No description available.

No description available.

No description available.

Congenital Heart Defects (CHDs), structural heart defects that are present at birth, are prevalent in approximately 1% of live births. While, historically, the presence of such defects was associated with a heightened risk of mortality, advances in medicine have allowed nearly 85% of individuals with CHDs to live into adulthood. As such, many of these individuals are reaching reproductive age and are becoming pregnant. In order to understand the implications of pregnancy among women with CHDs, a literature review was undertaken in order to elucidate the conditions that may present to the mother, as a product of the underlying defect, as well as to discern the impact of a maternal CHD on the child. Pregnancy induces hemodynamic alterations, such as an increase in stroke volume and heart rate (and thus cardiac output), and brings about an increase in blood volume. In populations with CHD, these hemodynamic changes may induce particular risks to the mother, as her cardiac condition may obstruct her ability to cope to with the heightened stress on the heart. Moreover, given that the heart may not function in an ideal manner, a less than ideal environment is present in-utero. Women with CHD suffer from elevated cardiovascular and obstetric complications, with the risk of some cardiovascular events extending into the post-partum period. During pregnancy, approximately 11% of women with CHD will experience a cardiovascular complication; this risk is highest among those with complex forms of the defect. Arrhythmia, specifically, is the most common complication, and will occur in 4.5% of pregnancies. Heart failure is also frequent, occurring in 4% to 5% of pregnancies, with the highest risk presenting in those patients with Eisenmenger syndrome and those with cyanotic forms of CHD. Pulmonary edema and thrombolytic events are also present in this population with a higher prevalence than in a healthy pregnant population. In the 6-month period after delivery, 12% of women with CHD will experience a cardiac event, with arrhythmia and heart failure as the most common complications. Obstetric complications occur in approximately 5% of pregnancies among women with CHDs, with 11% experiencing premature labor, 8.4% experiencing post-partum hemorrhage and 5.5% experiencing pregnancy-induced hypertension. Miscarriage occurs in 15% of pregnancies, with a dose-response type relationship associated with the severity of the underlying defect, as miscarriage occurs in up to 66% of pregnancies in those with Fontan palliation. Premature rupture of membranes occurs in 3.5% of pregnancies, with half of these cases occurring to patients with transposition of the great arteries. Termination of pregnancy also occurs in 5% to 8% of pregnancies, given high risk of complications to both mother and fetus. While preeclampsia is expected to occur in approximately 2-3% of pregnancies, this risk does not exceed what occurs in a healthy population. With regard to the impact of maternal CHD on the fetus, events occurring to the fetus/neonate include preterm delivery, small for gestational age, respiratory distress syndrome, intraventricular hemorrhage, and neonatal death. Between 1.5% and 2% of pregnancies will terminate in fetal mortality. Premature birth is likely in 12% to 20% births, with of 8% of neonates born as small for gestational age. Moreover, 3.5% of children born to mothers with CHD will present with CHD, themselves. Long-term effects of maternal CHD are not well-described. Despite these complications, pregnancy among women with CHD is well-tolerated and only in very few cases of those presenting with severe defects, is pregnancy counter-indicated. However, in order to mitigate risks, pre-pregnancy counseling is recommended in all women with CHD, regardless of severity of the defect. Counseling should include an overview of the form of defect, any surgical or medicinal interventions undertaken in response to the defect, an echocardiography, an exercise stress test, among other evaluations. Monitoring of the pregnancy should continue throughout gestation and delivery should occur in a specialized care facility and the mother and fetus should be monitored by a consortium of cardiologists, obstetricians, anesthesiologists, midwives. Monitoring of the mother should extend into the post-partum period until any cardiovascular or hemodynamic pregnancy-related alterations have returned to normal.

Thesis (M.S.)--Boston University

Thesis (Ph. D.)--University of Hong Kong, 2007. / Title proper from title frame. Also available in printed format.

There is relatively little epidemiologic information about Ebstein anomaly (EA) ─ a rare congenital heart defect. Thus, we analyzed characteristics of EA in a geographically and ethnically diverse population. Data from the National Birth Defects Prevention Study were used to study infants born from 1997-2007 with EA. Birth prevalence and prevalence ratio (PR) estimates were derived from the number of affected infants per 10,000 live births in the catchment area. Case characteristics were examined, stratified by the presence of other cardiac and extracardiac defects. Predictive modeling using logistic regression was conducted to understand infant mortality risk factors. There were 249 cases with EA, for a birth prevalence of 0.55/10,000 live births. Other cardiac defects were present in 41.0% and extracardiac defects in 10% of cases. Prevalence was higher among multiple births compared to singletons (PR 2.41, 95% confidence interval (CI) 1.46-3.92) and preterm compared to term infants (PR 1.84, 95% CI 1.27-2.64). Compared to EA cases without other defects, those with additional defects were more likely to die (crude Odds Ratio (cOR) 4.07, 95% CI 1.71-9.93) or undergo cardiac surgery (cOR 6.06, 95% CI 2.78-13.49). Risk for death during infancy was increased by being small for gestational age (adjusted (a) OR 2.97, 95% CI 1.13-7.76) and having extracardiac defects (aOR 6.31, 95% CI 2.28-17.52). Some findings are consistent with previous work, but further studies of EA could clarify risk factors for occurrence and mortality. Knowing population characteristics could guide development of prevention strategies and may improve clinical care.

published_or_final_version / Medicine / Master / Master of Medical Sciences

No description available.

Children with Down Syndrome (DS) experience cognitive delays with language being one of the most impaired domains. Exploring the effects of congenital heart defects (CHD), hospitalization, hearing impairment, and parental concern can provide a more precise view of factors affecting language development. Participants were 49 children with DS, 22 to 54 months of age. Expressive and receptive vocabulary size was obtained using a word count with the MacArthur Communication Development Inventory (MCDI). Medical information was obtained from the child’s medical file. Results showed expressive vocabulary was marginally significantly different between children with DS and no CHD, a CHD that did not require surgery, and a CHD that did require surgery, such that children with a CHD requiring surgery had the smallest vocabulary. Children had significantly more health problems when they had a CHD that required surgery. Expressive and receptive vocabularies were significantly smaller for children with hearing impairment.