Sedef Tunaoglu

The role of viral agents in aetiopathogenesis of acute rheumatic fever

The reason why abnormal immune response exists in acute rheumatic fever is not exactly explained. The influence of co-pathogens like certain viruses were mentioned regarding the initiation of the immunological reaction in acute rheumatic fever patients by several authors since 1970. This study was designed to find the role or effect of some viral infections in the development of rheumatic fever. In this study, 47 cases with acute rheumatic fever (acute rheumatic arthritis, acute rheumatic carditis, and chorea), 20 cases with chronic rheumatic fever, 20 cases with streptococcal pharyngitis, and 20 healthy age- and gender-matched control cases were involved. Serological and molecular tests were made including hepatitis B virus, hepatitis C virus, rubella virus, herpes simplex virus (HSV group 1), and Epstein-Barr virus (EBV). HBsAg, rubella IgM and EBV IgM positivity were not seen in any of patients with rheumatic fever. Although antiHBs seropositivity was higher in the control group, it was not statistically significant (p > 0.05). There was no difference in rubella IgG, HSV IgM seropositivity, either (p > 0.05). EBV DNA was searched by the polymerase chain reaction technique; due to the latent nature of the virus, no significant difference was found between the control group and the other groups (p > 0.05). In this study, no positive correlation could be found to support the synergism theories regarding the streptoccocus infection and viral infections in the development of acute rheumatic fever. Only EBV DNA positivity was found in all acute rheumatic fever cases but not in the control group may lead to further studies with larger series of patients.

Posttraumatic Left Ventricular Pseudoaneurysm in a Child

A 7-year-old boy developed a left ventricular pseudoaneurysm after being struck by a tractor. The pseudoaneurysm was diagnosed and successfully resected 5 years after the accident.

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

Serum and pulmonary vascular endothelial growth factor/receptors and haemodynamic measurements in cyanotic congenital heart disease with decreased pulmonary blood flow

Tetralogy of Fallot is the most common cyanotic congenital heart disease with decreased pulmonary blood flow. Right-to-left shunt and infundibular pulmonary stenosis in this disease lead to a decrease in arterial O(2) saturation. Hypoxia is a strong stimulus for angiogenesis; however, the reason for insufficiency in the pulmonary vascular growth in patients despite chronic arterial hypoxia is still not known. This study was planned considering that the impairment in vascular endothelial growth factor-receptor relationship or the vascular endothelial growth factor-receptor deficiency in the pulmonary vascular bed during development may cause insufficiency of pulmonary vascular growth. A total of 24 patients were grouped as cyanotic - including 13 patients with tetralogy of Fallot - and acyanotic - including 11 patients with left-to-right shunt lesions. During cardiac catheterisation, vascular endothelial growth factor measurements were performed; and oxygen saturations, pressures, and haemoglobin levels were measured. Perioperative lung biopsy for vascular endothelial growth factor receptors was performed in the cyanotic group. Vascular endothelial growth factor of the aorta was higher in the acyanotic group. There was a significant negative correlation between vascular endothelial growth factor levels and aortic O(2) saturation in the cyanotic group (p < 0.05). Vascular endothelial growth factor tissue staining was negative in 11 out of 13 (84.6%) patients. KDR/Flk-1 receptor was positive in four out of 13 (30.7%) patients; Flt-1 receptor was positive in six out of 13 (46.1%) patients. Vascular endothelial growth factor values were found to be lower than those of the acyanotic patients in this study. Low serum vascular endothelial growth factor levels of the cyanotic group, in spite of the hypoxia, demonstrated the importance of studying vascular endothelial growth factor tissue levels and vascular endothelial growth factor receptors in these patients.

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.

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 Ficks 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.

Turner syndrome with unusual clinical features: A case report

Turner Syndrome is a genetic disease with a frequency of 1 in every 1500–2500 live-born female babies. In this report, a 2 years 10 months old patient who had the genotype of 46,XOi (Xq) will be introduced, although her phenotype and cardiovascular system involvement are not seen in the classic Turner spectrum.