ABC | Volume 112, Nº5, May 2019

Guideline Brazilian Fetal Cardiology Guidelines – 2019 Arq Bras Cardiol. 2019; 112(5):600-648 Content 1. Introduction ..........................................................................................602 2. Screening and Diagnosis of Fetal Heart Disease ..................................603 2.1. Introduction ........................................................................................603 2.2.FetalHeartScreeningDuringMorphologicalUltrasound ...........................603 2.2.1. Step 1 – 1 st Level: Evaluation of the Abdomen to Identify the Abdominal Aorta and the Inferior Vena Cava ..................................605 2.2.2. Step 2 – 2 nd Level: Four Chamber View ............................................605 2.2.3. Step 3 – 3 rd Level: Left Ventricular OutflowTract ..................................606 2.2.4.Step4–4 th Level:RightVentricularOutflowTract ..................................606 2.2.5. Step 5 – 5 th Level: Three Vessels View .............................................606 2.2.6.Step6–6 th Level:ThreeVesselandTracheaView ..................................607 2.3. Screening for Congenital Heart Disease During the First Trimester .........607 2.4. Fetal Echocardiography ....................................................................608 2.5. Imaging Techniques Used on Fetal Echocardiography .......................608 3. Stratification of Centers that Work with Fetal Cardiology and Their Potential Therapeutic Facilities ......................................................611 4. Classification of Fetal Heart Disease ......................................................614 4.1. Group I − Heart Diseases without Fetal Hemodynamic Compromise .....614 4.1.1. Structural ..................................................................................614 4.1.2. Functional ...................................................................................616 4.2. Group II − Heart Diseases with Fetal Hemodynamic Compromises .......616 4.2.1. Structural ...................................................................................616 4.2.2. Functional ...................................................................................616 4.3. Group III − Fetal Heart Diseases with Limited Postnatal Prognosis .....616 5. Management of The Main Fetal Heart Diseases ..................................616 6. Fetal Ductal Constriction: Treatment and Prevention ..............................617 6.1. Prevalence, Diagnosis, Clinical Consequences, and Prognosis of Fetal Ductus Arteriosus Constriction .................................................620 6.2. The Role of Anti-Inflammatory Substances in the Genesis of Fetal Ductal Constriction ....................................................................621 6.3. Anti-Inflammatory and Antioxidant Action of Polyphenols ...............621 6.4. Summary of Evidence for Ductal Constriction Management ..............621 6.5. Conclusions .........................................................................................622 6.5.1. Recommendations for Ductal Constriction Treatment ................622 6.5.2. Recommendations for Ductal Constriction Prevention ...............623 7. Fetal Cardiac Arrhythmias: Diagnosis and Treatment ...........................623 7.1. Fetal Cardiac Rhythm and Fetal Cardiac Arrhythmias .......................626 7.2. Extrasystoles .........................................................................................627 7.2.1. Isolated Supraventricular Extrasystoles .........................................627 7.2.2. Ventricular Extrasystoles ..............................................................627 7.3. Fetal Bradycardia ...........................................................................627 7.3.1. Sinus Bradycardia .........................................................................627 7.3.2. Low Atrial Rhythm .........................................................................628 7.3.3. Blocked Atrial Bigeminy ..................................................................628 7.3.4. Complete Atrioventricular Block ......................................................628 7.4. Fetal Tachycardia ..............................................................................629 7.4.1. Intermittent Tachycardias ..............................................................629 7.4.2. Sustained Tachycardias .................................................................630 7.4.2.1. Diagnosis ...............................................................................630 7.4.2.2. Treatment ..................................................................................631 8. Fetal Cardiac Interventions ....................................................................636 8.1. Indications .....................................................................................636 8.1.1. Critical Aortic Stenosis with Impending Hypoplastic Left Heart Syndrome ............................................................................636 8.1.2. Hypoplastic Left Heart Syndrome with Intact Interatrial Septum or Significantly Restrictive Foramen Ovale ..................................637 8.1.3. Pulmonary Atresia with Intact Interventricular Septum or Critical Pulmonary Valve Stenosis with Signs of Evolving Right Heart Hypoplasia ................................................................................637 8.1.4. Critical Aortic Stenosis with Massive Mitral Regurgitation and Giant Left Atrium .............................................................................637 8.2. Technical Considerations ..................................................................637 8.3. Aortic Valvuloplasty ........................................................................638 8.4. Critical Aortic Stenosis with Giant Left Atrium ...................................638 8.5. Fetal Pulmonary Valvuloplasty .......................................................639 8.6. Fetal Atrial Septostomy ..................................................................639 8.7. Final Considerations of Fetal Cardiac Interventions ..........................640 9. Acknowledgments ...........................................................................640 References ..........................................................................................641 1. Introduction Over the years, Fetal Cardiology have been incorporated into the daily practice of Pediatric Cardiology. What was once restricted to a few fetal heart researchers, has slowly been incorporated into health institutions that deal with congenital heart diseases (CHD). Fetal echocardiography has generated extensive knowledge of the natural and modified history of heart diseases in utero, and normal fetal heart physiology and anatomy. The benefits of fetal diagnosis have become unquestionable over the years. Pioneers in the area succeeded in demystifying the fetal heart examination and proving the importance of screening for cardiac abnormalities during obstetric examinations. Prenatal detection rates have increased, and interest in fetal echocardiography is, thus, no longer merely a diagnostic tool; it has gone on to become a tool of the utmost importance in assisting medical and, progressively, interventional treatment of specific anomalies that occur in fetal life. A vast body of literature currently supports the practice of Fetal Cardiology. In addition to diagnosis, anatomical and functional particularities may be identified in utero, with implications on the delivery planning and pre and postnatal management. Prenatal diagnosis has certainly led to increase the number of babies with complex heart diseases in Pediatric Cardiology hospital beds. Prior to this, children with complex heart diseases did not survive the immediate neonatal period and died in neonatal intensive care units without being diagnosed. Nowadays, these children require increasingly careful and specific management involving Pediatric Cardiology and thus modifying the practice of Neonatal Cardiology. Despite the vast literature pertinent to Fetal Cardiology, due to the restricted number of cases, there is a lack of studies with large populations and randomization processes, being the information based on observational studies and description of small samples or cases reports. However, the accumulated knowledge is already enough to develop scientific statements or guidelines. In April 2014, the American Heart Association (AHA) published the first scientific statement for Fetal 602