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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."

"Latar belakang: Kekurangan gizi merupakan penyebab umum morbiditas pada anak dengan penyakit jantung bawaan (PJB). Data dari negara berkembang memperlihatkan prevalensi malnutrisi penderita dengan PJB sebelum dioperasi mencapai 45%. Penelitian ini bertujuan untuk mengetahui profil anhropometrik dan prevalensi kekurangan gizi pada anak dengan PJB dengan melakukan pengukuran anthropometrik.Metode: Penelitian ini merupakan penelitian dengan rancang bangun cross sectional pada anak berusia 0-2 tahun dengan PJB di RSCM. Pengukuran antropometri (berat badan, panjang badan, lingkar kepala) dilakukan pada seluruh pasien. Kekurangan gizi, failure to thrive/FTT, perawakan pendek, mikrosefali dinilai dengan menggunakan rekomendasi WHO tahun 2006, berupa perhitungan z-skor BB/PB, BB/U di 2 titik, PB/U dan LK/U < -2 SD.Hasil: Total subyek dalam penelitian ini berjumlah 95 orang, 73 orang dengan asianotik dan 22 orang dengan PJB sianotik. Prevalensi kekurangan gizi sebesar 51,1% dengan 22,3% diantaranya adalah gizi buruk. FTT terdapat pada 64,9%, perawakan pendek pada 49,5% dan mikrosefali pada 37% pasien. FTT ditemukan lebih banyak pada pasien dengan lesi asianotik (72,2%) dibandingkan dengan lesi sianotik (42,9). Pada lesi asianotik, berat badan lebih dipengaruhi daripada panjang badan (72,2% dengan 49,3%). Pasien dengan lesi sianotik, berat dan panjang badan akan dipengaruhi secara seimbang (42,9% dengan 54.5%). Konsultasi diet diberikan kepada pasien dengan kekurangan gizi. Terapi obat-obatan, intervensi transkateter atau bedah diindikasikan pada pasien tertentu.Kesimpulan: Prevalensi FTT lebih tinggi dibandingkan dengan kekurangan gizi pada anak dengan kelainan jantung kongenital. FTT ditemukan lebih banyak pada pasien dengan lesi asianotik. Pada lesi asianotik, berat badan lebih dipengaruhi daripada panjang badan. Pada lesi asianotik, berat badan lebih dipengaruhi daripada panjang badan.

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AbstractBackground: Undernutrition is a common cause of morbidity in children with CHD. Previous data from developing country showed prevalence of preoperative undernutrition in children with CHD was up to 45%. The aim of this study are to determine the anthropometric profi les and prevalence of undernutrition in children with CHD by using the anthropometric measurement.Methods: A cross-sectional study was carried out in children aged 0-2 years old with CHD in Cipto Mangunkusumo hospital. All patients underwent an anthropometric evaluation (weight, length and head circumference) at presentation. Undernutrition, failure to thrive /FTT, short stature and microcephaly were determined according to WHO, weight-forlength, weight-for-age at 2 points, length-for-age, head circumference-for-age z-score < -2SD accordingly.Results: We had total of 95 patients, 73 patients with acyanotic and 22 patients with cyanotic lesions. Prevalence of undernutrition in CHD was 51.1%, with 22.3% severe undernutrition. FTT was found in 64.9%, short stature in 49.5% and microcephaly in 37% patients. FTT was found higher in acyanotic (72.2%) compared to cyanotic lesions (42.9%). In acyanotic, weight was affected more than length (72.2% vs 49.3%). In cyanotic, weight and length affected equally (42.9% vs 54.5%). Diet counseling were done in patients with undernutrition. Medicines, transcatheter or surgery intervention were indicated in selected patients.Conclusions: Prevalence of FTT was higher than undernutrition in children with CHD. FTT was found higher in acyanotic lesions. In acyanotic, weight was affected more than length. In cyanotic, weight and length affected equally. "

"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."

"Pemberian air susu ibu ASI merupakan bentuk pemberian makanan yang paling disarankan untuk semua bayi, termasuk bayi dengan kebutuhan medis khusus seperti penyakit jantung bawaan. Penelitian ini bertujuan untuk memaparkan perilaku pemberian ASI pada 165 ibu yang memiliki bayi penderita penyakit jantung bawaan. Metode penelitian menggunakan survey deskriptif kuantitatif. Pengumpulan data menggunakan kuesioner pemberian ASI dan kuesioner perilaku pemberian ASI yang dimodifikasi dari penelitian Rickman 2017. Pemberian ASI eksklusif pada bayi penderita penyakit jantung bawaan hanya sebesar 36,4. Responden berusia 21-39 tahun tidak memberikan ASI eksklusif, begitupun dengan responden berpendidikan tinggi, tidak bekerja, berpendapatan cukup, multipara, dan berpengetahuan baik. Berdasarkan riwayat persalinan, responden yang melahirkan di fasilitas kesehatan, melahirkan secara sesar, melakukan inisiasi menyusu dini IMD. dan yang dirawat gabung tidak memberikan ASI eksklusif. Pada variabel dukungan sosial, responden yang mendapat dukungan suami dan ibu/mertua tidak memberikan ASI ekslusif. Sebanyak 62,2 bayi penderita kelainan asianotik dan 65,3 bayi penderita kelainan sianotik tidak mendapatkan ASI eksklusif. Kondisi medis bayi yang menyebabkan kendala menyusu pada bayi merupakan faktor utama tidak berhasilnya pemberian ASI eksklusif pada bayi penderita penyakit jantung bawaan. Hasil penelitian juga menunjukkan bahwa tenaga kesehatan kurang memberikan motivasi dan dukungan pada responden untuk memberikan ASI secara eksklusif. Hasil studi ini dapat menjadi informasi untuk menerapkan konseling ASI yang efektif dan tenaga kesehatan diharapkan mampu memberikan dukungan dan motivasi pada ibu untuk memberikan ASI secara eksklusif pada bayinya.

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Breastfeeding is the most recommended feeding for all infants, including infants with special medical needs such as congenital heart disease. This study aims to describe the breastfeeding behavior in 165 mothers who have infants with congenital heart disease. This research method used. quantitative descriptive survey. Data were collected using. modified breastfeeding and breastfeeding behavior questionnaire from Rickman 2017 study. Exclusive breastfeeding in infants with congenital heart disease is only 36.4. Respondents aged 21 39 years old did not provide exclusive breastfeeding, as did high educated, unemployed, fair income, multiparent, and knowledgeable respondents. Based on the history of labor, respondents who gave birth at. health facility, delivered by cesarean section, initiated breastfeeding, and who were treated together with their infants did not provide exclusive breastfeeding. In social support variables, respondents who have the support of husband and mother mother in law did not provide exclusive breastfeeding. As many as 62.2 of infants with asianotic abnormalities and 65.3 of infants with cyanotic abnormalities were not exclusively breastfed. The infant 39. medical condition that causes breastfeeding difficulties in infants is. major factor in the failure of exclusive breastfeeding in infants with congenital heart disease. The results also show that health workers less motivation and support to respondents to exclusively breastfeed. The results of this study can become an information to implement effective breastfeeding counseling and health workers are expected to provide support and motivation in mothers to exclusively breastfeed their babies. "

"Latar Belakang: Infeksi menjadi masalah pada anak dengan penyakit jantung bawaan (PJB),terutama pneumonia. Faktor risiko yang mendasari perjalanan pneumonia pada anak adalah:usia, jenis kelamin, status gizi, pemberian ASI, berat lahir rendah, status imunisasi,pendidikan orangtua, status sosioekonomi, penggunaan fasilitas kesehatan. Insidenspneumonia pada anak dengan PJB pirau kiri ke kanan meningkatkan morbiditas danmortalitas.Tujuan: Mengetahui insidens pneumonia anak dengan PJB pirau kiri ke kanan dan faktorrisiko yang terkait.Metode: Penelitian ini adalah studi analitik dengan rancangan cohort retrospectiveberdasarkan penelusuran rekam medis di RSUPN dr. Cipto Mangunkusumo dari tahun 2015 -2019, Jakarta. Diagnosis PJB pirau kiri ke kanan berdasarkan echocardiography. Dari hasilyang ada, dilakukan analisis multivariat dan dilaporkan sebagai odds ratio (OR).Hasil: Dari 333 subyek dengan PJB pirau kiri ke kanan, 167 subyek mengalami pneumonia(50,2%). Proporsi jenis PJB pirau kiri ke kanan terbanyak yang menyebabkan pneumoniaadalah defek septum ventrikel (VSD), yaitu 41,9%. Defek ukuran besar berhubungan denganangka kejadian pneumonia (p=0,001). Faktor risiko yang mempengaruhi kejadian pneumoniapada anak dengan PJB pirau kiri ke kanan antara lain: status gizi buruk [OR 5,152 (95% CI2,363-11,234)], status imunisasi tidak lengkap [OR 9,689 (95% CI 4,322-21,721)], statussosioekonomi rendah [OR 4,724 (95% CI 2,003-11,138)], dan ukuran defek yang besar [OR5,463 (95% CI 1,949-15,307)].Simpulan: Insidens pneumonia pada anak dengan PJB pirau kiri ke kanan sebesar 50,2 %.Tipe PJB dengan insidens pneumonia terbanyak adalah VSD. Status gizi, imunisasi, statussosioekonomi dan ukuran besar defek mempengaruhi angka kejadian pneumonia."

"[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.]"

"Latar belakang: Penyakit jantung bawaan (PJB) pirau kiri ke kanan adalah penyebab penting gagal tumbuh pada anak. Koreksi terhadap defek tersebut diketahui memperbaiki prognosis pertumbuhan berat maupun tinggi badan. Tujuan. Mengetahui korelasi usia koreksi defek dengan pertambahan tinggi badan pada pasien PJB pirau kiri ke kanan pasca-koreksi terhadap prognosis pertumbuhan. Metode: Penelitian dilakukan secara potong lintang dengan menggunakan rekam medis pada subyek dengan PJB pirau kiri ke kanan yang dikoreksi kurang dari 2 tahun di Rumah Sakit Cipto Mangunkusumo (RSCM) dengan variabel bebas usia koreksi defek dan variabel terikat z-skor dan Δz-skor TB/U pasca-koreksi. Referensi pertumbuhan menggunakan kurva WHO 2006. Perhitungan korelasi dilakukan menggunakan korelasi Spearman dan kemaknaan ditetapkan dengan p<0,05. Hasil: Median usia koreksi defek pada penelitian ini adalah 8 bulan dengan usia koreksi terbanyak adalah kurang dari 6 bulan dan usia 6-12 bulan masing-masing sebanyak 11 orang. Defek terbanyak adalah VSD. Usia koreksi defek tidak berkorelasi dengan z-skor TB/U pasca-koreksi berdasarkan uji korelasi Spearman (r= 0,093) dengan nilai p=0,642. Usia koreksi defek dengan Δ z-skor TB/U tidak ditemukan korelasi berdasarkan uji korelasi Spearman (r=0,143) dengan nilai p=0,452. Kesimpulan: Usia koreksi defek tidak terbukti berkorelasi baik dengan z-skor TB/U maupun Δ z-skor TB/U pasca-koreksi.

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Background: Congenital heart disease (CHD) left-to-right shunt is an important cause of growth failure in children. Correction of these abnormalities is known to improve the prognosis of growth in weight and height.

Objectives: Identify correlation between age of defect correction and height gain in patients with left-to-right shunt CHD after correction of growth prognosis Methods. This was a cross sectional study with reviewing medical records on subjects with CHD with left-to-right shunts who were corrected for less than 2 years at Cipto Mangunkusumo hospital with the independent variable being the age of defect correction and the dependent variable were z-score of post-correction height-for-age (H/A) and height gain (Δz-score H/A). The WHO 2006 growth chart were used as the growth reference. The correlation analysis was performed using the Spearman correlation and the significance was determined with p<0.05.

Results: The median age of defect correction in this study was 8 months. Most of the subjects were less than 6 months (11 subjects) and 6-12 months (11 subjects) in corrected ages. The most defects were ventricular septal defects (VSD). The age of defect correction did not correlate with the post-correction H/A z-score based on the Spearman correlation test (r= 0.093) with p value = 0.642 while the defect correction age with z-score H/A was not found to be correlated based on the Spearman correlation test (r = 0.143) with p value = 0.452.

Conclusion: The age of defect correction did not prove correlate with either the z-score for H/A or height gain."

"Latar Belakang: Penyakit jantung bawaan (PJB) dikoreksi dengan bedah jantung terbuka dengan bantuan alat mesin pintas jantung paru (cardiopulmonary bypass). Namun teknik ini dapat menyebabkan inflamasi pada paru yang menghasilkan kondisi Acute Respiration Dysfunction Syndrome (ARDS). Meskipun insidensinya pada pasien bedah dengan mesin pintas jantung paru hanya rendah, tingkat mortalitasnya dapat mencapai 50%. Tujuan: Mengetahui hubungan antara durasi penggunaan mesin jantung paru dengan insidensi ARDS pada pasien anak dengan PJB pasca bedah jantung terbuka. Metode: Studi kohort retrospektif dilakukan terhadap 194 anak yang menjalani bedah jantung terbuka atas indikasi PJB di Unit Pelayanan Jantung Terpadu (UPJT) RSCM periode Januari 2014-September 2015. Hasil: 64 (32,99%) pasien mengalami ARDS pasca bedah jantung terbuka dan sisanya sebanyak 130 (67,01%) tidak mengalami ARDS. Median penggunaan mesin pada golongan ARDS dan non-ARDS masing-masing sebesar 80 menit (23-219, IK90%) dan 70 menit (18-320, IK90%). Insidensi ARDS pada kelompok dengan durasi pendek (≤60 menit) adalah 27,5% dan dengan durasi panjang (> 60 menit) adalah 36%. Secara statistik dan klinis tidak terdapat hubungan bermakna antara durasi penggunaan mesin dengan munculnya ARDS (p = 0,298, uji chi square). Kesimpulan: Tidak terdapat hubungan bermakna antara durasi penggunaan mesin pintas jantung paru dengan kejadian ARDS pada pasien PJB pasca bedah jantung terbuka.

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Background: Congenital heart disease (CHD) is corrected by open thoracic surgery with the help of cardiopulmonary bypass machine (CPB). This technique can cause pulmonary inflammation resulting in Acute Respiratory Distress Syndrome (ARDS). Even though its incidence is low, the mortality rate of is up to 50%.

Aim: To find whether the duration of CPB using is related with incidence of ARDS in pediatric patients underwent open thoracic surgery.

Methods: Retrospective cohort study was done involving 194 pediatric patients underwent open thoracic surgery with CHD indication at Unit Pelayanan Jantung Terpadu (UPJT) RSCM within January 2014 and 2015 September.

Results: 64 (32,99%) patients had ARSD after open thoracic surgery. The mean of CPB machine duration was 80 minutes (23-219, CI90%) in patients with ARDS and 70 minutes (18-320, CI90%) in patients with no ARDS. The incidence of ARDS in patients with short duration of CPB (≤60 minutes) was 27.5% and long duration (>60 minutes) was 36%. There was no such correlation statistically and clinically between duration of CPB and ARDS occurence (p = 0.298, chi square test).

Conclusion: Duration of CPB using is not related with ARDS occurrence in pediatric patients with CHD underwent open thoracic surgery."