ABC | Volume 112, Nº5, May 2019

Guideline Brazilian Fetal Cardiology Guidelines – 2019 Arq Bras Cardiol. 2019; 112(5):600-648 particularities that benefit from fetal therapy. For this reason, the existence of more than 1 or 2 centers with these characteristics in Brazil is not justified. It is clear that Brazil needs more Pediatric and Fetal Cardiology centers as well as increase the number of cardiac surgeries and percutaneous interventions. Nevertheless, due to various political and environmental issues, these changes will only occur in medium to long term. Aiming to maximize referrals of fetuses with CHD to the existing centers, it is mandatory that all professionals involved in the screening of CHD know how to refer the patient to the appropriate care centers. 37,38 When fetuses with CHD are identified in places where there is no appropriate care, the doctor should promptly look for help to refer the patient to a specialized center according to the regulatory flow of the state. If the state has no specific hospital do refer the patient, the local health system should ask for outside treatment (tratamento for a de domicílio -TFD) which will look for the closest specialized center to take care of the mother and the fetus. This process is nowadays regulated by CNRAC (central nacional de regulação da alta complexidade) since directive instructions of the Ministry of Health to organize the health care for high- risk pregnancies were published. It is emphasized here that the high-risk pregnancies are “those in which the life or health of the mother, the fetus, or the newborn has higher chances of being affected when compared to the general population.” 39 When fetal cardiologist is dealing with a case of fetal heart disease, he or she needs to define whether there is any need of prenatal intervention or whether the treatment has to be started immediately after birth and if the patient needs to be referred to center levels 2 or 3 available in our country, reminding that not all the centers considered level 2 can treat all types of neonatal anomalies. It is known that HLHS and its variations, for example, have an extremely high fetal incidence, whereas few centers in our country have satisfactory operative results for this anomaly. The Figure 3.1 is a flowchart that standardizes the specific care according to the fetal heart disease. 4. Classification of Fetal Heart Disease With the development of fetal medicine as a medical subspecialty and with the recent advances in the ultrasound imaging, the detection of fetuses with congenital malformations has become increasingly frequent, making earlier treatment possible with significant reduction of fetal and neonatal mortality. 40 With prenatal diagnosis, diseases with potential risk to have hemodynamic compromise in utero and/or in the neonatal period can be followed up and have the specific pre and postnatal care planned. Taking into account the characteristics of the fetal circulation, it is particularly important to recognize the behavior of the different heart diseases in utero and after birth, identifying those that will require any kind of treatment (use of medications or invasive procedures) or anticipation of the childbirth. 41 Fetal cardiac disease may be classified as structural or functional. The majority of the structural heart diseases do not have hemodynamic compromise in utero due to the fetal circulation physiology. Clinical manifestations will occur after birth, when the physiological intracardiac shunts close. Cardiomyopathies, conditions like high output fistulas, significant abnormalities of the cardiac rhythm and restricted foramen ovale, ductal constriction or absent ductus venosus may also occur in utero and compromise the fetal hemodynamic requiring prenatal treatment. It is important to highlight the importance of a multidisciplinary team involved in the care of fetuses affected by heart diseases, since genetic syndromes or severe extracardiac malformations may be associated and significantly increase postnatal mortality. For these reasons, fetal heart diseases were classified into 3 groups according to possible clinical presentation and in utero hemodynamic manifestations and were separated in groups A - structural and B - functional (Table 4.1). 4.1. Group I − Heart Diseases without Fetal Hemodynamic Compromise 4.1.1. Structural This group includes simple or complex cardiac defects that do not usually present progression or hemodynamic decompensation during the fetal period and, thus, do not require treatment during pregnancy and do not change obstetric management. The main example of this group are diseases with left-to-right shunt, including atrial, ventricular and atrioventricular septal defects, and aortic to pulmonary window; heart diseases with mild obstruction of right or left outflow tracts, such as pulmonary valve stenosis, aortic stenosis, and localized coarctation of the aorta; and complex CHD such as TOF with mild pulmonary flow obstruction, corrected transposition of great arteries, double outlet right ventricle, and univentricular hearts without obstructions or with mild obstructions to systemic and pulmonary outflow tract flows. 614