Tetralogy Of Fallot Variant Research Articles

Impact of Underweight, Overweight, and Obesity on Health-Related Quality of Life in Children with Tetralogy of Fallot Variants.

Traditional cardiovascular risk factors put patients with congenital heart disease (CHD) at increased risk for cardiovascular morbidity and mortality. The aim of this study was to evaluate whether body mass index (BMI) is associated with health-related quality of life (HRQoL) in patients with variants of Tetralogy of Fallot (TOF). Patients and parents of children with variants of TOF-CHD were asked to fill out the PedsQL 4.0 questionnaire and provide weight and length. Patients were categorized into low, normal, and high BMI percentiles. Other demographic data were obtained from the Swedish national registry for congenital heart disease (SWEDCON). Statistical analyses included non-parametric Mann-Whitney U test, Fisher exact, and Chi-square tests. Eighty-five patients were included. Twelve were overweight or obese, 57 had a normal BMI, and 16 were underweight. There was a significant difference in age and gender between the groups. Comparing overweight/obese children to those with normal BMI, physical and social functioning were impaired, while emotional and school function were comparable between the groups. This applied to both child and parental assessment. When comparing underweight to normal weight children, school functioning assessed by the parent was the only domain significantly different from patients with a normal BMI. Children with variants of TOF and overweight/obesity have lower HRQoL, particularly in physical and social functioning, while underweight children may have impaired school functioning. We suggest that preventive measures aimed at maintaining a normal weight should be taken early in life to reduce long-term cardiovascular risk in the CHD population.

Validity of Pulmonary Valve Z-Scores in Predicting Valve-Sparing Tetralogy Repairs-Systematic Review †.

There is a lack of consensus regarding the preoperative pulmonary valve (PV) Z-score “cut-off” in tetralogy of Fallot (ToF) patients to attempt a successful valve sparing surgery (VSS). Therefore, the aim of this study was to review the available evidence regarding the association between preoperative PV Z-score and rate of re-intervention for residual right ventricular outflow tract (RVOT) obstruction, i.e. successful valve sparing surgery. A systematic search of studies reporting outcomes of VSS for ToF was performed utilizing PubMed, EMBASE, and Scopus databases. Patients with ToF variants such as pulmonary atresia, major aortopulmonary collaterals, absent pulmonary valve, associated atrioventricular septal defect, and discontinuous pulmonary arteries were excluded. Out of 712 screened publications, 15 studies met inclusion criteria. A total of 1091 patients had surgery at a median age and weight of 6.9 months and 7.2 kg, respectively. VSS was performed on the basis of intraoperative PV assessment in 14 out of 15 studies. The median preoperative PV Z-score was −1.7 (0 to −4.9) with a median re-intervention rate of 4.7% (0–36.8%) during a median follow-up of 2.83 years (1.4–15.8 years). Quantitatively, there was no correlation between decreasing preoperative PV Z-scores and increasing RVOT re-intervention rates with a correlation coefficient of −0.03 and an associated p-value of 0.91. In observational studies, VSS for ToF repair was based on intraoperative evaluation and sizing of the PV following complete relief of all levels of obstruction of the RVOT, rather than pre-operative echocardiography derived PV Z-scores.

315 One-stage percutaneous Melody pulmonary valve implantation: a single-center experience in 21 consecutive patients

Right ventricular outflow tract (RVOT) reconstruction in infancy or childhood leads to lifetime iterative surgery for pulmonary stenosis or regurgitation. Transcatheter pulmonary valve implantation (TPVI) was developed with the aim to reduce the number of reoperations and operative-related morbidity. From 12/2008 to 03/2010, 21 patients were referred to Marie-Lannelongue Hospital for TPVI. Mean age was 24 yrs (11.5–55 yrs), mean weight was 60 kg (20–85 kg). Most of them had a Tetralogy of Fallot variant (n = 12) and 70% had 3 or more previous surgery. RVOT stenosis was predominant in 90% of patients. RVOT reconstruction techniques were: valved conduit (n = 12), non-valved conduit (n = 1), homograft (n = 6), RVOT patch (n = 2). RVOT balloon inflation test was performed only if cardiac CT scan showed coronary arteries located next to the RVOT. Two patients were excluded, 1 because an aorto-pulmonary fistula after Ross operation, and 1 with previous arterial switch because of compression of the reimplanted left coronary artery at RVOT balloon inflation test. Initial stenting and TPVI were performed during the same procedure in 18/19. There was immediate significant decrease in right ventricular systolic pressure (mean 88.6 to 52.2 mmHg), mean RVOT gradient (mean 43.7 to 22 mmHg) and pulmonary regurgitation (grade 2 or more before, none after). One patient with a 16 mm homograft had significant residual stenosis (mean RVOT gradient 45 mmHg) after Melody implantation. All patients were discharged within 2 days. At a mean follow-up of 9.3 months (1 to 24 months) all patients are alive and well and neither Melody valve dysfunction nor stent fracture or migration was noticed. Percutaneous pulmonary valve implantation is a safe alternative to surgery. Our experience suggests that patients with RVOT conduit < 18 mm and those with previous arterial switch operation should be referred to surgery without considering transcatheter procedure.

Tracheostomy after pediatric cardiac surgery: Frequency, indications, and outcomes

This study was designed to review baseline characteristics and outcomes of children requiring tracheostomy after cardiac surgery. A retrospective review of children under age 2 requiring tracheostomy after cardiac surgery between January 1999 and December 2005 was performed. Indications for tracheostomy, survival, and completion of staged palliation were documented. After cardiac surgery, 59 (1.3%) of 4503 patients with a median age at surgery of 15 days and weight of 3.5 kg required tracheostomy. Median duration from surgery to tracheostomy was 36 days (range 10-145 days). Genetic syndromes or major noncardiac comorbidities were present in 40% of patients. Biventricular repair was performed in 34 patients and univentricular repair in 25. Tetralogy of Fallot variants (29%) and coarctation±ventricular septal defect (21%) constituted the majority of biventricular lesions associated with tracheostomy, whereas unbalanced atrioventricular septal defect and hypoplastic left heart syndrome with highly restrictive atrial septal defect accounted for 52% of the single ventricle group. Indications for tracheostomy included the following: multifactorial (37%), tracheobronchomalacia, (24%), cardiac (12%), bilateral vocal cord paralysis (10%), bilateral diaphragm paralysis (2%), and other airway issues (15%). Hospital survival was 75% with intermediate-term (median, 25.5 months; range, 1-122 months) survival of 53%. Of 25 single ventricle patients, 6 (24%) had successful completion of the Fontan procedure. Of 12 patients with single ventricle who were ventilator-dependent after initial repair, 10 died, 1 remains at hemi-Fontan, and 1 has undergone completion of the Fontan procedure. Requirement for tracheostomy in pediatric patients after cardiac surgery was associated with significant mortality. Patients with single ventricle have the highest late death rate and those with chronic ventilator dependency were unlikely to undergo successful Fontan completion.

Aorta Larger Than Pulmonary Artery in the Fetal 3‐Vessel View

Fetal great artery asymmetry may accompany congenital heart disease. The fetal echocardiographic 3-vessel view (3VV) allows assessment of the superior vena cava, ascending aorta (AA), and main pulmonary artery (MPA). Our aim was to determine the association of congenital heart disease in fetuses with an AA/MPA ratio of greater than 1. We electronically searched our fetal echocardiographic database for studies performed between March 2002 and January 2008 that showed 3VVs with AA/MPA ratios of greater than 1 and correlated the findings with the presence of congenital heart disease. In 2797 fetal echocardiograms, we identified 31 fetuses with normal 4-chamber views showing AA/MPA ratios of greater than 1 in the 3VV. Of 31 fetuses, 25 (81%) had tetralogy of Fallot (ToF) or a ToF variant, and 6 (19%) had an aortic valve abnormality or isolated dilatation of the AA. Screening obstetric fetal sonography showing a 3VV AA/MPA ratio of greater than 1 suggests congenital heart disease and indicates the need for comprehensive fetal echocardiography.

Cardiac anomalies in patients with congenital diaphragmatic hernia and their prognosis: a report from the Congenital Diaphragmatic Hernia Study Group

Background/Purpose Patients with congenital diaphragmatic hernia (CDH) are known to have associated cardiac anomalies. Data from the Congenital Diaphragmatic Hernia Study Group has allowed better definition of the types of heart defects (HDs) and survival for these patients. Methods Since 1995, 2636 patients were enrolled in the Congenital Diaphragmatic Hernia Study Group from 82 centers. Patients with hemodynamically significant HD, excluding patent ductus arteriosus (PDA); patent foramen ovale (PFO); and atrial septal defect (ASD), were selected. Cardiac anatomy and survival data for all patients were reviewed. Results Two hundred eighty (10.6%) patients had significant HDs: ventricular septal defect (VSD); (42.2%), aortic arch obstruction (15%), univentricular anatomy (13.9%), tetralogy of Fallot variants (11.1%), total anomalous pulmonary venous return (3.9%), double outlet right ventricle (RV) (3.2%), pulmonary stenosis (2.5%), transposition of the great arteries (2.5%), and various other defects in 5.7%. Survival for the entire group was 67.1%; survival for patients without HD was 70.2% and for patients with HD was 41.1% ( P < .001). Patients with biventricular cardiac anatomy had a 47% survival, whereas those with univentricular anatomy had a 5% survival ( P < .001). Conclusion Significant HD is associated with 10.6% of CDH. Survival for patients with HD is significantly lower than for patients with normal cardiac anatomy. Patients with CDH and univentricular cardiac anatomy have a poor prognosis.

Craniosynostosis and congenital heart anomalies associated with a maternal deletion of 15q15‐22.1

We report an infant with multiple congenital anomalies, including craniosynostosis, tetralogy of Fallot variant, and limb anomalies associated with a maternal deletion of 15q15-22.1. Only two other patients have been reported with a similar deletion, but the deletion was paternal in both cases. We review our patient's findings and compare them to previously reported individuals with similar 15q abnormalities. Our patient allows an expansion of phenotype associated with mid-15q deletions to include severe craniosynostosis, congenital heart disease, and limb anomalies. This will assist in prenatal counseling and predicting postnatal outcome for other affected individuals. The specific breakpoints in our patient and the other patients with similar deletions may also assist in determining a critical region for suture formation.