/ml. Statistically significant was observed (p=0,001) between NT-proBNP level of child without heart disease and child with heart disease.

There were 20 x times significantly increase levels of NT-proBNP of child with heart disease. There was statistically significant of levels NT-proBNP between child without heart disease and with heart disease. Study show high levels of NT-proBNP immediately after birth and gradually decline during childhood. The proportion of undernourished of child with congenital heart disease showed higher."

"Indonesian Journal of Dentistry 2006; Edisi Khusus KPPIKG XIV: 120-123Congenital heart disease is a condition of heart anomaly found since birth. The most common is ventricular septal defect whereby an aperture is found in the partition of heart chamber. It is estimated that 40,000 newborn in Indonesia have this defective condition. Dental treatment for patient with such condition must be undertaken in a very cautious way. Tooth with multiple caries can potentially lead to endocarditis bacteria. As such, dentist must be watchful on dental treatment that may worsen the patient's condition. To prevent endocarditis bacteria, a prophylaxis antibiotic is required as recommended by the American Heart Association (AHA). In this particular case, a dental treatment was undertaken to a child patient with congenital heart disease. Previously, the patient has undergone dental treatment in the form of multiple extraction under general anesthesia before conducting cardiac surgery. ln this case the treatment includes mortal pulpotomy, GIC restoration, and fissure sealant - all conducted in one visit under general anesthesia. After a year after the treatment,here are no complaint from the patient and no irregularity in x-ray results."

"Peran NT-proBNP sebagai penanda biologis untuk mengetahui terjadinya sindrom curah jantung rendah pada pasien pediatrik dengan penyakit jantung bawaan sianotik pascabedah jantung terbuka belumlah diketahui. NT-proBNP diharapkan dapat menjadi penanda sindrom curah jantung rendah sehingga dapat mengurangi morbiditas dan mortalitas. Penelitian cross sectional ini melibatkan 40 pasien pediatrik dengan penyakit jantung bawaan sianotik yang menjalani pembedahan jantung terbuka di Rumah Sakit Jantung dan Pembuluh Darah Harapan Kita, selama bulan Maret 2019-April 2019. Terdapat perbedaan bermakna antara kadar NT-proBNP prabedah, 4 jam pascabedah, 24 jam pascabedah dan 72 jam pascabedah terhadap kejadian sindrom curah jantung rendah (p<0,001). Kadar NT-proBNP tertinggi pada 24 jam pasca bedah dengan perbedaan bermakna terhadap kadar NT-proBNP prabedah (p<0,001), 4 jam pascabedah dan 72 jam pascabedah (p<0,001). Diperoleh pula variabel lain yang berhubungan secara bermakna dengan NT-proBNP yaitu usia, berat badan, jenis penyakit jantung bawaan sianotik, lama aortic cross clamp, lama cardiopulmonary bypass, lama ventilasi mekanik dan lama rawat PICU. Dapat disimpulkan bahwa kadar NT-proBNP yang tinggi sebagai penanda kejadian sindrom curah jantung rendah.

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The role of NT-proBNP as a biological marker to determined the occurrence of low cardiac output syndromes in pediatric patients with cyanotic congenital heart disease after open heart surgery was unknown. NT-proBNP was expected to be a marker of low cardiac output syndrome so that it can reduce morbidity and mortality. This cross-sectional study involved 40 pediatric patients with cyanotic congenital heart disease who underwent open heart surgery at National Cardiovascular Centre Harapan Kita, during March 2019-April 2019. There were significant differences between pre-operative levels of NT-proBNP, 4 hours postoperatively, 24 hours postoperatively and 72 hours postoperatively with the incidence of low cardiac output syndrome (p <0.001). The highest NT-proBNP level was 24 hours postoperatively with a significant difference in preoperative levels of NT-proBNP (p <0.001), 4 hours postoperatively and 72 hours postoperatively (p <0.001). Other variables that were significantly associated with NT-proBNP were age, body weight, type of cyanotic congenital heart disease, duration of aortic cross clamp, duration of cardiopulmonary bypass, duration of mechanical ventilation and length of stay of PICU. It can be concluded that high NT-proBNP level as a marker of low cardiac output syndrome."

"[ASTRAKLatar Belakang: Pengetahuan seseorang yang tidak memadai terhadap risiko suatu tindakan medis berpotensi menimbulkan kecemasan dan tuntutan sehingga diperlukan upaya komunikasi dan pemberian edukasi dengan baik. Pembedahan penyakit jantung bawaan (PJB) anak berisiko tinggi terjadi morbiditas hingga mortalitas. Terdapat kesan pemahaman ibu yang tidak optimal terhadap pembedahan PJB anak mereka. Penelitian ini bertujuan untuk menilai pengetahuan ibu terhadap pembedahan PJB.Metode: Penelitian ini menggunakan uji eksperimen tidak murni (kuasi eksperimen) untuk mengetahui perubahan pengetahuan dan uji potong lintang untuk mengetahui proporsi pengetahuan yang rendah sebelum diberikan edukasi pada ibu anak yang akan menjalani pembedahan jantung pada bulan Mei hingga Juli 2014 di Instalasi Pelayanan Jantung Terpadu (PJT) RSUPNCM. Pengetahuan ibu dinilai menggunakan kuesioner dan edukasi menggunakan media edukasi mini flipchart. Kuesioner dan media edukasi dikembangkan sendiri oleh peneliti. Pengetahuan akan dinilai saat praedukasi dan pascaedukasi.Hasil: Sebanyak 57 subyek diikutsertakan dalam penelitian dan tidak ada subyek yang dikeluarkan saat penelitian. Analisis menggunakan stratifikasi untuk tiap diagnosis dan jenjang pendidikan. Total 47 subyek yang dapat dianalisis. Didapatkan 49% subyek memiliki pengetahuan rendah praedukasi dan seluruh subyek (100%) mengalami peningkatan pengetahuan pascaedukasi.Simpulan: Pemberian edukasi mampu meningkatkan pengetahuan ibu anak dengan PJB yang akan menjalani pembedahan jantung.

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ABSTRACTBackground : Inadequate knowledge about risk of upcoming medical intervention may raiseanxiety and demands. Therefore good communication and education are required. CongenitalHeart Disease (CHD) children surgery has high risk of morbidity and mortality. There is animpression that the mother has lack understanding of their children?s surgery. This studyaimed to assess the mother?s knowledge of CHD surgery.Method : This study used quasi-experimental design to determine changes in maternalknowledge and cross-sectional tests to assess the proportion of low knowledge prior theprovision of education on the mothers whose children underwent heart surgery in May to July2014 in RSUPNCM. Knowledge of mothers was assessed using questionnaires before andafter the education. Education was provided using ?mini flipchart? media. Questionnaires andeducational media were developed by the researchers.Result : A total of 57 subjects were included in the study and no subjects were excludedduring the research. Analysis was done by using stratification for each diagnosis. A total of47 subjects can be analyzed. We found that 49% of the subjects had low knowledge prior theprovision of education and all subjects (100%) had increasing knowledge after education.Conclusion : The provision of education is able to increase the knowledge of mothers whosechildren will undergo congenital heart disease surgery.;Background : Inadequate knowledge about risk of upcoming medical intervention may raiseanxiety and demands. Therefore good communication and education are required. CongenitalHeart Disease (CHD) children surgery has high risk of morbidity and mortality. There is animpression that the mother has lack understanding of their children’s surgery. This studyaimed to assess the mother’s knowledge of CHD surgery.Method : This study used quasi-experimental design to determine changes in maternalknowledge and cross-sectional tests to assess the proportion of low knowledge prior theprovision of education on the mothers whose children underwent heart surgery in May to July2014 in RSUPNCM. Knowledge of mothers was assessed using questionnaires before andafter the education. Education was provided using “mini flipchart” media. Questionnaires andeducational media were developed by the researchers.Result : A total of 57 subjects were included in the study and no subjects were excludedduring the research. Analysis was done by using stratification for each diagnosis. A total of47 subjects can be analyzed. We found that 49% of the subjects had low knowledge prior theprovision of education and all subjects (100%) had increasing knowledge after education.Conclusion : The provision of education is able to increase the knowledge of mothers whosechildren will undergo congenital heart disease surgery., Background : Inadequate knowledge about risk of upcoming medical intervention may raiseanxiety and demands. Therefore good communication and education are required. CongenitalHeart Disease (CHD) children surgery has high risk of morbidity and mortality. There is animpression that the mother has lack understanding of their children’s surgery. This studyaimed to assess the mother’s knowledge of CHD surgery.Method : This study used quasi-experimental design to determine changes in maternalknowledge and cross-sectional tests to assess the proportion of low knowledge prior theprovision of education on the mothers whose children underwent heart surgery in May to July2014 in RSUPNCM. Knowledge of mothers was assessed using questionnaires before andafter the education. Education was provided using “mini flipchart” media. Questionnaires andeducational media were developed by the researchers.Result : A total of 57 subjects were included in the study and no subjects were excludedduring the research. Analysis was done by using stratification for each diagnosis. A total of47 subjects can be analyzed. We found that 49% of the subjects had low knowledge prior theprovision of education and all subjects (100%) had increasing knowledge after education.Conclusion : The provision of education is able to increase the knowledge of mothers whosechildren will undergo congenital heart disease surgery.]"

"Acute Kidney Injury (AKI) pada anak dengan penyakit jantung bawaan mencakup 5-33% dari seluruh pasien anak yang melalui bedah jantung terbuka, dengan dampak yang signifikan terhadap kualitas hidup dan luaran pasien. Salah satu faktor yang mempengaruhi kejadian AKI adalah durasi penggunaan mesin pintas jantung paru. Penelitian metode kohort retrospektif dilakukan terhadap 122 pasien dengan durasi panjang dan 73 pasien dengan durasi pendek pasca bedah jantung terbuka di PJT RSUPN Cipto Mangunkusumo. Data rekam medis yang dianalisis menunjukkan bahwa terdapat kemaknaan (p<0,05) hubungan antara durasi CPB dengan AKI dengan OR 2,95. Kesimpulan penelitian adalah durasi CPB >60 menit merupakan faktor risiko terjadinya AKI pasca bedah jantung terbuka.

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Acute kidney injury (AKI) in children with congenital heart disease consists of 5-33% pediatric patients who went through open heart injury, with significant impact on the quality of life and outcome of the patient. One of the factors affecting the incidence of AKI is the duration of cardiopulmonary bypass machine. Retrospective cohort study was done on 122 patients with bypass duration >60 minute and 73 patients with bypass duration <60 minute after open heart surgery in PJT RSUPN Cipto Mangunkusumo. Analysis of medical records shown that there was a significant difference (p<0,05) between the duration of cardiopulmonary bypass with the incidence of AKI with OR of 2,95. It was concluded that duration of bypass >60 minutes was a risk factor of post open heart surgery AKI."

"ABSTRACTPurpose: To evaluate the safety and efficacy of our new delayed sternal closure (DSC) method, involving sternal semi-closure using a bioresorbable osteosynthesis device and complete skin closure.Methods: Between 2013 and 2017, 36 patients underwent DCS at our hospital. The patients were divided into two groups based on the method used for DSC. The later conventional DSC group consisted of 18 patients undergoing late complete sternal closure following fixation of pulmonary and hemodynamic instability, and the new DSC group consisted of 18 patients undergoing early sternal semi-closure a few days after surgery. In the new DSC group, the sternum was fixed with Super Fixsorb MX40, followed by complete skin closure.Results: Respiratory and hemodynamic conditions, such as systolic blood pressure, cardiac index, tidal volume, and regional oxygen saturation, were significantly more stable in the new DCS group than in the conventional DSC group. The hospital stay was also significantly shorter in the new DSC group. Although there were no serious complications, one patient from the new DCS group suffered deformity of the sternum, which was managed successfully.Conclusion: The sternal semi-closure method decreases pulmonary and cardiac instability during DSC, making early DSC possible."

"Latar belakang: Society of Thoracic Surgeons – European Association of Cardiothoracic Surgeons (STS-EACTS) mortality score dan category merupakan sistem stratifikasi risiko terbaru yang dikembangkan sebagai prediktor mortalitas dan morbiditas pascaoperasi penyakit jantung bawaan (PJB). Namun belum pernah divalidasi di Indonesia. Tujuan: Melakukan validasi eksternal STS-EACTS mortality score dan category pada populasi Indonesia. Metode: Uji validasi dengan studi potong lintang, menggunakan data dari Rumah Sakit Pusat Jantung Nasional Harapan Kita bagian bedah jantung anak dan kongenital dari Januari 2015 – Desember 2019. Nilai sensitivitas, spesifisitas, dan area under curve – receiving operator characteristic (AUC-ROC) menjadi luaran utama dalam menilai kemampuan prediksi mortalitas dan pemanjangan lama rawat pascoperasi (PLRP). Hasil: Penelitian melibatkan 4139 subjek dengan tingkat mortalitas 5,4% (230 subjek). STS-EACTS score memiliki sensitivitas dan spesifisitas sebesar 65% dan 68% dalam memprediksi mortalitas, dengan 62% dan 71% dalam memprediksi PLRP. STS-EACTS category memiliki sensitivitas dan spesifisitas sebesar 63% dan 68% dalam memprediksi mortalitas, dengan 61% dan 75% dalam memprediksi PLRP. STS-EACTS category memperlihatkan kemampuan diskriminasi yang baik untuk mortalitas dan PLRP (AUC-ROC 0,704 dan 0,701). Sementara pada mortality score hanya memberikan hasil cukup untuk PLRP (AUC-ROC 0,704 dan 0,679). Kesimpulan: STS-EACTS category merupakan prediktor yang baik dalam menilai luaran mortalitas dan PLRP pascaoperasi PJB.

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Introduction: Society of Thoracic Surgeons – European Association of Cardiothoracic Surgeons (STS-EACTS) mortality score and category were the latest risk stratification for congenital heart disease (CHD) surgery, but it hasn't been validated in Indonesia.

Aim: Validate STS-EACTS score and category in Indonesian population.

Methods: Cross-sectional validation study was done using the dataset of Paediatric and Congenital Heart Surgery Department, National Cardiovascular Center Harapan Kita Indonesia, from January 2015 – December 2019. Sensitivity, specificity and area under curve – receiving operator characteristic (AUC-ROC) are used to assess its ability to predict mortality and PHLOS.

Results: This study enrolled 4139 subjects with 5.4% mortality rate. The STS-EACTS score have the sensitivity and specificity of 65% and 68% to predict mortality, with 62% and 71% to predict PHLOS. The STS-EACTS category have the sensitivity and specificity of 63% and 68% to predict mortality, with 61% and 75% to predict PHLOS. The STS-EACTS category shows good discrimination ability in predicting mortality and PHLOS (AUC-ROC 0.704 and 0.701), whereas the mortality score only gives sufficient results in predicting PHLOS (AUC-ROC 0.704 and 0.697).

Conclusions: The STS-EACTS category is a good predictor that can be used in Indonesian population to predict mortality and PHLOS following CHD surgery."