OBJECTIVE:To determine the frequence and profile of congenital heart defects in Down
syndrome patients referred to a pediatric cardiologic center, considering the age
of referral, gender, type of heart disease diagnosed by transthoracic
echocardiography and its association with pulmonary hypertension at the initial
diagnosis. METHODS:Cross-sectional study with retrospective data collection of 138 patients with Down
syndrome from a total of 17,873 records. Descriptive analysis of the data was
performed, using Epi-Info version 7. RESULTS: Among the 138 patients with Down syndrome, females prevailed (56.1%) and 112
(81.2%) were diagnosed with congenital heart disease. The most common lesion was
ostium secundum atrial septal defect, present in 51.8%,
followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects
were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases.
Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was
associated with 37.5% of the heart diseases. Only 35.5% of the patients were
referred before six months of age. CONCLUSIONS: The low percentage of referral until six months of age highlights the need for a
better tracking of patients with Down syndrome in the context of congenital heart
disease, due to the high frequency and progression of pulmonary hypertension.
Background: Down's syndrome (DS) affects one per 700 live births and congenital heart disease (CHD) occurs in 40-60% of these patients. Contributing factors to the association between DS and CHD are being unraveled. Gender could be one of them. Methods: We performed a meta-analysis of CHD prevalence in DS, separated by gender. Three search engines were used and 578 articles were reviewed. Twelve articles were included. Results: Quantitative analysis showed a higher prevalence of CHD, particularly atrioventricular septal defects (AVSD), in female patients. No differences were found in others forms of CHD. Conclusion: CHD, particularly AVSD, are more common in the female gender of Down's syndrome patients.
These findings suggest that the use of height-based equations may be a simple and feasible approach to improve the detection of high BP in the pediatric population.
ProblemProviding health care for children with congenital heart diseases remains a major challenge in low- and middle-income countries.ApproachIn October 2011, the Government of Paraíba, Brazil, established a paediatric cardiology network in partnership with the nongovernmental organization Círculo do Coração. A cardiology team supervised all network activities, using the Internet to keep in contact with remote health facilities. The network developed protocols for screening heart defects. Echocardiograms were performed by physicians under direct online supervision of a cardiologist; alternatively, a video recording of the examination was subsequently reviewed by a cardiologist. Cardiovascular surgeons came to a paediatric hospital in the state capital once a week to perform heart surgeries.Local settingUntil 2011, the State of Paraíba had no structured programme to care for children with heart disease. This often resulted in missed or late diagnosis, with adverse health consequences for the children.Relevant changesFrom 2012 to 2014, 73 751 babies were screened for heart defects and 857 abnormalities were identified. Detection of congenital heart diseases increased from 4.09 to 11.62 per 1000 live births (P < 0.001). Over 6000 consultations and echocardiograms were supervised via the Internet. Time to diagnosis, transfers and hospital stays were greatly reduced. A total of 330 operations were carried out with 6.7% (22/330) mortality.Lessons learntAccess to an echocardiography machine with remote supervision by a cardiologist improves the detection of congenital heart disease by neonatologists; virtual outpatient clinics facilitate clinical management; the use of Internet technology with simple screening techniques allows resources to be allocated more efficiently.
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