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 3.6. Other Diseases 3.6.1. Takayasu Arteritis Takayasu arteritis is a chronic, idiopathic vasculitis that predominantly affects the aorta and its main branches, coronary arteries, and the pulmonary artery. The resulting inflammatory process causes narrowing, occlusion, and aneurysm in the affected branches. 233 Etiology of the disease is unknown, but several studies have demonstrated an association with human leukocyte antigens, suggesting a predisposition to the immune-mediated process. 234 3.6.1.1. Prevalence Takayasu arteritis is a rare disease, with growing rates of prevalence. The highest rates occur in Japan, with 100 to 200 new cases annually. Women are more affected, in 80% to 90% of cases; the onset of the disease occurs between 10 and 40 years of age, overlapping with the fertile period of life, and gestation demands special attention. It is the most frequently observed form of vasculitis during pregnancy, precisely because it appears in young patients. 235 Maternal immune activation during pregnancy may influence the course of the disease and impair maternal and fetal outcome. 236 3.6.1.2. Prognosis Pregnancy in patients with Takayasu arteritis has an uncertain prognosis. Although the majority of gestations are successful, the incidence of severe hypertension and preeclampsia is 40% higher, when compared to 8% in the general population. Obstetric complications, such as premature delivery and stillbirth, are foreseen. 235 Patients with renal artery and abdominal aorta involvement more frequently have complications of preeclampsia and IUGR. 235 The rarest maternal complications, which are, however, very severe, are aortic aneurysm, stroke, HF, aortic insufficiency, myocardial infarction, and dissection of the aorta. 235 Other, more common complications include progression of renal insufficiency, anemia, thrombocytopenia, and elevated inflammatory markers. 235 3.6.1.3. Treatment Treatment of vasculitis during pregnancy is conventional, excluding three teratogenicmedications, namely, methotrexate, mycophenolate and cyclophosphamide. 236 Other medications are considered compatible with gestation. It is preferable to use immunosuppressive drugs to control active vasculitis, reserving prednisone for a short-duration regime in moderate doses during the acute phase or in cases where the disease worsens. Treatment may be initiated before conception and maintained during pregnancy and lactation. 237 Tumor necrosis factor inhibitors may be continued during the preconception, pregnancy and lactation. These inhibitors, when their composition is based on immunoglobulin G (IgG), cross the placenta from 16 th of gestation with a progressive increase in transference nearby term of gestation. Therefore, these drugs should not be administered after 30 th week of gestation, but they should be reintroduced in the postpartum period. 238 3.6.1.4. Key Points • Pregnancy is allowed when disease is in remission, because vasculitis has severe prognosis; • Treatment with corticosteroids and immunosuppressive drugs (azathioprine, cyclosporine, and tacrolimus) improves maternal-fetal evolution; • In cases of systemic vasculitis, seeing that the risk of thromboembolic events is elevated, prevention with aspirin or LMWH should be considered; • Takayasu arteritis should always be considered indifferential diagnosis of arterial hypertension during pregnancy; • Contraception should be efficacious and safe during treatment with high doses of cytotoxic drugs. 3.6.2. Kawasaki Disease Kawasaki disease is a systemic vasculitis of unknown etiology that occurs in children up to 5 years of age, with Asian prevalence and a male predominance of 1.5 para 1. During the acute phase, inflammatory involvement of coronary arteries results in clinical outcomes and provokes aneurysm formations in 15% to 25% of untreated children. It is one of the main causes of heart disease acquired during childhood. 239 Coronary artery aneurysms may be detected early on echo and loss of laminar flow in these arteries may favor clot formation. Disease prognosis is related to presence and size of coronary artery aneurysms. Small aneurysms have favorable prognosis, with low risk of myocardial ischemic events. In contrast, large and giant aneurysms (internal diameter > 8 mm) present a high risk of thrombosis and, consequently, myocardial infarction, arrhythmias, and sudden death. 240 Lack of diagnosis and treatment during the acute phase in childhood has contributed to the finding of women with vascular sequelae of Kawasaki disease during fertile age and pregnancy. 241,242 The influence of the hypercoagulable and hyperkinetic states inherent to pregnancy, delivery, and the postpartum period represents a potential risk of severe events, such as thrombosis, myocardial infarction, and sudden death, throughout the natural history of women with complicated Kawasaki disease with coronary aneurysms. In addition to this, pregnancy, per se , favors the risk of coronary artery rupture and/or dissection, as a result of specific changes in the artery walls, which include fragmentation of reticular fibers, reduction of mucopolysaccharides and loss of normal elastic fibers ripple. In keeping with this logic, it is accepted that the state of hypercoagulability during pregnancy and the postpartumperiod requires permanent anticoagulation. Therefore, low‑dose aspirin (80 mg daily) up 36 th week of gestation combined with anticoagulation, should be considered. LMWH is recommended during the first trimester and after week 36 of gestation, with low doses of warfarin in the interval between these 2 periods. In the literature, there is a lack of data regarding targets for prevention; nonetheless, the consensus is that INR around 2 is safe and presumably efficacious. Previous myocardial infarction increases the risk of gestation, and ventricular function is a determining factor for 889