Retroaortic innominate vein with coarctation of the aorta: surgical repair and embryology review.

A newborn girl with a retroaortic innominate vein, coarctation of the aorta, ventricular septal defect, and subaortic stenosis underwent a complete repair at 8 days of age. The ascending aorta was transected and the innominate vein was brought in front of it. We review the anatomy and embryology of this rare entity and describe the surgical technique to avoid recoarctation and innomante vein compression.

Subaortic stenosis: at risk substrates and treatment strategies.

Subaortic stenosis can be a complex disease of multiple anatomic etiologies. At the core is either an elongated and narrow outflow tract as compared to normal or a fully muscle-rimmed VSD used as an intraventricular routing pathway. An array of treatment modalities is needed for an effective management strategy.

Levels of exhaled nitric oxide before and after surgical and transcatheter device closure of atrial septal defects in children.

Objectives: We have shown that exhaled nitric oxide levels decrease after surgical closure of congenital left-to-right cardiac shunts. It remains unclear whether the change in exhaled nitric oxide levels reflects endothelial injury caused by the use of cardiopulmonary bypass or the decrease in pulmonary blood flow attendant on shunt closure. Transcatheter atrial septal defect closure permits shunt closure without the use of cardiopulmonary bypass.

Extending the usable size range of homografts in the pulmonary circulation: outcome of bicuspid homografts.

Background: Small-sized homografts are often not available, making the use of surgically reduced cryopreserved homograft conduits appealing.

Methods: From January 1993 to January 2000, 21 large homografts were size-reduced by excising one leaflet and were implanted in the pulmonary circulation. Valve function was compared with 21 children-matched for weight, homograft size, and year of operation-who received a standard homograft.

Extracardiac conduit versus lateral tunnel cavopulmonary connections at a single institution: impact on outcomes.

Objective: To compare outcomes of extracardiac conduit and lateral tunnel Fontan connections in a single institution over a concurrent time period.

Methods: Between January 1994 and September 1998, 60 extracardiac conduit and 47 lateral tunnel total cavopulmonary connections were performed. Age, sex, and weight did not differ between the 2 groups.

Improving outcomes of the Fontan operation in children with atrial isomerism and heterotaxy syndromes.

Background: The historic outcome of the Fontan procedure in children with single ventricle and heterotaxy syndrome has been poor, and in the current era it has been incompletely described.

Methods: From January 1993 through April 2000, 30 patients (age range, 5.3 +/- 3.

Evolving strategies and improving outcomes of the modified norwood procedure: a 10-year single-institution experience.

Background: This study reviews our 10-year experience with the modified Norwood procedure to determine its early and midterm outcomes. The focus is on the impact of evolving management strategies and accumulated institutional experience.

Methods: A modified Norwood operation was performed in 171 infants over a 10-year period.

Outcomes of intraoperative device closure of muscular ventricular septal defects.

Background: The surgical management of muscular ventricular septal defects (mVSD) in the small infant is a challenge particularly when multiple and associated with complex cardiac lesions. Devices for percutaneous implantation have the advantage of ease of placement and for the double umbrella designs a wide area of coverage. We reviewed our experience and clinical outcomes of intraoperative mVSD device closure for such defects in small infants.

One and one half ventricle repairs.

Patients with functionally or anatomically borderline pulmonary ventricles can be managed by a biventricular repair, a Fontan procedure, or by an intracardiac repair in association with a cavopulmonary anastomosis. The latter repair is known as a one and one half ventricle repair. Extending the limits of a biventricular repair can be associated with a high early mortality.

Total cavopulmonary connections in children with a previous Norwood procedure.

Background: Outcomes of the Fontan operation in children initially palliated with the modified Norwood procedure are incompletely defined.

Methods: From August 1993 to January 2000, 45 patients (mean age 2.6 +/- 1.

Pediatric heart transplantation: improving results in high-risk patients.

Objectives: Our institutional experience with 73 pediatric patients undergoing cardiac transplantation between January 1, 1990, and December 31, 1999, was reviewed to determine the impact of unconventional donor and recipient management protocols implemented to extend the availability of this therapy.

Methods And Results: The introduction of donor blood cardioplegic solution with added insulin was associated with a significant improvement in patient and graft survival (hazard ratio [Cox] = 0.25, P =.

Precoronary stenosis after stage I palliation for hypoplastic left heart syndrome.

We report a patient with stenosis of the native ascending aorta after palliation of hypoplastic left heart syndrome and aortic atresia. We describe the approach to diagnosis, temporary support with extracorporeal membrane oxygenation, and successful surgical reintervention. Stenosis of the native ascending aorta is an important, potentially reversible cause of acute, early postoperative ventricular dysfunction.

Independent factors associated with longevity of prosthetic pulmonary valves and valved conduits.

Objective: To evaluate the age dependence of variables predictive of pulmonary valve prosthesis replacement, we conducted the following analysis.

Methods: Retrospective analysis of 945 operations in 726 patients undergoing placement of pulmonary valve prostheses was performed. Age was identified as a strong independent predictor of valve failure.

What is the optimal age for repair of tetralogy of Fallot?

Background: Controversy regarding the timing for the repair of tetralogy of Fallot centers around initial palliation versus primary repair for the symptomatic neonate/young infant and the optimal age for repair of the asymptomatic child. We changed our approach from one of initial palliation in the infant to one of primary repair around the age of 6 months, or earlier if clinically indicated. We examined the effects of this change in protocol and age on outcomes.

Aortic valve repair for adult congenital heart disease: A 22-year experience.

Background: Aortic valve-preserving procedures have resulted in excellent outcomes in selected patients, particularly those with normal aortic valve leaflets and dilated aortic roots. However, several congenital heart lesions are associated with abnormal aortic valve leaflets. The long-term results of aortic valve repair for these lesions are not well defined.

Are bilateral superior vena cavae a risk factor for single ventricle palliation?

Background: Performing superior vena cava-to-pulmonary artery anastomosis, in the presence of bilateral superior vena cavae, can be technically challenging. Our clinical observation has been that bilateral superior vena cavae are a risk factor for poor outcome in children needing single ventricle palliation.

Methods: Detailed operative, angiographic, and follow-up data were analyzed in 39 children undergoing bilateral cavopulmonary anastomosis (b-CPA).

Interventions associated with minimal fontan mortality.

Background: The operative mortality rate for the first 400 Fontan procedures at this institution was 15% but declined to 4% for the next 100 procedures.

Methods: The cases of 100 consecutive patients receiving the Fontan procedure and associated with this change in mortality rate were reviewed to determine associations.

Results: The mortality rate in the first and second 50 patients was 16% and 0%, respectively.

A novel repair for patients with atrioventricular septal defect requiring reoperation for left atrioventricular valve regurgitation.

Objective: Left atrioventricular valve regurgitation (LAVVR) is the most frequent indication for reoperation following atrioventricular septal defect (AVSD) repair. We estimate from our experience that within 10 years of initial repair, 14% of patients undergoing repair of atrioventricular septal defect (AVSD) require reoperation for this complication. We have developed a novel leaflet augmentation technique for LAVVR which may avoid failure of conventional repair and/or the need for valve replacement.

Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.

Background: Congenital absence of the pericardium (CAP) is a rare clinical entity.

Methods: We identified from the two hospital databases all patients with isolated CAP, reviewed their data, and invited them for prospective clinical evaluation with electrocardiography, chest x-ray findings (CXR), echocardiography, and magnetic resonance imaging (MRI).

Results: Ten patients (3 males, 7 females) presented at a median age of 21 years (range, 2-53 years) with paroxysmal stabbing chest pain, largely nonexertional (9), and heart murmur with an abnormal CXR (1).

Does the degree of cyanosis affect myocardial adenosine triphosphate levels and function in children undergoing surgical procedures for congenital heart disease?

Objective: The outcome of children with cyanosis after cardiac surgical procedures is inferior to that of children who are acyanotic. Animal studies indicated detrimental effects of chronic hypoxia on myocardial metabolism and function. We studied whether the presence or the degree of cyanosis adversely affected myocardial adenosine triphosphate, ventricular function, and clinical outcome in children.

Surgical treatment of aortic arch hypoplasia in infants and children with biventricular hearts.

Background: Results of aortic arch reconstruction in the setting of biventricular physiology are well documented in the adult population, however, in children, surgical outcome of this subgroup of patients is less clear.

Methods: We studied the clinical outcomes of 37 children aged 8 days to 15 years (median 26 months), who underwent aortic arch reconstruction for arch hypoplasia from 1982 to 1997. The children were divided into three groups: Group 1 (20 patients) had isolated aortic arch lesions, Group 2 (13 patients) had associated intra-cardiac pathology yet conserving a biventricular physiology, Group 3 (4 patients) had Williams Syndrome.

Equivalent survival following cavopulmonary shunt: with or without the Fontan procedure.

Objectives: In 1992, an analysis of our experience with the cavopulmonary shunt (CPS) demonstrated equivalent long-term survival, with or without subsequent conversion to a Fontan circulation. Before 1992 (era 1) intervention was deferred until mandated by clinical deterioration. Since 1992 (era 2), timing of both CPS and Fontan was compressed in an effort to improve survival.

Autopsies in acute type A aortic dissection. Surgical implications.

Background: This study was undertaken to review autopsy findings in operative and nonoperative settings to define anatomic pathology, cause of death, and theoretical implications of operative and perfusion management strategies for acute Stanford type A dissection.

Methods And Results: Fifty autopsies for acute type A dissection performed between 1977 and 1995 were reviewed. Twenty-nine patients had no operative therapy (group A).