Pulmonary Arterial Hypertension（PAH）

Pulmonary arterial hypertension (PAH) is a rare but life-threatening condition characterized by elevated pulmonary vascular resistance and right heart failure. Pregnancy in PAH patients carries a maternal mortality risk of 30-50%, primarily due to cardiovascular collapse during delivery or postpartum. Physiological changes (e.g., increased blood volume, cardiac output) exacerbate right ventricular strain, leading to decompensation. If pregnancy occurs, multidisciplinary care (cardiology, obstetrics, anesthesia) is critical. Management includes optimizing PAH-specific therapies (e.g., sildenafil, prostacyclins) and avoiding teratogens. Anticoagulation is often recommended to mitigate thrombosis risks. Postpartum care requires intensive hemodynamic monitoring for 72 hours, as most deaths occur during this period. PAH patients who survive pregnancy face long-term cardiovascular risks, necessitating lifelong follow-up. Termination or advanced therapies (e.g., ECMO) may be considered in severe cases.

Marfan Syndrome

Marfan syndrome, a genetic disorder caused by FBN1 gene mutations, affects connective tissue, primarily impacting the cardiovascular system (aortic root dilation/dissection), skeletal structure, and eyes. Pregnancy significantly raises aortic dissection risk due to hemodynamic stress (↑blood volume, cardiac output), with mortality up to 10% if aortic root diameter exceeds 40 mm. Complications include preterm labor, fetal growth restriction, and 50% risk of transmitting Marfan syndrome.

Peripartum Cardiomyopathy (PPCM)

Peripartum cardiomyopathy (PPCM) is a rare, life-threatening condition occurring during late pregnancy or within 5 months postpartum, characterized by left ventricular systolic dysfunction (ejection fraction LVEF <45%) and symptoms of heart failure (e.g., dyspnea, edema, fatigue). Its etiology may involve hormonal shifts (e.g., abnormal prolactin cleavage), vascular endothelial injury, or immune dysregulation, though the exact mechanism remains unclear.