ABC | Volume 112, Nº5, May 2019

Guideline Brazilian Fetal Cardiology Guidelines – 2019 Arq Bras Cardiol. 2019; 112(5):600-648 Cardiology, encompassing all the practical aspects involved in this area, including screening, diagnosis, medical or interventional therapy, counseling, delivery planning, and neonatal treatment. Considering this extremely thorough and highly useful document, we have accepted the challenge of bringing together professionals dedicated to Fetal Cardiology from different regions of Brazil in order to jointly establish guidelines which are adapted to our reality and which also take into consideration knowledge created in Brazil. We believe that the information brought together in this document will be of great use to professionals who face the challenge of dealing with possible abnormalities that affect the fetal heart in their daily practice. 2. Screening and Diagnosis of Fetal Heart Disease 2.1. Introduction One of the main aims of prenatal diagnosis is the detection of severe CHD, whose diagnoses, in most cases, depend on delivery planning in a specialized referral center. 1-3 Although fetal echocardiography, which is traditionally designated for high-risk pregnancies, is quite accurate, the majority of newborns affected by heart diseases in most parts of the world, continue to be born without having been diagnosed. This occur because many cases of CHD affect low-risk groups and are not detected by screening prenatal ultrasound. 4,5 The concept of prenatal screening for CHD was first suggested in 1985, with the recommendation of incorporating the four-chamber view into routine obstetric ultrasound. 6 For more than 25 years, countries such as France, the United Kingdom and Spain have recommended examination of the fetal heart during the routine obstetric ultrasound. Nonetheless, after many years of investment in educational training programs, regional variation in detection rates of prenatal heart diseases continue to be high. The classic study by Garne et al., 7 conducted in 20 European centers showed that the global detection rate of fetal heart diseases was rather low (25%), France being the country with the best performance (48%), followed by Spain (45%), Germany (40%), and the United Kingdom (35%). Many studies have shown that detection rates of prenatal heart diseases significantly improve with the expansion of scanning planes for cardiac analysis, but they remain well below 50% and continue to lag behind in relation to prenatal detection of other forms of congenital malformation. 8,9 Faced with this situation, some have argued that fetal echocardiography should be indicated for all pregnancies, given that, in experienced hands, it is able to detect nearly 100% of all cardiac anomalies in fetal life and is considered the gold standard for fetal cardiac diagnosis. 10-13 Although it is almost intuitive that prenatal detection of heart diseases would improve perinatal results, it has not been easy to prove this observation scientifically, owing to the difficulty of comparing groups with pre- and postnatal diagnoses, which present rather peculiar and discrepant characteristics. The group with prenatal diagnosis often presents with fetal death or early neonatal death before surgery, as it pertains to the much more severe spectrum of fetal cardiac abnormalities, due to the inability of obstetric ultrasound to screen simpler heart diseases, thus resulting in higher global mortality. On the other hand, the group with postnatal diagnosis, that survives the fetal and early neonatal periods until the baby arrives in a tertiary center, has already demonstrated some constitutional advantages for survival. 2 A study conducted in France comparing perinatal outcome between babies with transposition of the great arteries, with and without prenatal diagnosis, showed, for the first time, that prenatal diagnosis significantly decreased pre- and postoperative mortality. 14 Other studies have suggested better results for hypoplastic left heart syndrome (HLHS) and coarctation of the aorta when they are diagnosed during fetal life. 15,16 Efforts and resources should be directed to teaching and training for prenatal screening of CHD by obstetric ultrasound to achieve a better and more uniform pattern of detection, since performing fetal echocardiography in all pregnancies is unrealistic and has yet to be adopted as a health policy in developed countries. 11,13,17 Table 2.1 shows the main risk factors for fetal heart diseases, divided into absolute risk of ≥ 2% and < 2%. 2.2. Fetal Heart Screening During Morphological Ultrasound Considering all these characteristics, we propose a very simple methodology for evaluating the fetal heart, which has been applied in various countries throughout the world. The main advantage of this systematized heart evaluation is that it eliminates the need for complex views and images, avoiding more difficult maneuvers, which is time-consuming and discourage the examiner who neglects this important part of the morphological exam. With this technique, the fetal heart is evaluated on transverse plane images of the baby only, with no need to rotate the transducer. It starts from the fetal abdomen, from the infradiaphragmatic region to the 603