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 Table 25 – Classification of pulmonary arterial hypertension Category 1 Idiopathic Hereditary Induced by drugs and toxins: anorectic agents, chemotherapy, serotonin reuptake inhibitors, cocaine Associated with congenital heart disease, collagen disease, HIV infection, portal hypertension, schistosomiasis Pulmonary capillary hemangiomas or veno-occlusive pulmonary disease Persistent pulmonary hypertension in the newborn Category 2 - Pulmonary hypertension due to left heart disease Diastolic dysfunction Systolic dysfunction Valve disease Congenital/acquired left heart obstruction and outflow tract obstruction and congenital cardiomyopathies Category 3 - Pulmonary hypertension due to pulmonary disease and/or hypoxemia Chronic obstructive pulmonary disease Interstitial pulmonary disease Pulmonary diseases with mixed patterns, i.e, restrictive and obstructive Obstructive sleep-disordered breathing Alveolar hypoventilation Chronic exposure to high altitudes Occupational pulmonary diseases Category 4 Pulmonary hypertension due to chronic thromboembolism Category 5 - Pulmonary hypertension with unclear multifactorial mechanisms Hematologic disorders: chronic hemolytic anemia, myeloproliferative disorders, splenectomy Systemic disorders: sarcoidosis, pulmonary histiocytosis, lymphangioleimoyomatosis Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders Others: tumoral obstruction, fibrosing mediastinitis, chronic renal insufficiency, segmental pulmonary hypertension HIV: human immunodeficiency virus. Parenteral prostaglandins are recommended in patients in NYHA FC IV or patients who show evidence of severe right ventricular involvement. Most experience is with intravenous epoprostenol. In patients with preserved ventricular function who are in NYHA FC I/II, inhaled prostaglandins, such as Ilosprost, may be indicated. Parenteral prostaglandins may be combined with oral phosphodiesterase inhibitors, with satisfactory results. 252 Endothelin receptor blockers and soluble guanylate cyclase stimulators are contraindicated in pregnancy. 251,252 Intravenous prostaglandins may be considered during delivery, with invasive monitoring via CVC and arterial access. It is worth remembering that a large number of maternal deaths occur during the postpartum period, with the following causes standing out: HF due to right ventricular failure, hypoxemia, and thromboembolism (pulmonary thrombosis in situ ). 248 Therefore, anticoagulation is essential, with therapeutic doses of LMWH (1 mg/kg every 12 h) during the first trimester and after 36th week of gestation and, warfarin in a daily dose, INR target of 2, another other periods (Figura 6). 3.6.3.1. Key Points • Diagnosis of PAH should be confirmed by right chamber catheterization. • Pregnancy should be advised against in women with PAH; • Categories of PH according to current classification have different prognoses and treatments; • The proposal to interrupt pregnancy should be considered in patients with PH during the first trimester, except in patients in category 2; • In pregnancy in maintained, the prenatal care and delivery should be at a tertiary hospital with specialized PH staff; • Current pharmacological therapy has assisted in successful pregnancy in PH. 3.6.4. Aortic Diseases Aortic diseases may be present in women of reproductive age, and they are considered to be important causes of complication and even death during gestation. 253 This is due to 3 physiological phenomena of pregnancy that have detrimental impacts of aortic diseases. These phenomena are hemodynamic (increased cardiac output), structural (progressive aortic root growth until the third trimester), and hormonal (vascular wall fragility). The following are the most frequent causes of aortic disease in pregnant women: bicuspid valve, Marfan syndrome, coarctation of the aorta, Ehlers-Danlos syndrome, Turner syndrome, and Loeys-Dietz syndrome. 891