Long-term outcomes of the classic Konno-Rastan procedure in paediatric and adult patients: impact of aortic annulus size on patient outcomes.

OBJECTIVES: The classic Konno-Rastan procedure may yield different outcomes regarding aortic annulus diameters ≤15 mm and larger. Focusing on the effect of the diameter of the aortic annulus, we described the long-term outcomes of our patients. METHODS: The outcomes of paediatric and adult patients who underwent surgery from 2000 to 2021 were studied retrospectively. The patient population was divided into 2 groups with aortic annulus diameters ≤15 mm and >15, and the outcomes were compared between the 2 groups. RESULTS: A total of 48 patients, with a mean age of 12.24 ± 9.42 years (2-53 years) and a median follow-up duration of 8 years (7 months to 20 years) with an IQR of 5.5, were enrolled. The mean peak instantaneous pressure gradient was 78.97 ± 25.29 mmHg, which decreased to 21.43 mmHg (P-value = 0.012). The maximum left ventricular outflow tract gradient at the last follow-up was 28.21 mmHg, with the exception of 1 case with a gradient of 68.45 mmHg. The mean diameter of the aortic annulus was 15.34 ± 3.87 mm (8-23 mm), and the mean prosthetic valve size was 20.31 mm, which was 5 mm (33%) larger than the native annulus diameter. The overall mortality rate was 6.3%, with 1 death in the hospital and 2 in the first year after the surgery. The major complication rate, including mortality, heart block and reintervention, was higher in patients with ≤15 mm annulus (P-value = 0.028.) However, there was no difference between the 2 groups in follow-up. Four (8%) late cardiac reoperations were performed, none of which were related to our surgeries. CONCLUSIONS: Kono-Rastan surgery for patients with aortic valve diameter of ≤15 mm can be performed with acceptable long-term outcomes.

Minimally invasive versus sternotomy approach for double-patch repair of partial anomalous pulmonary venous connection and sinus venosus defect in pediatric and adult patients: Mid to long-term outcomes.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Effect of coarctation of aorta anatomy and balloon profile on the outcome of balloon angioplasty in infantile coarctation.

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 < 0.001). A high-pressure balloon was used in 27, and a low-pressure balloon was used in 15 patients. COA's pressure gradient changed 30.89 ± 18.06 in the high-pressure group and 24.53 ± 20.79 in the low-pressure balloon group (P = 0.282). In the high-pressure balloon group, 14.81% and in the low-pressure group, 33.33% had recoarctation and need second balloon angioplasty (p < 0.021). The infant with discrete coarctation had a higher decrease in gradient and lower recoarctation. 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.

Age-adjusted Reference Values and Z-scores of the Coronary Sinus Diameter among Healthy Children and Adolescents in Southern Iran.

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<0.001). CONCLUSION: Coronary sinus diameter significantly correlates with the age, height, and body surface area of the cases. These features are useful in diagnosing some congenital heart diseases and insertion of suitable devices through it.

Midterm follow up of transcatheter closure of coronary artery fistula with Nit-Occlud® patent ductus arteriosus coil.

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.

Hidden pulmonary arteries in tetralogy of Fallot and pulmonary artery pressure in patients operated with a pulmonary artery.

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.

Surgical repair of tetralogy of Fallot using autologous right atrial appendages: short- to mid-term results.

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.

Japanese Kawasaki Disease Scoring Systems: Are they Applicable to the Iranian Population?

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Management of Persistent Pulmonary Hypertension After Correction of Congenital Heart Defect with Autologous Marrow-Derived Mononuclear Stem Cell Injection into the Pulmonary Artery: A Pilot Study.

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.

Hemolysis and its outcome following percutaneous closure of cardiac defects among children and adolescents: a prospective study.

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.

Follow-Up Results of Device Occlusion of Patent Ductus Arteriosus.

BACKGROUND: Transcatheter patent ductus arteriosus (PDA) closure is an established procedure. OBJECTIVES: The aim of the study was to assess midterm follow up of the Nit-Occlud coil and the amplatzer ductal occluder (ADO) closure of PDA. PATIENTS AND METHODS: In this cohort study, we collected the longitudinal data of patients who underwent percutaneous closure using coil or ADO from November 2005 to November 2013. A total of 404 patients with PDA closure by devices were included during the study period. Coil occlusion was performed in 220 patients and 184 patients underwent catheterization using ADO. Follow-up evaluations were performed with echocardiography at two weeks, two months, six months, and during the study period (in average 4.8 ± 3.8 years). RESULTS: The patients' mean age was 24 months (range: 1 - 312). The catheterization was successful in 393 (97.2%) patients and unsuccessful in 11 (2.7%). Immediate complete occlusion was seen in 290 (73.7 %) patients. The occlusion rates at two weeks, two months, six months, and during the study period were 73.7%, 84%, 93.6%, 98.7%, and 99.5%, respectively. Complications occurred in 23 (5.8%) patients during or immediately after the catheterization, and device embolization with 2.7% was the most common complication. Most complications occurred in a patient with pulmonary hypertension who was less than one year old and was undergoing the first year of experience with devices. CONCLUSIONS: Our findings showed that transcatheter occlusion of the PDA is an effective and safe intervention by coil or Amplatzer with excellent early and one-year outcomes. Pulmonary hypertension, age of less than 12 months and experience of less than one year may increase the complications of device closure.

Persistent Truncus Arteriosus With Intact Ventricular Septum: Clinical, Hemodynamic and Short-term Surgical Outcome.

INTRODUCTION: Truncus arteriosus with intact ventricular septum is a rare and unique variant of persistent truncus arteriosus (PTA) which usually presents with central cyanosis and congestive heart failure in neonate and early infancy. Associated cardiac and non-cardiac anomalies may affect morbidity and mortality of these patients. CASE PRESENTATION: We describe clinical presentation, echocardiography and angiographic features of a 7-month old boy with PTA and intact ventricular septum who underwent surgical repair of the anomaly at our institution. Operative findings, surgical procedure and short-term outcome are reported. CONCLUSIONS: While our patient had systemic pulmonary arterial pressure at the time of complete surgical repair, it was improved after surgery.

Evaluation of left ventricular function by tissue Doppler and speckle-derived strain rate echocardiography after percutaneous ductus closure.

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.

Acute hemodynamic effects of single oral dose of bosentan in patients with pulmonary arterial hypertension related to congenital heart disease.

BACKGROUND: 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). MATERIALS AND METHODS: 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). RESULTS: 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. CONCLUSION: 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.

Evaluation of electrocardiographic changes after arterial switch operation.

BACKGROUND: Transposition of Great Arteries (TGA) is a serious congenital heart disease which can be accompanied by good outcomes with anatomic correction within the first few weeks of life. OBJECTIVES: The present study aimed to evaluate electrocardiographic changes in the children with uncomplicated Arterial Switch Operation (ASO). PATIENTS AND METHODS: Twelve lead electrocardiograms were obtained from thirty-three patients with TGA after ASO. Then, the patients' QT-dispersion and P-wave dispersion were compared to those of 33 age- and gender-matched individuals as the normal control group. Both groups were also evaluated by M-mode echocardiography. Student's t-test and Pearson correlation were used to analyze the data. Besides, P < 0.05 was considered as statistically significant. RESULTS: The mean age of the patients and the control group was 41 ± 3.7 and 40.12 ± 4.2 months, respectively. Comparison of P wave, T wave, QRS complex, PR interval, QT segment, and corrected QT segment showed significant differences in the frequency of inverted T wave in pericardial leads [V3, V4, V5, and V6] (P = 0.004; more in patients), P wave amplitude in lead II (P < 0.001; more in patients), R wave amplitude in V1 (P = 0.016; smaller in patients), R and S waves amplitude in V6 (P = 0.004 and P = 0.001; taller in patients), corrected QT segment (in lead V5; P < 0.0001: prolonger in patients), and PR interval (P = 0.001; prolonger in patients). However, no significant differences were found regarding the vector axis and heart rate. Right bundle branch block (18% vs. 0%), Bifascicular (3% vs. 0%), and first-degree blocks (15% vs. 0%) were significantly more in the patients. Besides, the PR interval was longer in the corrected complex TGA (146 ± 24.4 vs. 127.7 ± 23.1, P = 0.001). Moreover, R/S ratio in lead V1 was significantly prolonger, among the patients (2.86 ± 3.35 vs. 0.706 ± 0.53, P = 0.002). Nonetheless, no significant was observed between the patients and controls concerning the mean of QT dispersion. On the other hand, the two groups were significantly difference in terms of P wave dispersion (25.7 ± 13.8 vs. 33.74 ± 12.9, P = 0.024). CONCLUSIONS: In this study, first-degree block and right bundle branch were detected in the operated patients with TGA. Increased P dispersion in these patients may increase the risk of atrial arrhythmia. Thus, long-term follow-up is necessary in these patients.

Distribution and Protective Role of HLAB40 in Iranian Patients with Kawasaki Disease; a Report from Southern Iran.

OBJECTIVE: 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. METHODS: 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. Findings : 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 CONCLUSION: 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.

Cardiovascular involvement in children with osteogenesis imperfecta.

OBJECTIVE: Osteogenesis imperfecta is a hereditary disease resulting from mutation in type I procollagen genes. One of the extra skeletal manifestations of this disease is cardiac involvement. The prevalence of cardiac involvement is still unknown in the children with osteogenesis imperfecta. The present study aimed to investigate the prevalence of cardiovascular abnormalities in these patients. METHODS: 24 children with osteogenesis imperfecta and 24 normal children who were matched with the patients regarding sex and age were studied. In both groups, standard echocardiography was performed, and heart valves were investigated. Dimensions of left ventricle, aorta annulus, sinotubular junction, ascending and descending aorta were measured and compared between the two groups. FINDINGS: The results revealed no significant difference between the two groups regarding age, sex, ejection fraction, shortening fraction, mean of aorta annulus, sinotubular junction, ascending and descending aorta, but after correction based on the body surface area, dimensions of aorta annulus, sinotubular junction, ascending and descending aorta in the patients were significantly higher than those in the control group (P<0.05). Two (8.3%) patients had aortic insufficiency and five (20%) patients had tricuspid regurgitation, three of whom had gradient >25 mmHg and one patient had pulmonary insufficiency with indirect evidence of pulmonary hypertension. According to Z scores of aorta annulus, sinotubular junction and ascending aorta, 5, 3, and 1 out of 24 patients had Z scores >2 respectively. CONCLUSION: The prevalence of valvular heart diseases and aortic root dilation was higher in children with osteogenesis imperfecta. In conclusion, cardiovascular investigation is recommended in these children.

Effects of omega-3 polyunsaturated Fatty acids on heart function and oxidative stress biomarkers in pediatric patients with dilated cardiomyopathy.

OBJECTIVES: Dilated cardiomyopathy is the most prevalent type of cardiomyopathy in children, which results in congestive heart failure and causes significant morbidity and mortality. This study, aims to investigate the effect of supplementation with omega-3 polyunsaturated fatty acids (n-3 PUFA) on heart function and oxidative stress biomarkers in these patients. METHODS: The present research was a case-control study on pediatric patients with dilated cardiomyopathy, who received n-3 PUFA and anti-failure therapy for 6 months (group 1, n = 6), or anti-failure therapy alone for 6 months (group 2, n = 6), as well as age matched normal individuals (group 3, n = 6), and evaluated the cardiac function and biomarkers of oxidative stress. RESULTS: Echocardiographic parameters, such as left ventricular ejection fraction, shortening fraction, tissue Doppler Ea and Aa waves of lateral annulus of tricuspid valve, and Ea and S wave of septum, were significantly improved in group 1 after n-3 PUFA compared to pre- treatment status, while they were not changed after treatment in group 2. Antioxidant enzymes, including catalase and glutathione peroxidase activities in erythrocytes were slightly decreased, while plasma 8-iso-prostaglandin F2α concentrations were somewhat increased in group 1 compared to groups 2 and 3, however these changes were not statistically significant. Total antioxidant capacity of plasma was similar in all 3 groups. CONCLUSIONS: The results indicate that some echocardiographic parameters were significantly improved in patients receiving omega-3 fish oil. However, omega-3 had no significant effect on oxidative stress biomarkers.

Evaluation of cardiac and valvular function after arterial switch operation: a midterm follow-up.

OBJECTIVES: Transposition of Great Arteries (TGA) is a serious congenital heart disease and anatomic correction in the first few weeks of life has revealed good outcomes nowadays. In this study, we aimed to evaluate the myocardial and valvular function at midterm postoperative follow-up. PATIENTS AND METHODS: In this study, thirty-three patients with TGA and Arterial Switch Operation (ASO) were evaluated by 2-dimensional, M-mode, Doppler, and pulsed Tissue Doppler. These patients were compared with 33 healthy children of the same age and gender as the normal control group. Student's t-test and Pearson correlation were used to analyze the data. Besides, P<0.05 was considered as statistically significant. RESULTS: The mean follow up time was 40.9±5.6 months. Among the 33 patients with ASO, 6% had mild pulmonary stenosis, while 3% had mild pulmonary insufficiency. Aortic stenosis and aortic insufficiency of trivial to mild degree was seen in 12% and 12% of the patients, respectively. The patients' systolic velocity of tricuspid (S), early diastolic velocity of tricuspid (Ea), and late velocity of tricuspid valve (Aa) were significantly different from those of the controls (P<0.001). Also, pulmonary annulus diameter was significantly dilated in the patients compared to the controls (1.67±0.41 vs. 1.29±0.28, P≤0.001). Besides, aortic annulus diameter (1.56±0.42 vs. 1.24±0.21, P=0.001) and also aortic sinus diameter (2.06±0.41 vs. 1.44±0.34, P=0.002) were significantly dilated, while sinutuboar junction diameter (1.65±0.5 vs. 1.28±0.29, P=0.094) was not dilated. Left ventricular function was in the normal range. CONCLUSIONS: This study showed good left ventricular function, but some abnormalities in lateral tricuspid tissue Doppler velocities. Neoaortic and pulmonary diameters were significantly dilated, while aortic and pulmonary insufficiencies were clinically insignificant in most of the patients. Long-term follow-up is necessary in these patients.