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Valvular heart disease in women of childbearing age poses an increased risk of adverse maternal and fetal outcomes, and management in pregnancy can be challenging. Ideally, patients with suspected valvular disease should have preconception counseling by a multidisciplinary team including cardiologists with expertise in pregnancy and a maternal-fetal medicine specialist. Preconception planning should include a cardiac assessment of maternal risk, determination of frequency of surveillance, and a cardiovascular management plan during delivery. Women with valvular heart disease should be followed closely by a cardiologist and monitored for signs and symptoms of congestive heart failure and arrhythmias. In general, stenotic lesions may become more symptomatic in pregnancy, whereas regurgitant lesions are generally well tolerated. Left-sided valvular lesions have higher complication rates than right-sided lesions. For patients with asymptomatic valvular stenosis, medical management during pregnancy may include beta blockade and/or diuretics. Exercise stress testing prior to pregnancy in sedentary patients can be helpful to unmask symptoms and determine functional capacity. Patients with symptomatic, severe left-sided valvular obstruction have a high maternal risk of cardiovascular events during pregnancy, and percutaneous balloon valvuloplasty or surgery is recommended prior to pregnancy. The type of prosthetic valve (mechanical vs bioprosthetic) should be selected after a careful discussion with the patient. Invasive procedures are generally reserved for when medical management fails. The second trimester may be the optimal time for intervention as fetal organogenesis is complete and the cardiac positioning has not been affected by the gravid uterus.

The arterial switch operation (ASO) is now the most frequently performed surgical correction in individuals with dextro-transposition of the great arteries (D-TGA). Patients who undergo this procedure as neonates have overall good clinical outcomes yet continued clinical follow-up is important to evaluate for postoperative complications. In this group, the highest mortality is in the immediate postoperative period and is generally associated with reimplantation of the coronary arteries. As these patients live into adulthood, longitudinal follow-up for other ASO complications including neo-pulmonary stenosis, right ventricular outflow tract (RVOT) obstruction, or neo-aortic root dilation and resulting aortic insufficiency should be performed. In adults, extra care should be taken to identify and treat traditional cardiovascular risk factors as individuals with coronary obstruction may not present with typical anginal symptoms. Management of these patients should be performed in collaboration with an adult congenital heart center of excellence. This population offers a unique opportunity to provide timely feedback to adult congenital heart community of providers regarding late outcomes from surgical intervention and in the next decade will hopefully demonstrate a model for clinical feedback cycles in lifelong congenital care.

Ebstein’s anomaly is a congenital malformation of the tricuspid valve and the right heart with a spectrum of clinical and morphologic presentations. Minor anomalies of the tricuspid valve may not be recognized until adulthood whereas major anomalies leading to heart failure and cyanosis require surgical intervention earlier in life. Echocardiography is the imaging modality of choice for both diagnosis and management of patients with Ebstein’s anomaly. Surgical correction includes tricuspid valve repair or replacement and associated findings such as interatrial communications and arrhythmias should be addressed at the time of surgery. Pre-pregnancy evaluation should be considered in all Ebstein’s anomaly patients and for those who are cyanotic, surgical correction must be considered due to the maternal and fetal ramifications of cyanosis in pregnancy. Most acyanotic Ebstein’s anomaly patients are able to tolerate pregnancy with manageable or no complications. Those patients with mild anomalies and no right heart dilation can participate in sports whereas those with severe anomalies are discouraged from competitive sports. Physical activity as tolerated is important in all patients with adult congenital heart disease. Adult congenital heart specialists should evaluate patients prior to cardiac or noncardiac surgery. Longitudinal clinical follow-up in all Ebstein’s anomaly patients (both repaired and unrepaired) is warranted to follow for signs and symptoms of heart failure, arrhythmias, cyanosis, and other associated findings. This should be ideally performed in collaboration with an adult congenital heart center of excellence.