Surgical improvement of hepatic venous mixing to resolve systemic arterial hypoxemia associated with post-Fontan pulmonary arteriovenous fistulae.

Pulmonary arteriovenous fistulae are known to develop in patients who have functional single-ventricle heart disease and interruption of the inferior vena cava with direct hepatic drainage to the heart, in which a bidirectional Glenn shunt is the only source of pulmonary blood flow. The progressive systemic arterial hypoxemia that is associated with pulmonary arteriovenous fistulae can have important clinical consequences. Baffling the hepatic venous return to the pulmonary circulation can alleviate pulmonary arteriovenous fistulae.Herein, we present the case of a 13-year-old patient with modified Fontan anatomy and pulmonary arteriovenous fistulae, in whom redirection of a previously placed hepatic venous-to-right pulmonary artery conduit was required in order to increase systemic arterial oxygen saturation. Revision of the conduit improved mixing of hepatic venous effluent with blood flow from the bidirectional Glenn shunt. Three years after this revision, the patient's oxygen saturation remained stable at 90%, and his physical activity was markedly improved. We present our rationale for selected redirection of the conduit and discuss other surgical options that can improve hypoxemia that is associated with pulmonary arteriovenous fistulae.

Bidirectional Glenn shunt as an adjunct to surgical repair of congenital heart disease associated with pulmonary outflow obstruction: relevance of the fluid pressure drop-flow relationship.

A bidirectional Glenn shunt (BGS) was successfully incorporated into a two-ventricle repair for 10 patients (age, 3-17 years) who had congenital heart disease associated with severe pulmonary outflow obstruction. The BGS was used to volume-unload the pulmonary ventricle faced with residual outflow obstruction, thereby avoiding the need for insertion of a ventricle-to-pulmonary artery conduit. Transthoracic Doppler flow velocity analysis was used to determine transpulmonary peak systolic pressure drops as a measure of obstruction. Preoperative values ranged from 70 to 100 mmHg, and postoperative values ranged from less than 10 to 16 mmHg. At this writing, all patients are doing well 15 to 52 months after surgery. To gain further insight into the reduced pressure drop that may be achieved by decreasing flow rate across obstruction, a computer-based description of fluid flow was used to simulate blood traversing circumferentially narrowed passages. Overall pressure drops and associated flow energy losses were determined from numeric solutions (using finite-element analysis) to the Navier-Stokes equations for the proposed fluid reactions. Pressure drops and flow energy losses were found to decrease dramatically as flow rate was progressively reduced. For selected patients, a BGS can be an effective adjunct to the surgical treatment of pulmonary outflow obstruction. This approach avoids the use of a ventricle-to-pulmonary artery conduit, and thus the inevitable need in most patients for reoperations because of somatic growth, conduit failure, or both.

Medtronic freestyle valves in right ventricular outflow tract reconstruction.

BACKGROUND: Various pulmonary valve substitutes, with their inherent limitations, have been used in children and young adults. We chose the Medtronic Freestyle (Medtronics, Minneapolis, MN) valve because of its excellent hemodynamics, known durability in adults, and design features that allow modifications during implantation. METHODS: Over a 3 1/2 year period the Freestyle valve was implanted in 47 patients age 2 to 58 years (mean 14.2, median 12.0) in the pulmonary position. All patients had pulmonic stenosis and(or) insufficiency from previous operations for tetralogy (27), pulmonary atresia (6), truncus (4), or other diagnosis (10). The indication for surgery was pure pulmonary insufficiency in 11 patients, pulmonic stenosis in 3, and mixed stenosis and insufficiency in 33. Root replacement technique was used with additional enlargement of the pulmonary artery branches in 10 patients. RESULTS: Intraoperatively, one patient sustained a right ventricle tear and one a circumflex coronary artery injury during the dissection. There was one postoperative death. Two patients developed late subvalvular pannus formation, one of whom required reoperation. One patient was found to have an echo gradient of 95 mm Hg due to decreased leaflet motion and underwent cardiac catheterization at which the peak systolic gradient was determined to be 50 mm Hg. He has not required reintervention during his 3 1/2 years of follow-up. The remaining 43 patients have minimal gradients or insufficiency. All surviving patients are in New York Heart Association (NYHA) Class I. CONCLUSIONS: The Medtronic Freestyle valve is an attractive alternative for RVOT (right ventricular outflow tract) reconstruction in children. It is readily available, versatile, and has excellent hemodynamic characteristics. Although long term follow-up is not yet available, longevity of this prosthesis, and freedom from complications, will hopefully be superior to valves with stents.

Multidose cardioplegia in a complex arterial switch procedure.

Integrated cardioplegia techniques have gained wide acceptance by surgeons performing adult cardiac surgery, because patients being referred are likely to have poor ventricular function and energy-depleted hearts. In addition, the increasing complexity of available procedures has led to an increased threat of reperfusion injury and calcium contracture ("stone heart") after prolonged ischemia. In this report, we describe the case of a newborn with transposition of the great arteries that survived almost 6 hours of ischemic time and has normal ventricular function postoperatively. We attribute this outcome to the myocardial protection employed throughout the procedure which allowed successful correction of a technical problem.