Effects of childhood obesity on myocardial performance, autonomic and conduction properties of the heart.

BACKGROUND: Childhood obesity tends to persist into adulthood and associated with increase in developing ischemic and non-ischemic cardiovascular diseases. We aimed to evaluate the effect of obesity on cardiac functions, atrial electromechanical coupling, and heart rate response, which are considered to be predictors of atrial fibrillation and sudden cardiac arrest. METHODS: Study population included 52 obese children and 52 healthy controls. We performed 12-lead electrocardiography, echocardiographic examination, and treadmill exercise testing. Mitral, septal, and tricuspid segments were analysed by tissue Doppler imaging. RESULTS: Myocardial performance index (p = 0.011, p < 0.001, and p = 0.001, respectively) was higher and E'/A' ratio (p = 0.011, p < 0.001, and p < 0.001, respectively) was lower in obese group than controls. Atrial electromechanical coupling was longer in the obese group at all three segments (p < 0.001, p = 0.009, and p = 0.04, respectively). They had significantly longer interatrial (p < 0.001) and intra-atrial (p = 0.003) electromechanical conduction delay. While chronotropic index was similar between two groups, heart rate reserve was lower in obese children than controls (p = 0.043). The 1st- and 2nd-minute heart rate recovery indices of the obese group were lower compared to controls (p < 0.001 and p = 0.03, respectively). Body mass index was positively correlated with intra- and inter-atrial conduction times, whereas it was negatively correlated with heart rate recovery indices. CONCLUSION: We showed a deterioration in the diastolic function, atrial conduction, and heart rate response properties in children with obesity. Given the prognostic importance of these parameters, obese patients are might be at risk for atrial fibrillation and severe dysrhythmias from a young age.

Clinical impact and efficacy of bedside echocardiography on patient management in pediatric intensive care units (PICUs): A prospective study.

OBJECTIVE: To determine the indication and necessity of echocardiographic assessment and therapeutic interventions in critically ill children. METHODS: A total of 140 children, including 75 mechanically ventilated (MV) and 65 spontaneously breathing (SB) children, who were admitted consecutively from March to August 2013 were evaluated prospectively. Data regarding the indication for echocardiography and therapeutic approaches used were documented. For evaluating disease severity, the Pediatric Risk of Mortality Score III (PRISM) was ascertained. The correlation between PRISM score and the requirement of echocardiographic evaluations were analyzed. RESULTS: Patients ages were between 45 days to 18 years. The male-to-female ratio was 1.33. In 35.4% patients who underwent echocardiographic evaluation, no definitive alteration occurred in treatment approach, whereas in the remaining 64.6% patients, decisive or supplemental information was gathered. Echocardiography was indicated in 88% MV children and 46.2% SB children. Echocardiographic evaluation was necessary in MV children and there was a positive correlation between the PRISM score and the requirement of echocardiographic assessment (p<0.001). CONCLUSION: Echocardiographic evaluation is an invaluable tool especially in MV children and the requirement of echocardiographic assessment increases according to clinical severity. Basic training for intensivists in this procedure is crucial and needs to be improved and supported in critically ill.

Unexplained cyanosis caused by hepatopulmonary syndrome in a girl with APECED syndrome.

Autoimmune polyendocrinopathy, candidiasis and ectodermal dystrophy (APECED) is a rare but devastating primary immunodeficiency disease caused by loss-of-function mutations in autoimmune regulator (AIRE) gene on chromosome 21q22.3. The clinical spectrum of the disease is characterized by a wide heterogeneity because of autoimmune reactions toward different endocrine and non-endocrine organs. Here, we report a 17-year-old Turkish girl diagnosed with APECED at 9 years in whom a novel homozygote mutation in AIRE gene p.R15H (c.44G>A) was found. In the clinical course of the patient, chronic liver disease due to autoimmune hepatitis has evolved resulting in hepatopulmonary syndrome (HPS) which has not been reported before in patients with APECED.

Decreased Deformation in Asymptomatic Children with Isolated Left Ventricular Non-compaction and Normal Ejection Fraction.

Echocardiography is the mainstay of screening and disease surveillance in isolated left ventricular non-compaction (iLVNC). The aim of our study is to determine the early regional and global myocardial functional changes and whether the myocardial changes that cannot be detected by conventional echocardiography could be detected by tissue Doppler imaging (TDI) or two-dimensional speckle-tracking echocardiography (STE) in iLVNC cases without symptoms. Longitudinal and circumferential strain (S) and strain rates (SR) as determined by STE in 20 children aged 12.1 ± 3.3 years was compared with those in 20 controls. All children underwent echocardiographic assessment using two-dimensional, tissue Doppler and speckle-tracking echocardiography. iLVNC patients who had normal systolic function by ejection and shortening fractions were included in this study. According to the TDI in all three segments [the non-compacted (NC), neighboring NC (NNC) and compacted (C) segments], isovolumic contraction time, isovolumic relaxation time and myocardial performance index values were significantly higher, while ejection time were significantly lower in the iLVNC group. According to STE in two segments (NC and NNC-segments) longitudinal S and SR values and also circumferential S and SR values were significantly lower in the iLVNC group compared with the control group; whereas, in the global measurements both longitudinal and circumferential S and SR values in all three segments were significantly lower in the iLVNC group compared with the control group. We believe that TDI and STE that evaluates myocardial deformation can be used for the detection of early myocardial dysfunction in the iLVNC patients who are subclinical and whose left ventricular functions were detected as normal by conventional methods with normal ejection and shortening fractions.

Cardiac Autonomic Dysfunction in Patients With Infantile Spasm and the Effect of Adrenocorticotropic Hormone Treatment.

Infantile spasm is an age-dependent epileptic-encephalopathy syndrome. Cardiac autonomic function is frequently altered in epilepsy. In this study, we examined heart rate variability in patients with infantile spasm before and after treatment. Nineteen patients with infantile spasm and 13 healthy comparisons were enrolled in the study. Cardiac rhythm was recorded with a Holter device for 24 hours before adrenocorticotropic hormone (ACTH) (Synacthen depot) and B6 vitamin administration and 1 month after treatment. Heart rate variability analysis found lower heart rate variability parameters in patients with infantile spasm at the onset of the syndrome, prior to treatment with ACTH. The time domain parameters of heart rate variability values showed a statistically significant increase following ACTH treatment. Our data suggest that patients with infantile spasm exhibit lower heart rate variability parameters, and the treatment of spasms with ACTH and B6 together diminished the autonomic dysfunction in our cohort.

The efficacy and safety of naproxen in acute rheumatic fever: The comparative results of 11-year experience with acetylsalicylic acid and naproxen.

The objective was to compare the efficacy and safety of naproxen (NXN) to acetylsalicylic acid (ASA) in the treatment of acute rheumatic fever (ARF). The data of 338 children were retrospectively analyzed. The patients were grouped according to joint and valve involvement and also drug chosen [methyl prednisolone (mPSL), ASA or NXN]. The treatment results and adverse events in each group were compared. The mean age was 10.3 years and the median follow-up was 62 months. Median time for normalization of acute phase reactants was 1 week in patients given steroids and 2 weeks in patients given ASA or NXN. ASA was replaced with NXN in 18 patients (10.2%) due to hepatic toxicity. The rate of rebound, recurrence and the prevalence of rheumatic valve disease were not different in patients given NXN, ASA or mPSL. In conclusion, NXN is a safe and effective alternative to ASA in the treatment of ARF in children.

Intracardiac thrombus in children: the fine equilibrium between the risk and the benefit.

The medical records of 16 patients diagnosed as intracardiac thrombus were searched. The size, location and outcome of thrombus together with demographic data of patients were assessed. The median age of the patients was 2.2 years. Six patients were newborn and two patients were infant. The median size of thrombus was 9 mm. The localization was right atrium in seven, right ventricle in five, left ventricle in one, pulmonary artery in one, and superior vena cava in two patients. There was prematurity in five, ciyanotic congenital heart disease in one, blood culture positivity in three, malignancy in four, nephrotic syndrome in one, indwelling catheters in 10, and acquired or genetic thrombophilia in six patients as risk factors. In the treatment, the first choice was tissue plasminogen activator in two patients, heparin infusion in one patient and low molecular weight heparin in remaining 12 patients. In nine patients, therapy included parenteral antimicrobials together with anticoagulants. The result was complete resolution in 15 patients and in one patient thrombus was surgically removed. The median time was 16 (2-70) days for 50% resolution and 26 (3-93) days for complete resolution. There was a statistically significant (P = .027 and r = 0.5) correlation between the size and the complete resolution time. There was no anticoagulant therapy related major complication. In patients with intracardiac thrombus, selection of anticoagulant therapy may decrease the risk of complications. Surgery is rarely required and thrombolytics are not usually necessary for resolution of thrombus.

Juvenile polyarteritis nodosa associated with toxoplasmosis presenting as Kawasaki disease.

Polyarteritis nodosa (PAN) is a vasculitis characterized by inflammatory necrosis of medium-sized arteries. Juvenile PAN and Kawasaki disease (KD) both cause vasculitis of the medium-sized arteries, and share common features. They have overlapping clinical features. Treatment should be managed according to the severity of symptoms and persistence of clinical manifestations. Herein is described the case of a 14-year-old boy first diagnosed with KD, who then fulfilled the criteria for juvenile PAN due to the development of severe myalgia, persistent fever, polyneuropathy and coronary arterial dilatation. He also had acute toxoplasmosis at the onset of vasculitis symptoms. The final diagnosis was of juvenile PAN associated with toxoplasmosis infection. Toxoplasma infection can be considered as an etiological agent for PAN and other vasculitis syndromes. Awareness of toxoplasmosis-related PAN facilitates early diagnosis, and instigation of appropriate treatment.

Multiple giant aneurysms and stenoses of the coronary and systemic arteries in an infant with kawasaki disease at the early stage of convalescent period.

Myocardial infarction and systemic arterial aneurysms are rarely seen during the course of the Kawasaki disease (KD). Herein, we report the case of a 4-month-old Turkish infant who was diagnosed with KD on the 17th day of the illness. On admission, echocardiogram showed multiple coronary arterial aneurysms (CAAs) and massive pericardial effusion. He was given intravenous immunoglobulin, aspirin and anticoagulant drugs. However, the aneurysms progressed to "super giant" CAAs, multiple huge coronary arterial thromboses developed recurrently and caused myocardial ischemia. Furthermore, the conventional angiography revealed multiple giant aneurysms and stenoses in the subclavian, celiac, and iliac arteries, besides CAAs.

Native aortic valve endocarditis complicated by pseudoaneurysm of mitral-aortic intervalvular fibrosa.

Pseudoaneurysm of mitral-aortic intervalvular fibrosa (PA-MAIVF) is a rare complication of native aortic valve endocarditis. This region is a relatively avascular area and prone to infection during endocarditis and subsequent aneurysm formation. The rupture into the pericardial cavity and left atrium or aorta, systemic embolism, myocardial infarction secondary to left coronary compression, and sudden death are the reported complications. Herein, we present a 9-year-old boy who was diagnosed with bicuspid aortic valve endocarditis complicated by PA-MAIVF, cerebral embolism, and hemorrhage. PA-MAIVF was visualized by both two- and three-dimensional transthoracic echocardiography and ruptured into pericardial space causing a fatal outcome.

Three-dimensional echocardiographic evaluation of a patient with double-orifice mitral valve, bicuspid aortic valve, and coarctation of aorta.

A 16-year-old male patient was diagnosed with bicuspid aortic valve and coarctation of aorta, and had a history of a subclavian patch plasty operation at 1 year of age. In addition, we detected double-orifice mitral valve (DOMV) at a routine evaluation and demonstrated by both two-dimensional and three-dimensional (3D) echocardiographic examinations. DOMV should be kept in mind in cases with left ventricular obstructive lesions and 3D echocardiography may provide a more detailed assessment of mitral valve and subvalvular apparatus.

Evaluation of cardiac functions and atrial electromechanical delay in children with adenotonsillar hypertrophy.

Deterioration of the right ventricular (RV) functions and the increase in the pulmonary arterial pressure (PAP) of children with moderate to severe adenotonsillar hypertrophy (ATH) have been well described. In addition to these complications, this study aimed to investigate the influence of ATH on the conduction system. The study investigated 46 patients with a diagnosis of ATH and 46 healthy control subjects. Conventional echocardiography, P-wave dispersion (Pd), tissue Doppler imaging (TDI) findings, and atrial electromechanical delay (AED) were compared between the patients and the control subjects before and after adenotonsillectomy. The maximum P-wave duration and Pd were significantly higher in the patients than in the control subjects (p < 0.001). The patient group showed significantly greater RV end-diastolic dimension (p = 0.01), right atrial area (p < 0.001), and mean PAP (p = 0.03) but lower E/A ratios for the mitral (p = 0.04) and tricuspid (p = 0.01) valves and a shorter pulmonary flow trace acceleration time (p = 0.03). The tricuspid annular-plane systolic excursion was similar between these groups (p = 0.21). In the patient group, TDI studies showed significantly lower E'/A' ratios for the tricuspid lateral (p = 0.006) and mitral septal (p = 0.003) segments than in the control group. The myocardial performance index was lower for the mitral lateral, mitral septal, and tricuspid lateral segments in patient group (p < 0.001). Similarly, AED was prolonged in the patient group at all three segments (p < 0.001). Also, the patient group showed a significantly longer interatrial (p = 0.03) and intraatrial (p = 0.04) electromechanical delay. However, all the electro- and echocardiographic parameters were similar between the patients and the control subjects after adenotonsillectomy (p > 0.05). The prolongations in P-wave duration, Pd, and inter- and intraatrial electromechanical delays were first shown in this population. The cardiac changes induced by ATH-associated hypoxia may facilitate arrhythmias during the long term.

Assessment of early-onset chronic progressive anthracycline cardiotoxicity in children: different response patterns of right and left ventricles.

We aimed to assess early-onset chronic progressive cardiotoxicity in the left and right ventricles with increasing cumulative anthracycline doses. We evaluated 72 patients within the first year after doxorubicin and/or daunorubicin treatment (median 1.3 months; range 0.3-11.5) and 31 healthy controls. Pretreatment and posttreatment QT interval analyzes were performed in 27 newly diagnosed patients. The echocardiographic data of all examinations of 72 patients were classified into three groups according to instant cumulative anthracycline doses: treatment group (TG)-I (≤120 mg/m(2); n = 26), TG-II (120-240 mg/m(2); n = 39), and TG-III (≥240 mg/m(2); n = 40). Diastolic and systolic parameters were analyzed by conventional echocardiography and tissue Doppler imaging (TDI) and compared with those of healthy controls. The mean age for patients and controls was 8.2 ± 4.5 and 9.6 ± 4.2 years, respectively (p > 0.05). QTc dispersion significantly increased after anthracycline treatment (p = 0.02). TDI showed decreased E' velocity (p < 0.001) and E'/A' ratio (p < 0.001) at lateral tricuspid annulus segment in TG-I, and these findings continued in TG-II and -III. In addition, S' velocity decreased in TG-I, -II, and -III at lateral mitral annulus (10.5 ± 2.6 cm/s, p < 0.05; 9.9 ± 2.2 cm/s, p < 0.001; and 10.1 ± 2.3 cm/s, p < 0.01, respectively). However, decrease in left-ventricular ejection fraction was statistically significant in TG-II and -III (p < 0.001). Although myocardial performance index was significantly increased in all treatment groups in both segments, it was primarily due to significant increases in isovolumic relaxation time at the lateral tricuspid annulus and isovolumic contraction time at the lateral mitral annulus. Abnormalities in diastolic function in right ventricle and systolic function in the left ventricle were observed even with a cumulative anthracycline dose <120 mg/m(2) by TDI. In addition, anthracycline treatment led to an increase in QTc dispersion.

Carotid intima media thickness and arterial stiffness in children with acute rheumatic fever.

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) constitute important public health problems in developing countries. Inflammation is present both in the early and late stages of the diseases. Chronic inflammation is known to be associated with atherosclerosis. We hypothesize that subclinical atherosclerosis and arterial stiffness may increase due to the ongoing inflammation as well as the increased pulse pressure and left-ventricular systolic dysfunction in RHD. The purpose of the present study was to investigate carotid intima media thickness (CIMT) and carotid artery stiffness in patients with ARF. Forty patients in follow-up due to ARF in the age group of 7-16 years (disease duration 1-10 years) and 36 volunteered subjects with similar body mass index were included in the study. The subjects included in the present study were compared regarding M-mode echocardiographic parameters and CIMT as well as carotid arterial strain (CAS), carotid artery distensibility (CAD), beta stiffness index (ßSI), and pressure-strain elasticity modulus (Ep) as carotid artery stiffness parameters. CIMT (0.52 ± 0.08 and 0.48 ± 0.07 mm, p = 0.01), ßSI (5.29 ± 2.98 and 3.02 ± 1.30, p < 0.001), and Ep (426.53 ± 210.50 and 254.44 ± 104.69 p < 0.001) were increased, whereas CAS (0.11 ± 0.01 and 0.19 ± 0.09, p < 0.001) and CAD (10.27 ± 4.69 and 17.76 ± 14.41, p < 0.001) were decreased in patients with ARF compared with the control group. There was a positive correlation between pulse pressure and ßSI (r = 0.25, p = 0.02) and Ep (r = 0.28, p = 0.01) in addition to a correlation between left atrial dilatation and CIMT (r = 0.55 p < 0.001) in patients with ARF. CIMT and carotid artery stiffness were increased in patients with ARF. Patients with ARF may have an increased risk of subclinical atherosclerosis and cardiovascular events.

Facial nerve palsy and Kawasaki disease.

Kawasaki Disease (KD) is a vasculitic disease and can affect any organ system in the body. The development of coronary artery aneurysms is the most common and life threatening complication of KD and makes this disease the leading cause of acquired heart disease in children in the developed world. Facial nerve palsy has been reported as a possible marker of more severe disease and increased risk of coronary artery involvement in KD. Herein, the authors report an 8-mo-old infant who had left sided facial nerve palsy and multiple coronary aneurysms associated with KD.

Chest pain with myocardial ischemia in a child: should we think about coronary slow flow phenomenon?

The coronary slow flow phenomenon (CSFP) is an angiographic finding characterized by delayed opacification of epicardial coronary arteries in the absence of stenotic lesion. Herein, we present a 13-year-old boy with recurrent chest pain who was diagnosed with acute ST-segment elevation myocardial infarction associated with CSFP, which has not been reported previously in the pediatric age group. Coronary angiography revealed only the presence of slow flow in the left anterior descending (LAD) coronary artery. Myocardial perfusion scintigraphy revealed a reversible perfusion defect in the LAD territory, which regressed partially at rest and showed complete improvement after dipyridamole infusion. All the symptoms, electrocardiogram abnormalities and cardiac markers returned to normal after dipyridamole treatment during the follow-up. We conclude that CSFP should be kept in mind in the differential diagnosis of chest pain with myocardial ischemia in the pediatric age group.