Transcatheter closure of partially ligated vertical vein after surgical correction of supracardiac total anomalous pulmonary venous connection

Total anomalous pulmonary venous connection [TAPVC] is an anomaly in which the pulmonary veins are directly connected to one of the systemic veins or drain into the right atrium. Management of pulmonary hypertension after the total correction of this congenital cardiac anomaly is very important. Unligation of the vertical vein in the supracardiac type of this anomaly can be a draining pathway for the prevention of postoperative pulmonary hypertension crisis. Late onset transcatheter closure of the unligated vertical vein after a decrease in pulmonary pressure with the Amplatzer vascular plug type 1 can prevent residual left-to-right shunting. Here we describe two patients who previously underwent surgical correction of supracardiac TAPVC and their vertical veinwas partially ligated due to severe pulmonary hypertension. Consequently, because of increased left-to-right shunting in the follow-up period, transcatheter occlusion of the vertical vein was done for them and this procedure seemed safe and less invasive compared to the surgical approach. At 2 years' follow-up, there was marked pulmonary artery pressure and clinical improvement

effect of educational program on the quality of life and self-efficacy of the mothers of the infants with congenital heart disease: a randomized controlled trial

Congenital heart disease causes large expenditures as well as mental pressures for the parents and, consequently, endangers the mothers' quality of life and self efficacy. Thus, the present study aimed to determine the effect of educational program on the quality of life and self efficacy of the mothers of the infants with congenital heart disease. The present randomized controlled trial was conducted on 56 mothers who had children with congenital heart disease [28 in the control and 28 in the intervention group] in Imam Reza Clinic, Shiraz, Iran in 2012. The mothers' quality of life and self-efficacy were assessed using SF-36 and Sherer's general self efficacy questionnaires before, immediately and 2 months after the training. The training was performed through four 90-minute sessions in 4 weeks. The data were analyzed using the SPSS statistical software and independent t-test, chi-square, and repeated measure analysis of variance. A significant differences was observed between the intervention and control groups regarding the mean of quality of life across the three study periods [F=59.91, P<0.0001]. A significant difference was also found between the two groups concerning the mean of self efficacy at these times [F=114.11, P<0.0001]. According to the results, providing appropriate training for the mothers of the infants with congenital heart disease increased their quality of life as well as self-efficacy.

Renal function in children with cyanotic congenital heart disease: pre- and post-cardiac surgery evaluation

Cyanotic congenital heart diseases [CCHDs] are a series of cardiac anomalies that have long been recognized as a potential cause of nephropathy. There have been few reports on renal impairment in patients with CCHD before and after corrective cardiac surgery. The aim of this study was to evaluate the prevalence of renal dysfunction before and after cardiac surgery and the impact of some risk factors on final renal outcome. Thirty children with CCHD who had done corrective cardiac surgery in the previous 6 months were enrolled in this study. All data prior to surgery were collected from the charts. Post-operation data including blood and spot urine samples were taken simultaneously for CBC, Cr, and uric acid and 24 hour urine was collected for microalbumin and Cr during the follow up visits. Pre- and post-operation parameters were compared to study the impact of cardiac surgery on renal function. Pre- and post-operative GFRs were not significantly different. Final GFR was significantly and inversely associated with pre- and post-operation age [P=0.008 r=-0.48, P=0.03 r=-0.38]. Three [10%] patients had microalbuminuria. The prevalence of microalbuminuria in children older than 10 years was 30%. There was no link between microalbuminuria and age, GFR, and hematocrit [P=0.1, P=0.3, P=0.3, respectively]. Patients with preoperation hematocrit >45 had a significantly lower final GFR compared to children with HCT <45 [83.7 +/- 6.5 vs 111.10.2, P=0.001]. The mean uric acid fraction [FEua] excretion was 8.21 +/- 4.75. Pre-operative HCT was inversely associated to FEua [P=0.01, r=-0.44]. There was no relationship between FEua and age, serum uric acid, and GFR [P=0.7, P=0.4, P=0.2]. Children with CCHD are at increased risk of renal injury which is related more to the duration of cyanosis and higher degree of hematocrit level. To lower the risk, corrective cardiac surgery is recommended to be done as soon as possible to improve renal function and stop more renal impairment

Distribution and protective role of hlab40 in Iranian patients with kawasaki disease; a report from southern Iran

Kawasaki disease [KD] clinically presents as a systemic vasculitis syndrome with significant cardiovascular involvement. With different incidence among different ethnic groups, the role of certain human leukocyte antigens and their products has been considered as a crucial predisposing factor in the immune responses in this disease. We determined the distribution of human leukocyte antigens type B for 90 Iranian patients with Kawasaki disease in order to evaluate a possible association between these antigens and this disease in our area. We used the polymerase chain reaction [PCR] sequence specific primers [PCR-SSP] technique for antigen typing. Distribution of these antigens for 89 healthy Iranians used as control. While 7 [3.9%] of our patients were positive for human leukocyte antigen type B 40, there were 18 [10.1%] subjects from the control group who had this antigen with statistically significant difference between patients and control group [CI= 95%, RR=1.15 and P= 0.02]. Data were analyzed by Pearson chi-square test and Fisher's exact test. SPSS version 15 was used for statistical analysis and a P value less than 0.05 considered statistically significant. The presence of higher frequency of allele type-B40 in the control group may represent a protective role for this antigen with resultant decreased susceptibility to KD in our area

prevalence of hypomagnesaemia in pediatric patients with mitral valve prolapse syndrome and the effect of Mg therapy

A paucity of data exists regarding the prevalence and relationship of hypomagnesaemia with clinical symptoms of mitral valve prolapse [MVP] in pediatric patients. In this study we evaluated the prevalence of magnesium [Mg] deficiency in pediatric patients with MVP syndrome and attempted to clarify the effect of Mg therapy on alleviating their symptoms. The present study was conducted from April 2010 to January 2012, and included 230 patients [90 males and 140 females] with symptoms of mitral valve prolapse and mean age of 11.6 +/- 3.66. Serum magnesium [Mg] level less than 1.5 mg/dl was defined as hypomagnesaemia. Patients with 2 mm leaflet displacement and maximum leaflet thickness of 5 mm in echocardiography were considered to have classic MVP, while those with leaflet thickness less than 5 mm were considered as non-classic MVP. Patients with hypomagnesaemia were orally treated with 4.5 mg/kg/day Mg chloride for 5 weeks followed by re-evaluation of symptoms of chest pain, palpitation, fatigue and dyspnea. Hypomagnesaemia was found in 19 [8.2%] of 230 patients with mitral valve prolapse. The re-evaluation of patients with Hypomagnesaemia after 5 weeks of Mg therapy, showed statistically significant relief of chest pain [P=0.01]. However, no significant changes was detected in regard to palpitation [P=0.06], fatigue [P= 0.5] and dyspnea [P=0.99]. This study revealed that the prevalence of hypomagnesaemia in pediatric patients with mitral valve prolapse is relatively low compared to adults, but treatment with oral Mg in patient with hypomagnesaemia decreases chest pain

T-peak to t-end abnormality in pediatric patients with syncope

The purpose of this study was to examine the relationship between T-peak-to-T-end interval and its dispersion in children with syncope to detect possible repolarization abnormalities in these patients. We enrolled 19 patients with a positive tilt test for syncope [7 boys, 12 girls] and 35 participants with normal results on the test. Mean age was 11.4 +/- 3.1 years in patients and 10.0 +/- 5.1 years in controls [P = 0.27]. The T-peak-to-T-end interval in lead V1 was significantly longer in patients with a positive tilt test [0.36 +/- 0.062 versus 0.32 +/- 0.071, P = 0.007]. T-peak-to-T-end interval dispersion was significantly greater in the group of patients [0.15 +/- 0.07 versus 0.11 +/- 0.04, P +/- 0.003]. The T-peak-to-T-end interval in lead V1 and T-peak-to-T-end dispersion were significantly larger in patients with a positive tilt test. Our findings suggest a depolarization abnormality in children with syncope

relationship of coronary sinus dilation with pulmonary artery pressure in pediatric patients

Increased right atrial pressure due to pulmonary hypertension may impair venous drainage with resultant coronary sinus dilatation. The aim of this study was to search for correlations between coronary sinus diameter and estimated pulmonary artery pressure in children. In a prospective study, 100 children who were referred consecutively for transthoracic echo car diography were included in this study. Ratios of coronary sinus diameter to weight, body surface area and aortic annulus were calculated for in each patient. Correlation between coronary sinus diameter and estimated pulmonary artery pressure was studied by person correlation. A tricuspid regurgitation peak gradient more than 36 mmHg or pulmonary regurgitation peak gradient more than 25 mmHg were considered as pulmonary hypertension. Sixty-eight of our participants had no pulmonary hypertension and 32 did. Mean age was 7.6 years in the patients without pulmonary hypertension and 8.0 years in the patients with pulmonary hypertension [P=0.11]. Mean coronary sinus diameter to aortic annulus diameter ratio was 0.49 +/- 0.13 in the patient with pulmonary hypertension versus 0.38 +/- 0.12 in the patient without pulmonary hypertension [P<0.001]. The coronary sinus diameter to body surface area ratio was 1.3 +/- 0.59 versus 0.7 +/- 0.28 [P<0.001], and coronary sinus diameter to weight ratio was 0.06 +/- 0.03 versus 0.02 +/- 0.01 [P<0.001]. coronary sinus dilation was documented in pediatric patients with pulmonary hypertension. The ratios of coronary sinus diameter to aortic annulus diameter, body surface area and weight correlated significantly with pulmonary hypertension

Aneurysmal aorto-left ventricular tunnel

Aorto-left ventricular tunnel [ALVT] is a congenital anomaly of aortic root which is an extra cardiac connection between the aorta and the left ventricle. It is usually short and direct but this report describes an aneurysmal aorto-ventricular tunnel which due to large left ventricular and small aortic hole culminated in aneurysm. This type of ALVT might be misdiagnosed as other cardiac lesions

Evaluation of pulsed doppler-versus tissue doppler-derived tei index of right and left ventricle in fetuses

The myocardial performance index [MPI], also known as the Tei index, was introduced by Tei et al. to evaluate cardiac function in adults with dilated cardiomyopathy. This index is defined as the sum of isovolumic contraction time [ICT] and isovolumic relaxation time [IRT], divided by ejection time [ET]. To determine the correlation between pulsed Doppler [PD]- and tissue Doppler imaging [TDI]-derived Tei indices in fetuses. Right and left ventricle PD and TDI echocardiographic data were obtained from 59 fetuses [11 pregnant women who were positive for anti-SSA-Ro or anti-SSB-La antibodies, 18 women who were referred due to dysrhythmia, and 30 women who had normal clinical findings]. Mean fetal gestational age was 27 +/- 6.4 weeks. Mean PD Tei index of the mitral and tricuspid valve was 0.58 +/- 0.05 and 0.53 +/- 0.08, respectively. Mean TDI indices for the mitral and tricuspid valve were 0.56 +/- 0.09 and 0.55 +/- 0.08, respectively. There were no significant differences between mitral and tricuspid PD- and TDI-derived Tei indices [P = 0.87, P= 0.21], but the Bland-Altman diagrams showed no fine agreement between the indices [the mean difference +/- 1 standard deviation of the right ventricular PD- and TDI-derived Tei indices was 0.24 +/- 0.02 and 0.29 +/- 0.04 for the left ventricle]. There were no significant differences in PD- and TDI-derived Tei indexes between groups of evaluated fetuses [Mitral valve: PD-Tei P=0.69, TDI-Tei P=0.49; Tricuspid valve: PD-Tei P=0.41, TDI-Tei P=0.36]. Although the mean values of the two indices did not differ significantly, the TDI-derived and PD-derived Tei indices did not have fine agreement

Efficacy of carvedilol in patients with dilated cardiomyopathy due to beta-thalassemia major; a double-blind randomized controlled trial

Dilated cardiomyopathy is the end result of chronic iron overload in patients with beta thalassemia major. The of the present study was to evaluate the safety and efficacy of Carvedilol in patients with beta thalassemia major and dilated cardiomyopathy. During a six-month period, fourteen patients with beta-thalassemia major and heart failure without diabetes mellitus referred to pediatric cardiology clinic enrolled in this double blind, randomly assigned study. All patients were on anti failure therapy with Digoxin, Captopril and Furosemide. Carvedilol was started at a dosage of 3.12 mg bid and for patients who had a systolic blood pressure >100 mmHg, heart rate >60/min and no signs of low cardiac output the dosage was increased every two weeks to a maximum of 25 mg bid. Clinical signs and symptoms, systolic and diastolic echocardiographic indexes and Tissue Doppler Imaging [TDI] data were collected from each patient. Eight patients received Carvedilol [Group 1] and six received placebo [Group 2]. The mean age of patients in Group1 and 2 were 16 +/- 0.7 years and 17 +/- 3 years respectively. Only one patent in Group 1 tolerated increasing Carvedilol dosage to more than 6.25 mg bid. Changes in New York Heart Association [NYHA] classification, Ejection fraction, End diastolic dimension changes, TDI systolic[S], early [Ea] and late [Aa] diastolic waves were not statistically significant in these two Groups [P>0.05]. Pulse Doppler E/A wave ratio of mitral valve in Group1 and Group 2 changed from 1.1 +/- 0.37 m/s to 1.8 +/- 0.40 m/s and from 1.34 +/- 0.30 m/s to 2.6 +/- 0.23m/s respectively [P=0.04]. Patients with thalassemia and dilated cardiomyopathy have poor tolerance to increasing Carvedilol dosage and develop decreased systolic blood pressure during advancement of the drug dosage. Carvedilol can be effective in prevention of progression of diastolic dysfunction in these patients

Estimation of right atrial pressure from the inspiratory collapse of the inferior vena cava in pediatric patients

Paucity of data exists between mean right atrial pressure [RAP] and inferior vena cava [IVC] size and collapsibility in pediatric patients with congenital heart disease. In a prospective study, fifty consecutive pediatric patients with different congenital heart diseases who had right side cardiac catheterization were studied, comparing right atrial pressure with simultaneous M-mode echocardiographic measurement of inferior vena cava diameter. Mean age of the patients was 4.96 +/- 4.05 years [30 male and 20 female]. Patients were categorized into two groups according to their right atrial pressure [RAP] as measured by cardiac catheterization: Group 1 [40 patients] were those with mean RAP <8 mmHg and group 2 [10 patients] who had a mean RAP> 8 mmHg. In M-mode echocardiography IVC size was statistically different [P=0.004 and 0.009] in inspiration and expiration in the two groups. Mean RAP was estimated to be > 8 mmHg when IVC diameter in inspiration was >3.6 [sensitivity of 100%, specificity of 47.5%, +LR=1.9] or if IVC diameter was >6mm in expiration [sensitivity of 70%, specificity of 87%, +LR=4.67]. This study showed that measurement of IVC size in inspiration and expiration can be used as a reliable method for estimation of mean right atrial pressure

Relation of mean right atrial pressure to doppler parameters of right atrial and hepatic venous flow in pediatric patients with congenital heart disease

A paucity of data exists regarding the relation of mean right atrial pressure [RAP] to Doppler parameters of right atrial and ventricular filling in pediatric patients with congenital heart disease. Fifty patients [30 male and 20 female] with mean age of 4.96 +/- 4.05 who were admitted in the pediatric cardiology ward of Nemazee Hospital affiliated to Shiraz University of Medical Sciences, were included in this study. Patients were categorized into two groups according to their RAP measured by cardiac catheterization: Group 1 [40 patients] were those with mean RAP <8 mmHg and group 2 [10 patients] who had mean RAP 38 mmHg. Data gathered from hepatic venous flow, tricuspid diastolic flow and pulse tissue Doppler of lateral tricuspid annulus of each patient were then compared with right atrial pressure obtained by cardiac catheterization. If change of peak S wave velocity of hepatic vein in respiration was more than 38%, sensitivity and specificity of a RAP more than 8 mmHg was 90% and 51.3% respectively with likelihood ratio [LR] equal to 1.85; a peak S wave velocity of less than 70 mm/sec also showed a RAP more than 8 mmHg with sensitivity and specificity of 70 and 82.1 respectively [LR=3.9]. A peak expiratory D wave velocity of hepatic vein more than 63 mm/sec was indicator of RAP more than 8 mmHg with sensitivity and specificity of 60% and 92.3% respectively [LR=7.8]. This study showed that hepatic venous flow can be valuable for estimation of mean RAP in pediatric patients with congenital heart disease

[Making a transcutaneus icterometer in neonates]

Approximately 50% of newborn develop jaundice, however only 6% of them have significant hyperbilirubinemia that need more evaluation. Actually, most of sampling [50-90%] for detection of hyperbilirubinemia is not necessary and cause some problems such as infection, tissue injury, pain, discomfort, wasting time and money. So non-invasive methods is desirable to decrease unnecessary sampling. This study performed on neonates who referred to Shiraz University of Medical Science affiliated Nemazee hospital neonatal emergency room due to jaundice. Initially 92 neonates were selected. After taking digital images and calculating mean and 95 percentile yellow wave's lengths of forehead skin and detection of total serum bilirubin, the correlation between wave's lengths and serum total bilirubin were determined. 95 percentile of skin yellow wave lengths that determined bilirubin level of 5, 10, 15 and 20 mg/dl were used for making 4 yellow strips of the icterometer. This icterometer was used for screening of 209 neonates who referred for evaluation of jaundice. Using icterometer in evaluation of 209 neonates with jaundice demonstrated sensitivity 94.7%, specificity 55.2%, positive predictive value of 85%, negative predictive value of 95% and accuracy 87%. This Icterometer can be applied as a screening tool in neonatal hyperbilirubinemia to detect less significant degree of hyperbilirubinemia that doesn't need sampling in Shiraz and even other regions of Iran with the same skin color