Off Pump Completion Extracardiac Fontan in Pulmonary Atresia With Intact Septum

The authors present an off pump extracardiac non-fenestrated Fontan in a child with pulmonary atresia and intact ventricular septum.

This is a 6-year old, 16-kg girl with a functional single ventricle. Her previous two palliations were a left MBT shunt followed by a bidirectional cavopulmonary anastomosis (Glenn). She presented with cyanosis (saturation of low-mid 70%) and polycythemia with preoperative hemoglobin level of 21 g/dl.

Echocardiography showed good ventricular function and widely patent Glenn. Cardiac catheterization showed satisfactory hemodynamics for completion Fontan but multiple veno-venous and arteriovenous collaterals. Coil embolization was performed.

A third time sternotomy was performed. The right and left main branch pulmonary arteries were dissected, the ascending aorta, the Glenn and the inferior vena cava (IVC) were all dissected and fully mobilized. Systemic heparinization was performed. A pressure monitoring line was placed in the SVC. This measured 10-12 mmHg. Proximal and distal control of the left branch pulmonary artery was achieved. A 20 mm Gore-Tex graft was chosen as the Fontan conduit.

An arteriotomy was performed on the inferior surface of the left main branch pulmonary artery. An end-to-side graft-to-pulmonary artery anastomosis was then constructed. The superior vena caval pressure remained no higher than 20 mmHg during the construction of the graft to pulmonary artery anastomosis with satisfactory cerebral NIRS.

In preparation for the IVC–to-graft anastomosis, a passive IVC drainage circuit was created by placing a single stage 18 Fr venous cannula in the right atrium and an 18 Fr. right angled venous cannula in the IVC. Both cannulae were connected with a Y-connector and the patient was placed in a Trendelenburg position. Low dose Dopamine was initiated. The IVC was then test clamped and the patient hemodynamics remained satisfactory. The IVC was then disconnected from the atrium between two vascular clamps. Its atrial end was oversewn. We then constructed the IVC-to-graft anastomosis. The graft length and orientation was adequate. The Fontan pressure measured 12-14 mmHg. Echocardiogram showed widely patent Fontan connections with laminar flow in both branch pulmonary arteries. The clamp time for the pulmonary-graft Anastomosis: 24 minutes, and the clamp time for the Graft-to-IVC Anastomosis: 13 minutes.

The patient was extubated in the operating room and received no transfusions. The remaining postoperative course was uneventful and chest tubes were removed on day 3 and 4 postoperatively.

References

1. Marcelletti C, Corno A, Giannico S, Marino B. Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc Surg.1990 Aug;100(2):228-32
2. Said SM, Burkhart HM, Dearani JA. The Fontan Connections: Past, Present and Future. World J Pediatr Congenit Heart Surg. 2012 Apr 1;3(2):171-82
3. Ovroutski S, Sohn C, Miera O, Peters B, Alexi-Meskishvili V, Hetzer R, Berger F, et al. Imporved early postoperative outcome for extracardiac Fontan operation without cardiopulmonary bypass: a single-center experience. Eur J Cardiothorac Surg. 2013 May;43(5):952-7
4. Mainwaring RD, Reddy VM, Hanley FL. Completion of the Three-Stage Fontan Pathway Without Cardiopulmonary Bypass. World J Pediatr Congenit Heart Surg. 2014 Jul;5(3):427-33

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