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Special considerations for the management of adult congenital heart disease include pregnancy-related 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). [ABSTRACT FROM AUTHOR]

One of the main challenges for clinicians evaluating patients with systemic sarcoidosis is determining when and how to investigate for cardiac involvement. Owing to a low index of suspicion, and in some cases lack of pulmonary involvement, it may take many years before cardiac sarcoidosis is finally diagnosed. This is regrettable as cardiac sarcoidosis is readily treatable with corticosteroids, and because prompt diagnosis and treatment may prevent severe complications such as left ventricular dysfunction, heart failure, malignant arrhythmias, and even death. The literature supports screening for cardiac sarcoidosis with a clinical approach that includes history taking, physical examination, an electrocardiogram, an echocardiogram, and Holter monitoring. When the results of these investigations indicate abnormalities, cardiac MRI should be considered. While endomyocardial biopsy can confirm cardiac sarcoidosis, in most cases it is not required. Once cardiac sarcoidosis is confirmed, the patient should be monitored closely for the development of lethal arrhythmias and referred to an electrophysiologist if these are detected. [ABSTRACT FROM AUTHOR]