Cardiovascular malformations in

Cardiovascular malformations in Case Report Fryns syndrome is characterized by multiple congenital anomalies including congenital diaphragmatic hernia (CDH), and congenital heart disease (CHD).The prognosis of infant with Fryns syndrome and left sided CDH when associated with pulmonary hypoplasia is grave. We report a 2- year old boy with Fryns syndrome who had right sided CDH, Tetralogy of Fallot, and other multiple congenital anomalies. The patient survived a successful operation of both heart and diaphragmatic defects. Diaphragmatic hernia, Fryns syndrome, Tetralogy of Fallot 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/269/

Aortic valve repair leads to a low incidence of valve-related complications Review Aortic valve replacement for aortic regurgitation (AR) has been established as a standard treatment but implies prosthesis-related complications. Aortic valve repair is an alternative approach, but its mid- to long-term results still need to be defined. Aortic valve • Aortic regurgitation • Aortic valve reconstruction • Valve-related complications • Aortic dilatation 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/266/

Four decades of experience with mitral valve repair: Analysis of differential indications, technical evolution, and long-term outcome Review To determine the long-term outcomes of mitral valvuloplasty for myxomatous valve disease, rheumatic valve disease, and functional mitral regurgitation. -- 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/265/

Patient-Prosthesis Mismatch in Patients with Aortic Stenosis Undergoing Isolated Aortic Valve Replacement Does Not Affect Survival Review Data suggest that patient-prosthesis mismatch (PPM) adversely effects late survival after aortic valve replacement (AVR). This study examined the incidence and implications of PPM in patients undergoing isolated AVR. -- 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/264/

The myocardial protective effect of adenosine as an adjunct to intermittent blood cardioplegia during open heart surgery Review Although adenosine (ADO) has been shown to have beneficial effects against tissue injury after myocardial ischaemia, the controversy still remains regarding the optimal timing, dose, temperature, method of ADO administration and duration of exposure to the drug. This study investigates the cardioprotective effect of exogenous ADO pretreatment as an adjunct to 1 mmol l-1 ADO cold (12 °C) blood cardioplegia during heart valve replacement surgery. Adenosine • Cardiopulmonary bypass 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/263/

Reoperations on the Aortic Root: Experience in 46 Patients Review The increasing use of biologic conduits and the advances in reparative aortic root procedures has increased the number of patients who may require reoperation on the aortic root. Although the primary operation yields excellent results with a low risk for morbidity and mortality, reoperation on the aortic root is still challenging. Reoperations-Aortic Root 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/258/

Is advanced age a contraindication for emergent repair of Review With the general increase in human lifespan, cardiac surgeons are faced with treating an increasing number of elderly patients. The aim of our study was to investigate whether advanced age poses an increased risk for major morbidity and mortality with repair of acute type A aortic dissection. Between 2000 and 2008, 119 patients underwent emergency operation for acute type A aortic dissection at two institutions; 90 were younger than 70 years of age and 29 patients were 70 years or older. Major morbidity, operative and 5-year actuarial survival were compared between groups. The operative mortality rates were comparable between the two groups (18.9% in patients <70 years vs. 24.1% for patients 70 years, P=0.6). There was no difference in the rates of reoperation for bleeding (<70 years 31.7% vs. 14.3% for 70 years, P=0.09), stroke (18.9% for those <70 years vs. 20.7% for those 70 years, P=0.79), acute renal failure (22.2% for those <70 years vs. 17.2% for those 70 years, P=0.79) or prolonged ventilation (34.4% for those <70 years vs. 24.1% for those 70 years, P=0.36) between the two groups. Actuarial 5-year survival rates were 77% for patients <70 years vs. 59% for patients 70 years (P=0.07). The mortality for patients who presented with hemodynamic instability was markedly higher (10 out of 14 patients, 71.4%) compared with the mortality of those who presented with stable hemodynamics (21 out of 88 patients, 23.9%, P<0.001), regardless of age group. No significant differences in operative mortality, major morbidity and actuarial 5-year survival were observed between patients 70 years and younger patients although there was a trend toward a lower actuarial 5-year survival in older patients. Surgery for type A acute aortic dissection in patients 70 years or older can be performed with acceptable outcomes. Hemodynamic instability portends a poor prognosis, regardless of age. Keywords: Morbidity; Outcomes; Mortality; Aortic dissection -- 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/256/

Five years follow-up after Y-graft arterial revascularization: Review We report our comparative experience of on-pump and off-pump full arterial coronary artery bypass grafting (CABG) using both internal mammary arteries (IMAs) anastomosed as a Y-graft. Methods: A single-center clinical study was conducted prospectively between January 2003 and May 2008. It compared the short- and mid-term clinical outcomes of on- and off-pump arterial revascularization where the left internal mammary artery (LIMA) was anastomosed to the left anterior descending (LAD) artery while the free right internal mammary artery (RIMA) graft taking off from the LIMA was used to bypass different coronary targets. Results: 192 patients were divided into 77 on-pump and 115 off-pump procedures based on the intention to treat. The mean age in both groups was 60.2±11.7 and 68.1±10.6 years, respectively (p<0.05). Mean predictive logistic EuroSCORE was 3.5±6.7% for the on-pump group and 7.3±8.6% for the off-pump group (p<0.0001). Mean number of distal anastomoses were 2.7±0.6 (group ON) and 2.5±0.6 (group OFF) (p=NS). Postoperative mortality was 2 patients (2.6%) in the on-pump group and 4 patients (3.4%) in the off-pump group (p=0.63). No major adverse cardiac event, no stroke and no late death were reported during the follow-up that averaged 36.5±18.6 months. Angina recurrence was 3 patients (2.6%) in off-pump and 2 patients (3.5%) in on-pump group (p=NS). Conclusions: The use of a free RIMA as Y-graft from the LIMA performed off pump eradicates aortic manipulations and provides complete revascularization to high-risk patients with mortality similar to the one of a lower risk population operated on pump. The morbidity and cost was lower in the off-pump group. This advocates for the widespread usage of the technique in high-risk patients. Keywords: Coronary; Off pump; On pump - 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/255/

Risk factors for deterioration of renal function Review Various definitions of impairment of renal function after coronary artery bypass grafting (CABG) are used in the literature. Depending on the definition, several risk factors are identified. We analysed our data to determine the risk factors for postoperative deterioration of the creatinine clearance of 10% or more. Coronary artery bypass grafting • CABG 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/254/

Safety and efficacy of sequential and composite arterial grafting to more than five coronary branches in off-pump coronary revascularisation: assessment of intra-operative and angiographic bypass flow Review Off-pump CABG • Complete revascularisation • Arterial graft • Graft flow • Angiography 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/253/

Stroke-Related Mortality in Coronary Surgery Is Reduced Review Stroke remains an important complication after coronary artery bypass graft surgery (CABG). We sought to determine the frequency and death-related incidence of stroke after on-pump and off-pump CABG. Stroke-Related-Off-Pump Approach 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/252/

Long-term cardiopulmonary exercise capacity Review Long-term cardiopulmonary 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/251/

Perioperative Hyperglycemia: Effect on Outcome Review Studies demonstrate that cardiopulmonary bypass (CPB) causes intraoperative and postoperative hyperglycemia. Hyperglycemia has been associated with morbidity and mortality after infant cardiac surgery. We studied the effects on early postoperative outcomes of glucose (GLU) changes during and after pediatric cardiac surgery. Perioperative Hyperglycemia 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/250/

Pulmonary Valve Replacement After Tetralogy of Review After tetralogy of Fallot (TOF) repair, severe pulmonary insufficiency is known to impair biventricular function. Pulmonary valve replacement (PVR) alleviates symptoms, normalizes right ventricular volumes, and improves ventricular function. Most studies addressing the role of PVR have examined older adolescents or adults. Less is known about the potential benefits of PVR in preadolescents with TOF and significant right ventricular dilatation. Pulmonary- Replacement - Tetralogy 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/249/

Outcomes of Biventricular Repair for Congenitally Review Corrected Transposition 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/248/

Evolution of treatment options and outcomes for hypoplastic Review CI = confidence interval; HLHS = hypoplastic left heart syndrome; ICD-9-CM 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/247/

Acute right ventricular failure after pediatric cardiac transplant: Predictors and long-term outcome in current era of transplantation medicine Review Abbreviations and Acronyms BSA = body surface area; CHD = congenital heart disease; DCM = dilated cardiomyopathy; ECMO = extracorporeal membrane oxygenation; iNO = inhaled nitric oxide; PVR = pulmonary vascular resistance; PVRI = pulmonary vascular resistance index; RCM = restrictive cardiomyopathy; RVF = right ventricular failure; TPG = transpulmonary gradient 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/246/

Cardiopulmonary Bypass Increases Permeability of Review The integrity of the blood-cerebrospinal fluid (CSF) barrier during cardiopulmonary bypass (CPB) with hypothermic circulatory arrest (HCA) has not been systematically studied, especially in children. We tested the hypothesis that the blood-CSF barrier is disrupted by CPB. Cardiopulmonary Bypass-Cerebrospinal Fluid Barrier 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/245/

Selective antegrade cerebral perfusion via right axillary artery cannulation reduces morbidity and mortality after proximal aortic surgery Review Selective antegrade cerebral perfusion is a well-described neuroprotective technique used in proximal aortic surgery. This study investigated whether selective antegrade cerebral perfusion is associated with improved outcomes in both emergency and elective settings compared with deep hypothermic circulatory arrest alone. Abbreviations and Acronyms AOR = adjusted odds ratio; CABG = coronary artery bypass grafting; CI = confidence interval; DHCA = deep hypothermia alone; SACP = selective antegrade cerebral perfusion; TND = temporary neurologic dysfunction 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/244/

Magnesium supplementation during cardiopulmonary bypass to prevent junctional ectopic tachycardia after pediatric cardiac surgery: A randomized controlled study Review We analyzed the role of magnesium sulfate (MgSO4) supplementation during cardiopulmonary bypass in pediatric patients undergoing cardiac surgery, assessing the incidence of hypomagnesemia and the incidence of junctional ectopic tachycardia. Methods: We performed a randomized, double-blind, controlled trial in 99 children. MgSO4 or placebo was administered during the rewarming phase of cardiopulmonary bypass: group 1, placebo group (29 patients); group 2, 25 mg/kg of MgSO4 (30 patients); and group 3, 50 mg/kg of MgSO4 (40 patients). Abbreviations and Acronyms CICU = cardiac intensive care unit; CPB = cardiopulmonary bypass; iMg = ionized magnesium; JET = junctional ectopic tachycardia; MgSO4 = magnesium sulfate; PRISM = Pediatric Risk of Mortality 0 0 http://www.iscs.org.ir/journals/vol.3/no.5/243/

Tissue engineered human heart valves Original Article The is no ideal heart valve substitute, both mechanical and biologic heart valve have significant limitation in term of durability, growth potential (for infant and children), compatibility, resistance to infection(1). Tissue-engineered heart valve have been proposed by physicians and scientists alike to be the ultimate solution for treating valvular heart disease rather than replacing a diseased or defective native valve with a mechanical or bioprosthesis, a tissue engineered valve would be a living organ with the capability for growth, repair and remodeling in the same way that the native heart valve does. Over the last decade attempts to create tissue engineered heart valve have been made with varying degrees of success, constructing these valve from a Varity of cell types and scaffolding materials. Early results in animals and limited clinical application are promising although it will take many years to demonstrate that long-term performance of this valve is comparable or better than conventional prosthetic heart valve. tissue engineering- heart valve- technique- future 10 13 http://www.iscs.org.ir/journals/vol.3/no.5/241/

Inadvertent treatment with a pure IKr blocker in LQT2 syndrome Original Article Long QT syndrome, torsades de points, Nifekalant, genotype, missense mutation 6 9 http://www.iscs.org.ir/journals/vol.3/no.5/239/

Coronary Artery Bypass Grafting In Familial Hypercholesterolemia Original Article Familial hypercholesterolemia is dominantly inherited disorder caused mutation by at the locus for the low-density lipoprotein receptor and is frequently associated with premature coronary artery disease. This study was performed to determine outcomes of coronary artery bypass grafting for patients with familial hypercholesterolemia.. familial hypercholesterolemia, coronary artery bypass grafting. 3 5 http://www.iscs.org.ir/journals/vol.3/no.5/238/