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  2. Keyword: Hypoplastic Left Heart Syndrome
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IJCS
Original Article

Associated Factors with Congenital Heart Disease in the Most Populated State of Brazil Between 2010 and 2018

Isabela Madruga et al. Tatiane Dunder de Moraes Caroline Prado Carlos Eduardo Slateff Baldini Alfésio Luís Ferreira Braga

Abstract

Background

Cardiac anomalies are the most prevalent congenital malformations among live births in the world. In Brazil, it is estimated that nearly 25,757 new cases occur each year, and the Southeast region presents the highest prevalence, with 10 new cases/1,000 live births.

Objective

The aim of this study is to evaluate the epidemiology of congenital heart disease (CHD) in the state of São Paulo.

Methods

This is a cross-sectional and time-series observational study with data from the Brazilian Information System on Live Births. Heart-related cardiac malformation cases — International Classification of Diseases (ICD) 10th revision Q20.0 to Q24.9 — were selected from January 2010 to December 2018, in the state of São Paulo, Brazil. This study analyzed rates of malformations per year and investigated associated factors, using single and multiple logistic regression models. The significance level adopted in this study was 5%.

Results

The highest cardiac malformation rate was in the São Paulo metropolitan region (2.84:1000), while the lowest was found in the region of Franca (0.3:1000). The most frequent defect was interatrial communication (38.2%). The main associated factors observed in this study were prematurity of 22 to 27 weeks (OR=4,401 95% CI: 3,796-5,104) — CI: Confidence Interval; OR: Odds ratio —, mother’s age between 35 and 49 years of age (OR=1,602 95% CI: 1,525-1,682), yellow race (OR=1,481 95% CI: 1,235-1,775), triple or more pregnancy (OR=1,438 95% CI: 1,004-2,060), and history of a dead child (OR=1,213 95% CI: 1,152-1,277).

Conclusion

The main factors associated with this outcome, which are part of the obstetric history of mothers, should be addressed and considered when pregnancy is a planned event.

  • Congenital Heart Disease
  • Epidemiology
  • Hypoplastic Left Heart Syndrome
  • Interatrial Block
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Creative Commons Attribution (CC BY) Creative Commons license
03/03/2023 DOI: 10.36660/ijcs.20210283 Views 2
Figure 1
ABC
Original Article

Early Outcomes of the Norwood Procedure in a Reference Center in Brazil

Rodrigo Freire Bezerra et al. Juliana Torres Pacheco Sônia Meiken Franchi Rosangela Belbuche Fittaroni … José Francisco Baumgratz Rodrigo Moreira Castro Luciana da Fonseca da Silva José Pedro da Silva

Abstract

Background

Only two papers have addressed the early outcomes of patients with hypoplastic left heart syndrome (HLHS) undergoing the Norwood operation, in Brazil.

Objectives

We evaluated patients with HLHS undergoing the first-stage Norwood operation in order to identify the predictive factors for early (within the first 30 days after surgery) and intermediate (from early survival up to the Glenn procedure) mortality.

Methods

Patients with HLHS undergoing the stage I Norwood procedure from January 2016 through April 2019, in our service, were enrolled. Demographic, anatomical, and surgical data were analyzed. Endpoints were early mortality (within the first 30 days after surgery), intermediate mortality (from early survival up to the Glenn procedure) and the need for postoperative ECMO support. Univariate and multivariate analyses were performed, and odds ratios, with 95% confidence intervals, were calculated. A p-value <0.05 was considered statistically significant.

Results

A total of 80 patients with HLHS underwent the stage I Norwood procedure. The 30-day survival rate was 91.3% and the intermediate survival rate 81.3%. Fourteen patients (17.5%) required ECMO support. Lower weight (p=0.033), aortic stenosis (vs aortic atresia; p=0.036), and the need for postoperative ECMO support (p=0.009) were independent predictive factors for 30-day mortality. Mitral valve stenosis (vs mitral valve atresia; p=0.041) was an independent predictive factor for intermediate mortality.

Conclusion

The present study includes the largest Brazilian cohort of patients with HLHS undergoing the stage I Norwood procedure in the recent era. Our survival rates were comparable to the highest survival rates reported globally. Low body weight, aortic valve stenosis, and the need for postoperative ECMO support were independent predictors for 30-day mortality. Mitral valve stenosis was the only independent predictive factor for intermediate mortality.

  • Extracorporeal Membrane Oxygenation
  • Hypoplastic Left Heart Syndrome
  • Mortality
  • Norwood Procedures
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Creative Commons Attribution (CC BY) Creative Commons license
10/06/2022 DOI: 10.36660/abc.20201226
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ABC
Research Letter

Left Heart Hypoplasia Evolving Up to 21 Years, After Total Cavopulmonary Operation Performed at 5 Years of Age

Edmar Atik

Introduction

Hypoplasia of the left heart is characterized by very small dimensions of the left cardiac cavities, represented by the left atrium, mitral valve, left ventricle, aortic ring and ascending aorta. Systemic circulation thus becomes dependent on the ductus arteriosus with reverse flow from the hypertensive pulmonary artery to the descending aorta, and also to the ascending aorta. Coronary circulation is also dependent on this reverse flow, which is why there is a high chance of myocardial infarction, even in the first days of life. It is easy to imagine that, on decreased pulmonary arterial pressure or ductus arteriosus caliber, there is an unfavorable evolution towards low cardiac output. The right heart is enlarged and atrial septal defect invariably accompanies this defect. When this atrial defect appears to be of reduced dimensions, known as restrictive, it causes retrograde pulmonary hypertension. On the other hand, when larger, it may favor the faster involution of pulmonary hypertension, with congestive symptoms and low output. In the rare cases of intact atrial septum, anomalous pulmonary vein drainage remains in order of maintenance and viability of circulation. Left heart hypoplasia is the fourth most common heart disease in the neonatal period, with high mortality, even in this period, and corresponds to 1.5% of all congenital heart diseases.

How it is externalized and evolves: Dynamic instability favors the advent of very early signs and symptoms in life, in the first hours or days of life. These signs are characterized by congestive condition (with large atrial defect) or low systemic output (decreased pulmonary arterial pressure and/or constriction of the ductus arteriosus), with pallor, dyspnea, discomfort, irritability and variable cyanosis. The most favorable picture corresponds to the one with restrictive atrial septal defect, which, in view of the maintenance of pulmonary hypertension, the cardiac output is maintained at a satisfactory level.

[…]

  • Cardiac Surgical Procedures
  • Fontan Procedure
  • Hypoplastic Left Heart Syndrome
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Creative Commons Attribution (CC BY) Creative Commons license
26/07/2021 DOI: 10.36660/abc.20201012 Views 1
ABC
Editorial

Mortality for Critical Congenital Heart Diseases and Associated Risk Factors in Newborns. A Cohort Study

Andressa Mussi Soares

Congenital heart disease (CHD) is any change in the anatomy of the heart and its blood vessels that occurs within the first 8 weeks of gestation. The manifestation of CHD is very variable and may occur soon after birth or appear later in childhood or adolescence.

The incidence of CHD is 8 to 10 per 1000 live births, or one in one hundred births. In Brazil, 28,900 children are born with CHD per year (1% of the total birth), of which about 80% (23,800) need cardiac surgery, and half of them need to be operated in the first year of life.

[…]

  • Health Programs and Plans
  • Heart Defects, Congenital/physiopathology
  • Heart Defects, Congenital/surgery
  • Hypoplastic Left Heart Syndrome
  • Infant,Newborn
  • Maternal and Child Health
  • Mortality
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Creative Commons Attribution (CC BY) Creative Commons license
01/11/2018 DOI: 10.5935/abc.20180203 Views 1

How to cite this article

EndNote RIS

Electronic Document Format (Vancouver)

Madruga I, Moraes TD, Prado C, Baldini CES, Braga ALF. Associated Factors with Congenital Heart Disease in the Most Populated State of Brazil Between 2010 and 2018. International Journal of Cardiovascular Sciences 2023;36:e20210283.

Electronic Document Format (ABNT)

Madruga, Isabela; Moraes, Tatiane Dunder de; Prado, Caroline; Baldini, Carlos Eduardo Slateff; Braga, Alfésio Luís Ferreira. Associated Factors with Congenital Heart Disease in the Most Populated State of Brazil Between 2010 and 2018. International Journal of Cardiovascular Sciences, v. 36, e20210283, Mar. 2023.

Electronic Document Format (APA)

Madruga, I., Moraes, T. D. , Prado, C., Baldini, C. E. S., & Braga, A. L. F. (2023). Associated Factors with Congenital Heart Disease in the Most Populated State of Brazil Between 2010 and 2018. International Journal of Cardiovascular Sciences, 36, e20210283.

Electronic Document Format (ISO)

Madruga, Isabela and Moraes, Tatiane Dunder de and Prado, Caroline and Baldini, Carlos Eduardo Slateff and Braga, Alfésio Luís Ferreira. Associated Factors with Congenital Heart Disease in the Most Populated State of Brazil Between 2010 and 2018. International Journal of Cardiovascular Sciences [online]. 2023, vol. 36, [cited 2026-10-14], e20210283. Available from: <https://journals.cardiol.br/en/article/associated-factors-with-congenital-heart-disease-in-the-most-populated-state-of-brazil-between-2010-and-2018/>. ISSN 2359-4802

EndNote RIS

Electronic Document Format (Vancouver)

Bezerra RF, Pacheco JT, Franchi SM, Fittaroni RB, Baumgratz JF, Castro RM, Silva LF, et al. Early Outcomes of the Norwood Procedure in a Reference Center in Brazil. Arquivos Brasileiros de Cardiologia 2022;119(2):282-91.

Electronic Document Format (ABNT)

Bezerra, Rodrigo Freire; Pacheco, Juliana Torres; Franchi, Sônia Meiken; Fittaroni, Rosangela Belbuche; Baumgratz, José Francisco; Castro, Rodrigo Moreira; Silva, Luciana da Fonseca da; Silva, José Pedro da. Early Outcomes of the Norwood Procedure in a Reference Center in Brazil. Arquivos Brasileiros de Cardiologia, v. 119, n. 2, p. 282-291, Jun. 2022.

Electronic Document Format (APA)

Bezerra, R. F., Pacheco, J. T., Franchi, S. M., Fittaroni, R. B., Baumgratz, J. F., Castro, R. M., Silva, L. F. , & Silva, J. P. (2022). Early Outcomes of the Norwood Procedure in a Reference Center in Brazil. Arquivos Brasileiros de Cardiologia, 119(2), 282-291.

Electronic Document Format (ISO)

Bezerra, Rodrigo Freire and Pacheco, Juliana Torres and Franchi, Sônia Meiken and Fittaroni, Rosangela Belbuche and Baumgratz, José Francisco and Castro, Rodrigo Moreira and Silva, Luciana da Fonseca da and Silva, José Pedro da. Early Outcomes of the Norwood Procedure in a Reference Center in Brazil. Arquivos Brasileiros de Cardiologia [online]. 2022, vol. 119, n. 2, [cited 2026-10-14], pp. 282-291. Available from: <https://journals.cardiol.br/en/article/early-outcomes-of-the-norwood-procedure-in-a-reference-center-in-brazil/>. ISSN 0066-782X

EndNote RIS

Electronic Document Format (Vancouver)

Atik E. Left Heart Hypoplasia Evolving Up to 21 Years, After Total Cavopulmonary Operation Performed at 5 Years of Age. Arquivos Brasileiros de Cardiologia 2021;117(1):142-5.

Electronic Document Format (ABNT)

Atik, Edmar. Left Heart Hypoplasia Evolving Up to 21 Years, After Total Cavopulmonary Operation Performed at 5 Years of Age. Arquivos Brasileiros de Cardiologia, v. 117, n. 1, p. 142-145, Jul. 2021.

Electronic Document Format (APA)

Atik, E. (2021). Left Heart Hypoplasia Evolving Up to 21 Years, After Total Cavopulmonary Operation Performed at 5 Years of Age. Arquivos Brasileiros de Cardiologia, 117(1), 142-145.

Electronic Document Format (ISO)

Atik, Edmar. Left Heart Hypoplasia Evolving Up to 21 Years, After Total Cavopulmonary Operation Performed at 5 Years of Age. Arquivos Brasileiros de Cardiologia [online]. 2021, vol. 117, n. 1, [cited 2026-10-14], pp. 142-145. Available from: <https://journals.cardiol.br/en/article/left-heart-hypoplasia-evolving-up-to-21-years-after-total-cavopulmonary-operation-performed-at-5-years-of-age/>. ISSN 0066-782X

EndNote RIS

Electronic Document Format (Vancouver)

Soares AM. Mortality for Critical Congenital Heart Diseases and Associated Risk Factors in Newborns. A Cohort Study. Arquivos Brasileiros de Cardiologia 2018;111(5):674-5.

Electronic Document Format (ABNT)

Soares, Andressa Mussi. Mortality for Critical Congenital Heart Diseases and Associated Risk Factors in Newborns. A Cohort Study. Arquivos Brasileiros de Cardiologia, v. 111, n. 5, p. 674-675, Oct. 2018.

Electronic Document Format (APA)

Soares, A. M. (2018). Mortality for Critical Congenital Heart Diseases and Associated Risk Factors in Newborns. A Cohort Study. Arquivos Brasileiros de Cardiologia, 111(5), 674-675.

Electronic Document Format (ISO)

Soares, Andressa Mussi. Mortality for Critical Congenital Heart Diseases and Associated Risk Factors in Newborns. A Cohort Study. Arquivos Brasileiros de Cardiologia [online]. 2018, vol. 111, n. 5, [cited 2026-10-14], pp. 674-675. Available from: <https://journals.cardiol.br/en/article/mortality-for-critical-congenital-heart-diseases-and-associated-risk-factors-in-newborns-a-cohort-study-2/>. ISSN 0066-782X

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