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.

Assessment of Microvolt T Wave Alternans in Children with Repaired Tetralogy of Fallot during 24-Hour Holter Electrocardiography.

BACKGROUND: We aimed to examine microvolt T wave alternans (MTWA) in 24-hour Holter electrocardiography (ECG) of children with repaired tetralogy of Fallot (TOF) to assess associations of MTWA with ventricular arrhythmias, ECG parameters, and echocardiographic findings. METHODS: Holter ECG records and archive files of 56 repaired TOF patients (62.5% male) who were analyzed retrospectively. Subjects' ECG parameters and MTWA values were compared with age-sex-matched control group. T wave changes were analyzed by time-domain-modified moving average method from the three channels of 24-hour Holter ECG. RESULTS: Mean age was 123.4 ± 48.3 months. Median MTWA value was 55.5 µV in the control group, whereas 95.5 µV in patients group (P < 0.001). A significant weak positive correlation was found between the presence of ventricular extrasystoles and tricuspid regurgitation. There was no correlation between ECG parameters, echocardiographic findings, and MTWA. CONCLUSIONS: MTWA was increased in children with repaired TOF as reported before. To our knowledge, this is the first study analyzing MTWA with 24-hour Holter ECG in repaired TOF patients.

Risk Factors for Thrombosis, Overshunting and Death in Infants after Modified Blalock-Taussig Shunt.

BACKGROUND: The Modified Blalock-Taussig shunt procedure can provide increased flow of blood to the lungs for babies born with certain congenital heart defects. We evaluated 44 subjects under 2 years of age who had a Modified Blalock-Taussig shunt (MBTS) procedure performed from 2009-2013, to investigate risk factors for thrombosis, overshunting and death. METHODS: The study subjects included in our investigation were severely cyanotic newborns with pulmonary stenosis or atresia and duct dependent circulation, and infants having Tetralogy of Fallot with small pulmonary arteries who underwent a MBTS procedure in our facility from 2009-2013. We duly noted patient preoperative characteristics such as hemoglobin, hematocrit, mean platelet volume, prothrombin time and partial thromboplastin time. Our study investigated the risk factors for post-operative overcirculation, thrombosis and death. RESULTS: The age and weight of patients in our study at the time of procedure ranged from 1 day to 20 months old (median 12 days), and 2.4 kg to 12 kg (mean 4.6 kg), respectively. A total of 8 patients died following surgery, and. 4 (9.1%) had shunt thrombosis, of which one died during shunt revision. Partial thromboplastin time was 28.7 seconds in patients with thrombosis, and 35 in all other patients (p = 0.04). Overcirculation was detected in 5 patients; shunt size/body weight was 1.25 in patients who had overcirculation, and 1.06 in all other patients. CONCLUSIONS: It is important to assess risk factors associated with the MBTS operation. The results of our study suggest that a preoperative low aPTT value may be an indicator for thrombosis in infants who have undergone MBTS surgery.

Massive pericardial effusion due to intrapericardial mixed germ cell tumor in a premature baby.

Primary cardiac tumor is uncommon in childhood, with an incidence of 0.06-0.32%, and intrapericardial teratoma represents an exceptional rarity among these entities. Germ cell tumors (GCT) are rare, representing only 1-3% of childhood tumors. Twenty per cent of GCT are malignant and are associated with age and location. Extragonadal involvement accounts for nearly half of the cases. Anterior mediastinum is a common location of malignant germ cell tumors, yet pericardial and aortic adventitia involvement have been rarely reported. Here we report the case of a preterm twin baby boy with intrapericardial mixed germ cell tumor who presented with hydrops fetalis and pericardial effusion.

Different haemodynamic patterns in head-up tilt test on 400 paediatric cases with unexplained syncope.

OBJECTIVE: To assess haemodynamic patterns in head-up tilt testing on 400 paediatric cases with unexplained syncope. METHODS: Medical records of 520 children who underwent head-up tilt testing in the preceding year were retrospectively evaluated, and 400 children, 264 (66%) girls and 136 (34%) boys, aged 12.6±2.6 years (median 13; range 5-18), with unexplained syncope were enrolled in the study. Age, sex, baseline heart rate, baseline blood pressure, frequency of symptoms, and/or fainting attacks were recorded. The test protocol consisted of 25 minutes of supine resting followed by 20 minutes of 70° upright positioning. Subjects were divided into nine groups according to their differing haemodynamic patterns. RESULTS: There were no statistically significant differences between the groups with regard to age, gender, baseline blood pressure, and frequency of syncope (p>0.05). The response was compatible with orthostatic intolerance in 28 cases (7.0%), postural orthostatic tachycardia syndrome in 24 cases (6.0%), asymptomatic postural orthostatic tachycardia syndrome in 26 cases (6.5%), orthostatic hypotension in seven cases (1.7%), vasovagal syncope in 38 cases (9.5%), and negative in 274 cases (69.2%). Vasovagal syncope response patterns were of type 3 in nine cases (2.2%), type 2A in 10 cases (2.5%), type 2B in two cases (0.5%), and type 1 (mixed) in 17 cases (4.25%). CONCLUSIONS: In the 400 paediatric cases with unexplained syncope, nine different haemodynamic response patterns to head-up tilt testing were discerned. Children with orthostatic intolerance syndromes are increasingly referred to hospitals because of difficulty in daily activities. Therefore, there is need for further clinical trials in these patient groups.

Unusual Presentation of Pre-T Cell Acute Lymphoblastic Leukemia: Massive Pericardial Effusion Only.

Despite the infectious and connective tissue diseases are most encountered, etiological factors, pericardial effusions, can also rarely emerge because of malign disease in childhood and it often develops during the course of chemotherapy and radiotherapy. Unfortunately, pericardial involvement is one of the frequently encountered symptoms of pediatric malignancies. Herein, we present a rare case who was admitted with pericardial effusion and whose disease was diagnosed as pre-T cell acute lymphoblastic leukemia without peripheral blood involvement.

A rare cause of respiratory distress in the emergency department: anomalous origin of the left coronary artery from the pulmonary artery.

Anomalous origin of the left coronary artery from the pulmonary artery is the most common cause of myocardial ischemia and infarction in children and infants. Most of the affected individuals become symptomatic, owing to dilated cardiomyopathy during the infant period. Because of congestive heart failure, pulmonary congestion and respiratory distress can be seen. Herein, we report a 35-day-old infant with anomalous origin of the left coronary artery from the pulmonary artery who presented with respiratory distress and dilated cardiomyopathy to draw attention to this rare surgically treatable anomaly.

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.

Are mean platelet volume and platelet distribution width useful parameters in children with acute rheumatic carditis?

Rheumatic fever (RF) is an inflammatory disease caused by autoimmune response to a preceding group A streptococcal infection. Mean platelet volume (MPV) reflects the platelet size and the rate of platelet production in bone marrow, and it may be used as an indicator of platelet activation and severity of inflammation. Fifty-three consecutive patients diagnosed with acute rheumatic carditis and 53 control subjects were enrolled into this study. Leukocyte and platelet counts were significantly higher in patients with acute carditis before treatment compared with controls, whereas MPV and platelet distribution width (PDW) values were not significantly different between groups. Platelet counts, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) values were decreased significantly in patients with RF after treatment. There was not a significant difference in terms of platelet count between the controls and the patient group after treatment. ESR was found to be correlated with CRP in patients before and after treatment. In conclusion, the results of our study showed that MPV and PDW levels do not change during acute rheumatic carditis before and after treatment.

The value of neurologic and cardiologic assessment in breath holding spells.

Objective : To evaluate the value of neurologic and cardiologic assessment and also the frequency of iron deficiency anemia in children with Breath Holding Spells (BHS). Methods : The hospital charts of patients diagnosed with BHS between 2011 and 2013 were reviewed retrospectively. Results : A total of 165 children (90 boys, 75 girls) with BHS comprised the study group. A matched group of 200 children with febrile convulsions served as controls. Among the first-degree relatives, 13.3% had BHS, 1.8% had febrile convulsions and 12.1% had epilepsy. The spells were cyanotic in 140 (84.8%) children and pallid or mixed in the remainder. BNS type was simple in 46.7% of patients and complicated in the remainder. Eighteen patients had abnormalities in electroencephalography, however only one patient was diagnosed with epilepsy. Sixty nine (47.9%) patients were found to have iron deficiency anemia. Conclusion : Referral of children with clinically definite BHS to pediatric neurology or pediatric cardiology clinics and performance of echocardiography and EEG investigations for exclusion of heart disease or epilepsy appear unnecessary. However, performance of an electrocardiogram to search for prolonged QT syndrome should be considered although no patient in our series had any cardiologic abnormalities.

Massive pericardial effusion caused by "inflammatory myofibroblastic tumour" in a 3-month-old child.

Tumours originating from cardiac tissues are rarely encountered during childhood, and fortunately most of these tumours are benign in nature. Inflammatory myofibroblastic tumour, which has unique clinical, pathological, and molecular characteristics, is a relatively new entity compared with previously mentioned tumoural processes originating from the heart. Most of the cardiac intima-media thickness patients are in the age group of 4 months to 17 years. This rarely seen tumoural process has not been subject of any specific research and the prognosis is not well known. Here we present the case of a 3-month-old child who was admitted to our outpatient clinic with massive pericardial effusion and who has shown excellent progress after surgical resection of over 1 year.

A patient with ischemic symptoms presents with left coronary artery-to-right ventricle fistula and agenesis of the right coronary artery.

Coronary artery fistulas are rare vascular anomalies characterized by abnormal communication, devoid of a capillary system between the coronary artery and the major vessels or cardiac chambers. In this report, we present a 14-year-old male patient with ischemic symptoms, a left coronary artery to right ventricle fistula and agenesis of the right coronary artery. The anatomy of the coronary arteriovenous fistula was determined in detail through a dual source CT coronary angiography. The patient underwent open cardiac surgery because of ischemic symptoms and a residual fistula was detected after the surgery.

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.

Right ventricular regional deformation analysis in patients operated for tetralogy of Fallot.

PURPOSE: We aimed to compare the findings of a segmental speckle-tracking strain of right ventricle with those of cardiac magnetic resonance imaging in this setting. METHODS: In 26 patients with operated tetralogy of Fallot (mean age, 15.35 ± 2.3 years; range 11-18 years), right ventricular segmental speckle-straining (the basal, mid, apical segments of right ventricular septum and lateral free wall, and right ventricular apex) were determined using two-dimensional echocardiography. The echocardiographic findings were compared to right ventricular ejection fraction, right ventricular indexed end-diastolic volume, indexed end-systolic volume and pulmonary regurgitation fraction at cardiac magnetic resonance. RESULTS: Right ventricular global speckle strain was -18.6 ± 3.7and lateral free wall strain was -17.8 ± 4.9. Indexed right ventricular end diastolic volume was 171.7 ± 23.3 ml/m2, indexed right ventricular end systolic volume was 95.1 ± 35.0 ml/m2 and right ventricular ejection fraction was 44.76 ± 9.39%. Basal inferior septum is correlated with indexed right ventricular end-diastolic volume (RVEDV; r = -0.521, p = 0.015) and pulmonary regurgitation fraction (r = -0.584, p = 0.015). Cardiac magnetic resonance (CMR)-derived RVEDV is correlated with lateral free wall (r = -0.465, p = 0.034) and Global RV (r = 0.442, p = 0.045). CONCLUSION: Right ventricular basal inferior septal and apical longitudinal strain correlates with the measures of right ventricular performance on CMR. These parameters would be useful to monitor right ventricular performance in patients after tetralogy of Fallot repair.

A case of Crimean-congo hemorrhagic fever with pleural effusion.

Crimean-Congo hemorrhagic fever (CCHF) is a tick-borne viral zoonosis with the potential of human-to-human transmission that affects wide areas in Asia, Southeastern Europe, and Africa. Hemorrhagic manifestations constitute a prominent symptom of late stage disease with case fatality rates from 3 to 50%. We present a case of CCHF complicated by hemorrhagic pleural effusion and resulting in resolution without chest tube drainage in a 9-year-old boy. The diagnosis of CCHF was confirmed by enzyme-linked immunosorbent assay tests. Both serum and pleural fluid CCHF IgM were positive at titers of 1/1,600 and 1/6,400, respectively.