Optimal surgical approach for repair of aortopulmonary window.

Background: This is a review of the experience over 26 year in a single institution with surgical repair of aortopulmonary window.

Methods: Between July 1973 and March 1999, 38 patients underwent surgery for aortopulmonary window at a median age of 5 weeks, and with a median weight of 3.9 kg.

Echocardiographic characteristics and outcome of straddling mitral valve.

Objectives: This study sought to characterize the echocardiographic features of straddling mitral valve (SMV) and to determine its surgical implications and midterm outcome in a large clinical cohort.

Background: Despite a relatively large body of literature on the postmortem anatomy of SMV, there is a paucity of information regarding its echocardiographic features, surgical implications and preoperative predictors of outcome.

Methods: A retrospective review identified 46 patients with SMV between 1982 and 1999 who underwent echocardiography and surgery and had follow-up data.

Minimal-access surgery for congenital heart defects.

Improved results with lower operative mortality and morbidity for corrective surgery for many congenital cardiac defects has stimulated a renewed interest in the use of surgical approaches other than a full midline sternotomy. In an effort to decrease pain and discomfort, shorten the recovery period, and improve the cosmetic result, several alternative approaches have been proposed and implemented, with varying results. Anterior thoracotomy in the inframammary area has been the most widely used incision and is most applicable to females patients past puberty, in whom the extent of breast tissue can be assessed more accurately.

Surgical management of double-outlet right ventricle with subaortic ventricular septal defect.

Double-outlet right ventricle (DORV) and subaortic ventricular septal defect (VSD) is defined anatomically as a defect where the entire pulmonary trunk and at least half of the aorta arises from the right ventricle (RV) and the VSD is most closely aligned with the aorta. The surgical management of DORV and subaortic VSD usually results in a 2 ventricle repair where the left ventricular outflow is diverted via the VSD to the aorta. Pulmonary and/or subpulmonary obstruction is found in a large portion of these patients and requires relief at the time of repair.

Situs inversus totalis and corrected transposition of the great arteries [I,D,D] in association with a previously unreported vascular ring.

A 3-month-old girl with "noisy breathing" was found to have situs inversus totalis, corrected transposition of the great arteries [I,D,D], and a vascular ring. The ring was composed of a left aortic arch with normal branching pattern and a right ligamentum arteriosum that extended from a diverticulum off the descending aorta and coursed retroesophageal and to the right to join the pulmonary artery. There was no circumflex component of the aorta or aberrant subclavian artery.

Long-term results after early primary repair of tetralogy of Fallot.

Objective: Early primary repair of tetralogy of Fallot has been routinely performed at Children's Hospital, Boston, since 1972. We evaluated the long-term outcome of this treatment strategy including the influence of a transannular patch.

Methods: Fifty-seven patients less than 24 months of age (median 8 months) underwent primary repair of tetralogy of Fallot between January 1972 and December 1977.

Post-ischemic PKC inhibition impairs myocardial calcium handling and increases contractile protein calcium sensitivity.

Objective: Protein kinase C (PKC) activation impairs contractility in the normal heart but is protective during myocardial ischemia. We hypothesized that PKC remains activated post-ischemia and modulates myocardial excitation-contraction coupling during early reperfusion.

Methods: Langendorff-perfused rabbit hearts where subjected to 25 min unmodified ischemia and 30 min reperfusion.

Surgery for coarctation of the aorta in infants weighing less than 2 kg.

Background: Low- and very low-birth weight infants are now candidates for reparative cardiac surgery. Outcomes after coarctation repair have not been characterized in this patient population.

Methods: We performed a retrospective review of 18 consecutive neonates less than 2 kg who underwent repair of aortic coarctation between August 1990 and December 1999.

Lipopolysaccharide internalization activates endotoxin-dependent signal transduction in cardiomyocytes.

We tested the hypothesis that bacterial lipopolysaccharide (LPS) must be internalized to facilitate endotoxin-dependent signal activation in cardiac myocytes. Fluorescently labeled LPS was used to treat primary cardiomyocyte cultures, perfused heart preparations, and the RAW264.7 macrophage cell line.

Minimal sternotomy approach for congenital heart operations.

Background: In recent years, minimal access cardiac operations have increased in application in both the adult and pediatric population. As our experience has grown with these approaches to atrial septal defect closure, we have expanded the same approach to the repair of more complex congenital heart disease.

Methods: At the Children's Hospital in Boston, from August 1996 to November 1999, a minimal sternotomy approach was used to surgically correct 104 children with congenital heart defects other than atrial septal defect.

Surgical outcome of double-outlet right ventricle with subpulmonary VSD.

Background: Optimal management of double-outlet right ventricle with subpulmonary ventricular septal defect remains controversial. We reviewed our 7-year experience with patients who had this anatomic configuration.

Methods: Between January 1992 and January 1999, 20 patients underwent an arterial switch operation (ASO group), and 12 underwent a bidirectional Glenn procedure followed by a modified Fontan in 10 (Glenn/Fontan).

Congenital supravalvar aortic stenosis: a simple lesion?

The underlying cause of congenital supravalvular aortic stenosis (SVAS) has recently been identified as a loss-of function mutation of the elastin gene on chromosome 7q11.23, resulting in an obstructive arteriopathy of varying severity, which is most prominent at the aortic sinutubular junction. The generalized nature of the disease explains the frequent association with stenoses of systemic and pulmonary arteries.

Ischemic dysfunction in transgenic mice expressing troponin I lacking protein kinase C phosphorylation sites.

To determine the in vivo functional significance of troponin I (TnI) protein kinase C (PKC) phosphorylation sites, we created a transgenic mouse expressing mutant TnI, in which PKC phosphorylation sites at serines-43 and -45 were replaced by alanine. When we used high-perfusate calcium as a PKC activator, developed pressures in transgenic (TG) perfused hearts were similar to wild-type (WT) hearts (P = not significant, NS), though there was a 35% and 32% decrease in peak-systolic intracellular calcium (P < 0.01) and diastolic calcium (P < 0.

Vesnarinone restores contractility and calcium handling in early endotoxemia.

Background: Endotoxin (lipopolysaccharide, LPS) is a trigger of the systemic inflammatory response. We have previously found that vesnarinone and amrinone, when given before LPS, prevented cytokine production and LPS-related cardiac dysfunction. We tested the hypothesis that vesnarinone would improve intracellular Ca(2+) handling and calcium-activated contractile force after the onset of endotoxemia.

Intraoperative recurrent laryngeal nerve monitoring during video-assisted throracoscopic surgery for patent ductus arteriosus.

Objective: To develop a technique to identify and localize the recurrent laryngeal nerve (RLN) during video-assisted thoracoscopic surgery (VATS) for patent ductus arteriosus.

Design: Prospective clinical study.

Setting: Children's hospital.

Coronary echocardiography in tetralogy of fallot: diagnostic accuracy, resource utilization and surgical implications over 13 years.

Objectives: This study sought to determine the diagnostic accuracy and impact of the systematic use of coronary echocardiography in a large group of preoperative patients with tetralogy of Fallot (TOF).

Background: Accurate preoperative identification of an anomalous coronary artery crossing the right ventricular outflow tract (RVOT) in patients with TOF is important to prevent coronary injury during surgical repair.

Methods: A retrospective review identified 598 patients with TOF between 1983 to 1995 who underwent an echocardiogram at <2 years old before complete surgical repair.

Surgery for bilateral outflow tract obstruction in elastin arteriopathy.

Objective: A number of patients with Williams syndrome or other forms of elastin arteriopathy have stenoses of pulmonary arteries in addition to supravalvular aortic stenosis. We sought to investigate the effect of the degree of pulmonary arterial stenosis on the prognosis after an operation for supravalvular aortic stenosis to help define the optimal treatment strategy for patients with severe forms of elastin arteriopathy.

Methods: Between 1960 and 1999, 33 patients underwent operations for supravalvular aortic stenosis while having significant stenoses of the pulmonary arteries.

Twenty-five-year experience with rastelli repair for transposition of the great arteries.

Objective: Our purpose was to describe the outcome of the Rastelli repair in D -transposition of the great arteries and to determine the risk factors associated with unfavorable events.

Methods: From March 1973 to April 1998, 101 patients with D -transposition of the great arteries and ventricular septal defect underwent a Rastelli type of repair. Median age and weight were 3.

CD14-independent activation of cardiomyocyte signal transduction by bacterial endotoxin.

In the heart, lipopolysaccharide (LPS) induces the production of proinflammatory cytokines that cause myocardial dysfunction; however, the signaling pathways involved in cardiomyocyte responses are poorly understood. We studied LPS-induced signaling by treating cardiomyocyte cultures with 0.01-10 microgram/ml LPS for 0-24 h in the presence or absence of 2.

Minimal access approach for the repair of atrial septal defect: the initial 135 patients.

Background: From May 1996 to August 1998 a minimal access approach was used for 135 of 200 consecutive surgical atrial septal defects closures in children through young adults ranging in age from 6 months to 25 years (median 5 years).

Methods: A 3.5- to 5-cm midline incision was centered over the xiphoid with division of the xiphoid alone (transxiphoid) or of the lower sternum (ministernotomy); both groups underwent bicaval venous cannulation through the incision.

Surgical management of complex and tunnel-like subaortic stenosis.

Background: Relief of primary or secondary subaortic stenosis (SAS) remains a surgical challenge. Heart block, aortic valve regurgitation and recurrent obstruction have been persistent problems.

Methods: Forty six patients who underwent surgery for complex and tunnel-like SAS between January 1990 and November 1998 were reviewed.

Postoperative recovery in children after minimum versus full-length sternotomy.

Background: Minimal access incisions for pediatric cardiac surgery have been reported to hasten postoperative recovery. This prospective study compared recovery after a minimum versus full-length sternotomy for repair of atrial septal defects in children.

Methods: We studied 35 children undergoing atrial septal defect repair using a full-length sternotomy (n = 18) or ministernotomy (n = 17) according to the surgeon's preference.

Repair of the truncal valve and associated interrupted arch in neonates with truncus arteriosus.

Objective: Truncal valve regurgitation and interrupted aortic arch have frequently been identified as risk factors in the repair of truncus arteriosus. We wished to examine these factors in the current era including the impact of truncal valve repair.

Methods: Between January 1992 and August 1998, 50 patients underwent surgical repair of truncus arteriosus.

Results of Norwood stage I operation: comparison of hypoplastic left heart syndrome with other malformations.

Objective: We compared the Norwood stage I operation for hypoplastic left heart syndrome and other complex malformations with ductus-dependent systemic circulation.

Methods: A retrospective study of 194 patients who underwent a Norwood stage I palliation between 1990 and 1998 was conducted. Malformations in 131 patients were classified as hypoplastic left heart syndrome, defined as aortic and mitral atresia or severe stenosis, normal segmental anatomy, intact ventricular septum, and hypoplasia of the left ventricle.