Select Articles Published on the Topic of Congenital Heart Disease in 2013

Function, and Pregnancy Outcome in Women With Congenital Heart DiseaseSummary-Women with congenital heart disease (CHD) are not only at risk of maternal cardiovascular complications during their pregnancies, but they also have an increased risk of obstetric and offspring complications.Offspring complications such as small for gestational age and premature birth are related to maternal cardiac function, but the mechanism underlying this relationship is unknown.In this clinical study, in 209 pregnant women with CHD and 70 healthy women, we demonstrated that uteroplacental Doppler flow parameters (uterine and umbilical artery pulsatility and resistance indices) are worse in pregnant women with CHD than in healthy women and are related to maternal cardiovascular function parameters, such as right ventricular function, valvular regurgitation, heart rate, and prepregnancy need for cardiac medication.Uteroplacental flow parameters were related to offspring outcome, as is also known in the general pregnant population.Therefore, we concluded that in women with CHD, uteroplacental flow may be compromised by maternal cardiac dysfunction and that impaired uteroplacental flow may be a key factor in the high incidence of obstetric and offspring complications.Our study improves the understanding of the pathophysiology of offspring events in women with CHD and may also contribute to a better insight into the pathophysiology of offspring complications in the general population.The results of this study are of importance for counseling of women with CHD who are contemplating pregnancy and will improve risk stratification leading to more adequate monitoring of pregnancies in these women.Conclusions-UDF parameters are abnormal in pregnant women with CHD.Cardiovascular function is associated with an abnormal pattern of UDF.Compromised UDF may be a key factor in the high incidence of offspring and obstetric complications in this population.1 Physiological and Phenotypic Characteristics of Late Survivors of Tetralogy of Fallot Repair Who Are Free From Pulmonary Valve ReplacementSummary-This article provides an account of the long-term survival (>45 years of follow-up) of a large, complete, consecutive cohort of patients with repaired tetralogy of Fallot from a single UK institution.Using the functionality of a unique identifier, the National Health Service number, we were able to locate patients lost to follow-up and to establish whether they were still alive.Their detailed survival data were compared with the age-and sex-matched normal population.Collaboration with specialist centers dealing with adult congenital heart disease nationally provided corresponding incidence data relating to the changing prevalence over time of secondary pulmonary valve replacement after Fallot repair.In the second part of the study, a subset of patients were chosen at random to represent those free from subsequent pulmonary valve replacement.These patients of all ages were assessed thoroughly with conventional noninvasive imaging and functional modalities to characterize their cardiac morphology and function.The currently published literature focuses primarily on patients who need reintervention, and less is formally documented about those who are "well."We also hoped to identify those characteristics that would suggest that the initial surgical repair has proved definitive, that is, patients with normal exercise capacity who are still free from pulmonary valve replacement at 35 years of age.The morphological characteristics of these patients with what seems to be an ideal outcome may serve as guide for today's surgeons and provide a good substrate for further longitudinal studies.Conclusions-An important proportion of patients require PVR late after tetralogy of Fallot repair.Patients surviving to 35 years of age without PVR and with a normal exercise capacity may have had a definitive primary repair; their right ventricular outflow tracts are characterized by mild residual obstruction and pulmonary annulus diameter <0.5z. 2 Increased Prevalence of Congenital Heart Defects in Monozygotic and Dizygotic TwinsSummary-Half of all twins are born prematurely, and therefore twins more often than singletons experience cardiopulmonary problems such as respiratory distress syndrome and patent ductus arteriosus.In addition, previous smaller studies indicate that congenital heart defects generally may be more common in monochorionic twins (who are all monozygotic) than in dichorionic twins and singletons.We investigated congenital heart defect occurrence in all twins and 5% of all singletons born in Denmark in 1977 to 2001.Among 41 525 twin individuals, 1.4% had congenital heart defects, whereas the corresponding number for singletons was 0.87%.Patent ductus arteriosus and coarctation of the aorta occurred >3 times as often in twins as in singletons.The increased occurrence in twins was also found in sensitivity analyses including only inpatients or only