This is a single center, retrospective study of adult patients with a diagnosis of un-repaired complex cyanotic congenital heart disease including Eisenmenger’s Syndrome, single ventricle, pulmonary atresia with aorticopulmonary collaterals, complete atrio-ventricular canal defects, double inlet left ventricle, heterotaxy syndromes and truncus arteriosus. Patients with surgically placed systemic to pulmonary artery shunts will be included.
Although generally incompatible with adult survival, rare patients with un-operated or palliated complex cyanotic congenital heart defects (CCHD) survive well into adulthood. Symptoms related to poor pulmonary blood flow and/ or increasing pulmonary vascular resistance progress with advancing age. Percutaneous interventions to improve symptoms and relieve hypoxemia have not been previously reported in adult patients with complex cyanotic congenital heart disease. Percutaneous interventions in patients with cyanotic congenital heart disease may be generally broken down into three types: percutaneous occlusion of shunt lesions, percutaneous balloon valvotomy or valvuloplasty, and balloon angioplasty and stenting of vascular structures (1). Percutaneous interventions for occluded or stenotic systemic to pulmonary artery shunts in childhood have been described (2,3). However, procedures to improve pulmonary blood flow in adults with CCHD have not previously been described.