Off-pump direct hepatic veins-to-hemiazygos vein anastomosis after primary Kawashima operation: long-term result

Direct hepatic veins-to-hemiazygos connection offers the balanced distribution of hepatic venous blood to both lungs, not requiring anticoagulation. We report a 13-year follow-up after this type of off-pump Fontan completion. Patient’s hepatic veins-to-hemiazygos confluence increased with growth to allow for unobstructed flow. This unique technique can be recommended in heterotaxy patients, if atrial hepatic venous drainage and hemiazygos vein are in close proximity.

Spontaneous closure of arterio-venous pulmonary fistulas by redirection of hepatic venous blood 9 years after Glenn anastomosis in a 12-year-old girl

We report on a 12-year-old girl with Ebstein’s anomaly after a unidirectional Glenn procedure with surgical ligation of the proximal right pulmonary artery, who suffered from significant central cyanosis caused by multiple arterio-venous fistulas in the right lung. The continuity between the right pulmonary artery and the pulmonary trunk was restored with the use of radiofrequency perforation and consecutive covered stent implantation.

Fontan Revision: Presurgical Planning Using Four-Dimensional (4D) Flow and Three-Dimensional (3D) Printing

Pulmonary arteriovenous malformations (AVMs) can be a complication of certain postoperative Fontan patients whose hepatic venous blood return is not distributed evenly to both lungs. A ten-year-old female, who had previously undergone staged single ventricle palliation for complex congenital heart disease, underwent a Fontan revision due to significant left-sided pulmonary AVMs and increasing arterial oxygen desaturation. The combination of four-dimensional flow cardiac magnetic resonance imaging and three-dimensional printing enabled presurgical planning for a Fontan takedown and diversion of hepatic venous flow to the azygous vein that resulted in significant clinical improvement.

Normal and abnormal pulmonary arteriovenous shunting: occurrence and mechanisms

Severe cyanosis due to pulmonary arteriovenous fistulas occurs often after a bidirectional superior cavopulmonary anastomosis (Glenn operation) and also in some congenital anomalies in which hepatic venous blood bypasses the lungs in the first passage. Relocation of hepatic flow into the lungs usually causes these fistulas to disappear. Similar pulmonary arteriovenous fistulas are observed in hereditary haemorrhagic telangiectasia, and in liver disease (hepatopulmonary syndrome). There is no convincing identification yet of a responsible hepatic factor that produces these lesions. Candidates for such a factor are reviewed, and the possibility of angiotensin or bradykinin contributing to the fistulas is discussed.