Cihat Sanli

Persistent left superior vena cava: Experience of a tertiary health-care center

Background:  The aim of this study was to assess the prevalence of persistent left superior vena cava (PLSVC) in patients with all types of congenital heart defects and to determine the congenital heart anomalies accompanying PLSVC.

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.

Association of MTHFR A1298C polymorphism with conotruncal heart disease

Congenital heart diseases are common congenital anomalies with 1% prevalence worldwide and are associated with significant childhood morbidity and mortality. Among a wide range of aetiologically heterogeneous conditions, conotruncal anomalies account for approximately one-third of all congenital heart defects. The aetiology of conotruncal heart diseases is complex, with both environmental and genetic causes. Hyperhomocysteinaemia, which is often accompanied by the defects of folic acid metabolism, is known to cause conotruncal heart anomalies. In this study, we have evaluated three polymorphisms in the following two hyperhomocysteinaemia-related genes: methylenetetrahydrofolate reductase (MTHFR C677T and A1298C) and nicotinamide N-methyl transferase (NNMT rs694539) in 79 children with conotruncal heart disease and 99 children without conotruncal heart disease. Genotype distribution of the MTHFR A1298C polymorphism showed a statistically significant difference between the two groups. In the case group, AC and CC genotypes were higher than the control group (p<0.05). We have found that MTHFR A1298C polymorphism is associated with conotruncal heart disease; C allele (p=0.028), AC (OR[95% CI]=2.48[1.24-4.95], p=0.010), CC (OR[95% CI]=3.01[1.16-7.83], p=0.023), and AC+CC (OR[95% CI]=2.60[1.36-4.99], p=0.004) genotypes are more frequent in the patient group. Genotype distributions of the MTHFR C677T and NNMT rs694539 polymorphisms were similar in the two groups when evaluated separately and also according to the dominant genetic model (p>0.05). Our results suggest that MTHFR 1298C allele is a risk factor for conotruncal heart disease.

Serum Levels of Soluble ICAM-1 in Children with Pulmonary Artery Hypertension

This prospective cross-sectional study attempted to determine both the usefulness of the serum intercellular adhesion molecule-1 (ICAM-1) as a biomarker for pulmonary artery hypertension secondary to congenital heart disease and the nature of this markers association with catheter angiographic findings. Our study included a total of 70 male and female children, comprising 30 patients with both pulmonary artery hypertension and congenital heart disease, 20 patients with congenital heart disease alone, and 20 healthy control subjects. Levels of ICAM-1 in plasma samples from all groups were measured by the enzyme-linked immunosorbent assay method. Cardiac catheterization was also performed in all patients. The mean serum ICAM-1 levels in pediatric patients who had congenital heart disease with and without pulmonary artery hypertension were 349.6 ± 72.9 ng/mL and 312.3 ± 69.5 ng/mL, respectively (P=0.002). In healthy control subjects, the mean serum ICAM-1 level was 231.4 ± 60.4 ng/mL. According to the results of this study, the ICAM-1 level of the pulmonary artery hypertension group was significantly higher than those of the congenital heart disease group and the healthy control group. Correlation analysis showed that ICAM-1 level was correlated with systolic and mean pulmonary artery pressures (r=0.62, P=0.001; r=0.57, P=0.001)-which are 2 important values used in diagnosis of pulmonary artery hypertension. Moreover, receiver operating characteristic analysis yielded consistent results for the prediction of pulmonary artery hypertension. Therefore, we conclude that ICAM-1 has potential use as a biomarker for the diagnosis and follow-up of pulmonary artery hypertension.

Clinicopathologic challenge : Linear brown macules on the chest and arm

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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