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Abstrak :
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.

Abstrak :
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. ......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.

Abstrak :
Latar Belakang: Penggunaan cardiopulmonary bypass dalam bedah jantung terbuka pada anak yang berkepanjangan dapat memicu koagulopati dan hemodilusi, serta menyebabkan perdarahan pasca operasi. Pasien anak dengan penyakit jantung bawaan sianotik memiliki risiko lebih tinggi karena sistem koagulasi darah mereka yang imatur. Meskipun demikian, tidak ada penelitian serupa yang betujuan untuk menilai hubungan antara keduanya. Tujuan: Penelitian ini bertujuan untuk meneliti korelasi antara CPB time dan perdarahan pasca operasi jantung terbuka pada pasien anak dengan penyakit jantung bawaan sianotik. Metode: Penelitian ini bersifat descriptive-analytical dengan metode cross- sectional. Rekam medis 100 pasien anak dari Rumah Sakit Cipto Mangunkusumo dari Januari 2016 sampai dengan Maret 2018 digunakan sebagai sampel. Pasien anak berusia 0 sampai 17 tahun dengan penyakit jantung bawaan sianotik, yang telah melalui bedah jantung terbuka elektif digunakan sebagai sampel. Korelasi Spearman digunakan untuk meneliti hubungan antara CPB time dengan perdarahan pasca operasi. Hasil: Dari 100 data yang diperoleh, tidak terdapat korelasi antara CPB time dan perdarahan pasca operasi (p = 0.087). Median dari CPB time adalah 87 menit (29 – 230). Perdarahan pasca operasi pasien memiliki median 15.3/kgBB dalam 24 jam (3.0 – 105.6). Konklusi: Tidak ada hubungan antara CPB time dan post-operative bleeding pada pasien anak dengan penyakit jantung bawaan sianotik. Faktor lain dapat mempengaruhi kedua variabel diteliti, termasuk dari pasien sendiri dan dari tindakan operasi, seperti kemampuan operator menangani perdarahan serta jenis prosedur operasi. Maka dari itu, CPB time tidak dapat dianggap sebagai faktor tunggal yang dapat mempengaruhi perdarahan pasca operasi.

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Background: Prolonged use of cardiopulmonary bypass during open heart surgery can induce coagulopathy and hemodilution, contributing towards post-operative bleeding. Pediatric patients with cyanotic congenital heart disease are susceptible due to presence of immature coagulation system. However, no similar studies have been done to assess the relationship between the two. Aim: This study aims to assess correlation between CPB time and post-operative bleeding in pediatric patients with cyanotic congenital heart disease undergoing open heart surgery. Method: This is a descriptive-analytical study, utilizing cross-sectional method. Medical records of 100 pediatric patients from Cipto Mangunkusumo General Hospital between January 2016 to March 2018 were used. Patients aged 0 to 17 years old with cyanotic congenital heart disease, who underwent elective open heart surgery were included as sample. Spearman’s correlation was used to determine correlation between CPB time and post-operative bleeding. Result: Data from 100 patients were obtained. No correlation was observed between CPB time and post-operative bleeding (p = 0.087). Patients’ CPB time has a median of 87 minutes (29 – 230). Patients’ post-operative bleeding has a median of 15.3 ml/kgBW in 24 hours (3.0 – 105.6). Conclusion: CPB time and post-operative bleeding has no correlation in pediatric patients with cyanotic congenital heart disease. Presence of various factors can influence both variables, including from the patients or operative factors, including dexterity of operator and applied procedure. Thus, CPB time cannot be held responsible as a single determining factor for post-operative bleeding.