Repair of anomalous aortic origin of a coronary artery in 113 patients: a Congenital Heart Surgeons' Society report.

Background: Anomalous aortic origin of a coronary artery (AAOCA) encompasses a wide morphologic spectrum, which has impeded consensus regarding indications for the diverse repair strategies. We constructed a profile of current surgical techniques and explore their application to morphologic variants.

Methods: Patients<30 years old (n=113) with isolated AAOCA who underwent operations at 29 Congenital Heart Surgeons Society (CHSS) institutions from 1998 to 2012 were identified from the CHSS AAOCA Registry.

Glucocorticoids improve calcium cycling in cardiac myocytes after cardiopulmonary bypass.

Background: Glucocorticoids can reduce myocardial dysfunction associated with ischemia and reperfusion injury following cardiopulmonary bypass (CPB) and circulatory arrest. The hypothesis was that maintenance of cardiac function after CPB with methylprednisolone therapy results, in part, from preservation of myocyte calcium cycling.

Methods: Piglets (5-7 kg) underwent CPB and 120 min of hypothermic circulatory arrest with (CPB-GC) or without (CPB) methylprednisolone (30 mgkg(-1)) administered 6h before and at CPB.

Improved current era outcomes in patients with heterotaxy syndromes.

Objective: Patients with heterotaxy syndrome have a myriad of visceral and cardiac malformations historically resulting in significant morbidity and mortality. We sought to assess whether current era management strategies have improved outcomes in patients with visceral heterotaxy.

Methods: A retrospective review (1994-2008) of our database identified 45 consecutive heterotaxy patients who underwent surgical palliation.

Modulation of nuclear factor-kappaB improves cardiac dysfunction associated with cardiopulmonary bypass and deep hypothermic circulatory arrest.

Objective: The hypothesis is that partial nuclear factor-kappaB (NF-kappaB) inhibition can alleviate cardiopulmonary dysfunction associated with ischemia and reperfusion injury following cardiopulmonary bypass and deep hypothermic circulatory arrest (CPB/DHCA) in a pediatric model.

Design: Animal case study.

Subjects: Two-week-old piglets (5-7 kg).

Computed tomographic appearance of Prolene Hernia System and polypropylene mesh plug inguinal hernia repair.

Purpose: To describe the normal postoperative computed tomographic (CT) appearance of inguinal hernia repair with the Prolene (polypropylene) Hernia System and polypropylene mesh plug.

Materials And Methods: The medical records for 480 consecutive patients who underwent inguinal mesh hernioplasty were reviewed to identify posthernioplasty pelvic CT scans. The presence or absence of the appearance and size of focal inguinal findings at CT for each groin was recorded by 2 radiologists in consensus.

Acute beta-blockade prevents myocardial beta-adrenergic receptor desensitization and preserves early ventricular function after brain death.

Objective: Beta-adrenergic receptor desensitization through activation of the G protein-coupled receptor kinase 2 is an important mechanism of early cardiac dysfunction after brain death. We hypothesized that acute beta-blockade can prevent myocardial beta-adrenergic receptor desensitization after brain death through attenuation of G protein-coupled receptor kinase 2 activity, resulting in improved cardiac function.

Methods: Adult pigs underwent either sham operation, induction of brain death, or treatment with esmolol (beta-blockade) for 30 minutes before and 45 minutes after brain death (n = 8 per group).

Factors affecting long-term risk of aortic arch recoarctation after the Norwood procedure.

Background: The purpose of this study was to identify factors predicting risk of aortic arch recoarctation after the Norwood procedure.

Methods: Patient records were reviewed retrospectively for consecutive patients who underwent the Norwood procedure from 1996 to 2005. Preoperative and intraoperative parameters were identified for analysis.

Beta-adrenergic receptor antagonism preserves myocardial function after brain death in a porcine model.

Background: Cardiac dysfunction after brain death decreases the already limited number of potential donors for cardiac transplantation. Acute beta-adrenergic receptor (betaAR) desensitization after the brain death-associated catecholamine surge is an important mechanism. We hypothesized that acute betaAR antagonism could improve myocardial function after brain death by preserving betaAR signaling.

Glucocorticoids alter the balance between pro- and anti-inflammatory mediators in the myocardium in a porcine model of brain death.

Background: Cardiac dysfunction after brain death (BD) limits donors for cardiac transplantation. Glucocorticoids ameliorate brain death-induced donor heart dysfunction. We hypothesized that glucocorticoid therapy alleviates myocardial depression through altering the balance between pro- and anti-inflammatory mediators via the nuclear factor-kappaB (NF-kappaB)/inhibitor of kappaB-alpha (IkappaBalpha) pathway and/or by preserving beta-adrenergic receptor (betaAR) signaling in the heart.

Pulmonary atresia with intact ventricular septum: initial management.

Background: Outcomes for pulmonary atresia with intact ventricular septum are suboptimal, while initial management remains controversial. This study was undertaken to determine the effect of catheter-based therapy on the need for early surgical intervention.

Methods: A single-institution retrospective chart review was made of all 25 neonates with pulmonary atresia with intact ventricular septum from 1999 to 2005.

Bilateral semilunar valve disease in a child with partial deletion of the Williams-Beuren syndrome region is associated with elastin haploinsufficiency.

Elastin is an extracellular matrix protein that is the primary component of elastic fibers, and is expressed in the great vessels as well as the semilunar and atrioventricular valves. Elastin haploinsufficiency, resulting from mutation or deletion of the elastin gene, is an important clinical problem that is typically characterized by arteriopathy. Herein is described a patient with elastin haploinsufficiency due to partial deletion of the Williams-Beuren syndrome region, resulting in bilateral semilunar valve disease and arteriopathy.

Current treatments for congenital aortic stenosis.

Purpose Of Review: Congenital valvar aortic stenosis is a challenging disease that often requires repeated palliative procedures. Stenosis can range from mild and asymptomatic, not requiring intervention, to severe, as seen in hypoplastic left heart syndrome. New advances such as fetal balloon valvuloplasty, improvements in the Ross technique, and long-term studies of trans-catheter balloon valvuloplasty and surgical valvotomy warrant a review of the outcomes and optimal timing of the various interventions.

Brain magnetic resonance imaging abnormalities after the Norwood procedure using regional cerebral perfusion.

Objectives: Neurologic deficits are common after the Norwood procedure for hypoplastic left heart syndrome. Because of the association of deep hypothermic circulatory arrest with adverse neurologic outcome, regional low-flow cerebral perfusion has been used to limit the period of intraoperative brain ischemia. To evaluate the impact of this technique on brain ischemia, we performed serial brain magnetic resonance imaging in a cohort of infants before and after the Norwood operation using regional cerebral perfusion.

Glucocorticoid administration reduces cardiac dysfunction after brain death in pigs.

Background: Traumatic brain injury and subsequent brain death (BD) account for nearly half of all organ donors, yet only 33% of available hearts are transplanted. Alterations in multiple physiologic pathways after BD can lead to cardiac dysfunction and exclusion from transplantation. Triple hormone resuscitation with methylprednisolone, thyroid hormone and vasopressin has had inconsistent results in the effort to reduce cardiac dysfunction associated with BD, but individual analysis of these agents is limited.

Heparin-induced thrombocytopenia complicating support by the Berlin Heart.

Since 1992, miniaturized pulsatile air-driven ventricular assist devices (VADs), "Berlin Heart," have been used at many institutions (36 cases in North America in 19 different institutions) for pediatric use. Heparin-induced thrombocytopenia (HIT II) is a significant complication rarely reported in the setting of adult VAD support; no similar report exists concerning pediatric VAD support. We report on a 13-month-old, 8.

Brain magnetic resonance imaging abnormalities after the Norwood procedure using regional cerebral perfusion.

Objectives: Neurologic deficits are common after the Norwood procedure for hypoplastic left heart syndrome. Because of the association of deep hypothermic circulatory arrest with adverse neurologic outcome, regional low-flow cerebral perfusion has been used to limit the period of intraoperative brain ischemia. To evaluate the effect of this technique on brain ischemia, we performed serial brain magnetic resonance imaging in a cohort of infants before and after the Norwood operation using regional cerebral perfusion.

Role of the beta-adrenergic receptor kinase in myocardial dysfunction after brain death.

Objective: Significant cardiac dysfunction after brain death leading to exclusion from procurement for cardiac transplantation is seen in up to 25% of potential organ donors in the absence of structural heart disease. The cause includes uncoupling of the myocardial beta-adrenergic receptor signaling system. The mechanism, however, has not yet been described.

Inhibition of p38 reduces myocardial infarction injury in the mouse but not pig after ischemia-reperfusion.

The MAPK family member p38 is activated in the heart after ischemia-reperfusion (I/R) injury. However, the cardioprotective vs. proapoptotic effects associated with p38 activation in the heart after I/R injury remain unresolved.

Calpain inhibition decreases endothelin-1 levels and pulmonary hypertension after cardiopulmonary bypass with deep hypothermic circulatory arrest.

Objective: Cardiopulmonary bypass in infants and children can result in cardiopulmonary dysfunction through ischemia and reperfusion injury. Pulmonary hypertension and injury are particularly common and morbid complications of neonatal cardiac surgery. Inhibition of calpain, a cysteine protease, has been shown to inhibit reperfusion injury in adult organ systems.

Sildenafil citrate alleviates pulmonary hypertension after hypoxia and reoxygenation with cardiopulmonary bypass.

Background: Sudden reoxygenation of hypoxic neonates undergoing cardiac operation exacerbates the systemic inflammatory response to cardiopulmonary bypass secondary to reoxygenation injury, worsening cardiopulmonary dysfunction. Reports suggest sildenafil decreases pulmonary hypertension and may affect myocardial function. Sildenafil's efficacy for treating postbypass cardiopulmonary dysfunction remains unknown.

Compression of an extracardiac fontan following classic fontan revision.

Background: The extracardiac Fontan arose as an alternative in order to prevent the classical procedure's sequelea. Complications of the extracardiac Fontan have been described, but this case report aims to highlight the devastating and infrequent complication of external conduit compression.

Methods And Results: A 25-year-old male underwent Fontan conversion to extracardiac Fontan for atrial arrhythmias and giant right atrium.

Risk of recoarctation should not be a deciding factor in the timing of coarctation repair.

To determine whether early coarctation repair is a significant risk for recoarctation in the modern era, 120 patients, including 87 infants, who underwent isolated coarctation repair at a single institution, were reviewed. At a mean follow-up of 44.4 months, there have were no late reoperations, and 2 patients required balloon aortoplasty.

Preoperative glucocorticoids decrease pulmonary hypertension in piglets after cardiopulmonary bypass and circulatory arrest.

Background: Glucocorticoids during cardiopulmonary bypass benefit pediatric patients undergoing repair of congenital heart defects and are routine therapy, but underlying mechanisms have not been fully examined. The hypothesis was that glucocorticoids could improve cardiopulmonary recovery after cardiopulmonary bypass and deep hypothermic circulatory arrest.

Methods: Crossbred piglets (5 to 7 kg) were cooled with cardiopulmonary bypass, followed by 120-min deep hypothermic circulatory arrest.