Long-term outcomes after Fontan surgery

Rychik, Jack

J Rychik is Director of the Fetal Heart Program at the Cardiac Center at The Children's Hospital of Philadelphia, and Associate Professor of Pediatrics at the University of Pennsylvania School of Medicine, Philadelphia, PA, USA.

Correspondence The Cardiac Center The Children's Hospital of Philadelphia 34th Street and Civic Center Boulevard Philadelphia PA 19104 USA [email protected]

Received 17 February 2008

Accepted 13 March 2008

Published online 13 May 2008

www.nature.com/clinicalpractice doi:10.1038/ncpcardio1220

Nature Clinical Practice Cardiovascular Medicine 5(7):p 368-369, July 2008. | DOI: 10.1038/ncpcardio1220

SYNOPSIS

BACKGROUND

Few studies have investigated long-term outcomes among patients who survive Fontan surgery in childhood.

OBJECTIVES

To elucidate causes of death, predictors of mortality, and survival rates among patients who have undergone Fontan surgery.

DESIGN

This retrospective review included patients who were born before 1 January 1985 and underwent a Fontan procedure at the Children's Hospital, Boston, MA, USA from April 1973 onwards. Fontan surgery was classified into four categories; right atrium (RA) to right ventricle (RV) connection, RA to pulmonary artery (PA) anastomosis, extra-cardiac conduit, and intra-atrial lateral tunnel. Perioperative death was defined as death within 30 days of the procedure or before hospital discharge, and further categorized as early (<30 days) or late (>30 days, during the same hospitalization).

OUTCOME MEASURES

The outcome measures were perioperative and postoperative death.

RESULTS

A total of 261 patients were included in the analysis, 53.6% of whom were male. The median age at which surgery was performed was 7.9 years. RA-PA anastomosis was performed in 51.7% of patients, 37.5% underwent intra-atrial lateral tunnel, 9.6% had an RA-RV connection and 1.1% had an extracardiac conduit. After a median follow-up of 12.2 years, 76 patients (29.1%) had died. Of these deaths, 52 (68.4%) occurred during the perioperative period, with 41 being classified as early and 11 as late. Sudden death occurred in 9.2% of patients, and death was caused by heart failure (HF), thromboembolism, and sepsis in 6.6%, 7.9%, and 2.6% of patients, respectively. There was a temporal reduction in peri-operative mortality from 36.7% for procedures performed before 1982, to 15.7% for procedures performed from 1982 to 1989, to 1.9% for procedures performed after 1990. There were three significant, independent predictors of perioperative mortality; RA-PA anastomosis (odds ratio [OR] 3.9, 95% CI 1.8-8.4; P = 0.0005), hypoplastic left heart syndrome (OR 15.2, 95% CI 4.0-57.5; P < 0.0001), and early surgical era (OR for surgery performed before 1982 3.5, 95% CI 1.7-7.5; P = 0.0011). In patients who survived the early perioperative period, the actuarial event-free survival rates at 1, 5, 10, 15, 20, and 25 years were 96.9%, 93.7%, 89.9%, 87.3%, 82.6%, and 69.6%, respectively. There was a notable increase in thromboembolic mortality 15 years after surgery, with lack of warfarin or aspirin therapy being a significant, independent predictor of death. The risk of death from HF increased after 10 years, and significant independent risk factors included single RV morphology, protein-losing enteropathy, and increased RA pressure. The annualized rate of sudden death was 0.15%. No significant independent predictors of sudden death were identified.

CONCLUSION

Most deaths among patients undergoing Fontan surgery occur during the perioperative period, although the proportion of perioperative deaths has decreased steadily since 1982. The risk of death from other causes (HF, thromboembolism, and sudden death) increases in the years following the procedure.