Excellent 25-Year Survival After Tetralogy of Fallot Correction

Marlene Busko

December 24, 2018

Long-term survival in a large cohort of young patients who underwent surgery to repair tetralogy of Fallot, the most common type of congenital heart disease, is excellent, researchers report in a new study.

Overall, 94.5% of individuals in a US registry who underwent this surgery were alive and had not received a heart transplant 25 years later. Most of the patients were infants at the time they underwent the surgery.

The study by Clayton A. Smith, MD, Emory University School of Medicine, Atlanta, Georgia, and colleagues was published online December 19 in JAMA Cardiology.

Individuals who underwent surgery to repair this cyanotic heart disorder had a biphasic increased risk for mortality, the investigators report, with "an early steep hazard during the first 4 years,...and a late hazard with a slow increase in mortality after 6 years and acceleration 20 years later."

The risk for mortality within 6 years was greater in patients who underwent a two-stage procedure, with a palliative shunt placed first, or who underwent non-valve-sparing surgery or had a genetic abnormality. The risk for dying later was tied only to genetic abnormality.

"The data highlight the shortcomings of early repair, staged, and non-valve-sparing approaches in the early period following repair," Smith and colleagues write. "Long-term transplant-free survival beyond the first 6 years following repair is excellent and influenced primarily by the coexistence of an associated genetic condition."

Most individuals who died had arrhythmias or congestive heart failure, so "continuous surveillance of this cohort is important," the authors note, "in identifying additional risks resulting from the interaction of underlying conditions with cardiovascular morbidities expected with aging."

This study "highlights the value of teams that provide intensive post discharge care, a growing trend in congenital cardiology," Roger Andrew de Freitas, MD, Ann and Robert H. Lurie Children's Hospital of Chicago, writes in an accompanying commentary.

"This robust analysis of a large, contemporary, multicenter database answers an important question about how surgical factors affect mortality over the first several decades of life," he notes, "an essential outcome to be considered along with functional outcomes in evaluating the long-term value of any surgical strategy."

Long-term Survival

Until now, there had been no information about long-term transplant-free survival following repair of tetralogy of Fallot in a large, multicenter US cohort, the authors write.

For this analysis, the investigators linked data from 3283 patients in the Pediatric Cardiac Care Consortium registry who had undergone surgery to repair tetralogy of Fallot from 1982 to 2006 with data from the National Death Index and the Organ Procurement and Transplantation Network.

About half of the patients (56%) were male, and about half (54%) underwent the surgery when they were younger than 1 year; the rest underwent the surgery when they were 1 to <5 years old (38%) or 5 to 21 years old (8%).

Most underwent nonstaged (81%) and non-valve-sparing (68%) surgery.

Most procedures were performed during 1990 to 1999 (59%), and the rest were done during 2000 to 2006 (25%) or 1982 to 1989 (17%).

Most patients did not have a genetic condition (89%), but a few had trisomies (6%), DiGeorge syndrome (2%), or other genetic conditions (3%).

After they were discharged following tetralogy of Fallot repair, 98.6%, 97.8%, 97.1%, 95.5%, and 94.5% of the patients were still alive without a transplant 1, 5, 10, 20, and 25 years later, respectively.

After a median follow-up of 18.5 years, five patients had received heart transplants, and 145 patients had died.

Most deaths were directly or indirectly associated with the surgery and were reported as sudden death or the result of arrhythmia or congestive heart failure.

The median age at death was 1 year (range, 3 days to 20 years).

A total of 4% of patients died in hospital following surgery. Deaths were more common in infants (especially if they were premature) and in patients who had a genetic condition or who had undergone staged surgery or surgery before the year 2000.

The risk for early mortality was three to four times higher with staged repair (hazard ratio [HR], 2.68; 95% confidence interval [CI], 1.59 - 4.49) or a non-valve-sparing operation (HR, 3.76; 95% CI, 1.53 - 9.19).

"The choice between staged vs single-stage repair and between valve-sparing vs not may reflect nonmeasured covariables such as more severe underlying pathology or other comorbidities," the authors acknowledge.

Having a genetic abnormality was tied to a threefold to fourfold increased risk for early death (HR, 3.64; 95% CI, 2.05 - 6.47) and death at 6 years or longer after surgery (HR, 4.41; 95% CI, 2.62 - 7.44).

"Overall long-term transplant-free survival in repaired tetralogy of Fallot is excellent," the authors conclude, "with several factors affecting survival, some of which may be modifiable such as planning of the surgical strategy."

The study was supported by grants from the National Heart, Lung, and Blood Institute and by a National Institutes of Health CTSA award. The authors and the editorialist have disclosed no relevant financial relationships.

JAMA Cardiol. Published December 19, 2018. Full text, Commentary

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