Results of 100 Consecutive Extracardiac Conduit Fontan Operations

Abstract

Throughout the years, the experience with Fontan's operation has increased and has opened the way to a much wider application of this principle. A number of major risk factors have been identified and managed by several modifications of the original Fontan procedure. In the past 15 years, operative and postoperative risks have been controlled to a major extent by the application of intermediate surgical procedures. Modifications to the original technique have been designed to simplify surgery and better protect the myocardium by avoiding, as much as possible, prolonged ischemic time. In 1988, we developed a new form of total extracardiac right heart bypass by means of associating an extracardiac conduit placed between the inferior vena cava (IVC) and the pulmonary artery with a bidirectional cavopulmonary anastomosis (BCPA)--the so-called total extracardiac cavopulmonary connection (TECC). Between November 1997 and October 2003, 100 patients with complex cardiac anomalies underwent a modified Fontan operation by TECC. In 88 patients, the repair was staged by preliminary BCPA that was bilateral in 15 patients and associated with a modified Damus-Kaye-Stansel procedure to bypass a subaortic obstruction in 24 patients. Early (in hospital) deaths occurred in 6% of patients and the extracardiac conduit was taken down in 2 additional patients for a total early failure rate of 8%. The cause of death was myocardial failure in 5 patients. Pulmonary artery distortion or hypoplasia appeared to be the cause of death in 1 patient and the cause of failure in 1 patient. Atrioventricular valve regurgitation was the cause of failure in 1 patient. Follow-up to December 2003 was available in all surviving patients. There were no late deaths. At follow-up, 87 patients (89%) were in New York Heart Association (NYHA) functional class I, 4 in class II, and 3 in class III due to moderate to severe atrioventricular valve regurgitation. Use of the following guidelines can result in the achievement of orthoterminal repair with complete separation of pulmonary and systemic circulation, with negligible early mortality and a long-term NYHA class I: (1) Not all patients with univentricular heart (UVH) should undergo the extracardiac Fontan procedure; (2) in UVH with favorable streaming, a Q (p)/Q (s) ratio of approximately 1 to 1, and mild cyanosis, natural history might be preferable to surgical history; (3) any form of fenestration is contradictory to orthodox application of Fontan's principle. Children in whom a planned fenestration seems necessary because of suboptimal conditions should instead undergo a combination of BCPA and associated forward pulmonary blood flow; and (4) negligible mortality should be considered mandatory in UVH, normal pulmonary arteries, and absent cardiomegaly after appropriated and correct staging.

Pacemaker treatment after Fontan surgery—A Swedish national study

“…This finding is in contrast to a study byBalaji 9 et al, where no difference regarding pacemaker implantation was found, but in line with the results reported by Downing et al, who also found an association between LT and need for pacemaker 6. EC type TCPC, which minimizes the ischemic time and atrial suture lines, has been suggested to reduce the incidence of atrial tachycardia 11. However, as the surgical procedure switched from LT to EC in Sweden beginning in 1998, we cannot yet estimate the long-term need for a pacemaker in patients who received an extracardiac tunnel.…”

The Spectrum of Long-term Electrophysiologic Abnormalities in Patients with Univentricular Hearts

“…Although some patients may remain as unreparied or palliated single ventricle, the standard cardiovascular surgical approach to patients with a univentricular heart is a staged repair to a Fontan procedure. The Fontan surgery has undergone multiple modifications from the initial atrail appendage to pulmonary artery anastamosis, to the lateral tunnel, to the more recent extracardiac conduit 1–10 . From an electrophysiologic viewpoint, over half of these patients will develop a cardiac rhythm abnormality 5–10 years after the Fontan procedure 1–22 .…”

Mechanically Assisted Total Cavopulmonary Connection With an Axial Flow Pump: Computational and In Vivo Study

“…Total cavopulmonary connection (TCPC) represents an efficient palliative surgery in single‐ventricle pathologies . TCPC produces a unique physiology in which pulmonary blood flow is driven primarily by the residual force of the systemic ventricle, causing a substantial increase of systemic venous pressure.…”