Special considerations in the management of adult congenital heart disease

Special considerations for the management of adult congenital heart disease include pregnancyrelated cardiovascular disease, pulmonary arterial hypertension, and arrhythmias. Improvements in the treatment of congenital heart disease mean that more women with congenital heart disease are reaching childbearing age. In BC the Cardiac Obstetrics clinic at St. Paul’s Hospital provides coordinated care for pregnant women with cardiac conditions. The clinic also offers preconception counseling so that couples can make informed choices about pregnancy. Another service based at St. Paul’s Hospital, the Pacific Adult Congenital Heart clinic, helps manage patients with pulmonary arterial hypertension, a progressive condition affecting around 10% of adult congenital heart disease patients. Agents used to treat pulmonary arterial hypertension include endothelin receptor antagonists and phosphodiesterase type 5 inhibitors. The Pacific Adult Congenital Heart clinic also manages patients with a significant arrhythmia, often the first manifestation of deterioration in complex congenital heart disease. Complications that can result from arrhythmias include heart failure, thromboembolism, and sudden cardiac death. A range of complementary imaging modalities aid in the management of all these conditions by enabling the assessment of ventricular and valvular function (echocardiography and magnetic resonance imaging), quantification of right ventricular volume (multidetector computed tomography), and exclusion of coronary stenosis (coronary CT angiography). Complications in adult congenital heart disease (CHD) include pregnancy-related cardio vascular disease (CVD), pulmonary arterial hypertension (PAH), and arrhythmias. Patients with these complications can benefit from a variety of treatments and management approaches. They can also benefit from advances in diagnostic imaging. Pregnancy-related CVD At present, 0.2% to 4.0% of all pregnancies in Western industrialized countries are complicated by cardiovascular disease, and the number of patients who develop cardiac Dr Grewal is a clinical associate professor at the University of British Columbia, and director of the Cardiac Obstetrics Program and cardiologist at the Pacific Adult Congenital Heart (PACH) clinic. She is also an echocardiographer at St. Paul’s Hospital. Dr Brunner is a clinical assistant professor at UBC, and a cardiologist and pulmonary hypertension specialist at Vancouver General Hospital. Dr Ellis is a cardiothoracic radiologist at St. Paul’s Hospital. Dr Swiston is an associate professor at UBC, and director This article has been peer reviewed. of the Pulmonary Hypertension Program. Dr Leipsic is Canada Research Chair in Advanced Cardiopulmonary Imaging, and a radiologist at St. Paul’s Hospital. Dr Levy is a professor of medicine in the Respiratory Division, UBC, and medical director of the Lung Transplant Program. Dr Barlow is director of the Heritable Aortopathy Program at St. Paul’s Hospital, and a cardiologist in the PACH clinic. Dr Chakrabarti is a clinical assistant professor at UBC and a cardiologist in the PACH clinic.