ABC | Volume 114, Nº5, May 2020

Statement Brazilian Cardiology Society Statement for Management of Pregnancy and Family Planning in Women with Heart Disease – 2020 Arq Bras Cardiol. 2020; 114(5):849-942 Another important issue is the risk of transmitting the disease to the fetus, because HCM is an autosomal dominant Mendelian trait, which is also caused by mutations that encode the components of the cardiac sarcomere. 196 The complexity of this disease still does not allow for determination of its true incidence in apparently healthy newborns who do not present abnormalities on 2-dimensional echo. In most cases, echo during the neonatal period does not identify HCM because myocardial hypertrophy occurs over the course of development, only becoming apparent after adolescence. Nonetheless, it is worth highlighting that the disease’s obstructive form and family history of sudden cardiac death (SCD) are risk factors for early manifestation of hypertrophy in children. 131,197 Genetic study of asymptomatic children and adolescents with family history of HCM may identify “healthy” carriers of the mutation. There are, however, important obstacles to the clinical application of this investigation, such as genetic plurality, low frequency of the mutation responsible in the diseased population, difficulties in techniques for identifying the pathogenic mutation, and high costs. In symptomatic patients, initial pharmacological treatment is with the use beta-blockers, propranolol, or metoprolol succinate, which may or may not be associated with CCB, such as verapamil. 52 Association of these drugs requires caution with respect to maternal tolerance, arterial pressure, and fetal vitality. The use of prostaglandins to induce delivery is not advisable, due to their vasodilating effects. Vaginal delivery is considered to be safe, whereas cesarean delivery is reserved for special situations. Epidural or spinal anesthesia should be contraindicated in severe obstructive forms. During pregnancy planning in patients with arrhythmias that are difficult to control pharmacologically, it is necessary to consider discussing the possibility of percutaneous intervention with an electrophysiologist. Examples include radiofrequency ablation in cases of complex and/or symptomatic tachycardias or even ICD in patients included in conventional class IA recommendations. 3.3.3. Arrhythmogenic Right Ventricular Dysplasia Arrhythmogenic right ventricular dysplasia is a hereditary, autosomal dominant cardiomyopathy, with reduced penetrance and variable expressivity. For these reasons, genetic counseling is mandatory. Pregnancy is well tolerated in women with this disease, but patients with preexisting biventricular disease have a higher risk of developing HF as pregnancy progresses. 198 Symptom control and prevention are done with beta-blockers (propranolol, metoprolol succinate). In the event that they are necessary, antiarrhythmic drugs should be maintained, respecting fetal toxicity limits. If indicated, ICD implantation should preferably take place before gestation. 199 3.3.4. Non-compaction Cardiomyopathy The non-compacted myocardium is characterized by distinctly trabeculated myocardial morphology. It is a family disease in up to 60% of cases, with autosomal dominant inheritance. Its prevalence is unknown, and evidence regarding practice during pregnancy is limited. 200 The clinical picture is highly variable, ranging from asymptomatic patients to patients with refractory HF and severe arrhythmias. There is no specific treatment for non-compaction cardiomyopathy, and therapeutic conduct should be supported by experience with other cardiomyopathies. The risk of thromboembolism, however, is considered to be greater due to the myocardial morphology in itself, which justifies permanent anticoagulation during gestation. 3.3.5. Restrictive Cardiomyopathy Idiopathic restrictive cardiomyopathy is characterized by non-hypertrophic, non-dilated ventricles, with diastolic dysfunction, resulting in atrial dilatation. It may be idiopathic or associated with other diseases, such as amyloidosis, endomyocardial fibrosis, sarcoidosis, and hemochromatosis. Scarcity of experience in the literature, limited and controversial therapy, and frequently severe clinical evolution are factors which make pregnancy unadvisable. 3.3.6. Key Points • Women with cardiomyopathy should participate in family planning, including genetic counseling; • Risk stratification for subsequent pregnancies should consider the functional and structural status of the cardiomyopathy; • Children of women with HCM, even when they are apparently healthy, should receive differentiated follow up until adolescence; • Therapeutic optimization should follow conventional guidelines, considering classical drug contraindications during gestation; • Permanent anticoagulation should be practiced in pregnant women with noncompaction or dilated cardiomyopathy, intracavitary thrombus, or prior embolic event; • Genetic studies are promising for changing prognosis in cardiomyopathies. 3.3.7. Peripartum Cardiomyopathy PMFC is defined as an idiopathic form of cardiomyopathy that manifests with HF secondary to left ventricular systolic dysfunction, with LVEF (< 45%), which occurs in late pregnancy or months after delivery or miscarriage, when none other cause of HF has been found. 201 The pathophysiology of PPCC, not yet fully understood, is based on hypotheses that suggest hormonal, inflammatory, autoimmune, infectious, genetic and environmental mechanisms. 201 New concepts on etiopathogenesis have been presented, involving oxidative stress, angiogenic imbalance, and prolactin in the genesis of PPCM. 202,203 The most recent studies show that PPCM is triggered by increased oxidative stress in pregnancy, 204 in combination with lower expression of angiogenesis regulators. Oxidative cleavage of prolactin by cathepsin D, the major endoprotease responsible for the generation of adenohypophyseal vasoinibins, generates an antiangiogenic subfragment, 884