Hemoptysis in a single lung Fontan: percutaneous approach

The Fontan procedure is sometimes the last palliative surgery for complex congenital heart disease with single-ventricle physiology. According to some authors, atrial-baffle fenestrations with right-to-left shunts have improved the surgical outcome of the Fontan operation. Percutaneous fenestration closure at a later stage may increase saturations and reduce the risk of embolic complications. A case could be made that the fenestration should be left patent if the patient, having lost the continuity of the pulmonary artery to one lung, only has a single functional lung.

The case of an 11-year-old boy with a severe form of Ebstein anomaly is reported. He had undergone a fenestrated Fontan procedure and had lost the continuity to the left pulmonary artery. Further intervention was discouraged at other centers but he was eventually referred to our institution due to increasing cyanosis and severe hemoptysis. Several right-sided arteriovenous pulmonary malformations and collateral arteries were embolized percutaneously; a major right-to-left shunt at the fenestration in the atrial baffle was also occluded with an Amplatzer device. His saturation rose from 78 to 94% and his exercise tolerance increased, without further hemoptysis.