Search

Several surgical techniques have been proposed to repair right partial anomalous pulmonary venous connection (PAPVC) along with sinus venosus defect (SVD). This study aimed to compare the perioperative data and outcomes of double-patch repair using a minimally invasive approach versus conventional sternotomy in pediatric and adult patients.This retrospective study was conducted on 48 minimally invasive cases and 35 sternotomy cases, undergoing surgery by a single surgeon between July 2002 and August 2020. For all patients, repair was performed using the double-patch technique. In the minimally invasive approach, right mini-thoracotomy was performed with central cannulation for children and with peripheral cannulation for adults. The patients were classified into two pediatric and adult groups, and each group was categorized into minimally invasive and sternotomy approaches. They were followed-up by transthoracic echocardiography and electrocardiography before and early after surgery, 3 and 6 months after surgery, and then annually. The relative data were compared between the two approaches in terms of perioperative findings, postoperative pulmonary vein or superior vena cava (SVC) stenosis, and sinus node dysfunction.This study included 25 minimally invasive cases and 19 sternotomy cases in the pediatric group (mean age, 4.99 ± 4.28 and 6.10 ± 4.39 years, respectively) and 23 minimally invasive cases and 16 sternotomy cases in the adult group (mean age, 35.73 ± 8.06 and 32.62 ± 9.80 years, respectively). The mean and median follow-ups were 6.31 ± 4.92 years and 6 years (range: 6 month-18 year) in the pediatric group and 6.15 ± 4.53 years and 5 years (range: 6 month-18 year) in the adult group, respectively. The mean chest tube drainage was significantly lower in the minimally invasive pediatric group (p = .03), and the mean blood transfusion volume was significantly lower in the minimally invasive adult group compared to the other groups (p = .03). No stenosis occurred in the pulmonary veins. Mild SVC stenosis occurred in one patient in the minimally invasive pediatric group, with no need for reintervention. All patients had a normal sinus rhythm, except for the mentioned case with a transient, first-degree atrioventricular block, which spontaneously reverted to the normal sinus rhythm.The minimally invasive approach can be a safe and practical alternative for the double-patch repair of PAPVC and SVD. It ensures a repair with comparable quality to sternotomy, but with better cosmetic and psychological outcomes.

Objective Coarctation of the Aorta (CoA) is a relatively common cardiovascular disorder. The present study aimed to evaluate the effect of COA anatomy and high versus low-pressure balloons on the outcome of balloon angioplasty among neonates and infants. Methods In this retrospective study, the neonates and infants undergoing balloon angioplasty at Namazi hospital were enrolled. After balloon angioplasty, immediate data results were promptly recorded.Moreover, midterm echocardiographic information was collected via electronic cardiac records of pediatric wards and clinical and echocardiographic data at least 12 months after balloon angioplasty. Finally, data were analyzed using SPSS-20. Results In this study, 42 infants were included. The median age at the time of balloon angioplasty was 1.55 (range 0.1–12) months and 66.7% of the patients were male. The mean pressure gradient of coarctation was 38.49 ± 24.97 mmHg, which decreased to 7.61 ± 8.00 mmHg (P Conclusion Recoarctation rate was lower in the high-pressure balloon. The infant with discrete COA had a better response to the balloon with more decrease in gradient and lower recoarctation rate. Therefore, the stenotic segment anatomy needs to be considered in the selection of treatment methods.

Background: Coronary sinus dimension is an important factor for diagnosing some types of cyanosis as well as congenital heart diseases and insertion of some devices into the coronary sinus if required. This study was designed to access the diameter and Z-score of the coronary sinus among children under 18 years of age. Methods: In this cross-sectional study on 95 individuals, the coronary sinus diameter was measured by transthoracic echocardiography in the four-chamber view at the connection to the right atrium, middle part, and distal end. The linear regression equation was utilized to determine age-adjust reference values, Z-scores, and the relationship between the individuals’ coronary sinus diameter age, height, and body surface area. The study was conducted from March to July 2020 in Namazi hospital clinic of Shiraz University of Medical Sciences, Shiraz, Iran. Results: The mean age of the persons who entered this study was 5.87±4.25 years. The mean coronary sinus diameter was 4.91±1.29 mm at the site of connection to the right atrium, 4.50±1.44 mm at the middle part, and 3.74±1.32 mm at the distal end. Coronary sinus diameter correlates positively with the participants’ age, weight, height, and body surface area (P

Background Closure of perimembranous ventricular septal defects (pmVSD), either surgical or percutaneous, might improve or cause new-onset mitral regurgitation (MR) and aortic regurgitation (AR). We aimed to evaluate the changes in MR and AR after pmVSD closure by these two methods. Material and method We performed a comparative retrospective data review of all pediatric patients with pmVSDs treated at our institution with surgical or antegrade percutaneous methods from 2014 to 2019 and 146 consecutive patients under 18 years were enrolled. We closely looked at the mitral and aortic valve function after repair. Included patients had no or lower than moderate aortic valve prolapse and baseline normal mitral or aortic valve function or less than moderate MR or AR. Results Out of 146 patients, 83 (57%) pmVSDs were closed percutaneously, and 63 (43%) pmVSDs were closed surgically. 80 and 62 patients were included for MR evaluation, and 81 and 62 patients for AR evaluation in percutaneous and surgical groups. The mean follow-up time was 40.48 ± 21.59 months in the surgery group and 20.44 ± 18.66 months in the transcatheter group. Mild to moderate degrees of MR and AR did not change or decreased in most patients. In detail, MR of 70% and AR of 50% of the valves were resolved or decreased in both groups. 13% of patients with no MR developed trivial to mild MR, and 10% of patients with no AR showed mild to moderate AR after pmVSD closure in both methods. There was no significant difference between the two methods regarding emerging new regurgitation or change in the severity of the previous regurgitation. Conclusion pmVSD closure usually improves mild to moderate MR and AR to a nearly similar extent in both percutaneous and surgical methods among children and adolescents. It might lead to the onset of new MR or AR in cases with no regurgitation.

Objective Spontaneous closure of the ductus arteriosus often fails to occur in premature newborns and this condition can be associated with increased morbidity and mortality. The initial treatment to achieve closure of the opening is pharmacological, and various nonsteroidal anti-inflammatory drugs may be used. The aim of this study was to determine whether combining acetaminophen with ibuprofen is more effective than the individual use of these drugs to treat patent ductus arteriosus (PDA). Methods The present randomized, controlled trial study included 154 premature newborns with PDA. The patients were randomized into 3 groups: the acetaminophen group (n=67), ibuprofen group (n=68), and combination drug group (n=19). Echocardiography was performed before initiating the medication and after completing a first and second course of treatment. Blood markers were measured to assess the safety of the 3 types of therapy. Results After the first course of treatment, PDA closure was seen in 76.1% of the infants in the acetaminophen group, 76.4% of those in the ibuprofen group, and 78.9% of the combination therapy group (p=0.97). The closure rate after a second course of treatment was 43.7% in the acetaminophen group, 62.5% in the ibuprofen group, and 100% in the combination group. There were no complications attributed to the 3 methods of treatment used. Conclusion Concomitant use of acetaminophen and ibuprofen can be an effective option for closure of PDA. Other studies with a larger sample size are recommended in order to confirm these results.

Background Transcatheter closure of intracardiac defects might be complicated by intravascular hemolysis. We evaluated hemolysis and its outcome after transcatheter closure of these defects. Methods and patients All patients who underwent transcatheter closure of patent ductus arteriosus, ventricular septal defect and atrial septal defect were included in this prospective study. Clinical data were obtained before and after the catheterization. Results One hundred and thirty-eight patients were enrolled; and four (3%) patients developed intravascular hemolysis; while two cases had residual shunt and two other cases had not residual flow. Although residual shunt occurred in ten patients, only 2 of these cases developed hemolysis. Patent ductus arteriosus closure was done for one of these cases and the other one, underwent perimembranous ventricular septal defect closure. Moreover, hemolysis occurred after device closure of patent ductus arteriosus in 2 of the other patients with no residual shunt. In this study the hemolysis was eliminated by conservative management within 2 weeks although residual shunt continued in this time. We observed a decline in lactate dehydrogenase value after catheterization in comparison with precatheterization, which was mainly among ventricular septal defect patients that might be due to mild chronic hemolysis in these patients. Conclusion Incidence of hemolysis after device closure was low, and occurred with and without residual flow and was eliminated by conservative management in 2 weeks, without the need for surgery, although the residual shunt was continued.

Background Coronary artery fistula (CAF) is a rare congenital anomaly with a challenging scenario in children. This study reports our experience in transcatheter closure of CAF with Nit-Occlude PDA coil and midterm clinical and imaging follow-up. Methods Twelve children with congenital CAF between 2009 and 2019, mean age 2.05 ± 2.05 years (4 days to 7.2 years), mean weight 8.8 ± 4.83 (2.8–17 kg), who underwent transcatheter closure with PFM coil at the Namazi hospital, Shiraz, Iran, were reported. Echocardiography and electrocardiogram were done before and after the procedure (early, 3, and 6 months after), and Multi-slice computerized tomography or conventional coronary angiography was performed at least one year after closure. Results In a median follow-up of 5.5 years (range 13 months to 8 years), retrogradely closed fistula had no residual, and the fistula tract was wholly occluded, but in most anterogradely closed fistula, had a small residual, which made the fistula tract open and need additional coil closure. Conclusions Transcatheter closure of CAF with PFM coil is feasible and effective with low mortality and morbidity, although antegrade closure with this device may be accompanied by residual shunt and need for multiple coil insertion.

Background Repair of the absence of the whole or major parts of pulmonary arteries is a challenge, and the choice of conduit material to reconstruct the pulmonary arteries is under dispute. We used the autologous innominate vein to construct pulmonary arteries. Case presentation l We present a novel technique using the autologous innominate vein as a free graft in a 6-month-old infant with pulmonary atresia and absence of central pulmonary arteries. Double ductus arteriosus were the only source of perfusion of the lungs. The innominate vein was substituted for the central pulmonary artery between the two lung hila. Total repair by using Contegra graft was performed 9 months later. The patient has been followed for 5 years. Conclusions The autologous innominate vein could be used as inter-hilar pulmonary arteries with no calcification and fibrosis in 5-year follow-up.

Introduction The absence of a pulmonary artery is a rare congenital anomaly that occurs isolated or with other congenital cardiac disorders, particularly tetralogy of Fallot (TOF); meanwhile, a hidden pulmonary artery might exist and originate from a closed ductus arteriosus (DA), which can be stented to reach the artery. Material and methods This prospective study describes cardiac catheterization of nine TOF patients diagnosed with the absence of the left pulmonary artery before the operation. The patients were stratified into three groups: group one, whose closed DA was found and connected to the hidden pulmonary artery with a stent; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful. We also evaluated outcomes of six other surgically-corrected TOF patients who were operated with the absent left pulmonary artery. Results The first group included the patients aged 1, 24, and 30 months, whose CT angiography 6–9 months after stenting showed acceptable left pulmonary artery diameter for surgical correction, and the pulmonary vein angiography of the second group showed a hidden left pulmonary artery with a suitable diameter for surgical correction. However, we were unable to find or stent the DA of group three patients, aged 12, 38, 60, and 63 months. Earlier Angiography might have increased the chance of access to the hidden vessel. Apart from these three groups, follow-ups of six other patients previously corrected with only the right pulmonary artery revealed pulmonary artery hypertension in all patients. Conclusion The concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery. This procedure may allow TOF total surgical correction with two pulmonary arteries. Besides, pulmonary vein angiography can reveal the hidden pulmonary artery.

Objectives The aim of this study was to collect consistent data on the efficacy and safety and evaluation hepatotoxicity of intravenous acetaminophen for the treatment of PDA in preterm infants. Methods This is an observational longitudinal prospective study on 46 preterm infants with PDA who treated with high dose of acetaminophen and evaluated with echocardiography and serum liver enzymes at Hafez and Zeinabiyeh hospitals from January 2016 to December 2019. Result Forty-six preterm infants with PDA treated with intravenous acetaminophen. Rate of closure of PDA was 82.6. There was no significant difference after treatment regarding AST, ALT, Albumin, total and direct bilirubin (P value > 0.05) and no adverse side effects were observed in association with intravenous acetaminophen. Conclusion High dose of acetaminophen is not more effective than that with standard doses although without hepatotoxic side effect for PDA closure.

Pulmonary arterial hypertension (PAH) related to left-to-right shunt can progress to Eisenmenger syndrome, a serious and fatal disease that is not yet curable. This pilot study considered stem cell injection as a new treatment modality in persistent pulmonary hypertension after the correction of a congenital heart defect. Three patients with persistent pulmonary hypertension after ventricular septal defect repair were included in this pilot study for a clinical trial. Mononuclear stem cells derived from patients’ bone marrow specimens were injected into the right and left pulmonary arteries via cardiac catheterization. The patients were followed over a 6-month period, with six-minute walk test, echocardiography and repeated angiography performed in the sixth month after treatment. The results of the study showed improvement of 40 m, 280 m and 100 m in 6-minute walk distance in patients 1 to 3, respectively. The peak PR gradient decreased 2, 5 and 9 mmHg by echocardiography, and mean PA pressure decreased 21, 22 and 9 mmHg by catheterization in patients 1 to 3, respectively. Pulmonary artery resistance decreased 4, 4.5 and 1.3 Wood units after 6 months of stem cell therapy in the three patients. No short-term complications were detected in this pilot trial, and all patients tolerated the procedure without any complications. Intrapulmonary artery injection of stem cells may have a role in the treatment of persistent PAH secondary to congenital heart disease. This procedure is feasible, with no significant complications, and this study can be considered as a platform for larger studies.

Kawasaki disease (KD) is the most frequent cause of coronary artery aneurysm (CAA) in children. This study tried to evaluate the accuracy of different KD scores developed for prediction of CAA, in an Iranian population.This is a cross-sectional retrospective investigation on pediatric patients with a diagnosis of KD. Clinical manifestations, laboratory, and echocardiographic data were recorded. Five Kawasaki scores, including Kobayashi, Egami, Sano, Nakano, and Harada, were assessed and analyzed in relation to CAA and intravenous immunoglobulin (IVIG) resistance.During five years, we recruited 121 cases of KD under 13 years of age. The rates of CAA and IVIG resistance were 16.5%, and 13.2% respectively. The IVIG resistance group was significantly younger than responder patients. All five scores had low sensitivity in predicting CAA or IVIG resistant cases; the highest sensitivity pertained to the Harada score with 50% sensitivity and 59% specificity (the area under the curve: 0.545, with a 95% confidence interval: 0.423 to 0.667) in predicting CAA, which is lower than the usual acceptable criteria for a screening test. The specificity of all other scores were more than 85% in predicting CAA or IVIG resistance. Gender, fever before therapy and laboratory data showed no significant difference between the groups.The Kobayashi, Egami, Sano, Nakano and Harada scores have limited usefulness in the Iranian population to predict high risk patients for coronary artery involvement or IVIG resistance; in our study, age under one year was a risk factor for IVIG resistance.

The goal of this study was to compare heart function and arrhythmia in clinically asymptomatic patients with beta thalassemia intermedia and beta thalassemia major.In this cross-sectional study, 60 patients with beta thalassemia major and 60 patients with beta thalassemia intermedia who had clinically no symptoms of arrhythmia and clinically normal heart function were evaluated using 24-hour ambulatory electrocardiogram monitoring and echocardiography. For data analysis SPSS ver.20 software was used. A P-value of less than 0.05 was considered statistically significant.The mean age of the beta thalassemia intermedia patients was 24.18 ± 7.9 years and the mean age in beta thalassemia major was 24.38 ± 7.7 years (P0.05). Premature atrial contractions (PACs) were observed in 14 (23.3%) patients with beta thalassemia intermedia and in 22 (36.6%) beta thalassemia major patients. Premature ventricular contractions (PVCs) were detected in 8 (13.3%) patients in the beta thalassemia intermediate group and 16 (26.6) patients in the beta thalassemia major group, respectively. The left ventricular diastolic dimension, end-diastolic volume, and stroke volume were significantly higher in beta thalassemia intermedia group (P0.05). Pulmonary acceleration time as an indicator of pulmonary pressure was lower in beta thalassemia intermedia group.Both atrial and ventricular arrhythmias were more common in the beta thalassemia major group. Higher end-diastolic volume and stroke volume were detected in the beta thalassemia intermedia group. Pulmonary acceleration time was lower in the beta thalassemia intermedia group, which can be an indicator of higher pulmonary pressure.

FH is a genetic disorder characterized by an increase in serum LDL and total cholesterol values. The afflicted patients are at increased risk of premature atherosclerosis and myocardial infarction. Different treatment modalities are present, including pharmacological agents and surgical procedures. The most effective method of therapy in refractive cases is liver transplantation. Herein, we report our experience on 36 cases of patients with FH undergoing liver transplantation in our center, the main referral center of liver transplantation in Iran. The clinical findings, hospital courses, post-operative complications, and patient follow-up are also described.

The aim of this study was to determine the left ventricular myocardial deformation and segmental myocardial dysfunction by speckle tracking echocardiography and tissue Doppler imaging among the operated patients with anomalous origin of the left coronary artery from the pulmonary artery. The study was conducted on 12 patients diagnosed with anomalous origin of the left coronary artery from the pulmonary artery, who had been operated upon between 2001 and 2013 at the medical centres of Shiraz University of Medical Sciences, Shiraz, Iran. The mean age of the patients at the time of surgical correction was 12.6 years ranging from 6 months to 43 years, and the duration of postoperative follow-up was between 1 and 12 years. Comparison of the strain rate between the patients with acceptable ejection fraction and the control group by tissue Doppler imaging showed significant differences between the two groups regarding the lateral wall (p

Changes in cardiac structure and function are common among patients with end-stage renal disease. The aim of this study was to evaluate the effect of hemodialysis on global and segmental cardiac function in children with end-stage renal disease.Fifty-two children with ESRD who were on long-term hemodialysis at Nemazee Hospital, Shiraz, were enrolled. They underwent echocardiography (M-mode, 2-dimensional, Doppler, and speckle echocardiography) 30 minutes prior to and after hemodialysis, and the values were compared.The mean age of the patients was 13.00 ± 3.53 years. There was a significant reduction in left ventricular systolic and diastolic dimensions and volume (P.001) and the E:A ratio after dialysis (P = .03). Global and longitudinal strain and strain rate showed no significant changes before and after hemodialysis.Hemodialysis with volume reduction decreases left ventricular volume but not regional strain and strain rate significantly in children. This study showed preload independency of speckle echocardiography in children.

To evaluate the cardiac conduction system using P wave dispersion on electrocardiogram and its relationship with the short term mortality and development of arrhythmia in asphyxiated neonates. Thirty term babies with evidence of asphyxia and without any congenital abnormalities were consecutively evaluated as cases. They were compared with thirty healthy term babies without asphyxia. Twelve-lead surface electrocardiography was obtained from all the patients and the controls, and P wave dispersion was calculated according to its definition as the difference between P maximum duration and P minimum duration in 12-lead electrocardiogram. A statically significant difference of P wave dispersion was observed between the patients and the control group (0.027 ± 0.011 mm/s and 0.016 ± 0.006 mm/s, respectively; P value = 0.0001). The P wave dispersion had a statistically significant correlation with the grade of asphyxia (P = 0.004, r = 0.62), the P wave dispersion had no statistically significant correlation with Apgar scores, short term arrhythmia, and troponin I level in asphyxiated neonates (P

There are conflicting reports about the protective effect of hemophilia on the occurrence of ischemic heart disease. This study focuses on evaluation of heart function in patients with hemophilia. Cross-sectional, case–control study was done on all patients with hemophilia A or B who came to hemophilia center, and data were compared to controls. The data were collected from their charts, and heart function was evaluated by 2-dimensional, Doppler and pulse tissue Doppler. The serum troponin I level was measured in all patients as a marker of myocardial damage. Fifty patients with hemophilia took part in this study. All of them were male with mean age 29.1 years. Systolic blood pressure (mean = 121.52 ± 11 vs 115.61 ± 9.81, P = .038) and diastolic (mean = 81.94 ± 4.51 vs 75.21 ± 3.95, P = .042) blood pressure were higher in the patients. Five (10%) patients had systolic hypertension and 7 (14%) patients had diastolic hypertension. The M-mode echocardiography results showed that interventricular septum in diastole in patients with hemophilia (mean 1.143 ± 0.29) was significantly thicker than the control group (mean 0.828 ± 0.22, P < .001). Tissue Doppler echocardiography showed that late diastolic velocity of septum (Aa; P = .030), systolic velocity (S) of lateral mitral valve ( P = .006), late diastolic velocity of lateral mitral (Aa) annulus ( P = .038), and late velocity of (Aa) tricuspid ( P = .004) had significant difference compared with the control group ( P < .05). Troponin enzyme level was < 0.1 in all patients. Patients with hemophilia had higher blood pressure and more hypertension. Echocardiographic study of patients with hemophilia showed some increase in septal thickness and changes in diastolic dysfunction

Hypertension is a common complication after renal transplantation and is associated with increased risk of cardiovascular disease. The aim of the current study was to investigate the diurnal blood pressure pattern and its relation to structural and functional cardiac changes in renal transplant recipients. Sixty-six stable renal transplant patients (34 female, 32 male), aged 7 to 25 years (mean 17.4 ± 4.3 years) were enrolled in this study. Cardiac function assessed by tissue Doppler echocardiography and blood pressure measurement performed using both the ambulatory and the casual method. Hypertension was demonstrated in 57% of recipients by the casual method and in 75.7% by ambulatory blood pressure monitoring (ABPM). The efficacy of BP control among patients on antihypertensive drugs was 60%. The prevalence of non-dipping was 73%. There was significant inverse correlation between systolic or diastolic day-time or night-time BP index and post-transplant duration (p < 0.001, r =−0.386), but no correlation between ABP parameters and BMI, gender, and eGFR. There was a significant relationship between all ABP parameters and left ventricular mass index (LVMI) (p = 0.025–0.007, r = 0.28–0.38). LVMI was significantly higher in hypertensive than in normotensive cases (p = 0.034). There was no difference in diastolic function between hypertensive and normotensive patients or between patients with and without left ventricular hypertrophy (LVH). In conclusion, our study showed the advantage of ABPM over the casual method of diagnosis of hypertension. LVH is common in transplant patients and is likely associated with arterial hypertension. Hypertension and LVH cannot differentiate transplant patients with diastolic malfunction.

Transcatheter closure of atrial septal defect (ASD) is the most common approach to close the defects worldwide. In this approach, persistent conduction disturbance is extremely rare, but an acute increase in supraventricular ectopy and minimal risk of atrioventricular conduction disturbance, as complete heart block, has been seen. Here, we report a patient who underwent ASD closure with device and presented with persistent complete heart block 10 days after device occlusion due to inflammatory tissue formation just near the atrioventricular node area at the floor of the right atrium without any direct compression on the triangle of Koch.

Heart complications are among the serious problems of patients with β-thalassemia intermedia. This study aimed to evaluate myocardial function in these patients. Clinical parameters and both standard Doppler and pulsed Doppler tissue imaging parameters were compared in 51 β-thalassemia intermedia patients (mean age, 17.05 ± 5.8 years) and 20 normal subjects (mean age, 17.81 ± 7.35 years, p = 0.98). In 11 patients (21.5%), pulmonary artery hypertension was detected. M-mode echocardiographic findings such as ejection fraction and fractional shortening did not show statistically significant changes (p0.005). Pulsed Doppler showed a significant difference in the early (E) to late diastolic (A) velocity ratio of the tricuspid and mitral valve between the patients and the control subjects (p0.05). In the pulsed tissue Doppler study, the peak systolic velocity of the septum (Ss), the peak atrial velocity of the septum (Aas), the peak systolic velocity of the tricuspid annulus (St), the peak early diastolic velocity of the tricuspid annulus (Eat), and the peak late diastolic velocity of the tricuspid annulus (Aat) were increased significantly (p0.05). The pulse tissue Doppler of the lateral mitral annulus did not change significantly (p0.005). The peak systolic velocity of the posterior wall and the peak late diastolic velocity of the anterior wall changed significantly (p0.05). This study showed that β-thalassemia intermedia patients with normal M-mode and two-dimensional echocardiography had statistically significant changes in pulsed Doppler and pulsed tissue Doppler imaging.

Management of patent ductus arteriosus (PDA) in full-term neonates remains controversial. We evaluated the effects of oral ibuprofen on PDA closure in 51 full-term neonates. All neonates were >3-days-old and had a gestational age ≥37 weeks. Patients with ductal-dependent congenital heart disease or severe pulmonary artery hypertension (gradient >40 mmHg) were excluded. Patients were randomly assigned to the treatment group (n = 30) or the control group (n = 21). The treated group received ibuprofen suspension (initially 10 mg/kg, then two 5-mg/kg doses 24 h apart), and control neonates received a placebo. Physicians who treated the patients were blinded to group assignment. There was a statistically significant difference in the closure rate of PDA between the treated (73.3%) and control groups (42.9%, P = 0.029). Among neonates with PDA closure, there was a significant difference in the day of closure between the two groups (25.49 and 17.65% on the days 4 and 14 after diagnosis of PDA in treated neonates versus 1.96 and 15.69% in controls, respectively). This study showed the efficacy of oral ibuprofen in achieving earlier closure of PDA in full-term neonates.

Doppler tissue imaging (DTI) has been proposed as a tool for evaluation of diastolic function. Controversy exists regarding whether DTI measurements are influenced by preload. To determine the influence of preload reduction on DTI, we studied 17 patients (mean age 17 +/- 1.82 years) with end-stage renal disease and without overt heart disease, using DTI before and after hemodialysis (HD). Peak systolic (S), peak early diastolic (Ea), and peak late diastolic (Aa) velocities, and tissue Doppler-derived Tei index (the sum of the isovolumetric contraction and relaxation times divided by the ejection time: TDE-Tei), were determined for each patient from the septum, and lateral mitral and lateral tricuspid annuluses. The Ea of the mitral annulus (12.47 +/- 4.51 cm/s to 10.71 +/- 5.18 cm/s, P = 0.023), isovolumetric contraction time (ICT) of the mitral annulus (85.29 +/- 33.66 ms to 68.76 +/- 17.24 ms, P = 0.031) and Aa of the lateral tricuspid annulus (15.94 +/- 6.46 cm/s to 12.47 +/- 3.85 cm/s, P = 0.044) decreased significantly after HD. With weight loss of more than 1.5 kg, significant changes in the TDE-Tei index of the septum was observed (P = 0.036). From these findings, it is concluded that the Ea of the mitral annulus and the Aa of the tricuspid annulus are decreased by acute preload reduction. The TDE-Tei index is affected by preload reduction of more than 1.5 kg; therefore, it seems necessary to consider the preload status of HD patients when myocardial function is assessed by DTI.

The presence or absence of pulsus paradoxus (PP), defined as an inspiratory decrease greater than 10 mmHg in systolic blood pressure, can have significant diagnostic and therapeutic implications for many clinical conditions including acute asthma, pericardial tamponade, heart failure, hypovolemia, shock states, and the like. However, PP may be difficult to measure in children. Indwelling arterial catheters facilitate the measurement of PP, but this invasive technique generally is reserved for critically ill patients. This study aimed to assess the use of the pulse oximetry plethysmographic waveform (POPW) for the detection of PP in pediatric patients after cardiac surgery. The study enrolled 40 pediatric patients 18 years of age and younger who had invasive blood pressure monitoring with an intraarterial cannula. Systolic pressure variability (SPV) and changes in POPW amplitude (DeltaPOPW%), calculated using five consecutive snapshots from every patient's monitor, were compared using linear regression, Pearson product-moment correlation, the Spearman rank method, and receiver operating characteristic (ROC) curve analysis. A strong correlation existed between respiratory SPV and DeltaPOPW% for the detection of PP (r = 0.682; p < 0.0001). A respiratory variation in DeltaPOPW% exceeding 25.44% (about one-fourth the amplitude of the tallest POP waveform) allowed detection of PP with a sensitivity of 86.7% and a specificity of 88%. Pulse oximetry is a readily available and easily performed noninvasive means for detecting PP in children.

It is shown that phosphodiesterase type 5 (PDE5) inhibitors such as sildenafil can modulate pulmonary arterial hypertension (PAH) via increasing the level of guanosine-3,5-cyclic monophosphate (cGMP) and decreases pulmonary artery pressure (PAP). In this study we determined the effectiveness of sildenafil and compared its efficacy with inhaled nasal oxygen (O2) during cardiac catheterization in patients with congenital heart diseases (CHD) and PAH, as a test of feasibility for surgical repair of the patients. We studied 15 patients, 9 male and 6 female, with a mean age of 8.3 years. Hemodynamic measurements were made at baseline, after O2 administration for 20 min (5 L/min by mask), and then 45 min after administration of a single dose of sildenafil (0.5 mg/kg orally or via nasogastric tube). Mean PAP at baseline was 72.2 +/- 12.54 mm Hg and was reduced by sildenafil to 52.5 +/- 9.6 and by O2 to 61.3 +/- 10.39. Both sildenafil and O2 decreased PAP effectively (p = 0.08 and p = 0.04, respectively). Pulmonary vascular resistance (PVR) was calculated for 12 patients, with a baseline level of 9.08 +/- 1.09 mm Hg . L(-1) . min, which was significantly decreased by O2, to 3.74 +/- 0.43, and by sildenafil, to 5.93 +/- 0.75 (p = 0.005 and p = 0.05, respectively). Sildenafil, as a single oral dose, can effectively reduce PAP and PVR. This novel PDE5 inhibitor can be used for assessment of feasibility of operation for patients with CHD and PAH when inhaled NO is not available.

Central venous pressure (CVP) measurement is a reliable method for evaluating intravascular volume status and cardiac function, but it is an invasive method that results in some complications such as arterial puncture, pneumothorax, and development of infection. The current study was performed to compare CVP measurements between central and peripheral catheters in infants and children with congenital heart disease referred for right-sided heart catheterization. The CVP and peripheral venous pressure (PVP) in 45 patients were measured simultaneously. The mean difference between CVPs measured from the central and peripheral catheters was 8 +/- 4 cm H(2)O. The linear regression equation showed that CVP = 0.32 PVP + 3.8 (r = 0.67; p0.005). There was no difference in CVP measurements depending on the intravenous cannula and chest diameters, arm diameter, arm length, body surface area, patient's age (or =10 years and10 years), and type of congenital heart disease (cyanotic or noncyanotic). In conclusion, although CVP measured from a peripheral intravenous catheter in infants and children with congenital heart disease is not as accurate as the measurement in adults, the aforementioned linear regression equation based on measurement of PVP gives a reliable estimate of CVP.

The aim of this study was aim to evaluate cardiac function by means of echocardiography in patients with sickle cell anemia.This was a case control study that evaluated a total of 44 patients with sickle cell anemia, who were on regular follow up, and 44 age/sex-matched normal healthy control subjects. M-mode, two-dimensional, Doppler and pulse tissue-Doppler echocardiographic measurements were performed in both groups.The mean age was 14.06 ± 6.4 years (55% female, 45% male). The mean hemoglobin, hemoglobin F, and serum ferritin levels were 9.4 ± 1.20 g/dL, 22.7 ± 12.9 g/dL, and 391 ± 590 ng/dL respectively. Pulsed Doppler echocardiography showed that the early diastolic trans-tricuspid peak flow velocity was greater in the patients than in the control subjects. Assessment of the lateral mitral and tricuspid annulus peak velocities by pulsed tissue Doppler imaging showed that the patients had significantly greater systolic, and early and late diastolic velocities than the controls. The left ventricular diameter, interventricular septum diameter, and posterior wall diameter were statistically significantly greater in the patients compared with the control group, whereas there was no difference in ejection fraction.Sickle cell anemia in children results in a volume-overloaded heart with a significant increase in left ventricular dimensions. However, left ventricular systolic dysfunction and pulmonary hypertension were not frequent findings in our patients.

The aim of this study is to compare the efficacy and possible adverse effects of the oral high-dose ibuprofen regimen to that of standard regimen in closing patent ductus arteriosus (PDA).This clinical trial study was performed from April 2012 to May 2013 on preterm infants with gestational age37 weeks and postnatal age 3 to 7 days with echocardiographic diagnosis of hemodynamically significant PDA. These neonates were randomly assigned to two treatment groups that respectively received high dose (20-10-10 mg/kg/d) and standard dose (10-5-5 mg/kg/d) oral ibuprofen regimen for 3 days. Effect of ibuprofen therapy was evaluated by echocardiography and neonates were followed for renal dysfunction, gastrointestinal complication, bleeding, and hyperbilirubinemia.From a total of 60 enrolled infants, 30 cases received the high dose of ibuprofen and the remaining 30 received the standard dose. Complete ductal closure was observed in 20 (70%) infants treated with high-dose regimen in comparison with 11 (36.7%) in the standard-dose regimen group (p=0.010). No gastrointestinal, renal, or hematological adverse effects were reported.The high-dose oral ibuprofen seems to be more effective than the current standard dose regimen for PDA closure in premature neonates without increasing the adverse effects.

Endothelin-1 is a potent endogenous vasoconstrictor and an important remodeling factor in the pathogenesis of pulmonary arterial hypertension (PAH). Bosentan, a nonselective and active dual endothelin receptor antagonist, is used as a vasodilator in treatment of such patients. This study aimed to evaluate acute response to a single oral dose of bosentan as a vasodilator agent in comparison with nasal oxygen (O2) in patients with PAH related to congenital heart disease (CHD).We enrolled 20 patients with PAH-CHD, with a mean age of 5.45 ± 4.5 years. Hemodynamic variables were measured at baseline state, after administration of nasal O2 (5 L/min) for 20 minutes, and then when hemodynamic variables returned to the baseline, the measurements were repeated for the third time 3 hours after administration of a single oral dose of bosentan (2 mg/kg).Mean pulmonary vascular resistance was 9.92 ± 2.97 Wood units · m(2) at baseline and was lowered by O2 to 6.17 ± 2.71 Wood units · m(2) (P = .001) and by bosentan to 5.90 ± 2.69 Wood units · m(2) (P = .0001). Mean pulmonary artery pressure was 71.2 ± 15.4 mm Hg at baseline and was reduced to 62.6 ± 15.2 mm Hg (P = .001) by O2 and to 61.6 ± 14.8 mm Hg (P = .0003) by bosentan.A single oral dose of bosentan has the same acute vasodilatory effect on the pulmonary vascular bed as nasal O2 in patients with PAH related to CHD. Such patients may benefit from long-term therapy with this novel medication.

Pediatric kidney transplant recipients are at increased risk of CVD. Exercise test is a good method to evaluate exercise capacity, cardiorespiratory fitness, and risk of potential CVDs. The aim of this study was to assess the exercise capacity in this population and determine its relationship with their cardiac function using conventional and tissue Doppler echocardiography. Exercise test, conventional and tissue Doppler echocardiography were performed on 44 kidney transplant children (age ranging 11-20, 59% male) with acceptable renal function, and the results were compared with their normal healthy counterparts. Our transplant patients achieved significantly lower maximal heart rate, maximal heart rate ratio, total energy expenditure during the exercise, and maximal O2 consumption (Max VO2 ) than the normal group (p < 0.05). No correlation was found between hemoglobin (Hb) level, dialysis duration, kidney function, and the exercise test parameters. Kidney transplant patients had preserved systolic despite diminished diastolic cardiac function compared to the normal children. Our pediatric renal transplant recipients had severely impaired diastolic dysfunction and significantly reduced MaxVO2 compared with their healthy counterparts. No correlation was found between MaxVO2 and measured indices of systolic and diastolic cardiac function.

Pulmonary vascular resistance (PVR) is a critical and essential parameter during the assessment and selection of modality of treatment in patients with congenital heart disease (CHD) accompanied by pulmonary arterial hypertension (PAH). Cardiac catheterization is the "gold standard" but is an invasive method for PVR measurement. A noninvasive and reliable method for estimation of PVR in children has been a major challenge and most desirable during past decades, especially for those who need repeated measurements. In a prospective study and among consecutive patients who were referred for cardiac catheterizations, PVR was calculated as the ratio of the transpulmonary pressure gradient (∆P) to the amount of the pulmonary flow (QP) accordingly for 20 patients with CHD and high PAH. Subsequently and noninvasively, PVR was assessed for these patients by a Doppler echocardiography-derived index defined as the ratio of the tricuspid regurgitation velocity (TRV(m/s)) to the velocity time integral (VTI(cm)) of the right-ventricular outflow tract (RVOT). There was a good correlation between PVR measured at catheterization (PVR(cath)) and TRV/VTI(m) ratio; the mean of three measurements of VTI (VTI(m)) with R (2) = 0.53 (p = 0.008). In addition, a TRV/VTI(m) value of 0.2 provided a sensitivity of 71.4% and a specificity of 100% for PVR6 Woods units (WU) as well as sensitivity of 90% and specificity of 90% for a PVR equal to 8 WU. PVR value between 6 and 8 WU by catheterization has been considered as a cut-off point for intervention in children with left-to-right shunts and PAH. In conclusion, Doppler-derived TRV/VTI(m) ratio is a reliable index that may be helpful as a supplementary diagnostic tool for the selection of modality of treatment and follow-up of patients with PAH and increased PVR.

ObjectiveTo investigate whether accurate estimation of the diameter of the patent ductus arteriosus can be obtained by colour Doppler echocardiography.MethodsThe minimum and maximum diameters of the patent ductus arteriosus were measured by colour Doppler echocardiography and compared with its angiographic size.ResultsWe studied 40 patients, aged between 4 months and 18 years. The measured pulmonary side diameter in echocardiography was 33.6% larger than that in angiography, and the aortic side was 7.8% smaller. We found a correlation between the measurements of the minimum and maximum size of the patent ductus arteriosus (r = 48.8 and 52.6) by colour Doppler echocardiography and angiographic findings, respectively.ConclusionColour Doppler echocardiography significantly overestimates the minimum size of the patent ductus arteriosus; therefore, reconsideration of the respective size is suggested.

The patient is a 17-year-old boy who was referred to our center with complaints of easy fatigability and exertional dyspnea. In echocardiography, the left ventricle was identified to be composed of a small functional cavity, and 2 sequestrated apical cavities that communicated with the functional cavity only through few stenotic orifices. The mitral valve apparatus was also abnormal: there was no definite papillary muscle, and chordae tendineae arose from the aberrant fibromuscular ridge that partitioned the left ventricular cavity. Imaging findings and management of this patient with a triple-cavity left ventricle are discussed.

Myocarditis is a well-recognized component of Kawasaki disease, with left ventricular dysfunction occurring in more than half of the patients during the acute phase of the disease. The purpose of our study was to evaluate myocardial function in these patients using the myocardial performance index, also known as the Tei index. In a prospective study, 14 patients underwent echocardiographic evaluation, first at the time of diagnosis of the disease, in its acute phase before treatment with intravenous immunoglobulin and then 2 weeks later after treatment with immunoglobulin. We assessed the Tei-index, the ejection fraction, shortening fraction, and the presence of valvar regurgitation, pericardial effusion, or coronary arterial involvement. As a control, we also assessed 22 healthy children, matched for age and sex with the study population. Of the patients, half had an increased left ventricular Tei-index in the acute phase, as compared with the data obtained after treatment, the index changing from 0.43 ± 0.08 to 0.35 ± 0.06 (p equal to 0.003). An increased index for the right ventricle was found in 5 patients (36%), values being 0.30 ± 0.05 as opposed to 0.26 ± 0.04 (p equal to 0.009). Of the patients, 5 (35.7%) also had decreased ejection fractions and proportional shortening fractions during the acute phase, confirming left ventricular dysfunction. We concluded that the Tei-index, which measures combined systolic and diastolic function, is a simple, sensitive, and accurate tool for estimating global myocardial dysfunction in patients with Kawasaki disease.

Despite increasing reports on nonionic contrast media-induced nephropathy (CIN) in hospitalized adult patients during cardiac procedures, the studies in pediatrics are limited, with even less focus on possible predisposing factors and preventive measures for patients undergoing cardiac angiography. This prospective study determined the incidence of CIN for two nonionic contrast media (CM), iopromide and iohexol, among 80 patients younger than 18 years and compared the rates for this complication in relation to the type and dosage of CM and the presence of cyanosis. The 80 patients in the study consecutively received either iopromide (group A, n = 40) or iohexol (group B, n = 40). Serum sodium (Na), potassium (K), and creatinine (Cr) were measured 24 h before angiography as baseline values, then measured again at 12-, 24-, and 48-h intervals after CM use. Urine samples for Na and Cr also were checked at the same intervals. Risk of renal failure, Injury to the kidney, Failure of kidney function, Loss of kidney function, and End-stage renal damage (RIFLE criteria) were used to define CIN and its incidence in the study population. Accordingly, among the 15 CIN patients (18.75%), 7.5% of the patients in group A had increased risk and 3.75% had renal injury, whereas 5% of group B had increased risk and 2.5% had renal injury. Whereas 33.3% of the patients with CIN were among those who received the proper dosage of CM, the percentage increased to 66.6% among those who received larger doses, with a significant difference in the incidence of CIN related to the different dosages of CM (p = 0.014). Among the 15 patients with CIN, 6 had cyanotic congenital heart diseases, but the incidence did not differ significantly from that for the noncyanotic patients (p = 0.243). Although clinically silent, CIN is not rare in pediatrics. The incidence depends on dosage but not on the type of consumed nonionic CM, nor on the presence of cyanosis, and although CIN usually is reversible, more concern is needed for the prevention of such a complication in children.

l-Carnitine and magnesium have antioxidant properties. They have the potential to stimulate production of fetal hemoglobin and stabilize the RBC membrane, respectively. Several studies have also shown the beneficial effects of hydroxyurea in thalassemic patients. We assessed the effect of combination therapy of hydroxyurea with l-carnitine and magnesium chloride on hematologic parameters and cardiac function of patients with beta-thalassemia intermedia.One-hundred-and-twenty patients with thalassemia intermedia (range, 4-35 yr; mean, 19 +/- 6.4 yr) who had no need for blood transfusion or requirement for blood transfusion with an interval of6 months were randomly selected. All patients had been on hydroxyurea for6 months. They were randomly divided into four groups: group A (hydroxyurea alone); group B (hydroxyurea and l-carnitine); group C (hydroxyurea and magnesium chloride); and group D (hydroxyurea, l-carnitine and magnesium chloride).In groups B, C, and D, mean Hb and hematocrit increased during 6-month treatment (P0.001). Echocardiographic studies revealed a significant decrease in left ventricular end-diastolic diameter in group B (P = 0.032), increase in pulmonary acceleration time in group C (P = 0.012), and increase in left ventricular ejection fraction in groups C and D (P0.000 and 0.006, respectively).Combination of hydroxyurea with l-carnitine or magnesium could be more effective in improving hematologic parameters and cardiac status in patients with beta-thalassemia intermedia than hydroxyurea alone.

Myocarditis is a well-recognized component of Kawasaki disease, with left ventricular dysfunction occurring in more than half of patients during the acute phase. The purpose of this study was to evaluate myocardial function in patients with Kawasaki disease using pulsed tissue Doppler imaging (TDI). Twenty-five patients with the diagnosis of acute Kawasaki disease were enrolled in the study. All patients underwent echocardiographic studies at the time of diagnosis of the disease, in its acute phase, prior to treatment, and then 4 weeks later. For an aged-matched control group with fever and no cardiac disease, the same echocardiographic evaluations were performed. Peak velocities of systolic (Sa), early diastolic (Ea), and late diastolic (Aa) motion of the annulus were obtained at the lateral and septal sides in apical four-chamber view, and TDI-derived myocardial performance index (TDI-MPI) was also calculated. Peak Ea velocity of lateral mitral annulus was decreased significantly during the acute phase of illness (14 +/- 4.40 vs. 17.67 +/- 4.41; P = 0.028). In seven patients with carditis, changes in Ea-to-Aa ratio of septum (1.28 +/- 0.278 vs. 1.78 +/- 0.49; P = 0.018) and lateral mitral annulus (1.23 +/- 0.496 vs. 2.11 +/- 0.822; P = 0.014) were statistically significant but TDI-MPI showed no statistically significant changes. This study showed that peak mitral annular Ea velocities obtained by TDI were significantly altered in the acute phase of Kawasaki disease. TDI- MPI does not add an incremental benefit to other indexes of myocardial performance for comprehensive myocardial function in the acute phase of Kawasaki disease.

This study was conducted to determine the epidemiology, clinical manifestations, cardiac involvements and laboratory findings of Kawasaki disease (KD) in Fars Province, Iran from January 1991 to December 2002. One hundred and thirteen patients with KD were confirmed. This syndrome occurred mostly in males (ratio=2.1:1). The most commonly affected age group was one to five years old. There was no secondary case in families, none of the patients were related and recurrence of disease was seen in only one patient. Prolonged fever and thrombocytosis were significant risk factors for developing coronary artery disease (p0.05, respectively). Among the 10 patients with cardiac involvement, seven patients had dilated coronary arteries (4 mm) and three patients had aneurysm of coronary artery in their first echocardiography. It has been suggested that a high index of clinical suspicion of KD could improve diagnosis and implementation of preventive treatment.