The aim of this study was to evaluate the left ventricular systolic and diastolic function before and after transcatheter percutaneous patent ductus arteriosus (PDA) closure. 21 children (age >6 months old) diagnosed with hemodynamically significant PDA underwent percutaneous PDA closure. Conventional, Doppler and tissue Doppler imaging and speckled-derived strain rate echocardiography were done at pre-closure, 1 day (early) and 1 month (late) post-closure. Mean age of the patients (female/male: 1.3) was 17.54 ± 24.7 months with the mean PDA diameter of 3.6 ± 0.8 mm. Systolic measures (ejection fraction, shortening fraction) reduced significantly early after PDA closure (P < 0.05). After 1 month, both improved significantly; ultimately, after 1 month no change was observed in systolic function measures compared with the pre-closure status. Early and late diastolic flow velocities of mitral (E M and A M) reduced considerably in early and late post-closure time (P < 0.05). Both early tissue Doppler early velocity of lateral mitral annulus (E'M) and early to late velocity ratio (E'M/A'M) of lateral mitral annulus decreased significantly (P = 0.02) in early post-closure. After 1 month, E'M increased considerably. (P = 0.01) but E'M/A'M had an insignificant rise (P > 0.05). E M/E'M ratio did not change in early post-closure but it had a considerable reduction in the subsequent month compared with the pre- and early post-closure (P < 0.001 for both occasions). Global and segmental longitudinal strain measures reduced significantly early after PDA closure (P < 0.05) but it improved remarkably in the subsequent month. Transcatheter PDA closure causes a significant decrease in left ventricular performance early after PDA closure which recovers completely within 1 month. Also PDA size can affect post-closure left ventricular function.

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.

OBJECTIVES The prevention of pulmonary insufficiency (PI) is a crucial part of the tetralogy of Fallot repair. Many techniques have been introduced to construct valves from different materials for the right ventricular outflow tract, including the most commonly constructed monocusp valves. We are introducing a new bicuspid valve made intraoperatively using the autologous right atrial appendage (RAA) to prevent PI in these patients. METHODS The RAA valve was constructed and used in 21 patients with tetralogy of Fallot. The effective preservation of the native valve was impossible in all patients because of either a severe valve deformity or a small annulus. The RAA valve was created after ventricular septal defect closure and right ventricular outflow tract myectomy and was covered with a bovine transannular pericardial patch. The perioperative data were evaluated, and the echocardiography results were assessed immediately after operations and in follow-up with a median of 10.5 months. The data were retrospectively compared with 10 other patients with similar demographic data but with only transannular patches. RESULTS The mean age of the patients was 13.3 months. No mortality or related morbidity occurred after repair using the RAA valve. The PI severity early after the operation was trivial or no PI in 18 patients, and mild PI was observed in 3 patients, which progressed to moderate PI in one of them in the mean 12-month follow-up period. Fifteen patients had mild or no pulmonary stenosis, while moderate pulmonary stenosis was observed in 6 others. Compared with the other 10 patients with only transannular patches, the RAA valve patients had prolonged operative and clamping times, but no difference in postoperative course and shorter hospital stays. The degree of PI was, of course, significantly less in the RAA valve patients, but pulmonary stenosis was the same. CONCLUSIONS The RAA valve construction is a safe and effective technique to prevent PI after the tetralogy of Fallot repair, at least in terms of short- and mid-term results. A longer follow-up period is needed to confirm if this new valve can eliminate or significantly delay the need for pulmonary valve replacement in these patients.

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: Ordinary pressure dressing for hemostasis after cardiac catheterization is time consuming and might cause some problems, such as pain or loss of blood, which has to be controlled, particularly in pediatric patient. Using Celox® (chitosan) powder dressing might cause quicker initial hemostasis. Methods: In this prospective study, we assessed Celox® powder among patients in hospitals affiliated with Shiraz University of Medical Sciences, from November 2017 to February 2018. The patients were stratified in two groups. The case group included patients for whom Celox® powder was used along with sterile gauze pressure at the puncture site to achieve hemostasis, and the control group those in whom hemostasis was achieved by standard sterile gauze pressure method. Results: Sixty patients under 16 years of age with congenital heart diseases were evaluated and underwent cardiac catheterization. We stratified the patients in two groups called case (30 patients) and control group (30 patients). Considering both arterial and venous initial hemostasis, in the case group, the minimum and maximum, median and mean coagulation time were less than those in the control group; however, the initial hemostasis was statistically significant only in venipuncture site. Also, the venous coagulation time was shorter among the patients weighing less than 10 kilograms in comparison to those with higher weight. In the case group, using Celox® stirred hemostasis toward the lower percentiles, but based on 50th percentile, the distribution in each group was identical. Conclusions: Celox® powder dressing in children led to reduced coagulation time in venipuncture site, and we might recommend utilizing this type of dressing for venous hemostasis in children after venipuncture.

Background and Aims: We reinvestigated the causes, symptoms, and management of childhood pericardial effusion (PE) and its gradual changes during recent years in a referral pediatric cardiology center in the south of Iran. Methods:We retrospectively analyzed the profile of PE patients who were under 18 years old from 2015 to 2020. The patient's demographic, clinical, and paraclinical information was extracted and analyzed using SPSS software.Result: In general, 150 out of 63,736 admitted patients (0.23% of the total pediatric admissions) were diagnosed with PE (male/female 1:1.17). The median age was 3.25 years (range:\ 2 days to 18 years; interquartile range: 9.5), and 50% of them were under 3 years of age. 32.6% had moderate to severe PE. Most patients presented with acute symptoms (68%) and respiratory problems, as the most common symptoms (30.6%). Tamponade signs were presented in 2% (n = 3) of the patients, and 80.7% (n = 121) were in a stable hemodynamic condition. In total, renal failure (22%) and parapneumonic effusion were the leading etiologies. Viral (7%) and bacterial (5%) pericarditis were the seventh and eighth causes; however, in severe cases, renal failure (22%) and bacterial pericarditis (14%) were dominant. In total, 14.1% (n = 21) of the patients needed pericardiocentesis that increased to 78.3% (n = 18) in severe cases. Only 6% had persistent PE for more than 3 months. Conclusion:Childhood PE is mostly a result of renal failure and noninfectious causes.True pericarditis cases are not common, except in severe cases. It is more common in less than 3-year-old patients, and chronicity is rare. Severe cases had a high chance of pericardiocentesis, but other cases were mainly managed by treatment of the underlying causes.

Background:Patent ductus arteriosus (PDA) is an important risk for heart failure due to left to right shunt in term neonates.Objectives:In this study, we evaluated the effect of high dose ibuprofen in closure of PDA in term neonates.Patients and Methods:We used double dose ibuprofen (20 mg/kg, 10 mg/kg, and 10 mg/kg) for 3 - 30 day old term neonates with PDA who were admitted in the neonatal wards of Shiraz University of Medical Sciences. The results of this study were compared to the data of the previous study in our center which used the low dose of ibuprofen (10 mg/kg, 5 mg/kg, and 5 mg/kg).Results:29 full term neonates received high-dose ibuprofen, in 18 neonates, PDA was closed after 4 days (62.1% versus 43.3% for the standard dose and 4.7% for the control group in the previous study) (P = 0.001). The results showed no significant correlation between the closure rate and gestational age, postnatal age, sex, and weight. In the 4th day of treatment, size of the pulmonic end of ductus arteriosus decreased from 2.09 mm to 0.77 mm compared to 1.68 mm to 0.81 mm in the standard dose of oral ibuprofen and 2.1 mm to 1.4 mm in the control group (P = 0.046).Conclusions:This study indicated that high-dose oral ibuprofen was more effective in closing or decreasing the size of PDA.

Introduction: The absence of a pulmonary artery is a rare congenital anomaly that occurs on its own or with some congenital cardiac disorders, particularly tetralogy of Fallot (TOF), while the hidden pulmonary artery might originate from a closed ductus arteriosus (DA) that can be stented to reach the artery.Material and methods: This prospective study describes cardiac catheterization of our nine TOF patients who had the absence of the left pulmonary artery before the operation. The patients were stratified in three groups: group one, whose closed DA were found and stented successfully to the hidden pulmonary artery; 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 the other surgically-corrected TOF patients who were operated with the absent left pulmonary artery.Results: The first group included patients aged 1, 24, and 30 months, whose CT angiography 6-9 months after stenting showed acceptable left pulmonary artery diameter for surgically correction, while the pulmonary vein angiography of the group two patients showed a hidden left pulmonary artery with a suitable diameter for surgical correction.However, we were unable to find or stent the DA of the group three patients, aged 12, 38, 60, and 63 months. Moreover, evaluation of the other six previously corrected patients who were operated with a right pulmonary artery revealed pulmonary artery hypertension of the entire 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.