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Prevalence of haemoptysis in adults with pulmonary atresia and ventricular septal defect, and the role of mammary artery collateral vessels
  1. K Greaves1,
  2. P Bye2,
  3. G Parker3,
  4. D S Celermajer1,*
  1. 1Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
  2. 2Department of Respiratory Medicine, Royal Prince Alfred Hospital
  3. 3Department of Radiology, Royal Prince Alfred Hospital
  1. Correspondence to:
    Professor David S Celermajer, Department of Cardiology, Royal Prince Alfred Hospital, Page Chest Pavilion Building, Missenden Road, Camperdown, Sydney, NSW 2050, Australia;

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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.1

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.1 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.


The records of all patients with PA-VSD regularly attending the adult CHD clinic at our tertiary referral centre (established in 1991) were examined to determine the number of patients who were recorded to have had a past history of haemoptysis. Twenty six patients, of whom eight were …

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  • * Also Department of Medicine, University of Sydney