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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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).

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.

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.

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.

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.

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.

The prevalence of hypomagnesaemia in pediatric patients with mitral valve prolapse syndrome and the effect of mg therapy.

Background: A paucity of data exists regarding the prevalence and relationship of hypomagnesaemia with clinical symptoms of mitral valve prolapse (MVP) in pediatric patients.

Objective: In this study we evaluated the prevalence of magnesium (Mg) deficiency in pediatric patients with MVP syndrome and attempted to clarify the effect of Mg therapy on alleviating their symptoms.

Methods: The present study was conducted from April 2010 to January 2012, and included 230 patients (90 males and 140 females) with symptoms of mitral valve prolapse and mean age of 11.

Short-term result of ductus arteriosus stent implantation compared with surgically created shunts.

This report aims to compare the researchers' early experience with the safety, efficacy, short-term outcomes, and complications of patent ductus arteriosus (PDA) stents in neonates having duct-dependent pulmonary circulation with those of surgically created shunts. Between April 2009 and April 2011, 18 infants with duct-dependent pulmonary circulation underwent cardiac catheterization for PDA stenting as the first palliative procedure in a referral center. For comparison, 20 infants who underwent surgical aortopulmonary shunt placement in another center were used.

Efficacy of Carvedilol in Patients with Dilated Cardiomyopathy due to Beta-thalassemia major; a Double-blind Randomized Controlled Trial.

Objective: Dilated cardiomyopathy is the end result of chronic iron overload in patients with beta thalassemia major. The objective of the present study was to evaluate the safety and efficacy of Carvedilol in patients with beta thalassemia major and dilated cardiomyopathy.

Methods: During a six-month period, fourteen patients with beta-thalassemia major and heart failure without diabetes mellitus referred to pediatric cardiology clinic enrolled in this double blind, randomly assigned study.

Estimation of Right Atrial Pressure from the Inspiratory Collapse of the Inferior Vena cava in Pediatric Patients.

Objective: Paucity of data exists between mean right atrial pressure (RAP) and inferior vena cava (IVC) size and collapsibility in pediatric patients with congenital heart disease.

Methods: In a prospective study, fifty consecutive pediatric patients with different congenital heart diseases who had right side cardiac catheterization were studied, comparing right atrial pressure with simultaneous M-mode echocardiographic measurement of inferior vena cava diameter. Mean age of the patients was 4.

Evaluation of myocardial function using the Tei index in patients with Kawasaki disease.

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.