Rupture of pulmonary aneurysms in association with long-standing Waterston shunts

Abstract

Anastomosis of the ascending aorta to the right pulmonary artery, the so-called Waterston shunt, was undertaken as a palliative procedure for children with cyanotic congenital heart disease due to obstruction of the pulmonary outflow tract with reduced pulmonary blood flow. We present the clinicopathological correlations in two patients who underwent construction of Waterston shunts as neonates, and subsequently died of ruptured pulmonary aneurysms in adult life. Rupture should, therefore, be recognized as a late complication of this procedure, and be considered in the long-term follow-up of such patients, especially when the shunted lung is hypertensive.

Echocardiogram‐gated multislice spiral CT in functionally univentricular heart following long‐lasting Waterston–Cooley anastomosis

“…10,11 Rupture of pulmonary aneurysms and dissecting haematomas of the pulmonary artery in association with longstanding Waterston shunts have been described as well. 12,13 Reviewing the published reports, patients with tricuspid atresia only had a 10% chance of survival beyond the first year without surgery. 14 Fig.…”

“…These complications include preferential distribution of shunt flow to the right lung, thrombosis, embolism, kinking, narrowing or obstruction of the right pulmonary artery at the anastomotic site, increasing stenosis or atresia of the right ventricular outflow tract, hypoplasia of the left pulmonary artery and obstruction of the shunt itself 10,11 . Rupture of pulmonary aneurysms and dissecting haematomas of the pulmonary artery in association with long‐standing Waterston shunts have been described as well 12,13 …”

Just because you can, doesn't mean you should: The flipside of technical feasibility

“…The risk of rupture of PA aneurysms is associated with pulmonary arterial hypertension (PAH) 4 ; therefore, it is reasonable to aim at reduction of PA pressure. On the other hand, patients with PAH that persists after corrective surgery have poorer survival compared with those with other types of PAH associated with congenital heart disease, arguing against surgical repair.…”