Congenital Heart Disease Is Possible ? Experience from Eight Brazilian Cities

Fundamentos: As cardiopatias congênitas são causa de alta morbimortalidade na faixa etária pediátrica. Em regiões em desenvolvimento, a falta de diagnóstico precoce pode acarretar a piora do quadro; uma busca ativa por cardiopatias congênitas pode colaborar na resolução dessa situação. Objetivo: Descrever os principais achados de uma busca ativa por cardiopatias na infância, realizada em oito cidades brasileiras do estado da Paraíba. Métodos: Estudo realizado em oito cidades brasileiras do estado da Paraíba. A busca ativa compreendeu duas fases. Na primeira, os centros de saúde locais realizaram a triagem de crianças com sintomas ou história clínica de doenças cardiovasculares. Na segunda fase, realizada uma consulta clínica com posterior realização de ecocardiograma para identificação de cardiopatias. As frequências obtidas foram analisadas. Variáveis categóricas foram comparadas utilizando-se o teste do qui-quadrado. Resultados: Atendidas 440 crianças. Destas, 192 (43,63%) apresentaram alterações ao ecocardiograma. As presenças de sopro e da síndrome de Down mostraram correlação significativa (p<0,05) com a cardiopatia congênita. A maioria dos casos era de cardiopatia congênita por shunt (64,1%), seguida pelos defeitos valvares (12,5%) e pelas cardiopatias obstrutivas acianogênicas (8,3%). Conclusão: A busca ativa realizada foi capaz de identificar um número expressivo de cardiopatias congênitas na infância, não reconhecidas previamente, e inserir esses pacientes nos serviços de saúde para o tratamento adequado, garantindo-lhes melhor qualidade de vida.


Introduction
Heart diseases in children are divided into two main groups: congenital and acquired.Congenital heart diseases affect about 1.0% of the total number of live newborns and it is an important morbidity and mortality factor in newborns 1 .Acquired heart diseases include rheumatic fever and the Kawasaki disease.The former is a serious public health problem in poor regions 2 .What both groups have in common is the need of echocardiography for diagnosing or evaluating cardiac after-pains for properly managing the patient.
Despite the importance of heart diseases in childhood, poor regions still have great difficulty in diagnosing and properly treating the patients 3 .This can be explained by the lack of specialized professionals in remote areas and the difficulties of access to health services by the population, leading to the false indication of low incidence 4 , which may influence the presence of undiagnosed congenital heart diseases in older age groups.
In this scenario, the active search for congenital heart diseases is an option, since such patients can be identified and included in the health system for proper treatment.

ABBREVIATIONS AND ACRONYMS
• ASD -atrial septal defect This study aims to describe the main findings of an active search for heart diseases in childhood in eight Brazilian cities in the state of Paraíba.

Methods
A study conducted in eight Brazilian cities in the state of Paraíba: Cajazeiras, Souza, Itaporanga, Patos, Monteiro, Esperança, Picuí e Guarabira, located between the Far East and west of the state.
The active search consisted in two phases.The first phase took place in the Health Centers of the selected cities and surrounding areas, where children with cardiovascular symptoms or history of heart disease were searched.The cardiovascular symptoms included heart murmur, dyspnea, tachycardia, syncope, cyanosis, poor weight gain, recurrent infections of the upper airways and chest pain.The history of heart disease included previous diagnosis of rheumatic fever, Kawasaki disease, congenital heart diseases and genetic syndromes.
In the second phase, a team of experts in pediatric cardiology conducted clinic visits and transthoracic echocardiograms in all patients selected in the first phase.This phase lasted eight days (one for each city, in July 2013) by a multidisciplinary team.Data were tabulated and then subjected to statistical analysis.An analysis of the frequencies obtained; for categorical data, we used the chi-square test.A p value < 0.05 was considered statistically significant.
This study was approved by the Ethics Committee in Research of the hospital complex HUOC/PROCAPE, under no.18024513.3.0000.5192and performed in accordance with Resolution CNS 466/12.

Results
In this study, 440 children were assisted.Of these, 192 (43.63%) had cardiac abnormalities on the echocardiogram (Table 1).
Most patients were male (51.8%) and the predominant age group was between 6-12 years of age (42.0%).Among the reasons for the medical appointment, it was observed that the presence of murmurs and the Down's syndrome had a sufficient statistical value (p < 0.05) to be correlated with congenital heart disease.Table 2 shows the profiles of patients according to the presence or absence of a heart disease, and Table 3 shows the reasons for the medical appointment and the presence or absence of a heart disease, of the patients studied.
Most heart diseases diagnosed were from the group with left-right shunt, followed by valve problems.Fortyfour 44 (10.0%) patients had diagnosis and surgical treatment performed before the active search.Table 4 shows the heart disease groups according to the echocardiographic findings and the period of diagnosis.
Subsequently, all clinical follow-up patients had medical appointments in their respective cities via telemedicine by pediatric cardiology specialists.Surgery patients were followed up and subsequently subjected to cardiac surgery.

Discussion
Congenital heart diseases affect approximately 1.0% of all live newborns 1 .Many regions of developing countries have difficulties in diagnosing and treating these conditions 3 .This is due to two basic factors: lack of pediatric cardiology specialists in vast areas with difficult access to health services.This is particularly serious when one considers that in the long run, congenital heart diseases may bring severe consequences, including, for example, pulmonary hypertension by hyperresistance.This condition appears, in most cases, when cardiac diseases with shunts are not corrected, leading to pulmonary overflow.This overflow, over time, leads to changes in pulmonary microvasculature, preventing pulmonary hypertension from disappearing even with surgical repair.In addition, some populations have higher risk of pulmonary hypertension and congenital heart disease at the same time, such as the Down's syndrome, for example 5 .
Other changes may result from lack of treatment of congenital heart diseases.Prolonged hypoxia, for example, may be associated with cognitive and growth impairment and exercise intolerance.Others, such as the tetralogy of Fallot, may lead to severe cyanosis.Note, therefore, that failure to diagnose congenital heart diseases in the neonatal period may lead to irreversible consequences.
The main heart diseases acquired in childhood include rheumatic fever and the Kawasaki disease.The former is a public health problem in developing countries 6 , one of the biggest causes of disability among young people.Its main cardiac side effect is mitral regurgitation; however, other valves can be affected simultaneously.
On the other hand, its prevention is simple, consisting of the administration of penicillin regularly.Despite the easy prevention, the difficulty in diagnosis, search for cardiac after-pains and treatment adherence in Brazil still make rheumatic fever a serious health problem in the population.
In this context, the lack of access to pediatric cardiologists and, more specifically, to echocardiogram tests, leads to late diagnosis or lack of follow-up of heart diseases in the childhood.Therefore, the application of an active search of these cases is important as it allows the diagnosis and the application of an appropriate management.The ideal time would be the neonatal period, but even late diagnosis allows treatment, minimizes after-pains and ensures better quality of life for the patients.
This study consists of an active search for heart diseases in childhood in eight Brazilian cities in the state of Paraíba.Initially, local services conducted a search for children at higher risk for heart diseases in childhood based on symptoms and medical history.This was necessary because these services have greater knowledge of local patients and inform them on the action.Then, a group of pediatric cardiology specialists visited each of the cities for the medical appointment and echocardiography, in order to diagnose cases of heart diseases.This was particularly relevant as it prevented hundreds of people from going to reference health services and greatly helped such patients to have access to the health services.
In the study, 440 children were treated, and abnormalities were identified in 192 (43.6%).This figure is extremely high compared to the incidence of congenital and acquired heart diseases, but this was due to the initial screening performed by the health services.Note that the cities had different rates of heart diseases, possibly due to the different quality of the initial screening leading to greater or lesser numbers of patients with heart diseases.
Another factor that may have contributed is the higher incidence of congenital heart diseases in certain regions of the state, a fact that needs to be further elucidated with further studies 7 .
Among the reasons for the medical appointment, it was observed that only the isolated factors of heart murmur and Down's syndrome had a positive correlation with the presence of congenital heart disease.Other studies showed that the presence of heart murmur is the signal mostly related to the presence of congenital heart disease 8 ; however, it is necessary to differentiate it from an innocent heart murmur, a non-pathological condition.
Approximately half of patients with the Down's syndrome have some type of congenital heart disease 9 .
In this study, this is possibly due to the initial screening.
The other reasons for the medical appointment were not statistically significant, despite a positive correlation.
Tachycardia and chest pain are symptoms reported by adolescents and, in most cases, are not related to heart disease in that age 10 .Nevertheless, cardiac causes have been excluded.Dyspnea may be present in both cardiovascular and respiratory causes.It is important to distinguish between both for the full management of the patient.The presence of cyanosis or cyanosis crisis is usually related to serious heart problems.However, the term "turn purple" can be confused with the coloring from temper tantrums, for example.Even so, the correlation between history of cyanosis and congenital heart disease was close to the significance value (p=0.057).
Approximately 32.25% of patients with history of rheumatic fever had cardiac after-pains.Thus, it was observed that there is a failure inherent in the prevention of cardiac after-pains in rheumatic fever in this population.
In this study, 192 children showed abnormalities on echocardiogram.Of these, 44 (22.91%) had diagnoses and treatments before the active search, totaling 148 newly diagnosed cases.Most abnormalities (n=124) were by shunt heart disease, which are the most common ones 11,12 .Atrial septal defect (ASD) is more frequent than ventricular septal defect (VSD) (most common congenital heart disease 11,12 ).Among the cases of valvular heart diseases, mitral regurgitation was the most frequent cardiac consequence of rheumatic fever.A higher prevalence in the non-diagnosis of valvular heart diseases and shunt was observed.It is known that these heart diseases have less obvious symptoms compared to complex heart diseases.However, non-diagnosis may lead to important consequences in the long term, which makes them important in terms of public health.
Hence, the active search for heart diseases in childhood has a good impact in regions with diagnostic difficulties in the neonatal stage.The search for patients with cardiovascular symptoms is a good initial screening method, especially with the collaboration of local services.More important than the active search is the maintenance of patient care and the inclusion of this patient in the health services, thus ensuring better quality of life and minimization of the deleterious effects of heart disease in childhood.

Conclusion
The active search was able to identify a significant number of congenital heart diseases in childhood not previously recognized, and it included these patients in the health services for appropriate treatment, ensuring them a better quality of life.

Potential Conflicts of Interest
No relevant potential conflicts of interest.

Sources of Funding
This study had no external funding sources.

Academic Association
This study is not associated with any graduate programs.

Table 3 Reasons for the medical appointment and presence or absence of heart disease in the population studied Reasons for the medical appointment Tests p-value Odds ratio (CI 95%) Normal Abnormal
URTI -upper respiratory tract infection