Abstract

BACKGROUND
Single ventricle hearts can be surgically palliated by a series of operations culminating in the Fontan procedure, which establishes a total cavopulmonary connection. The second-stage procedure creates a physiologic connection between the superior vena cava and the pulmonary artery.
METHODS
From 1998 to 2010, 557 patients with single ventricle heart disease underwent second-stage surgical palliation. This cohort was retrospectively analyzed to assess patient outcome by a number of anatomic, physiologic, and procedural factors. The analysis excluded patients undergoing hybrid first-stage procedures.
RESULTS
The median age at operation was 165 days (range, 59 days to 49 years). The most common anatomic subtypes were hypoplastic left heart syndrome (52%), tricuspid atresia (12%), unbalanced atrioventricular septal defect (10%), double inlet left ventricle (9%), or other (17%). Left ventricular hypoplasia was present in 70%. A hemi-Fontan procedure was done in 89%, and 11% received a bidirectional Glenn. Concomitant atrioventricular valve repair was necessary in 9%. Early mortality was 4.7%, and 5.9% died after discharge but before Fontan. No early or late deaths occurred in patients with tricuspid atresia and double inlet left ventricle. Multivariate analysis demonstrated ventricular dysfunction, atrioventricular valve regurgitation, and unbalanced atrioventricular septal defect were significant adverse risk factors for survival to Fontan.
CONCLUSIONS
Second-stage palliation can be performed at low risk for patients with left ventricular dominance, but significant risk remains for patients with left ventricular hypoplasia and unbalanced atrioventricular septal defect. Atrioventricular valve insufficiency is a persistent problem that has not been neutralized by repair strategies.

Links

Publisher Full Text

Publisher Full Text

Authors

Lee TM, Aiyagari R, Hirsch JC, Ohye RG, Bove EL, Devaney EJ

Institution

Division of Pediatric Cardiovascular Surgery, Section of Cardiac Surgery, University of Michigan Medical School, Ann Arbor, Michigan 48109, USA.

Source

The Annals of thoracic surgery 93:2 2012 Feb pg 614-8; discussion 619

MeSH

Adolescent
Adult
Anoxia
Child
Child, Preschool
Female
Fontan Procedure
Heart Septal Defects
Heart Valve Prosthesis Implantation
Heart Ventricles
Heterotaxy Syndrome
Humans
Hypoplastic Left Heart Syndrome
Infant
Male
Middle Aged
Mitral Valve Insufficiency
Palliative Care
Postoperative Complications
Pulmonary Artery
Retrospective Studies
Risk Factors
Survival Rate
Treatment Outcome
Tricuspid Atresia
Tricuspid Valve Insufficiency
Vena Cava, Superior
Young Adult

Pub Type(s)

Journal Article

Language

eng

PubMed ID

22197533