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A 21-month-old boy was diagnosed with partial anomalous pulmonary venous connection, with the right upper pulmonary veins draining to the superior vena cava (SVC). Intraoperatively, it became evident that the right upper pulmonary veins connected to the azygous vein before draining to the SVC.

Partial anomalous pulmonary venous return is a congenital anomaly in which some of the pulmonary veins drain into the right atrium or one of its venous tributaries. Although excellent long-term results have been described for the classic two-patch technique repair in older patients, The Warden procedure has an important role in smaller and younger patients and those patients in whom the superior vena cava (SVC) may be small and the right-sided pulmonary veins may connect high, at or above the azygos vein. In addition to prevention of early and late sinus node dysfunction, the Warden procedure helps to avoid systemic or pulmonary venous obstruction if care is taken to ensure a tension-free Warden anastomosis. Patch extension or interposition of prosthetic grafts with no growth potential have been used in instances of high partial anomalous pulmonary venous connection (PAPVC) to SVC. We report three cases of high PAPVC to SVC using an innovative technique of incorporating the azygos vein into the Warden anastomosis for a tension-free suture line.

Patients with functionally single ventricle and interrupted inferior vena cava may develop progressive cyanosis soon after the Kawashima operation. Therefore, early redirection of the hepatic venous return to the pulmonary circulation is recommended. To avoid performing an early redo sternotomy, we propose to prepare these patients for the interventional Fontan-Kreutzer at the time of the Kawashima operation using a technical modification of the approach reported by Prabhu and coworkers in 2017. The technique described here uses an expanded polytetrafluoroethylene conduit interposed between the hepatic veins and the right pulmonary artery. This graft is everted and divided into two portions with a pericardial patch. The lower one is widely opened and anastomosed side-to-side to the atrium. A few months after the operation, percutaneous Fontan-Kreutzer completion can easily be performed using covered stents to open the patch and at the same time close the opening between the conduit and the atrium.

Background: Patients with heterotaxy, single ventricle and interrupted inferior vena cava are at risk of developing significant pulmonary arteriovenous malformations and cyanosis, and inequitable distribution of hepatic factor has been implicated in their development. We describe our experience with a technique for hepatic vein incorporation that reliably provides resolution of cyanosis and presumably equitable hepatic factor distribution., Methods: A retrospective review of a single-surgeon experience was conducted for patients who underwent this modified Fontan operation utilizing an extracardiac conduit from the hepatic veins to the dominant superior cavopulmonary connection. Preoperative characteristics and imaging, operative details, and postoperative course and imaging were abstracted., Results: Median age at operation was 5 years (2-10 years) and median weight was 19.6 kg (11.8-23 kg). Sixty percent (3/5) of patients had Fontan completion without cardiopulmonary bypass, and follow-up was complete at a median of 14 months (range 1-20 months). Systemic saturations increased significantly from 81% ± 1.9% preoperatively to 95% ± 3.5% postoperatively, P = .0008. Median length of stay was 10 days (range: 7-14 days). No deaths occurred. One patient required reoperation for bleeding and one was readmitted for pleural effusion. Postoperative imaging suggested distribution of hepatic factor to all lung segments with improved pulmonary arteriovenous malformation burden., Conclusions: Hepatic vein incorporation for patients with heterotaxy and interrupted inferior vena cava should optimally provide equitable pulmonary distribution of hepatic factor with resolution of cyanosis. The described technique is performed through a conventional approach, is facile, and improves cyanosis in these complex patients.

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.

Introduction: A peripherally inserted central catheter (PICC) is widely used for long chemotherapy regimens or total parenteral nutrition. However, spontaneous migration of the catheter into the azygos vein after satisfactory initial placement has occurred in rare cases., Methods: We discuss three such cases in esophageal cancer patients., Results: In two patients, the migrated catheters were found during surgery and were repositioned without complications. However, in the third case, the migrated catheter had to be removed to avoid severe complications, as the catheter and the azygos vein were ligatured during operation without notice., Conclusions: Migrations might be related to the material properties of silicone PICCs, the short insertion length, the left-sided placement, the changes of thoracic pressure and the intraoperative traction by thoracic surgeons. In conclusion, we recommend routine checks before cutting off the azygos vein during the operations for esophageal cancer patients with left-sided PICCs.

We report on an emergent use of hepatic-to-azygous vein connection using a 10-mm polytetrafluoroethylene graft in a 6-year-old girl who developed acute thrombosis of an extracardiac graft conduit from the hepatic veins to the pulmonary arteries. She had left atrial isomerism, interrupted inferior vena cava with azygous continuation, and had undergone surgical creation of bilateral superior cavopulmonary connections 5 years earlier. The emergency procedure to create hepatic-to-azygous connection resulted in successful salvage of the patient who was subsequently able to maintain stable oxygen saturations and experienced rapid regression of hepatic congestion and pleural effusions. Six months following the procedure, oxygen saturation was above 90%. Repeated echocardiography and computed tomography angiography showed patency of the hepatic-to-azygous connection.

Central venous catheters are established as vascular access in hemodialysis therapy. Vascular catheter misdirection may occur and is a well known problem. We present a rare catheter malposition in a young dialysis patient with consequent dilatation of the azygos vein system, simulating the appearance of an azygos continuation syndrome.