Deniz Oguz

Chest pain in children referred to a cardiology clinic

One hundred consecutive patients (54 girls, 46 boys) referred to a pediatric cardiology department with the primary complaint of chest pain were evaluated. The age distribution was 2.5–16.0 years (mean 11.3 years for girls and 9.9 years for boys). The history showed 17% of patients with chest pain, 22% with heart disease, and 19% with recent death in the family. The time course of the pain was longer than 1 week in 92 patients. Localization was on the left precordium in 60 patients, and there was no radiation from the original site in 66 cases. Ninety-two percent of cases were idiopathic in origin. Of the 74 patients who had a psychiatric interview, 55 (74%) had psychiatric symptoms and 5 required psychiatric care. Anxiety, conversion disorder, and depression were the main psychiatric symptoms.

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.

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.

A Comparison Between MUGA and Echocardiography in Patients with Muscular Dystrophy in the Early Detection of Cardiac Involvement

Abstract. Muscular dystrophies are a group of sex-linked diseases with frequent myocardial involvement. In this study 14 patients with Duchenne Muscular Dystrophy (DMD), 7 with Becker Muscular Dystrophy (BMD), and 8 female carriers who were asymptomatic were evaluated with echocardiography and multigated radionuclide ventriculography (MUGA). All showed predominant systolic and minor diastolic left ventricular dysfunction determined both by echocardiography and MUGA when compared with healthy controls. In conclusion, it is recommended that DMD, BMD, and female carriers be evaluated and closely monitored for cardiac functions. Though MUGA and echocardiography are both effective and sensitive techniques, echocardiography is more convenient for availability and serial evaluation.

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.

Evaluation of pulmonary vascular resistance and vasoreactivity testing with oxygen in children with congenital heart disease and pulmonary arterial hypertension.

Estimating pulmonary vascular resistance index (PVRI) is of critical importance in determining the type of cardiac surgery, the decision to perform heart transplantation, the choice between surgery and drug treatment or combined modalities, even though it is not the only criterion for judgment (1, 2). A positive pulmonary vasoreactivity test (PVT) is accepted as an indicator of low perioperative risk and good prognosis. An acute positive response to PVT is reported in only 40% of patients (3). This test has been applied in many centers, following different protocols and different evaluation criteria. Most centers use nitric oxide (NO) or oxygen (O2) inhalation, iloprost nebulization, or a combination thereof. A reduction by 20% of mean pulmonary artery pressure (PAPmean) or in the ratio of pulmonary resistance to systemic vascular resistance index (PVRI/SVRI) will define the patient as being a “responder” (4, 5). Reports on PVT performed with different drugs have been published recently (5-8). The aim of this study was to define the hemodynamic parameters of patients undergoing cardiac catheterization in our center for congenital heart disease and pulmonary arterial hypertension (PAH), characterize the efficacy of O2 use in the PVT, and present the clinical findings in these patients with congenital intracardiac shunts. The present study was conducted on a cohort of 30 children diagnosed with PAH and congenital intracardiac shunts and placed under close surveillance at the pediatric cardiology department of the study center between October 2009 and November 2011 (Table 1). As described previously the criteria used for PAH definition and patient selection were considered as mean pulmonary arterial pressure (PAPmean) of ≥25 mm Hg, pulmonary capillary wedge pressure (PCWP) of ≤15 mm Hg, and PVR index (PVRI) of > 3 WU/m2 at rest (6). The PVRI was calculated conventionally as the ratio of the difference between PAP and left atrial pressure or the pulmonary capillary wedge pressure to mean pulmonary flow, and the values were expressed as units per square meter. These parameters were also obtained before and after pulmonary vasoreactivity testing using 100% O2 by simple face mask for 10 min when a high PAPmean was suspected. The PVT was considered positive if PAPmean or the PVRI/SVRI ratio exhibited a reduction by more than 20% (7, 8). Patients were evaluated in two groups according to PVT results, responders and non-responders (Table 2). The median age, height, weight, body surface area (BSA) and heart rate of the recruited patients were respectively 20.0 months, 76.5 cm, 9.2 kg, 0.41 m2 and 112.0 beats/min. No significant difference was found in systolic PAP (PAPsystolic), SVRI, systemic flow (Qs) before and after PVT (p>0.05). The values of the other parameters before and after PVT were significantly different, with p<0.05. Average diastolic pulmonary arterial pressure (PAPdiastolic), PAPmean and median PVRI, PVRI/SVRI showed a significant decrease following PVT. Pulmonary blood flow (Qp) and its ratio to systemic blood flow (Qp/Qs) underwent a significant increase. The fall by more than 20% of PVRI and PVRI/SVRI was especially significant with regard to their PVT positivity (Table 3). No complication occurred in any patient during PVT testing with oxygen. No statistically significant difference in PVT-related measurements before and after the test was apparent within the non-responder patient group. All values in the responders, except Qs and SVRI (p=0.541 and p=0.984, respectively) were significantly different before and after the test (p≤0.05). All of the significantly different parameters except the Qp/Qs ratio in the responders showed a reduction after the test, whereas Qp/Qs was increased (p=0.019). While 11 of 13 nonresponders received medical treatment and the other two underwent full surgical correction, 14 of 17 responders were subjected to full surgical correction. Three patients of the recruited 30 patients were lost. Two of the deceased three patients had undergone surgery and one had had medical treatEvaluation of pulmonary vascular resistance and vasoreactivity testing with oxygen in children with congenital heart disease and pulmonary arterial hypertension

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.

[The role of dobutamine stress echocardiography in early diagnosis of cardiac toxicity in long-term survivors of asymptomatic children treated with anthracycline].

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.

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.

330 Hepatopulmonary syndrome may mask cystic fibrosis

Clinically significant hepatobiliary manifestations of CF are reported to occur in 15–30% of children and cirrhosis is reported in 5–10% of pediatric populations. A 7 year-old boy consultated to pediatric pulmonology clinic with chronic cough, decreased exercise tolerance for six months and cyanosis for 15 days, in terms of hepatopulmonary syndrome. He was under investigation because of hepatosplenomegaly for one year. Physical examination demonstrated tachypnea, mild clubbing, orthodeoxia (saturation 81–82% in the upright position and 86–88% in the supine position) and common crepitan crackles on the auscaltation. On abdominal examination hepatosplenomegaly were present. The calculated alveolar-arterial oxygen gradient was 43 mmHg. Chest x-ray and CT demonstrated dilated peripheral vasculature and bronchiectasis. Liver function tests, coagulation profiles, serum ceruloplasmin, alpha-1 antitrypsin, antinuclear antibodies, anti smooth muscle antibody were all normal. Endoscopy of upper gastrointestinal tract showed no esophageal varices. Liver biopsy findings resulted as cirrhotic liver showing signs of ascending cholangitis. But definitive diagnosis was not determined for one year. Contrast-enhanced echocardography with agitated saline was compatible with intrapulmonary shunting. Although patient denied recurrent lower respiratory tract infection, diarrhea, malnutrition, CF was diagnosed with p.G85E (c.254G>A), p.I148T (c443T>C) mutation. Cystic fibrosis should be investigated in cirrhotic patients with respiratory signs. Hepatosplenomegaly may be the first sign of CF.