Cardiac evaluation of patients with juvenile dermatomyositis.

BACKGROUND: The present study aims to evaluate possible cardiac involvement in juvenile dermatomyositis (JDM) patients by conventional methods and cardiac magnetic resonance imaging (MRI) along with a systematic review of the literature on cardiac features in JDM. METHODS: The study group consisted of JDM patients who underwent cardiac MRI. We conducted a systematic review of the published literature involving JDM patients with cardiac involvement. RESULTS: In the present study, although baseline cardiologic evaluations including electrocardiography and echocardiography were within normal limits, we showed late gadolinium enhancement on cardiac MRI in 3 of 11 JDM patients. In the literature review, we identified 25 articles related to cardiac involvement in JDM. However, none of them, except one case report, included cardiac MRI of JDM patients. CONCLUSION: Cardiac abnormalities have been reported among the less frequent findings in patients with JDM. Cardiovascular complications during the long-term disease course are a leading cause of morbidity and mortality in these patients. Early detection of cardiac involvement by cardiac MRI in patients with JDM and aggressive treatment of them may improve the clinical course of these patients. IMPACT: The myocardium in patients with JDM may be involved by inflammation. Myocardial involvement may be evaluated by using contrast-enhanced cardiac MRI. This is the first study evaluating cardiac involvement by cardiac MRI in JDM patients. MRI may show early cardiac involvement in patients whose baseline cardiologic evaluations are within normal limits. Early detection of cardiac involvement by cardiac MRI may improve the long-term prognosis of patients with JDM.

Left atrial appendage aneurysm in newborns: a report of two cases.

Left atrial appendage aneurysm is an extremely rare cardiac anomaly with serious complications. It is rarely diagnosed in childhood. Herein, we reported two newborns diagnosed with left atrial appendage aneurysm.

Cardiac involvement in multisystem inflammatory syndrome in children: single-centre experience.

BACKGROUND: This study aimed to evaluate electrocardiographic and echocardiographic findings, Holter recordings of the multisystem inflammatory syndrome in children, and to identify prognostic factors for cardiac involvement. METHODS: We retrospectively reviewed demographic characteristics, medical data, laboratory findings, electrocardiogram and echocardiographic findings, 24-hour Holter recordings, need for an ICU, and extracorporeal membrane oxygenation in multisystem inflammatory syndrome in children. Acute left ventricular systolic dysfunction was defined as left ventricular ejection fraction (EF) ≤%55 on echocardiography. RESULTS: Sixty-seven children were included in the study. 24-hour Holters were recorded in 61.2% of the patients and 49.2% were normal. On echocardiographic examination, 14.9% of the patients had systolic dysfunction (EF ≤ 55%). While 32.8% of patients had mild mitral regurgitation, 3% had moderate mitral regurgitation, and 6% had mild aortic regurgitation. There was no statistically significant difference in EF values between the group with arrhythmia in Holter and the group with normal Holter results (p ≥ 0.05). B-type natriuretic peptide was positively correlated with C-reactive protein, ferritin, and fibrinogen. Significant effectivity of the B-type natriuretic peptide value was observed in the differentiation of those with EF ≤ and > 55%. Extracorporeal membrane oxygenation support was needed for three (4.5%) patients. One patient who died had systemic juvenile idiopathic arthritis. CONCLUSIONS: Neutrophil/lymphocyte ratio, C-reactive protein, D-dimer, ferritin, troponin, and B-type natriuretic peptide were found to be significantly higher in patients with systolic dysfunction. Also, the cut-off value of 1700 pg/ml for B-type natriuretic peptide was significantly effective. These parameters may indicate the severity of the disease but should be supported by prospective studies.

A rare cause of hemoptysis in childhood: peripheral pulmonary artery pseudoaneurysm to bronchial fistula in a case with tetralogy of Fallot.

We herein report a patient complaining from significant hemoptysis due to secondary fistulisation of the peripheral branch of the left pulmonary artery and the left bronchial tree, which was successfully treated with a transcatheter angiography.

Usefulness of ischemia-modified albumin for assessment of the effects of small ventricular septal defects on the pulmonary vascular bed.

BACKGROUND: Pulmonary vascular damage may be associated with oxidative stress in congenital heart diseases. We investigated whether small ventricular septal defects have an effect on the pulmonary bed. METHODS: This prospective cohort study included 100 patients with small ventricular septal defects and 75 healthy controls. Ischemia-modified albumin, high-sensitivity C-reactive protein, and various cardiovascular parameters were assessed in both groups. RESULTS: The mean ischemia-modified albumin level was significantly higher in patients with small ventricular septal defects (0.62 ± 0.17 absorbance units) than in the control group (0.51 ± 0.09 absorbance units; p < 0.001). The mean high-sensitivity C-reactive protein level was significantly higher in the ventricular septal defects group (3.72 ± 1.57) than in the control group (2.45 ± 0.89; p < 0.001). The ischemia-modified albumin levels in patients with left ventricular internal diameter end diastole and end sistole and main pulmonary artery z-scores ≥ 2 were significantly higher than patients whose z-scores were <2. The ischemia-modified albumin and high-sensitivity C-reactive protein levels were positively correlated in the small ventricular septal defects group (rho = 0.742, p < 0.001). Receiver operating characteristic analyses showed that at the optimal cut-off value of ischemia-modified albumin for the prediction of pulmonary involvement was 0.55 absorbance units with a sensitivity of 60%, specificity of 62% (area under the curve = 0.690, p < 0.001). CONCLUSIONS: We demonstrated the presence of oxidative stress and higher ischemia-modified albumin levels in small ventricular septal defects, suggesting that ischemia-modified albumin might be a useful biomarker for evaluating the effects of small ventricular septal defects on the pulmonary bed.

Risk Factors Associated with Ascending Aortic Aneurysms and Aortic Elasticity Parameters in Children with a Bicuspid Aortic Valve.

Aneurysms of the ascending aorta are frequently found in patients with a bicuspid aortic valve (BAV). This study assessed the risk factors of ascending aortic aneurysms and aortic elasticity in children with BAV. The study included 66 patients with no history of transcatheter intervention or surgical procedure who had been diagnosed with isolated BAV. Echocardiographic, blood pressure, and pulse measurements were obtained for all patients. The BAVs were classified as described by Sievers et al. (J Thorac Cardiovasc Surg 133:1226-1233, 2007), and aortic elasticity parameters were calculated using various formulas. The patients were divided into groups with and without cusp fusion, aortic stenosis (AS), aortic regurgitation (AR), or mixed lesions; the groups were then compared. The mean patient age was 10.43 ± 3.91 years; 15%he patients had no AS or AR, 33% had both AS and AR, 17% had AS alone, and 35% had AR alone. The most common type of BAV was type 5, and the ascending aorta z-scores were higher in children with mixed lesions and without a cusp fusion. Aortic distensibility (AD) was significantly higher, and the stiffness index was significantly lower, in patients with an ascending aorta z-score > 4. The ascending aortic z-scores were higher in the no-fusion and mixed lesion (AS + AR) groups, especially those originating from post-stenotic dilation due to AS. The AD was increased in patients with an ascending aorta z-score > 4. Patients should thus be monitored closely for dissection risk, and preventive medical treatment should be started early in those with AS without cusp fusion.

A cardiac hydatid cyst mimicking a pericardial tumour in a paediatric case.

Cardiac hydatid cysts are a rare presentation of hydatid cyst disease in the body, with a reported cardiac involvement rate of <2%. The left ventricle is the most common site of cardiac involvement. Here, we report a patient with a hydatid cyst that ruptured into the pericardium after producing an aneurysm on the right ventricular free wall, appearing as fibrinated fluid and a solid mass lesion in the pericardium. Our aim in this case report was to emphasise that the possibility of a hydatid cyst should not be overlooked in the differential diagnosis of pericardial tumours.

Rare presentation of Candida albicans: infective endocarditis and a pulmonary coin lesion.

We present a case of a rare association of infective endocarditis and a coin lesion in the lung caused by Candida albicans. The lesion disappeared after 6 weeks of treatment with 5 mg/kg/day amphotericin B.

Cardiac function in children with premature ventricular contractions: the effect of omega-3 polyunsaturated fatty acid supplementation.

BACKGROUND: Premature ventricular contractions are accepted as benign in structurally normal hearts. However, reversible cardiomyopathy can sometimes develop. Omega-3 polyunsaturated fatty acids have anti-arrhythmic properties in animals and humans.AimWe evaluated left ventricular function in children with premature ventricular contractions with normal cardiac anatomy and assessed the impact of omega-3 fatty acid supplementation on left ventricular function in a prospective trial. METHODS: A total of 25 patients with premature ventricular contraction, with more than 2% premature ventricular contractions on 24-hour Holter electrocardiography, and 30 healthy patients were included into study. All patients underwent electrocardiography, left ventricular M-mode echocardiography, and myocardial performance index testing. Patients with premature ventricular contraction were given omega-3 fatty acids at a dose of 1 g/day for 3 months, and control echocardiography and 24-hour Holter electrocardiography were performed. Neither placebo nor omega-3 fatty acids were given to the control group. RESULTS: Compared with the values of the control group, the patients with premature ventricular contraction had significantly lower fractional shortening. The myocardial performance index decreased markedly in the patient groups. The mean heart rate and mean premature ventricular contraction percentage of Group 2 significantly decreased in comparison with their baseline values after the omega-3 supplementation. CONCLUSION: In conclusion, premature ventricular contractions can lead to systolic cardiac dysfunction in children. Omega-3 supplementation may improve cardiac function in children with premature ventricular contractions. This is the first study conducted in children to investigate the possible role of omega-3 fatty acid supplementation on treatment of premature ventricular contractions.

Predictive Factors for Determining the Clinical Severity of Pediatric Scorpion Envenomation Cases in Southeastern Turkey.

OBJECTIVE: The aim of this study was to define the epidemiological, clinical, and laboratory manifestations of scorpion envenomation and to identify factors that are predictive of severe cases. METHODS: The medical files of 41 scorpion envenomation cases were reviewed retrospectively. The cases were classified as mild-moderate or severe. The epidemiological, clinical, and laboratory findings of patients were recorded. RESULTS: There were 27 patients (65.9%) in the mild-moderate group and 14 patients (34.1%) in the severe group. The median age of all patients was 48 months. The most common systemic finding was cold extremities (41.5%). In all patients, the most commonly observed dysrhythmia was sinus tachycardia (34.1%). Two patients (4.9%) had pulseless ventricular tachycardia and died. Pulmonary edema and myocarditis were observed in 9 patients (22%). Median values of leukocyte and glucose levels were markedly increased in the severe group. Additionally, the mean thrombocyte level (540,857 ± 115,261 cells/mm(3)) in the severe group was significantly increased compared with the mild-moderate group (391,365 ± 150,017 cells/mm(3)). Thrombocyte levels exhibited a positive correlation with leukocyte and glucose values and a negative correlation with patient left ventricular ejection fraction. Multivariate analysis of laboratory parameters indicated that the most predictive factor for clinical severity is thrombocytosis (odds ratio 23.9; 95% CI: 1.6-353.5, P = .021). CONCLUSIONS: Although our results share some similarities with those of other reports, thrombocytosis was markedly increased in the severe group and served as the most predictive laboratory factor of clinical severity.

Atrial Functions and Aortic Elasticity in Children with Aortic Coarctation.

BACKGROUND: Coarctation of the aorta (CoA) is a chronic vascular disease characterized by a persistence of myocardial and vascular alterations. We aimed to evaluate children who have had successful coarctation surgery or balloon dilatation to evaluate the elasticity of the aorta, left atrial ejection force (AEF) and myocardial performance collectively at midterm follow-up. METHODS: Nineteen patients (7.15 ± 0.9 years of age) and 21 age-sex matched healthy children were included in this study. Left AEF index is defined as the product of mass and acceleration of blood expelled from the left atrium. Aortic stiffness and distensibility were estimated using ascending and descending aorta diameters. RESULTS: The left atrial force index [(g∙cm/s(2))/m(2)] in the patient group was found to be significantly higher (12.69 ± 7.29, 5.74 ± 2.59, respectively, p = 0.001). Distensibility of the ascending aorta (cm(2)/dynes 10(-6)) was significantly lower in the patient group than in the control group (42.13 ± 24.02, 78.79 ± 20.49, respectively, p < 0.001). The stiffness index of the ascending aorta was significantly higher in the patient group (p < 0.001). We also documented that atrial force index is associated with peak E velocity, right arm systolic blood pressure and left ventricular mass index. CONCLUSIONS: Our investigation showed that AEF is higher in children who have had successful coarctation surgery or balloon dilatation, and AEF is associated with systolic blood pressure, peak E velocity and left ventricular mass index. Distensibility of the ascending aorta was lower, and stiffness index was higher in children with corrected CoA than in healthy subjects. KEY WORDS: Atrial ejection force; Balloon dilatation; CoA; Coarctation surgery; Distensibility; Stiffness index.

Pulmonary and ventricular functions in children with repaired tetralogy of Fallot.

OBJECTIVE: This study aimed to evaluate biventricular function, brain natriuretic peptide levels, respiratory function test and 6 minute walking test (6MWT) in children with repaired tetralogy of Fallot (TOF), and analyse the correlation between these variables and clinical status. METHODS: Twenty-five children (14 boys, 11 girls; aged 6 to 17 years) with repaired TOF (Group 1) and 25 age-sex matched healthy controls (Group 2) were enrolled in the study. Tissue Doppler echocardiography, respiratory function test, 6MWT distance and brain natriuretic peptide levels were measured. RESULTS: Mean ages of the children at TOF corrective surgery and at study time were 5.1±3.5 years and 11.6±2.7 years respectively. The duration between palliative operation and corrective surgery was 4.3±2.0 years, and the follow-up period after corrective surgery was 6.3±3.0 years. The right ventricular and left ventricular myocardial performance indices (MPIs), and isovolumic relaxation and contraction times were significantly higher in Group 1 than in Group 2 (p<0.01). Spirometry displayed significantly reduced forced vital capacity (FVC), forced expiratory volume in one second (FEV1), forced expiratory flow 25-75% (FEF25-75) and inspirational capacity in Group 1 compared to Group 2 (p<0.01). In Group 1, 6MWT distances were significantly lower than in Group 2 (p=0.001). Right ventricular MPI is correlated with FEV1, FVC and 6MWT distance in the current study. CONCLUSION: The children with repaired TOF had impaired ventricular and pulmonary functions. Hence, right ventricular MPI along with FEV1, FVC and 6MWT distance may be useful in the follow-up of children with repaired TOF.

Arterial function and anatomy in obese children with cardiovascular risk factors.

BACKGROUND: Limited data are available related to the effects of cardiovascular risk factors on abdominal arterial stiffness using carotid and brachial artery indices. Therefore, we aimed to determine arterial changes in obese children and investigate any relation with cardiovascular risk factors. METHODS: Thirty-eight obese children (mean body mass index: 28.35 ± 4.65 kg/m(2) ) and 34 age- and sex-matched healthy subjects underwent ultrasound measurements of abdominal, carotid and brachial arteries. Aortic strain, pressure strain elastic modulus (Ep), pressure strain normalized by diastolic blood pressure (Ep*), carotid intima-media thickness, carotid artery compliance, brachial artery flow-mediated dilatation, and well-known cardiovascular risk factors were assessed in the obese children. RESULTS: Obese children had significantly higher Ep and Ep* parameters than the healthy controls (mean: 242.6 [107.1-666.6], 164.2 [110.6-231.5]; P < 0.001, and mean: 3.39 [1.76-7.5], 2.64 [1.46-4.2]; P < 0.001, respectively). Ep and Ep* were significantly correlated with the homeostasis model assessment of insulin resistance (r = 0.587, P = 0.001; r = 0.467, P = 0.004, respectively). Receiver-operator curve analysis of Ep for identification of children with cardiovascular risk factors showed that the area under the curve for hyperinsulinemia was 0.80 (P < 0.001) and for hypertriglyceridemia was 0.62 (P < 0.01). CONCLUSIONS: Abdominal arterial stiffness parameters as well as carotid intima-media thickness and brachial arterial flow-mediated dilatation assessment were similarly useful in identifying obese children with cardiovascular risk factors. Insulin resistance is related with the augmented rigidity of the aortic wall in obese children.

Predictors of chronic valvular disease in patients with rheumatic carditis.

This study investigated the predictors of chronic valvular disease in children with rheumatic carditis. The short- to mid-term follow-up records of 88 patients (mean age, 10.68 ± 2.5 years) with chronic rheumatic heart disease were reviewed. The mean follow-up period was 2.95 ± 1.4 years. Valvular involvement completely improved for 24 of the patients (27%) during the follow-up period. The multivariate logistic regression analysis found initial left ventricular dilation to be a significant independent risk factor associated with the persistence of either valvular involvement or mitral regurgitation. Furthermore, persistence of mitral regurgitation was found to be strongly correlated with cardiac murmur at admission. No significant correlation was detected between age, gender, severity of valvular involvements at initial evaluation, and chronic valvular disease. The majority of patients with rheumatic carditis had normal left ventricular systolic function. However, a significant proportion of patients had left ventricular dilation, reported in the medical literature to be associated with the severity of valve involvement. This study found no relation between initial severity of valve involvements and chronic valvular disease. For this reason, increased left ventricular end-diastolic diameter may be secondary to myocardial involvement independent of valvular regurgitation. The findings in this study also suggest that subclinic carditis had a better outcome than clinically evident carditis.

Complications of cardiac catheterization in pediatric patients: a single center experience.

We analyzed 519 catheterization procedures performed over a period of two years retrospectively. Several risk factors related to the patient or catheterization were analyzed. The incidence of complications was 6.2%. The most common major and minor complications were arterial thrombosis that required intervention and transient arrhythmias, respectively. The incidence of complications during interventional studies was higher (9.7%) when compared to that in diagnostic procedures (5.4%). The independent risk of any complication was greatest up to 1 year of age (p = 0.02). The risks of a major complication (p = 0.003) and development of arterial thrombosis (p = 0.02) were significantly greater in patients <1 year of age by univariate analysis. The risks of pediatric cardiac catheterization continue to decline. The complication rates associated with interventional catheterization were significantly higher than with diagnostic catheterization in this study. Younger age, particularly <1 year of age, is the strongest predictor of development of any complication.

The clinical course and short-term health outcomes of multisystem inflammatory syndrome in children in the single pediatric rheumatology center.

OBJECTIVES: Multisystem inflammatory syndrome in children (MIS-C) is a rare but severe condition resulting in excessive response of the immune system after SARS-CoV-2 infection. We report a single-center cohort of children with MIS-C, describing the spectrum of presentation, therapies, clinical course, and short-term outcomes. METHODS: This is a prospective observational study from to a tertiary pediatric rheumatology center including patients (aged 1 month to 21 years) diagnosed with MIS-C between April 2020-April 2021. Demographic, clinical, laboratory results and follow-up data were collected through the electronic patient record system and analyzed. RESULTS: A total of 67 patients with MIS-C were included in the study. Fever was detected in all patients; gastrointestinal system symptoms were found in 67.2% of the patients, rash in 38.8%, conjunctivitis in 31.3%, hypotension in 26.9% myocarditis, and/or pericarditis in 22.4%, respectively. Respiratory symptoms were only in five patients (7.5%). Kawasaki Disease like presentation was found 37.3% of the patients. The mean duration of hospitalization was 11.8 7.07 days. Fifty-seven patients (85%) received intravenous immunoglobulin (IVIG), 45 (67%) received corticosteroids, 17 (25.3%) received anakinra, and one (1.5%) received tocilizumab. Seven of the patients (10.4%) underwent therapeutic plasma exchange (TPE). In 21 (31.3%) patients, a pediatric intensive care unit (PICU) was required in a median of 2 days. The first finding to improve was fever, while the first parameter to decrease was ferritin (median 6.5 days (IQR, 4-11.2 days)). Sixty-five patients were discharged home with a median duration of hospital stay of 10 days (IQR, 7-15 days). CONCLUSION: Patients with MIS-C may have severe cardiac findings and intensive care requirements in admission and hospital follow-up. The vast majority of these findings improve with effective treatment without any sequelae until discharge and in a short time in follow-up. Although the pathogenesis and treatment plan of the disease are partially elucidated, follow-up studies are needed in terms of long-term prognosis and relapse probabilities.

[Evaluation of unresponsiveness to standard high-dose gamma globulin therapy in Kawasaki disease]. / Kawasaki hastaliginda standart yüksek doz intravenöz gama globulin tedavisine yanitsizligin degerlendirilmesi.

OBJECTIVES: We investigated the incidence of unresponsiveness to intravenous gamma globulin (IVIG) treatment in Kawasaki disease (KD) and evaluated its relation with coronary artery involvement. STUDY DESIGN: The study included 20 children (13 boys, 7 girls; mean age 4.2+/-3.4 years; range 9 months to 12 years) with KD. The mean disease duration on admission was 7.3+/-2.4 days (range 5 to 14 days). Initial treatment consisted of a single dose of IVIG and high-dose of aspirin. Unresponsiveness was defined as the persistence of fever and other symptoms within the first 48 hours of treatment. All the patients were evaluated by two-dimensional echocardiography before and after treatment. The mean follow-up period was 16.5+/-2.8 months (range 9 to 24 months). RESULTS: Unresponsiveness was seen in five patients (25%), who received a subsequent dose of IVIG, which improved fever in two patients. The remaining three patients received high-dose methylprednisolone. One patient who showed no response to either IVIG or methylprednisolone was treated with low-dose oral methotrexate. Six patients (30%) had coronary artery involvement (4 dilatations, 2 aneurysms), five patients on admission echocardiography, and one patient on control echocardiography seven days after treatment. Of five unresponsive patients, four (80%) had coronary artery involvement on admission. Patients with coronary involvement underwent coronary angiography after a mean of one year. Five patients had normal coronary arteries, whereas no angiographic regression was observed in one patient who had a giant coronary artery aneurysm on admission and was treated with oral methotrexate. CONCLUSION: The incidence of unresponsiveness to treatment was markedly high in KD patients who had coronary artery involvement on admission.

Short-term efficacy of ORS formulation and propranolol regimen in children with POTS.

BACKGROUND: To evaluate the short-term effectiveness of reduced-osmolarity oral rehydration salt formulation (ORS) and propranolol in children diagnosed with postural orthostatic tachycardia syndrome (POTS) in head-up tilt testing (HUTT). METHODS: Children were admitted with symptoms of orthostatic intolerance (OI) occurring in a standing position and disappearing in the supine position. Patients with heart rate increments of ≥40bpm and symptoms of OI constituted the pediatric POTS group in HUTT. A total of 70 pediatric patients with POTS were included in the study. POTS patients were divided into two groups based on whether they were prescribed reduced-osmolarity ORS and propranolol or not. The study group comprised patients on a regimen of reduced-osmolarity ORS and propranolol (n=34), while the control group comprised patients who were not prescribed any medication (n=36). The frequency of symptoms and standardized symptom scores were analyzed before and after 3 months of treatment in both groups. RESULTS: The post-treatment frequency of syncopal attacks was significantly reduced in both groups (P<0.01 for both groups), but the post-treatment standardized symptom scores were significantly reduced in the pediatric study group compared with the control group (P<0.01). CONCLUSION: The frequency of syncopal attacks was significantly reduced and the symptom scores for OI were improved in the study group. The improvement in OI symptom scores was better in the treatment group than in the control group. The control group symptoms persisted and caused extreme difficulty in their daily activities. In view of its clinical efficacy, we strongly advocate the use of combined treatment of reduced-osmolarity ORS and low-dose propranolol in pediatric patients with POTS.

Systolic dysfunction of systemic ventricle in patients who underwent a Fontan operation.

Öner T, Balli S, Hekim Yilmaz E, Bulut MO, Sasmazel A, Çelebi A. Systolic dysfunction of systemic ventricle in patients who underwent a Fontan operation. Turk J Pediatr 2019; 61: 925-930. The aim of the study was to report the clinical and echocardiographic data of patients who underwent a Fontan operation, and define the group with systolic dysfunction in the systemic ventricle observed during postoperative follow-up. The medical records of 183 patients [mean age: 10.93 ± 5.89 years (range: 2.5-45 years)] who were referred to our center and underwent a Fontan operation were retrospectively reviewed. The clinical, echocardiographic, and postoperative follow-up data of the patients were recorded. Preoperatively, 68 (37.2%) patients experienced pulmonary stenosis, while 41 (22.4%) had pulmonary atresia and 74 (40.4%) had pulmonary hypertension. The most common pathology in patients who were ineligible for biventricular repair was tricuspid atresia, seen in 51 patients (27.9%), followed by double-inlet left ventricle pathologies in 40 patients (21.9%). In total, 38 (20.7%) patients had a biventricular structure; among those with a single ventricular structure, the systemic ventricle involved was the right ventricle in 51 (27.9%) patients and the left ventricle in 94 (51.4%) patients. During follow-up, a total of 31 (16.9%) patients underwent catheterization and ventricular systolic functions were preserved in 168 (91.8%) patients. In Fifteen (8.2%) patients developed systolic dysfunction (ejection fraction < 50%). The mean age of the group developing systolic dysfunction was 15.6 ± 2.63 years (median, 13 years; range: 5-45 years). Of this group, 10 patients had a left ventricular structure of the systemic ventricle, 14 had atrial situs solitus, and 4 had moderate atrioventricular valve insufficiency. Even if the systemic ventricle is in the left ventricular structure, systolic dysfunction in the systemic ventricle develops, especially after the first 10 years, and this makes us think rudimentary ventricle function should also be carefully monitored for intra-univentricular diastolic asynchrony.