ABC | Volume 111, Nº5, November 2018

Original Article Mortality for Critical Congenital Heart Diseases and Associated Risk Factors in Newborns. A Cohort Study Selma Alves Valente do Amaral Lopes, 1,2 Isabel Cristina Britto Guimarães, 1 Sofia Fontes de Oliva Costa, 4 Angelina Xavier Acosta, 1 Kyoko Abe Sandes, 3 Carlos Maurício Cardeal Mendes, 2,3 Departamento de Pediatria, Faculdade de Medicina da Bahia, Universidade Federal da Bahia (UFBA), 1 Salvador, BA – Brasil Programa de Pós Graduação em Processos Interativos dos Órgãos e Sistemas, Instituto de Ciências da Saúde, Universidade Federal da Bahia (UFBA), 2 Salvador, BA - Brasil Instituto de Ciências da Saúde, Universidade Federal da Bahia (UFBA), 3 Salvador, BA – Brasil Faculdade de Medicina da Bahia, Universidade Federal da Bahia (UFBA), 4 Salvador, BA – Brasil Mailing Address: Selma Alves Valente do Amaral Lopes • Largo do Terreiro de Jesus S/N, Centro Histórico, Salvador, BA – Brazil E-mail: selma.lopes@ufba.br Manuscript received March 05, 2018, revised manuscript May 23, 2018, accepted June 06, 2018 DOI: 10.5935/abc.20180175 Abstract Background: Congenital heart diseases are the most common type of congenital defects, and account for more deaths in the first year of life than any other condition, when infectious etiologies are ruled out. Objectives: To evaluate survival, and to identify risk factors in deaths in newborns with critical and/or complex congenital heart disease in the neonatal period. Methods: A cohort study, nested to a randomized case-control, was performed, considering the Confidence Interval of 95% (95% CI) and significance level of 5%, paired by gender of the newborn and maternal age. Case-finding, interviews, medical record analysis, clinical evaluation of pulse oximetry (heart test) and Doppler echocardiogram were performed, as well as survival analysis, and identification of death-related risk factors. Results: The risk factors found were newborns younger than 37 weeks (Relative Risk - RR: 2.89; 95% CI [1.49-5.56]; p = 0.0015), weight of less than 2,500 grams (RR: 2.33 [; 95% CI 1.26-4.29]; p = 0.0068), occurrence of twinning (RR: 11.96 [95% CI 1.43-99.85]; p = 0.022) and presence of comorbidity (RR: 2.27 [95% CI 1.58-3.26]; p < 0.0001). The incidence rate of mortality from congenital heart disease was 81 cases per 100,000 live births. The lethality attributed to critical congenital heart diseases was 64.7%, with proportional mortality of 12.0%. The survival rate at 28 days of life decreased by almost 70% in newborns with congenital heart disease. The main cause of death was cardiogenic shock. Conclusion: Preterm infants with low birth weight and comorbidities presented a higher risk of mortality related to congenital heart diseases. This cohort was extinguished very quickly, signaling the need for greater investment in assistance technology in populations with this profile. (Arq Bras Cardiol. 2018; 111(5):666-673) Keywords: Heart Defects Congenital/mortality; Infant Newborn/mortality; Risk Factors; Survival Analysis. Introduction Before the age of cardiac surgery, less than 50 years ago, just over 30% of children with severe Congenital Heart Diseases (CHD) survived into adulthood. This change was due to the evolution not only in the technique of cardiac surgery, and adaptation of cardiac catheterization to newborns, but also in the anesthetic technique, as well as the improvements in neonatal and pediatric intensive care units. Thus, the countries that have organized their care network, following this evolution pattern, have been able to considerably increase survival with quality of life for children with severe CHD. In these countries, mortality from heart disease has dropped dramatically, with up to 85% of these newborns surviving adulthood. 1-3 In spite of all this progress, CHDs are related to increased fetal losses, 4 being present in up to 85% of the deaths in necropsy findings in stillbirths, newborns, and infants, 5 being the main cause of cardiac arrest up to 24 years of age, ranging from 84% in the first two years to 21% in the second decade of life. 6 In addition, CHD mortality has a great variability worldwide. Low-industrialized or developing countries, where access to health is precarious, have substantially higher mortality rates than developed countries, which are consistent with national studies. 7-9 In the statistics with more methodological strictness, it is expected that, for serious heart diseases, such as conotruncal defects, tetralogy of Fallot, transposition of large arteries, and truncus arteriosus, survival in the first year of life fluctuates from 62.8% to 79, 6%, with a worse result for truncus arteriosus. 10 For hypoplastic left heart syndrome, data are more discouraging even in the main centers, with neonatal mortality of 68%, and mortality up to 3 months of 81%, depending on the moment that this newborn is seen. The later the care in a reference center, the greater the mortality. 6,11 666