Prognostic Value of Tpeak-Tend Interval in Early Diagnosis of Duchenne Muscular Dystrophy Cardiomyopathy.

The most common cause of death in patients with Duchenne muscular dystrophy (DMD) is cardiomyopathy. Our aim was to investigate the relationship between the Tpeak-Tend (Tp-e) interval and the premature ventricular contraction (PVC) burden and therefore early arrhythmic risk and cardiac involvement in DMD patients. Twenty-five patients with DMD followed by pediatric cardiology were included in the study. Those with a frequency of <1% PVC in the 24 h Holter were assigned to Group 1 (n = 15), and those with >1% were assigned to Group 2 (n = 10). Comparisons were made with healthy controls (n = 27). Left ventricular ejection fraction (LVEF) was lowest in Group 2 and highest in the control group (p < 0.001). LV end-diastolic diameter was greater in Group 2 than in Group 1 and the control group (p = 0.005). Pro-BNP and troponin levels were higher in Group 1 and Group 2 than in the control group (p = 0.001 and p < 0.001, respectively). Tp-e interval was longer in Group 2 compared to Group 1 and the control group (p < 0.001). The LVEF (OR 0.879, 95% CI 0.812-0.953; p = 0.002) and Tp-e interval (OR 1.181, 95% CI 1.047-1.332; p = 0.007) were independent predictors of PVC/24 h frequency of >1%. A Tp-e interval > 71.65 ms predicts PVC > 1%, with a sensitivity of 80% and a specificity of 90% (AUC = 0.842, 95% CI (0.663-1.000), p = 0.001). Determination of Tp-e prolongation from ECG data may help in the determination of cardiac involvement and early diagnosis of arrhythmic risk in DMD.

Transcatheter Closure of Atrial Septal Defect in Children: Single-Center Experience, Mid-Term Follow-up Results.

OBJECTIVE: Atrial septal defect is a congenital heart disease usually diagnosed in childhood. This study aimed to evaluate the mid-term follow-up results of patients who underwent trans- catheter closure of atrial septal defect by comparing the devices and methods used in the procedure and investigating the complications of this procedure in children. MATERIALS AND METHODS: This study evaluated 232 patient files retrospectively. Of the 232 patients, 24 were excluded from the study due to missing files or data. Also, patients with multi-fenestrated atrial septal defect and aneurismatic septal tissue were excluded from the study. The following data were evaluated: follow-up time, patient complaints, symptoms, trans- thoracic echocardiography, and transesophageal echocardiography findings (if performed), the size of the defect as measured by balloon-sizing, the size of the device used in the proce- dure, and major and minor complications. RESULTS: The study included 208 children who were diagnosed with atrial septal defect. The mean age of the patients was 88.0 ± 56.5 months. Of the patients, 170 (81.7%) had no com- plaints. The success rate of the procedure was found to be 95.7%. While device embolization was the most common major complication, arrhythmia was the most common minor complica- tion. The complication rate was statistically different according to the device type used in the procedure. CONCLUSION: Transcatheter closure of atrial septal defect is a safe method for atrial septal defect closure in pediatric patients. The study found that defect diameters measured by differ- ent methods were not correlated with each other. The procedure complication rates differed according to device type.

Quantification of Hepatic and Splenic Stiffness After Fontan Procedure in Children and Clinical Implications.

We aimed to investigate Fontan associated liver disease in children by shear wave elastography (SWE). This is a single-center, prospective case-control study included 41 patients with Fontan physiology and 30 healthy controls. Hepatic and splenic shear wave elasticity values were exhibited both as kPa and m/s. The mean hepatic SWE values of Fontan patients (n = 41; 15.8 ± 3.2 kPa or 2.5 ± 1.8 m/s) were significantly higher than the control group (n = 30; 5.59 ± 0.6 kPa or 1.37 ± 0.07 m/s) (P < 0.001). The mean splenic SWE values of Fontan patients were (25.6 ± 4.61 kPa or 2.85 ± 0.22 m/s) significantly higher than the control group (15.9 ± 1.44 kPa or 2.29 ± 0.1 m/s) (P < 0.001). There were statistically significant positive correlations among the follow-up duration after the Fontan procedure with NT-proBNP (P = 0.008, r = 1) and prothrombin time (P = 0.009, r = 0.4) as well as the hepatic SWE values with alanine aminotransferase (P = 0.039, r = 0.32), gamma-glutamyl transferase (P = 0.045, r = 0.31), and PT (P = 0.011, r = 0.39). There has been statistically significant moderate positive correlations of splenic stiffness values with PT (P = 0.047, r = 0.34), and INR (P = 0.038, r = 0.35). The sensitivity and specificity of liver stiffness cutoff value as 11.1 kPa for detection of Fontan associated liver disease were 95% and 100%, respectively. The hepatic and splenic stiffness increase independently in Fontan patients due to parenchymal disease. Hepatic SWE is a reliable and noninvasive predictor of early hepatic alterations that could not be detected only by biochemical results or routine ultrasound examinations.

The effect of DALI lipid apheresis in the prognosis of homozygous familial hypercholesterolemia: Seven patients' experience at a DALI apheresis center.

INTRODUCTION: Familial hypercholesterolemia (FH) is characterized by severe hypercholesterolemia that can result in coronary artery disease occurring at an early age. If patients are not cured with lipid-lowering drugs and diets, lipid apheresis may be an effective treatment option in these cases. Here, we evaluate the efficacy, selectivity and safety of the DALI apheresis technique. MATERIALS AND METHODS: Seven pediatric patients (2 girls; 5 boys) with ages between 7 and 14 years (mean age: 6.5±2.1 years) with HFH were included in this study. We restrospectively evaluated clinical and laboratory findings. We used the DALI system for lipid apheresis concomitant with medical treatment and diet for hyperlipidemia. RESULTS: The cohort's mean T.cholesterol level prior to apheresis was 700.57±136.36 mg/dl,the mean LDL-C value was 526.86±131.56 mg and the mean HDL-C level was 36.57±4.58 mg/dl.The mean cholesterol levels after apheresis were consecutively 317.57±93.70 /257.29±90.38 / 33.36±4.78 mg/dl.We noted a 51.1% reduction in LDL-C level and an 8.7% reduction in HDL-C level in our apheresis sessions.The reduction in LDL-C was statistically significant (p<0.05). During 1025 apheresis therapy, the most frequent mild and moderate adverse events were deviceaccess problems and hypotension (in all patients);severe adverse events were mainly due to cardiac problems(myocardial infarct and arrhythmia) and hypotension. CONCLUSION: Lipid apheresis is an inevitable alternative treatment for HFH. Despite all of its application problems, DALI system is an effective therapy for decreasing atherogenic lipids from circulation.

Resistant gram-negative infections in a pediatric intensive care unit: a retrospective study in a tertiary care center.

AIM: Healthcare-associated infections cause increased morbidity and mortality in intensive care units. In this study, it was aimed to compare infections with multi-drug resistance and extended drug resistance, while evaluating the characteristics of resistant Gram-negative infections in the pediatric intensive care unit in our university hospital. MATERIAL AND METHODS: In this study, pediatric patients who were found to have Gram-negative infections during hsopitalization in the pediatric intensive care unit in our faculty between January 2011 and December 2015, were evaluated retrospectively. RESULTS: One thousand thirty patients were internalized in our unit in the study period. The incidence for healthcare-associated infection was found as 17.2% and the incidence density was found as 32.7 per 1000 patient days. The incidence for healthcare-related infection per 1000 device days and the rate for device use were calculated as 66.9 and 0.59, respectively. One hundred thirty Gram-negative infection episodes were found in 79 patients whose median age was 22 (1-205) months. The most common infections included ventilator-related pneumonia (n=78, 60%) and bloodstream infections (n=38, 29.2%). The most common causative agents included Pseudomonas aeruginosa (n=50, 38.5%), Kleibsiella pneumonia (n=32, 24.6%) and Acinetobacter baumannii (n=28, 21.5%). Among A. baumannii isolates, the rates for resistance against piperacillin-tazobactam and meropenem were found as 96.4% and 89.3%, respectively. Empirical use of carbapenems, aminoglycosides, and fluoroquinolones, the presence of total parenteral nutrition and history of Gram-negative bacterial infections prior to pediatric intensive care unit admission were significantly more common among extended-drug Gram-negative bacterial infections. The late mortality rate was found to be higher in presence of extended drug resistance. History of Gram-negative infection was found to be an independent risk factor in terms of extended drug resistance. CONCLUSION: Healthcare-associated infections are an important health problem and it is important for infection control committees of hospitals to determine and apply strategies according to hospital colonization in prevention.

AMAÇ: Saglik bakimi iliskili enfeksiyonlar yogun bakim birimlerinde yüksek hastalik ve ölüme neden olmaktadir. Bu çalismada, üniversite hastanemiz çocuk yogun bakim birimindeki dirençli gram negatif enfeksiyonlarin özellikleri degerlendirilirken; çok ilaca dirençli ve genisletilmis ilaç direnci olan enfeksiyonlarin karsilastirilmasi amaçlanmistir. GEREÇ VE YÖNTEMLER: Bu çalismada Ocak 2011­Aralik 2015 yillari arasinda, fakültemiz çocuk yogun bakim biriminde yatisi sirasinda gram negatif enfeksiyon saptanan çocuk hastalar geriye dönük olarak degerlendirildi. BULGULAR: Çalisma döneminde birimimize 1 030 hasta yatirildi; saglik bakimi iliskili enfeksiyon sikligi %17,2, siklik yogunlugu 1 000 hasta günü basina 32,7 idi. 1 000 cihaz günü basina saglik bakimi iliskili enfeksiyon sikligi ve cihaz kullanim orani sirasiyla 66,9 ve 0,59 olarak hesaplandi. Yas ortancasi 22 (1­205) ay olan 79 hastada, 130 gram negatif enfeksiyon atagi saptandi. En sik saptanan enfeksiyonlar, ventilatör iliskili pnömoni (n=78, %60) ve kan akim enfeksiyonu (n=38, %29,2) idi. En sik etkenler Pseudomonas aeruginosa (n=50, %38,5), Kleibsiella pneumonia (n=32, %24,6) ve Acinetobacter baumannii (n=28, %21,5) idi. A. baumannii izolatlari arasinda piperasilin-tazobaktam ve meropenem direnci sirasi ile %96,4 ve %89,3 saptandi. Ampirik karbapenem, aminoglikozid ve florokinolon kullanimi, total parenteral nütrisyon varligi, yogun bakim öncesi geçirilmis gram negatif enfeksiyon öyküsü anlamli olarak daha yüksekti. Geç dönem ölüm hizi genisletilmis ilaç direnci varliginda daha yüksek saptandi. Geçirilmis gram negatif enfeksiyon öyküsü, genisletilmis ilaç direnci açisindan bagimsiz risk etmeni olarak bulundu. ÇIKARIMLAR: Saglik bakimi iliskili enfeksiyonlar önemli bir saglik sorunudur ve önlemede mümkünse her hastanenin kendi enfeksiyon kontrol kurulunun hastane kolonizasyonuna göre stratejiler belirleyip uygulamasi önemlidir.

Clinical Features and Indications Associated with Mortality in Continuous Renal Replacement Therapy for Pediatric Patients.

OBJECTIVES: To identify prognostic factors and indications in patients receiving continuous renal replacement therapy (CRRT) in the pediatric intensive care unit (PICU), and to demonstrate their effect on mortality. METHODS: A total of 63 patients admitted between 2011 and 2014 were included in the study. The demographic information, pediatric risk of mortality (PRISM) scores, vasoactive-inotropic score, indication for CRRT, time of starting CRRT, presence of fluid overload, durations of CRRT, and pediatric intensive care unit (PICU) stay were compared between survivors and non-survivors. RESULTS: The overall rate of survival was 69,8%. The most common indication for CRRT was fluid overload (31.7%) followed by acute attacks of metabolic diseases (15.9%), and resistant metabolic acidosis (15.9%). The median duration of CRRT was 58 (IQR 24-96) h. The most common CRRT modality was continuous venovenous hemodiafiltration. The CRRT modality was not different between survivors and nonsurvivors. Sepsis, as the diagnosis for admission to intensive care unit was significantly related to decreased survival when compared to acute kidney injury and acute attacks of metabolic diseases. Patients with fluid overload had significantly increased rate of death, CRRT duration, use of mechanical ventilation, and PICU stay. CONCLUSIONS: The CRRT, can be effectively used for removal of fluid overload, treatment of acute attacks of metabolic diseases, and other indications in critically ill pediatric patients. It has a positive effect on mortality in high-risk PICU patients. This treatment modality can be used more frequently in pediatric intensive care unit with improved patient outcomes, and should be focused on starting therapy in early stages of fluid overload.

Determinants of Increased Aortic Diameters in Young Normotensive Patients With Turner Syndrome Without Structural Heart Disease.

Factors associated with aortic dilation and dissection in patients with Turner syndrome (TS) remain unclear. We assessed magnetic resonance imaging-based aortic diameters at nine predefined anatomic positions and examined associations of increased aortic diameters with B-type natriuretic peptide (BNP), A-type NP (ANP), growth hormone treatment, insulin-like growth factor 1 (IGF1), and estrogen status. Forty-seven patients with TS aged 7.3-21 years and 34 healthy peers were enrolled in this study. Aortic diameters were higher in patients with TS at three positions than in controls (p < 0.05). History of GH treatment, pubertal status, and serum estradiol levels were not associated with increased aortic diameters. Patients with TS had higher plasma BNP and ANP levels than controls. BNP and IGF1 were independently associated with the increase in aortic diameters in TS at three positions of the ascending aorta (R2 = 0.361-0.458, p < 0.05 for all). At two positions of the descending aorta, only BNP emerged as an independent variable (R2 = 0.130-0.139, p < 0.05). We conclude that young, normotensive patients with TS had greater aortic diameters at several positions than healthy controls. BNP and IGF1 were independently associated with increased aortic diameters in TS.

Evaluación del lipidograma y ciertos factores de riesgo de ateroesclerosis en niños cuyos padres tuvieron arteriopatía coronaria de inicio temprano / Assessment of lipid profile and some risk factors of atherosclerosis in children whose parents had early onset coronary artery disease

Antecedentes/Objetivo. El objetivo de nuestro estudio fue analizar el lipidograma y ciertos factores de riesgo de ateroesclerosis, tales como las lipoproteínas de baja densidad oxidadas (ox-LDL, por su sigla en inglés) y las lipoproteínas de baja densidad pequeñas y densas (sdLDL, por su sigla en inglés) en los hijos de pacientes con cardiopatía coronaria (CC) prematura. Población y métodos. Hijos de padres con CC de inicio temprano emparejados con pares de su misma edad y mismo sexo. Se analizaron las concentraciones de lípidos, apolipoproteínas (ApoA, B, E), ox-LDL, sdLDL y lipoproteína (a) [Lp(a)] en los niños de estudio y de referencia. Los datos se evaluaron con el programa SPSS, junto con la prueba t de Student y la prueba U de Mann-Whitney. Resultados. Los niños del grupo de estudio (n: 43) tenían niveles más elevados de LDL, Lp(a) y ox-LDL y cocientes mayores de CT/HDL, ApoB/ApoA, LDL/HDL y ox-LDL/HDL (p < 0,05) que los del grupo de referencia. Conclusión. Con base en estos hallazgos, se sugiere que la dislipidemia y las concentraciones elevadas de LDL, Lp(a) y ox-LDL son frecuentes en los hijos de pacientes con CC de inicio temprano y representan gran parte de la predisposición familiar a tener CC

Background/Aim: The objective of our study was to analyze the lipid profile and some risk factors of atherosclerosis such as oxidized-low density lipoprotein (ox-LDL), small dense LDL (sd LDL) in the offspring of patients with premature coronary heart disease (CHD). Population and Methods: Children whose parents had early onset CHD were matched with age and sex pairs. Study and controls were analyzed for lipid levels, apolipoproteins (Apo- A,B,E), ox-LDL, sd LDL and lipoprotein (a) [Lp(a)]. The data were evaluated with SPSS using "Student tand Mann-Whitney U" tests. Results: Thestudy group children (n: 43) had higher LDL, Lp(a) and ox-LDL levels, ratios of TC/HDL, Apo-B/A, LDL/HDL and ox-LDL/HDL (p<0.05) than control group. Conclusion: These findings suggest that dyslipidemia and increased LDL, Lp(a) and ox-LDL levels are common in the offspring of patients with early onset CHD and account largely for their familial predisposition for CHD.

Assessment of lipid profile and some risk factors of atherosclerosis in children whose parents had early onset coronary artery disease. / Evaluación del lipidograma y ciertos factores de riesgo de ateroesclerosis en niños cuyos padres tuvieron arteriopatía coronaria de inicio temprano.

BACKGROUND/AIM: The objective of our study was to analyze the lipid profile and some risk factors of atherosclerosis such as oxidized-low density lipoprotein (ox-LDL), small dense LDL (sd LDL) in the offspring of patients with premature coronary heart disease (CHD). POPULATION AND METHODS: Children whose parents had early onset CHD were matched with age and sex pairs. Study and controls were analyzed for lipid levels, apolipoproteins (Apo- A,B,E), ox-LDL, sd LDL and lipoprotein (a) [Lp(a)]. The data were evaluated with SPSS using "Student t and Mann-Whitney U" tests. RESULTS: The study group children (n: 43) had higher LDL, Lp(a) and ox-LDL levels, ratios of TC/HDL, Apo-B/A, LDL/HDL and ox-LDL/HDL (p<0.05) than control group. CONCLUSION: These findings suggest that dyslipidemia and increased LDL, Lp(a) and ox-LDL levels are common in the offspring of patients with early onset CHD and account largely for their familial predisposition for CHD.

Antecedentes/Objetivo. El objetivo de nuestro estudio fue analizar el lipidograma y ciertos factores de riesgo de ateroesclerosis, tales como las lipoproteínas de baja densidad oxidadas (ox-LDL, por su sigla en inglés) y las lipoproteínas de baja densidad pequeñas y densas (sdLDL, por su sigla en inglés) en los hijos de pacientes con cardiopatía coronaria (CC) prematura. Población y métodos. Hijos de padres con CC de inicio temprano emparejados con pares de su misma edad y mismo sexo. Se analizaron las concentraciones de lípidos, apolipoproteínas (ApoA, B, E), ox-LDL, sdLDL y lipoproteína (a) [Lp(a)] en los niños de estudio y de referencia. Los datos se evaluaron con el programa SPSS, junto con la prueba t de Student y la prueba U de Mann-Whitney. Resultados. Los niños del grupo de estudio (n: 43) tenían niveles más elevados de LDL, Lp(a) y ox-LDL y cocientes mayores de CT/HDL, ApoB/ApoA, LDL/HDL y ox-LDL/HDL (p < 0,05) que los del grupo de referencia. Conclusión. Con base en estos hallazgos, se sugiere que la dislipidemia y las concentraciones elevadas de LDL, Lp(a) y ox-LDL son frecuentes en los hijos de pacientes con CC de inicio temprano y representan gran parte de la predisposición familiar a tener CC

Electrocardiographic changes in children with diabetic ketoacidosis and ketosis.

AIM: We aimed to study electrocardiographic changes in children with diabetic ketoacidosis and ketosis and to evaluate the relation of the changes with serum electrolyte levels and ketosis. MATERIAL AND METHODS: This study was performed in Istanbul Medical Faculty, Pediatric Emergency and Intensive Care Department between May 2008 and May 2009. The electrocardiographic parameters and QT length of children with diabetic ketoacidosis and ketosis were evaluated at diagnosis and after the treatment. RESULTS: Forty patients were included in the study; 16 (40%) were diagnosed as having diabetic ketosis and 24 (60%) had diabetic ketoacidosis. Twenty-four (60%) patients were male and 16 (40%) were female and the mean age was 9.21±4.71 years (range, 1-16 years). Twelve (30%) cases of diabetic ketoacidosis were mild, three (7.5%) were moderate, and nine (22.5%) were severe. One patient had premature ventricular beats, and four had ST depression. The electrocardiographic parameters were all normal beyond the QTC length prolongation. The mean QTC length was 447±45 ms (380-560 ms) at diagnosis and 418±32 ms (350-500 ms) after treatment. The change in the QTC length was statistically significant. None of the patients had significant electrolyte disturbance and the prolongation of QTc length was not correlated with serum electrolyte levels. The prolongation of QTc length was statistically correlated with anion gap (r=0.33, p=0.03). CONCLUSIONS: In our study, we showed QTc length prolongation and the importance of performing electrocardiography during the diagnosis of diabetic ketoacidosis and ketosis. We also demonstrated that ketosis was responsible for the prolongation of QTc length.

Balloon angioplasty of severe aortic coarctation in a newborn, using axillary artery access: The first experience in Turkey.

Axillary artery access for demanding interventions in newborns is rarely described, in spite of the many clear advantages. This access route in newborns with critical congenital heart disease is a real alternative to the more commonly used femoral or carotid artery routes. In brief, axillary access is an attractive alternative approach in newborns with complex heart diseases. It is advisable to stop an elective procedure if axillary access is not successful. Presently described is the use of axillary artery access for balloon angioplasty in a newborn with critical aortic coarctation. To the best of our knowledge, it was the first instance in Turkey.

[Balloon valvuloplasty for aortic stenosis using umbilical vein access in a newborn: First experience in Turkey]. / Yenidoganda umblikal ven yoluyla aort balon valvüloplastisi: Ülkemizdeki ilk tecrübe.

Balloon valvuloplasty is an effective therapy for severe congenital aortic valve stenosis, with mild aortic insufficiency and minimal intermediate-term restenosis. No consensus currently exists regarding optimal vascular approach for balloon dilatation in newborns with critical or severe aortic valve stenosis. Critical aortic valve stenosis in newborns must be treated promptly and effectively. Transcatheter therapy may offer marked advantages, as surgical therapy has been associated with significant rates of morbidity and mortality. Percutaneous balloon dilatation is usually performed as emergent therapy of valve stenosis, with various options for vascular approach. While umbilical artery and vein access is rarely used in the treatment of critical aortic valve stenosis and aortic coarctation, this approach is a safe, simple, and effective choice for balloon dilatation in newborns, even in those weighing under 2.5 kg.

Cardiac rhythm abnormalities during intravenous immunoglobulin G(IVIG) infusion in two newborn infants: coincidence or association?

We report the occurrence of supraventricular tachycardia during intravenous immunoglobulin (IVIG) infusion. Supraventricular tachycardia was observed in two newborn patients during IVIG infusion. Both of the babies responded to adenosine treatment. Cardiorespiratory monitoring during IVIG infusion can be recommended because of the possibility of this potentially lifethreatening adverse effect.

Increased arterial stiffness in young normotensive patients with Turner syndrome: associations with vascular biomarkers.

OBJECTIVES: Factors contributing to arteriopathy in patients with Turner syndrome (TS) remain unclear. We assessed arterial stiffness in young, normotensive patients with TS and correlated arterial stiffness with vascular biomarkers, GH treatment and oestrogen exposure. Sixty-one patients with TS (mean age, 12·6 years; range 6·6-21·3 years) were matched for age and sex with 61 healthy peers. Associations between arterial stiffness and high-sensitivity C-reactive protein (hsCRP), B-type natriuretic peptide (BNP), atrial NP (ANP), plasma aldosterone/plasma renin activity (PRA), IGF1 and IGFBP3 were examined after adjusting for well-established confounders of vascular disease. RESULTS: Carotid intima media thickness standard deviation score (SDS), arterial stiffness index SDS and incremental modulus of elasticity SDS were higher, and distensibility coefficient SDS was lower in patients with TS. The duration of GH treatment and oestrogen exposure was not associated with indices of arterial stiffness. TS patients had higher hsCRP, BNP and ANP. Plasma aldosterone/PRA, IGF1 and IGFBP3 were similar in patients and controls. Multivariable regression analyses (R(2) = 0·200-0·668, P < 0·01) showed that BNP was associated with all indices of arterial stiffness. We found that hsCRP was associated with distensibility coefficient SDS (ß = -0·16, P < 0·01). TS was independently associated with increased arterial stiffness (ß = 0·420-3·424, P < 0·001 for all, R(2) = 0·06-0·31). CONCLUSIONS: Young, normotensive TS patients had increased arterial stiffness than that of healthy peers. BNP, and possibly hsCRP, was independently associated with arterial stiffness in TS. Further research will determine any causal inference of these relationships.

Investigation of cardiomyopathy in children with cirrhotic and noncirrhotic portal hypertension.

BACKGROUND: Cirrhotic cardiomyopathy (CCMP) is a functional disorder characterized by electrophysiological disturbances, and diastolic and/or systolic dysfunction in patients with liver disease. This disorder is a well-defined entity in adults, but pediatric data are limited. The aim of the study was to determine the incidence, features, and risk factors of CCMP in children with portal hypertension (PHT). METHODS: This study included 50 children with cirrhotic PHT (40/50) and noncirrhotic PHT (10/50). Fifty healthy children were also selected for the control group. Electrocardiography and echocardiography were used to evaluate cardiac functions. Corrected QT (QTc) ≥ 0.45 was accepted as prolonged on electrocardiography. The study group was divided into 3 groups: cirrhotic, noncirrhotic, and control. Then, the CCMP group was created according to the diagnostic criteria. Latent CCMP was diagnosed in the presence of prolonged-QTc along with a minor criterion (tachycardia). Manifest CCMP was diagnosed in the presence of at least 2 major criteria (prolonged-QTc along with abnormal echocardiographic findings). Moreover, in this study, the risk factors for CCMP were investigated. RESULTS: The CCMP group included 10 cases (20%). Nine of these cases had latent CCMP (18%), and the remaining one (2%) had manifest CCMP. All of the cases with CCMP had cirrhosis and ascites. None of the patients with CCMP had severe cardiac symptoms, but they were already using some cardioprotective drugs such as propanolol and spironolactone. As risk factors for CCMP, pediatric end-stage liver disease scores, Child-Pugh scores, and ascites grades were found to be significant for the determination of CCMP. The most important risk factor was ascites severity (P = 0.001, odds ratio 9.4). CONCLUSIONS: Approximately 20% of children with PHT have CCMP. A detailed cardiac examination should be carried out periodically in children with cirrhotic PHT, especially in the presence of ascites and high Child-Pugh score.