ABC | Volume 111, Nº5, November 2018

Original Article Lopes et al Mortality for critical congenital heart diseases and newborns Arq Bras Cardiol. 2018; 111(5):666-673 Figure 1 – Distribution of recording density of pre- and post-ductal pulse oximetry levels, according to the presence or absence of congenital heart diseases (CHD). 0.3 0.2 0.1 0.0 0.2 0.1 0.0 75 80 85 90 95 100 75 80 85 90 95 100 Density Density CHD Absent Present CHD Absent Present Pre-ductal oximetry (%) Pre-ductal oximetry (%) reinforces the importance of adequate prenatal diagnosis and follow-up. Studies in Brazil have already indicated that low access to prenatal and/or at birth diagnosis makes the treatment of CHD considerably difficult, which leads to a worse clinical condition at birth. 9 The frequency of twin pregnancies among the cases was proportionally higher within the comparison group. This data was reportedwith controversy in other studies, due to the difficulty of concomitantly evaluating the association of other risk factors, but for the outcome death, this finding was determinant. 21 The early and high mortality rate found here was one of the most discordant data in the world literature. In developed countries, it is expected that the CHD fatality in the neonatal period will only exceed 60% for the late diagnoses of the hypoplastic left heart syndrome (HLHS); for the other types of CHD, the expected fatality rate does not exceed 40%, when the diagnosis of CHD is made before hospital discharge. 22 Countries with socioeconomic classification similar to that of Brazil, although also coping with glaring regional differences in relation to neonatal care, have an overall incidence rate of CHD deaths of 20 to 30/100,000 births. 2 Fixler et al. 3 measured the mortality rate according to the time of referral, considering first day, up to 5 days, 4 to 27 days, and no referral after 27 days, and found mortality near 38% when the newborn was not referred before 27 days of life. In addition, mortality increased considerably at 3 months, getting close to 80% for HLHS. 3 The literature has shown a significant improvement in the quality of care, which has led to a decrease in morbidity and mortality in developed countries, 3,4 but this is not a reality for developing countries, as can be seen in the high mortality and lethality rate despite the same incidence of CHD described herein. Table 2 – Causes of death, according to the type of cardiopathy Type of cardiopathy Cause of death n (%) PVA, IVCa, PVAD, HLV, GAT, AVI, and TrA Cardiogenic shock 7 (41,1) Ebstein's anomaly Supraventricular tachycardia 1 (5,9) RVDO Sepsis 3 (17,6) LHHS, pentalogy of Cantrell Through CHD (basic cause/palliative care) 3 (17,6) PVAD, GAT Ill-defined causes 3 (17,6) PVA: post-varicella angiopathy; IVC: interventricular communication; PVAD: Pulmonary vein anomalous drainage; HLV: hypoplasic left ventricle; GAT: great arteries transposition; AVI: aortic valve insufficiency; TrA: Truncus arteriosus; RVDO: right ventricle double outlet; CHD: congenital heart disease. 669