40-Year Follow-up after the Fontan Operation: Long-term Outcome of 1,052 Patients

Educational Objectives

Report on benefits of performing a Fontan procedure in infants who require it and identify those infants at highest risk for postoperative complications.

Summary

Take-home Messages:

• With better patient selection and an evolution of surgical techniques and medical management of patients after the Fontan operation, there has been improvement in survival over the last 40 years.
• There are certainly variables that increase risk and pose significant management challenges.
• There remains a need to further improve the procedure itself as well as management of these patients to ensure that outcomes continue to improve for future generations.

Since its introduction in 1971, the Fontan operation has become a mainstay of the palliative strategy for infants born with the heterogeneous collection of congenital heart defects characterized by a functional single ventricle. Theoretically, the Fontan operation separates the systemic and pulmonary venous returns to ameliorate the disadvantages of long-term hypoxemia, reduce thromboembolic events, preserve ventricular function, and prolong survival for patients with single-ventricle physiology.

However, there have been some negative consequences of this approach, including premature death, ventricular failure, thromboembolic disease, arrhythmia, liver disease, and protein-losing enteropathy. Recently, a team of investigators sought to determine long-term outcomes for all patients who have had a Fontan operation at the Mayo Clinic, Rochester, Minnesota, over a 40-year period, covering almost the entirety of the history of this operation.¹

The myriad of small advances in surgical approach and postoperative management over the last 40 years have, in sum, led to important overall improvements in outcomes. In an accompanying editorial comment, David J. Goldberg, MD, Children’s Hospital of Philadelphia and Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, wrote that perhaps the most striking, but not surprising, is the improvement in short- and long-term outcomes in more recent eras.² The Mayo analysis demonstrated a late survival of 95% at 10 years for the cohort that underwent a Fontan operation after 2001. Survival in the initial era (1973 to 1990) was 69%, and survival in the middle era (1991 to 2000) was 89%.

Reason for Optimism

The overall 10-, 20-, and 30-year survival in the Mayo data from the time of operation was 74%, 61%, and 43%, respectively. While it is too soon to predict the 20- and 30-year survival rates for the most recent cohort, the 10-year data provide a reason for optimism.

Over time, congenital heart surgeons have moved away from the atriopulmonary connection, instead generally favoring an intra-atrial lateral tunnel or an extracardiac conduit. In the Mayo experience, the extracardiac conduit appeared to have the lowest mortality, although it may be that the lateral tunnel suffered in this comparison due to its use in an earlier era.

The authors also documented the increased mortality associated with performing the Fontan operation in “high-risk” patients. Patients who went into the Fontan operation with an elevated pulmonary arterial pressure fared less well than their counterparts with lower pulmonary arterial pressures. The development of ventricular failure, cirrhosis, arrhythmias, and the need for reoperation during long-term follow-up seemed to pose significant management challenges. Similarly, the need for atrioventricular valve surgery at the time of Fontan procedure conferred increased risk, as did the absence of normal sinus rhythm.

Dr. Goldberg noted, “Ultimately, the Fontan operation was a paradigm-shifting advancement in the care of children born with single-ventricle congenital heart disease. Over the years, technical adjustments and improvements in anesthesia and postoperative care have led to substantial improvements in short- and long-term survival.”

However, he continued, “As a community, our work is not done. We need to maintain our focus on ways to improve the efficiency of this circulation to ensure that outcomes continue to improve for future generations.”

Readings

1. Pundi KN, Johnson JN, Dearani JA, et al. 40-Year Follow-Up After the Fontan Operation: Long-Term Outcomes of 1,052 Patients. J Am Coll Cardiol 2015;66:1700-10. http://content.onlinejacc.org/article.aspx?articleID=2449093

2. Goldberg DJ. The Fontan Operation: Improved Outcomes, Uncertain Future. J Am Coll Cardiol 2015;66:1711-3. http://content.onlinejacc.org/article.aspx?articleID=2449092

Disclosures

Valentin Fuster, MD, PhD, MACC
This author has nothing to disclose.

The planning committee reported nothing to disclose.

A = Advisory panel B = Speakers’ bureau C = Consultant fees/honoraria D = Data and Safety Monitoring Board E = Equity interests/stock options F = Fellowship support G = Grant support L = Licensing Agreement O = Other relationship R = Royalties S = Salary W = Expert witness

Acknowledgements

CME/CE INFO

Accreditation:

The Audio- Digest Foundation is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.

The Audio- Digest Foundation designates this enduring material for a maximum of 0 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Audio Digest Foundation is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center's (ANCC's) Commission on Accreditation. Audio Digest Foundation designates this activity for 0 CE contact hours.

Lecture ID:

AC481116

Expiration:

This CME course qualifies for AMA PRA Category 1 Credits™ for 3 years from the date of publication.

Instructions:

To earn CME/CE credit for this course, you must complete all the following components in the order recommended: (1) Review introductory course content, including Educational Objectives and Faculty/Planner Disclosures; (2) Listen to the audio program and review accompanying learning materials; (3) Complete posttest (only after completing Step 2) and earn a passing score of at least 80%. Taking the course Pretest and completing the Evaluation Survey are strongly recommended (but not mandatory) components of completing this CME/CE course.

Estimated time to complete this CME/CE course:

Approximately 2x the length of the recorded lecture to account for time spent studying accompanying learning materials and completing tests.

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