ABC | Volume 112, Nº5, May 2019

Guideline Brazilian Fetal Cardiology Guidelines – 2019 Arq Bras Cardiol. 2019; 112(5):600-648 Table 5.5 – Group IIA. Structural fetal heart diseases that inevitably require neonatal care. Class of recommendation/level of evidence: IB. 17,41,57-59 Heart disease In utero outcome In utero follow-up Delivery Postnatal assessment Simple TGA HLHS IAA Severe CoA TAPVR Truncus Complex heart diseases with severely restricted systemic or pulmonary outflow tracts FO may be restrictive during gestation Although they are complex heart diseases, they tend to remain stable, without hemodynamic compromise during gestation Repeat study every 4 to 6 weeks is recommended In HLHS or anatomical variations with restrictive ASD, consider fetal intervention Perform a new evaluation a few weeks before delivery Induced vaginal delivery or programmed C-section Level 2 or 3 center Immediate neonatal cardiac evaluation The majority are duct dependent CHD and require prostaglandin infusion + interventional or surgical treatment during the first week of life TAPVR and Truncus are diseases with early presentation of HF and PH, and thus require treatment during the first weeks of life, even when they are not duct dependent CoA: coarctation of the aorta; FO: foramen ovale; HF: heart failure; HLHS: hypoplastic left heart syndrome; IAA: interrupted aortic arch; PH: pulmonary hypertension; TAPVR: total anomalous pulmonary venous return; TGA: transposition of great arteries. Table 5.6 – Group IIB. Functional fetal heart diseases with hemodynamic compromise. Class of recommendation/level of evidence: IIb C. 17,41,57-59 Heart disease In utero outcome In utero follow up Delivery Postnatal assessment Restricted FO Ductal constriction Pericardial effusion Extrinsic compressions Anemia High-output AV fistulas TTTS May evolve with ventricular dysfunction or fetal hydrops Serial echocardiogram every 4 to 6 weeks is recommended May need fetal treatment With hydrops, programmed C-section; Without hydrops, induced vaginal delivery or programmed C-section Level 2 or 3 centers Evaluate the need for preterm delivery Immediate neonatal cardiac evaluation May require clinical, interventional or surgical treatment immediately after birth AV: arteriovenous; FO: foramen ovale; TTTS: twin-twin transfusion syndrome. Table 5.7 – Group IIB. Nonstructural fetal heart diseases which may evolve with hemodynamic compromise. Class of recommendation/level of evidence: I C. 17,41,57-59 Heart disease In utero outcome In utero follow up Delivery Postnatal assessment Cardiomyopathies Arrhythmias Tumors May evolve with fetal hydrops May require medical treatment Frequent follow-up (weekly or biweekly), depending on diagnosis and hemodynamic compromise Vaginal delivery in a level 1 center if well controlled tachyarrhythmias or cardiomyopathies without fetal hemodynamic compromise; Programmed C-section in a level 2 or 3 center in cases of arrhythmia or hydrops which have not been resolved in utero Cardiac management according to diagnosis Treatment is usually with medication, with the exception of some tumors which need to be removed due to obstructive or compressive character, which compromises hemodynamics Table 5.8 – Group III. Fetal heart diseases associated with genetic syndromes or extracardiac malformations. Class of recommendation/level of evidence: IIb C. 17,41,57-59 Heart disease In utero outcome In utero follow up Delivery Postnatal assessment Multiple malformations Associative syndromes Trisomies Triploidy Other genetic anomalies May evolve with fetal hydrops depending on the genetic of extracardiac anomaly Depends on fetal or neonatal viability and extracardiac anomalies prognosis For non-viable fetuses or newborns, delivery may be in a level 1 center, preferably by spontaneous vaginal birth. For viable fetuses or newborns, delivery may be vaginal or programmed C-section in a level 2 or 3 center Consider palliative care team support Cardiac management according to prognosis of associated anomalies or chromosome diseases 619