Evaluation of handgrip strength in children with pulmonary hypertension.

BACKGROUND: Handgrip strength is a crucial indicator of upper extremity muscular strength and is vital for monitoring disorders like cardiac diseases that restrict a patient's physical activity and result in muscle atrophy. The aim of our study was to evaluate whether muscle strength loss is present in patients with pulmonary hypertension and whether this test can be an alternative to 6-minute walk test. MATERIALS AND METHODS: The study included 39 healthy children who were admitted to the outpatient clinic and 16 children with a diagnosis of pulmonary hypertension who were being followed in our centre. We assessed the differences in upper extremity handgrip strength using the Jamar Hydraulic Hand Dynamometer device among both healthy children and those diagnosed with pulmonary hypertension. Moreover, we compared the handgrip strength of pulmonary hypertension patients with significant prognostic indicators such as NYHA class, 6-minute walk test, and pro-brain natriuretic peptide. RESULTS: The mean dominant handgrip strength was 20.8 ± 12 kg in the patient group and 21.6 ± 12.4 kg in the control group (p = 0.970). Handgrip strength was shown to be negatively connected with pro-brain natriuretic peptide (r = -0.565, p = 0.023) and positively correlated with 6-minute walk test (r = 0.586, p = 0.022) during the patient group evaluation. CONCLUSION: Six-minute walk test needs a customised physical area (30 m of a straight hallway) and trained personnel for applying the test. The handgrip strength test, a different muscle strength indicator, can be used to more clearly and simply indicate the decline in patients' ability for effort. Additionally, it was found in our study that handgrip strength decreased as pro-brain natriuretic peptide levels rose, a crucial measure in the monitoring of pulmonary hypertension.

Comparison of echocardiographic aortic stiffness index measurements and pulse wave velocity measurements in obese and overweight children.

BACKGROUND: Aortic/arterial stiffness is a reliable, independent predictor and a risk factor for cardiovascular mortality. Arterial stiffness is assessed by pulse wave velocity and echocardiography. The purpose of this study is to analyse aortic/arterial stiffness in patients using echocardiographic and pulse wave velocity techniques. MATERIALS AND METHODS: The participants of this study consisted of 62 patients who presented to the Gazi University Pediatric Endocrinology and Pediatric Cardiology outpatient clinics, including 21 obese, 20 overweight, and 21 normal-weight patients. Echocardiography was performed on all patients, and echocardiographic measurements were compared to pulse wave velocity measurements. RESULTS: The mean (min-max) arterial strain measurements were 0.146 ± 0.0 (0.06-0.3) in the obese group and 0.106 ± 0.0 (0.05-0.18) in the overweight group. In comparison to the overweight group, the obese group had greater arterial strain measurements. The pulse wave velocity measurements in the obese and overweight groups were greater than those in the normal weight group (p > 0.05). Elastic modulus and aortic stiffness ß index values were shown to be positively correlated with pulse wave velocity measurements in the obese group (r = 0.56, r = 0.53, respectively; p = 0.008, p = 0.01, respectively). Systolic and diastolic blood pressure measurements were correlated with pulse wave velocity measurements in the obese group (r = 0.98, p = 0.0001, respectively). CONCLUSION: In our study, echocardiographic aortic measurements showing the vessel wall were correlated with pulse wave velocity measurements. Echocardiographic evaluation should be included in the routine follow-up of patients because pulse wave velocity measurement devices are not available in all centres, echocardiography is available in many centres, it is easily applicable, and it facilitates the follow-up of patients.

High take-off right coronary artery in a patient with tetralogy of Fallot.

High take-off coronary artery anomaly is a quite rare anomaly which is usually seen in isolated form and diagnosed incidentally. Association with tetralogy of Fallot is also rare and it is not one of the well-known coronary anomalies seen in this disease. Here, we describe high take-off right coronary artery in a 10-month-old female patient with tetralogy of Fallot which was diagnosed during catheter angiography. It is very important to show this anomaly sometimes with additional imaging techniques as it alters all the surgical approach including aortic cannulation.

Beneficial Effects of Modified Atkins Diet in Glycogen Storage Disease Type IIIa.

INTRODUCTION: Glycogen storage disease Type III (GSD III) is an autosomal recessive disease caused by the deficiency of glycogen debranching enzyme, encoded by the AGL gene. Two clinical types of the disease are most prevalent: GSD IIIa involves the liver and muscle, whereas IIIb affects only the liver. The classical dietetic management of GSD IIIa involves prevention of fasting, frequent feeds with high complex carbohydrates in small children, and a low-carb-high-protein diet in older children and adults. Recently, diets containing high amount of fat, including ketogenic and modified Atkins diet (MAD), have been suggested to have favorable outcome in GSD IIIa. METHODS: Six patients, aged 3-31 years, with GSD IIIa received MAD for a duration of 3-7 months. Serum glucose, transaminases, creatine kinase (CK) levels, capillary ketone levels, and cardiac parameters were followed-up. RESULTS: In all patients, transaminase levels dropped in response to MAD. Decrease in CK levels were detected in 5 out of 6 patients. Hypoglycemia was evident in 2 patients but was resolved by adding uncooked cornstarch to diet. CONCLUSION: Our study demonstrates that GSD IIIa may benefit from MAD both clinically and biochemically.

An adolescent case of extensive Behçet`s disease successfully treated with Infliximab.

Isiyel E, Bakkaloglu S, Oguz D, Yenicesu I, Boyunaga Ö, Özdemir Y, Damar Ç, Kandur Y, Akçaboy M, Aslan AT, Sismanlar T, Hasanoglan E, Buyan N. An adolescent case of extensive Behçet`s disease successfully treated with Infliximab. Turk J Pediatr 2019; 61: 585-588. Cardiac involvement is an uncommon and life-threatening complication of Behçet`s Disease. We present a 14-year-old boy, admitted to our hospital for recurrent hemoptysis. In his radiologic evaluation, a right ventricular thrombus and pulmonary arterial aneurysm were identified. He was diagnosed with Behçet`s Disease, and then he received prednisone and cyclophosphamide. However, his cardiac thrombus enlargened. After his treatment was replaced with infliximab, the pulmonary aneurysms regressed, and the cardiac thrombus disappeared. In conclusion, infliximab should be considered as a reliable option for vascular Behçet`s Disease resistant to conventional treatment.

The role of inflammatory biomarkers in CHD-associated pulmonary hypertension in children.

OBJECTIVE: The present study aims to identify the role of inflammatory markers such as C-reactive protein, interleukin-6, and fractalkine in CHD-associated pulmonary hypertension in children. METHODS: This is a prospective review of 37 children with CHD-related pulmonary hypertension, 21 children with congenital heart defects, and 22 healthy children. RESULTS: Serum C-reactive protein and interleukin-6 levels were significantly higher in the children with CHD-related pulmonary hypertension (respectively, p=0.049 and 0.026). Serum C-reactive protein concentrations correlated negatively with ejection fraction (r=-0.609, p=0.001) and fractional shortening (r=-0.452, p=0.007) in the pulmonary hypertension group. Serum fractalkine concentrations correlated negatively with ejection fraction (r=-0.522, p=0.002) and fractional shortening (r=-0.395, p=0.021) in the children with pulmonary hypertension. Serum interleukin-6 concentrations also correlated negatively with Qs (r=-0.572, p=0.021), positively with Rs (r=0.774, p=0.001), and positively with pulmonary wedge pressure (r=0.796, p=0.006) in the pulmonary hypertension group. A cut-off value of 2.2 IU/L for C-reactive protein was able to predict pulmonary hypertension with 77.5% sensitivity and 77.5% specificity. When the cut-off point for interleukin-6 concentration was 57.5 pg/ml, pulmonary hypertension could be predicted with 80% sensitivity and 75% specificity. CONCLUSION: Inflammation is associated with the pathophysiology of pulmonary hypertension. The inflammatory markers C-reactive protein and interleukin-6 may have a role in the clinical evaluation of paediatric pulmonary hypertension related to CHDs.

Case report: Neuromuscular block induced by rocuronium following sugammadex administration.

We present a case in which rocuronium was applied for muscle relaxation following the administration of sugammadex. An emergency surgery under general anesthesia was planned for a 43-year-old male patient due to an L1 vertebral corpus and right tibia-fibula shaft fracture. Anesthesia was induced with fentanyl, propofol and lidocaine. After applying only 30mg of the total induction dose of rocuronium, it was learned that the neurological examination should be controlled again from the surgeon because of the controversial of the neurological deficit. As a result, patient awakened from anesthesia. We administered 2mg/kg sugammadex and spontaneous breathing of patient returned immediately. The patient became conscious and orientated immediately afterwards. The neurological examination of the lower extremities was performed. The patient was anesthetized once again and 0.6mg/kg rocuronium was given in order to gain neoromuscular block approximately 10min after sugammadex administration. 2min later, the patient was smoothly intubated. Neuromuscular monitorization was not used because of emergency. We administered 2mg/kg sugammadex at the end of the procedure and the patient was extubated. The most suitable time for the re-establishment of rocuronium following sugammadex is currently unclear. This case showed that neuromuscular block can be effectively re-induced by rocuronium following the reversal of rocuronium-induced neuromuscular block with sugammadex. In this case, we consider that the ability to effectively reuse normal induction doses of rocuronium is an important clinical observation.

Left-to-Right Shunt with Congenital Heart Disease: Single Center Experience.

Objective. The objective of this study was to determine the frequency of pulmonary arterial hypertension (PAH) in congenital heart disease (CHD) with an isolated, large left-to-right shunt and to indicate the factors in the development of PAH. Methods. The pressure measurements in the cardiac chambers and the calculations based on the Fick's principle were compared among 3 separate groups of patients, respectively, with PAH, with hyperkinetic pulmonary hypertension (HPH), and with neither PAH nor HPH. Results. PAH was diagnosed in 30 (12.3%) patients, HPH in 35 (14.4%), while 177 (73.1%) were free of either. The highest risk for the development of PAH was found in the presence of perimembranous ventricular septal defect. A statistically significant difference was seen among these groups as to their left atrial pressure (p = 0.005) and the mean pulmonary arterial pressure (PAPmean; p < 0.001). While a correlation was present between RpI on one hand and age on the other (p = 0.014), a multiple linear regression could not evidence any correlation among age (p = 0.321), gender (p = 0.929). Conclusion. Our findings do not allow establishing a correlation between the duration of the high pulmonary flow and pulmonary vascular resistance increase or PAH development in isolated left-to-right shunts with congenital heart diseases.

Doppler tissue imaging provides an estimate of pulmonary arterial pressure in children with pulmonary hypertension due to congenital intracardiac shunts.

OBJECTIVE: The aim of this study is to determine the relationship between the cardiac catheterization findings and pulsed-wave (PW) Doppler and Doppler tissue imaging (DTI) in pulmonary arterial hypertension patients with congenital heart disease with intracardiac shunts. DESIGN AND PATIENTS: The present study aims to determine the relationship between the cardiac catheterization findings and PW Doppler and Doppler tissue imaging (DTI) in patients who have pulmonary arterial hypertension patients due to congenital heart disease with intracardiac shunts. Echocardiographic measurements were performed at the catheter angiography laboratory with concurrent catheterization. Left and right ventricle inflow velocities were recorded with PW Doppler and DTI studies. Maximum tricuspid regurgitation velocity (TS) was recorded in cases with measurable levels by continuous-wave Doppler. Moreover, the correlations among the echocardiographic values and invasive hemodynamic measures such as systolic pulmonary arterial pressure (PAPsystolic), mean pulmonary arterial pressure (PAPmean), diastolic pulmonary arterial pressure (PAPdiastolic) and pulmonary vascular resistance index (PVRI) were evaluated. RESULTS: A negative correlation was found between TE'/TA' and PAPsystolic, PAPdiastolic and PAPmean (P = 0.008, r = -0.480; P = 0.001, r = -0.584; P = 0.001, r = -0.567, respectively). ME/ME' was also found to be negatively correlated with PAPdiastolic, PAPmean and PVRI (P = 0.002, r = -0.556; P = 0.005, r = -0.502; P = 0.027, r = -0.411, respectively). The concurrent use of TE'/TA' (cut-off value <2.6) and TS had a sensitivity of 79% and a specificity of 93% for distinguishing between patients with healthy controls. CONCLUSION: When used in conjunction with conventional methods, TE'/TA' has the highest sensitivity and specificity in distinguishing between patients and healthy controls.

Elevated homocysteine and asymmetric dimethyl arginine levels in pulmonary hypertension associated with congenital heart disease.

Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality among patients with congenital heart disease (CHD). This study was designed to determine biomarker levels in patients with PAH associated with CHD (PAH-CHD) and CHD patients without PAH and to investigate the relationship of these potential biomarkers with hemodynamic findings. In this prospective single-center study, patients with CHD were analyzed according to the presence or absence of PAH and compared with healthy control subjects. Cardiac catheterization and echocardiographs were performed. Plasma homocysteine, asymmetric dimethyl arginine (ADMA), and nitric oxide (NO) levels were determined by enzyme-linked immunosorbent assay. Homocysteine and ADMA levels were higher in the PAH-CHD group (n = 30) than among CHD patients with left-to-right shunting but no PAH (n = 20; P < 0.001) and healthy control subjects (n = 20; P < 0.001). There was no difference in NO levels. Cyanotic PAH-CHD patients had significantly higher homocysteine than acyanotic patients in the same group. No correlation was shown between echocardiographic/hemodynamic parameters and homocysteine, ADMA, and NO levels. Homocysteine and ADMA levels are increased in patients with PAH-CHD. These parameters have the potential to be used as biomarkers in the diagnosis and follow-up evaluation of patients with PAH-CHD. However, large, multicentered prospective studies are required to facilitate routine use of these biologic markers in the clinical setting.

Profile of vitiligo patients attending a training and research hospital in Central Anatolia: a retrospective study.

Clinical characteristics of vitiligo had been reported in different countries previously. This is the second report from Turkey and it is from a different region of Turkey, middle Anatolia, and a more detailed study. The age and sex of patients, onset age, duration, areas of involvement, course, clinical types and severity of the disease, family history of vitiligo, associated disorders, the values of vitamin B12, folic acid, ferritin, iron and hemoglobin, antithyroglobulin and antithyroid peroxidase antibodies and positivity of viral markers were obtained from the records of the patients retrospectively. The study involved 93 vitiligo patients with a mean age of 37.4±17.05 years. The median age of onset disease was 33 years and onset age of disease was significantly lower in women. Of the patients, 24.8% had family history. The most frequent form of vitiligo was vitiligo vulgaris. Facial involvement was the most common localization. Trunk involvement was significantly higher in women but anogenital involvement was significantly higher in men. Of the patients, 40.3% had another autoimmune disease, 19.7% of the patients had low ferritin levels, 12% had low iron levels, 9.9% had anemia, 4.9% had low vitamin B12 levels, 3.8% had low folic acid levels and 4.3% had positivity of viral markers. The age of onset and the incidence of family history were higher and sites of involvement according to sex were different from the published work. The knowledge of clinical characteristics, course and associated conditions of vitiligo may improve the approach of dermatologists to vitiligo patients.

Hepatopulmonary syndrome in children with cirrhotic and non-cirrhotic portal hypertension: a single-center experience.

BACKGROUND: Hepatopulmonary syndrome (HPS) is defined as an arterial oxygenation defect induced by intrapulmonary vascular dilatation (IPVD) associated with hepatic disease. The prevalence and clinical characteristics of HPS in portal hypertensive children is not well characterized. AIMS: The aim of this study was to investigate the prevalence and clinical characteristics of HPS in 40 portal hypertensive children. METHODS: We studied 40 children (11 girls and 29 boys; mean age, 111 months ± 52 months; range, 24-216 months) with portal hypertension (24 cirrhotic, 16 non-cirrhotic) for the presence of HPS using blood gas analysis, contrast-enhanced echocardiography (CEE), and Tc99m-macroaggregated albumin scintigraphy. Clinical and laboratory characteristics of patients were recorded. HPS was considered to be present in a patient with hypoxemia and/or an elevated alveolar-arterial oxygen gradient (PAaO(2)) ≥ 15 mmHg) and positive CEE and/or scintigraphy. RESULTS: Elevated PAaO(2) was detected in 7 of 24 patients with cirrhosis. Four of them also had IPVD with CEE. An intrapulmonary shunt in Tc99m-MAA with CEE was shown in one patient. A diagnosis of HPS was made in 16.7% of the patients with cirrhosis. Cirrhotic patients without IPVD had significantly better hepatic function and lower pediatric end-stage liver disease scores. Although, 2 of the 16 patients with non-cirrhotic portal hypertension had elevated PAaO(2,) none of them showed IPVD. One normoxemic patient in the non-cirrhotic portal hypertension group showed IPVD with CEE. None of the non-cirrhotic patients fulfilled the diagnostic criteria of HPS. CONCLUSION: Hepatopulmonary syndrome particularly occurs in cirrhotic portal hypertensive patients with severe hepatic dysfunction.

An unusual case of heterotaxy and polysplenia syndrome associated with hemiazygous continuation of the left-sided vena cava inferior, dilated azygous vein and large venous ectasia.

We report a 13-year-old male patient with heterotaxy and polysplenia syndrome associated with hemiazygous continuation of the left-sided vena cava inferior, dilated azygous vein, and large venous ectasia. This is the first report of heterotaxy and polysplenia syndrome associated with this particular venous arrangement.

Cardiac troponin T in children with acute rheumatic carditis.

The existence of cardiac damage in active rheumatic carditis patients is unknown, especially in those without pericarditis. The aim of this study was to determine cardiac myocyte damage using cardiac troponin T (cTnT) measurements in active rheumatic carditis. The levels of creatine kinase MB isoenzyme (CK-MB), cTnT, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), cardiothoracic ratio (CTR), and fractional shortening (FS) were compared using Mann-Whitney U test between 28 patients with active rheumatic carditis and 32 controls (healthy children). Association of cTnT levels with ESR, CRP, and CTR was evaluated with Spearman correlation analysis. ESR, CRP, cTnT levels, and CTR were statistically higher in the patients than in the controls. There were no differences between the groups for CK-MB levels and FS. No relationships were found between cTnT levels and ESR, CRP, and CTR in the patient group. The results of the study suggest that when cTnT levels are within non-pathological range, there is no serious cardiac damage; however, statistically significant increases in cTnT levels may indicate minor damages in patients with active carditis due to acute rheumatic fever.

Early diagnosis of anthracycline toxicity in asymptomatic long-term survivors: dobutamine stress echocardiography and tissue Doppler velocities in normal and abnormal myocardial wall motion.

AIMS: Asymptomatic long-term cancer survivors treated with anthracycline were investigated for late anthracycline cardiotoxicity using dobutamine stress echocardiography (DSE) and tissue Doppler (TD) velocities. METHODS AND RESULTS: The study comprised 20 asymptomatic patient and 18 healthy children as the control group. Twenty patients were divided into two groups according to the myocardial wall motion during DSE: Group 1 (normal myocardial wall motion; six girls and five boys) and Group 2 (abnormal myocardial wall motion: nine boys). Intravenous dobutamine infusion was started at a dose of 5 µg/kg/min (D5) and gradually increased to 10 (D10), 15 (D15) and 20 µg/kg/min (D20). Echocardiographic assessment was performed at rest and after each dose of dobutamine infusion. Abnormal myocardial wall motion was observed at rest in 3 patients and during DSE in six patients. There were no significant differences between the patients and control groups at rest except the end systolic wall stress and mitral deceleration time measured by conventional methods; however, both patients group showed significant differences of systolic and diastolic functions at D20. In patients groups, systolic and diastolic functions of interventricular septum (IVS) and systolic function of left ventricle (LV) and right ventricle (RV) TD velocities showed significant changes compared with control group at rest. Significant differences of diastolic functions of IVS and RV were noted during dobutamine infusion in abnormal myocardial wall motion compared with other groups. CONCLUSION: LV, RV and IVS TD velocities systolic function at rest and during DSE can provide valuable information for early detection of subclinical cardiac toxicity. TD velocities of diastolic functions during DSE are a valuable parameter for assessment of subclinical cardiac toxicity in patient with abnormal wall motion.

[The role of dobutamine stress echocardiography in early diagnosis of cardiac toxicity in long-term survivors of asymptomatic children treated with anthracycline]. / Antrasiklin ile tedavi edilen yasayan asemptomatik çocuklarda uzun dönemde gelisebilecek kardiyotoksisitenin erken tanisinda dobutamin stres ekokardiyografinin yeri.

OBJECTIVE: Long-term survivors of asymptomatic children treated with anthracycline may have cardiac toxicity without clinical findings. The subclinical cardiac toxicity could be evaluated by dobutamine stress echocardiography (DSE) with exploring effective and safe doses of dobutamine. METHODS: Twenty asymptomatic survivors (mean age: 19.2+/-4.0 years) treated with cumulative dose of 282.1+/-125.9 mg/m2 of anthracycline were compared with 18 age-matched healthy volunteers. Total time completed this treatment was 10.2+/-2.2 years. This was a cross-sectional case-controlled study and patient and control groups were evaluated at the time of routine appointments. Echocardiographic studies were performed before and after each dobutamine infusion of 5, 10, 15, 20 microg/kg/min. STATISTICAL ANALYSIS: Mann-Whitney U test was used to evaluate the difference between the groups. ANOVA for repeated measurements test was used to compare each measurement of control and patients groups and Bonferroni posthoc test was used for correction. RESULTS: Hemodynamic changes are observed at the dobutamine doses of 15 microg/kg/min in the patient group. Before dobutamine infusion in the patient group only isovolumic relaxation and contraction times values were prolonged comparing to the control group. After the infusion of dobutamine ejection fraction, shortening fraction, left ventricular posterior wall thickening (%LVPWt), end-systolic wall stress (ESS), interventricular septum systolic thickening, left ventricular end-systolic and end-diastolic diameters, mitral acceleration (AT) and deceleration times values were deteriorated in the patient group compared to the control group (p=0.05 for all). The highest differences between the groups were observed in the %LVPWt, ESS and AT values at the end of test. CONCLUSION: The DSE is an effective and safe method to demonstrate the late anthracycline cardiotoxicity. Echocardiographic evaluation should be made at rest and dobutamine dose of 20 microg/kg/min. In the early diagnosis of late cardiac toxicity; assessment of %LVPWt, AT and ESS values in addition to standard echocardiographic examination could be the guidance for early diagnosis of late cardiac toxicity.

Woven right and aneurysmatic left coronary artery associated with Kawasaki disease in a 9-month-old patient.

Woven coronary artery disease is an extremely rare congenital abnormality with unusual findings of branching thin channels and distal reanastomosis. This pathologic finding was reported earlier in a few adult patients. In Kawasaki disease, coronary arterial system is commonly affected, which causes a necessity of cardiac imaging. We report a 9-month-old infant with Kawasaki disease in which left coronary artery aneurysm and woven right coronary artery were coincidentally detected during coronary angiography. After 1 year, coronary angiogram was re-performed and showed no changes in the coronary arteries. During the follow-up period of 4 years, the patient remained asymptomatic. In Kawasaki disease, there is a tendency for thrombus formation and a woven coronary artery can be easily misinterpreted as a thrombus. Woven coronary artery is a benign condition and it should not be confused with a thrombus or a stenosis related finding which needs a medical or surgical intervention.