Prevalence of haemoptysis in adults with pulmonary atresia and ventricular septal defect, and the role of mammary artery collateral vessels.

Recurrent or massive haemoptysis is a potentially lethal complication occurring in patients with congenital heart disease (CHD). In particular, it is most frequently described in patients with pulmonary vascular obstructive disease such as tetralogy of Fallot and pulmonary atresia with ventricular septal defect (PA-VSD). Although widely believed to be a rare complication of PA-VSD, the true prevalence of this complication and anatomic vessels involved are unknown. Most of the patients who survive to adolescence or adulthood have had palliative or corrective surgery, and have developed an alternative blood supply to the lungs through systemic-to-pulmonary collateral vessels. These collateral vessels are prone to rupture and may result in serious and even fatal haemoptysis. Selective embolisation of these systemic vessels is a widely accepted and successful technique used in the control of recurrent or massive haemoptysis, but identification of the culprit collateral(s) can be difficult. We sought to determine the prevalence of haemoptysis in a cohort of adult patients with PA-VSD and report the collateral vessels involved.