Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection.

Background: A superior cavopulmonary connection is commonly performed before the Fontan procedure in patients with a functionally univentricular heart. Data are limited regarding associations between a prior superior cavopulmonary connection and functional and ventricular performance late after the Fontan procedure.

Methods: We compared characteristics of those with and without prior superior cavopulmonary connection among 546 subjects enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study.

Late status of Fontan patients with persistent surgical fenestration.

Objectives: This study was undertaken to determine the effects of creating a systemic-to-pulmonary venous atrial-level communication (fenestration) at the time of the Fontan procedure on late outcomes.

Background: Fenestrations are frequently performed during Fontan procedures, but late consequences are not well described.

Methods: Patient characteristics were compared between those with and without surgical fenestration among 536 subjects (mean age 11.

The impact of prenatal diagnosis of complex congenital heart disease on neonatal outcomes.

Prenatal diagnosis of congenital heart disease (CHD) is increasingly common. However, the current impact of prenatal diagnosis on neonatal outcomes is unclear. Between January 2004 and January 2008, a retrospective chart review of infants who underwent surgical repair of CHD before discharge at our institution was conducted.

Laboratory measures of exercise capacity and ventricular characteristics and function are weakly associated with functional health status after Fontan procedure.

Background: Patients after the Fontan procedure are at risk for suboptimal functional health status, and associations with laboratory measures are important for planning interventions and outcome measures for clinical trials.

Methods And Results: Parents completed the generic Child Health Questionnaire for 511 Fontan Cross-Sectional Study patients 6 to 18 years of age (61% male). Associations of Child Health Questionnaire Physical and Psychosocial Functioning Summary Scores (FSS) with standardized measurements from prospective exercise testing, echocardiography, magnetic resonance imaging, and measurement of brain natriuretic peptide were determined by regression analyses.

A new diagnostic algorithm for assessment of patients with single ventricle before a Fontan operation.

Objectives: Cardiac catheterization has a low diagnostic yield before a Fontan operation, and magnetic resonance imaging and computed tomography are reliable alternatives to invasive angiography. A new diagnostic algorithm to avoid cardiac catheterization in "low-risk" subjects before a Fontan operation is proposed.

Methods: The proposed algorithm would identify "high-risk" subjects on the basis of risk factors on medical history, echocardiography, and noninvasive angiography.

Atherosclerosis in survivors of Kawasaki disease.

Objectives: To test the hypothesis that long-term survivors of low-risk Kawasaki disease (KD) have ongoing vascular inflammation and dysfunction and a higher risk of accelerated atherosclerosis than healthy control subjects.

Study Design: Twenty-eight patients with KD (7-20 years after acute illness) and 27 age-matched healthy control subjects were examined for medical and dietary history, serum markers of atherosclerotic risk and inflammation, carotid intimal-medial thickness (CIMT) with vascular ultrasound scanning and arterial stiffness with applanation tonometry.

Results: Patients and control subjects were similar in age, sex, body mass index, waist-to-hip ratio, blood pressure, cigarette smoking, family history, diet, high-density lipoprotein cholesterol level, lipoprotein (a) level, homocysteine level, glucose level, insulin level, CIMT, arterial stiffness, C-reactive protein level, and inflammatory cytokine level.

Functional state following the Fontan procedure.

Background: Despite improvements in outcomes after completion of the Fontan circulation, long-term functional state varies. We sought to identify pre- and postoperative characteristics associated with overall function.

Methods And Results: We analyzed data from 476 survivors with the Fontan circulation enrolled in the Pediatric Heart Network Fontan Cross-sectional Study.

Incidence of aortic root dilatation in pectus excavatum and its association with Marfan syndrome.

Objective: To investigate the incidence of aortic root dilatation in pectus excavatum.

Design: Retrospective medical record review and echocardiographic reanalysis.

Setting: Morgan Stanley Children's Hospital of New York-Presbyterian.

Contemporary outcomes after the Fontan procedure: a Pediatric Heart Network multicenter study.

Objectives: We characterized a large cohort of children who had a Fontan procedure, with measures of functional health status, ventricular size and function, exercise capacity, heart rhythm, and brain natriuretic peptide (BNP).

Background: The characteristics of contemporary Fontan survivors are not well described.

Methods: We enrolled 546 children (age 6 to 18 years, mean 11.

Parental understanding of neonatal congenital heart disease.

Background: This study aimed to evaluate the impact of prenatal diagnosis on parental understanding of congenital heart disease (CHD) in newborns.

Methods: Consenting parents of newborns with CHD answered questions about the cardiac lesion, surgical repair, follow-up management, risk for CHD in future children, and maternal education before neonatal intensive care unit (NICU) discharge. A total understanding score was calculated (0-10) as the sum of five subscores: physician score, CHD score, surgery score, follow-up score, and reproduction score.

Coronary artery involvement in children with Kawasaki disease: risk factors from analysis of serial normalized measurements.

Background: Most studies of coronary artery involvement and associated risk factors in Kawasaki disease have used the Japanese Ministry of Health dichotomous criteria. Analysis of serial normalized artery measurements may reveal a broader continuous spectrum of involvement and different risk factors.

Methods And Results: Clinical, laboratory, and echocardiographic measurements obtained at baseline and 1 week and 5 weeks after presentation were examined in 190 Kawasaki disease patients as part of a clinical trial of primary therapy with pulse steroids in addition to standard intravenous immunoglobulin.

Closure of unroofed coronary sinus with a covered stent in a symptomatic infant.

A covered stent was used for percutaneous closure of an unroofed coronary sinus in an infant with congestive heart failure secondary to increased pulmonary flow. Prior to the stent deployment, the location of the entrance of the coronary vein into the coronary sinus was demonstrated with a selective left coronary artery angiogram to facilitate placement of the stent. The procedure was well tolerated without complications.

Frequency of development of aortic cuspal prolapse and aortic regurgitation in patients with subaortic ventricular septal defect diagnosed at <1 year of age.

The natural history of aortic cuspal prolapse and aortic regurgitation (AR), studied most commonly in subpulmonic ventricular septal defect (VSD), has not been well defined in isolated, unrepaired VSD diagnosed during infancy. This study aimed to define the incidence and progression of aortic cuspal prolapse and AR in patients with subaortic VSDs diagnosed at <1 year of age who had no aortic cuspal prolapse or AR at presentation and did not require surgery within the first year of life. Patients had yearly follow-up, and data regarding clinical course, physical examination, and echocardiography were obtained.

Spontaneous regression of left ventricular dilation in children with restrictive ventricular septal defects.

Objectives: To test the hypothesis that left ventricular (LV) dilation associated with pressure-restrictive ventricular septal defect (VSD) often remains stable or regresses spontaneously, calling into question the role of interventional management for such defects.

Study Design: We analyzed 96 serial echocardiograms from 33 unoperated patients with a moderate-to-large VSD with LV dilation (LV end-diastolic dimension [LVED] z score >2.0) at enrollment who were followed for more than 2 years.

Beta-blocker therapy does not alter the rate of aortic root dilation in pediatric patients with Marfan syndrome.

Objective: To test the hypothesis that chronic beta-blocker therapy in pediatric patients with Marfan syndrome alters the rate of aortic root dilation. Beta-blockade has been advocated as preventive therapy for Marfan syndrome based on reports indicating a decreased rate of aortic root dilation in treated patients.

Study Design: Patients with Marfan syndrome (n = 63) followed at Children's Hospital of Pittsburgh or Children's Hospital of New York-Presbyterian who had > or =18 months of echocardiographic follow-up were studied.

Anomalous right coronary artery arising from the pulmonary artery: a report of 7 cases and a review of the literature.

Objective: To determine the experience with anomalous right coronary artery arising from the pulmonary artery (ARCAPA) at our institution.

Background: Anomalous right coronary artery from the pulmonary artery is a rare congenital defect affecting 0.002% of the population.

The effect of beta-blocker therapy on clinical outcome in patients with Marfan's syndrome: a meta-analysis.

Objective: To assess the effect of beta-blockade therapy on clinical outcome in patients with Marfan's syndrome.

Background: Despite the lack of definitive evidence to support its efficacy, beta-blocker therapy is widely used prophylactically in patients with Marfan's syndrome.

Methods: A meta-analysis was instituted, which included studies identified by a systematic review of MEDLINE of peer-reviewed publications and by abstracts from annual scientific meeting.

Report of the National Heart, Lung, and Blood Institute Working Group on research in adult congenital heart disease.

The Working Group on research in adult congenital heart disease (ACHD) was convened in September 2004 under the sponsorship of National Heart, Lung, and Blood Institute (NHLBI) and the Office of Rare Diseases, National Institutes of Health, Department of Health and Human Services, to make recommendations on research needs. The purpose of the Working Group was to advise the NHLBI on the current state of the science in ACHD and barriers to optimal clinical care, and to make specific recommendations for overcoming those barriers. The members of the Working Group were chosen to provide expert input on a broad range of research issues from both scientific and lay perspectives.

Ross procedure in infants and toddlers followed into childhood.

Background: The Ross procedure is commonly used to treat aortic valve disease in pediatric and adult patients. For infants, data are limited regarding survival, reintervention, autograft growth, and function.

Methods And Results: The Ross procedure was performed in 27 infants <18 months of age (median age 5.

Pulmonary position cryopreserved homografts: durability in pediatric Ross and non-Ross patients.

Objective: The purpose of this study was to evaluate the outcome and risk factors for implant failure in pediatric patients who underwent pulmonary position homograft placement for right ventricular outflow tract obstruction compared with conduit placement as a component of the Ross operation. Actuarial 5-year survivals for cryopreserved right ventricle-to-pulmonary artery homografts range from 55% to 94% at all ages. It is not known whether there is a difference in homograft durability when utilized for right ventricular outflow tract obstruction or as part of the Ross operation.