Clinical characteristics and outcomes in middle-aged Fontan patients.

BACKGROUND

Middle-aged Fontan patients are underrepresented in contemporary studies. The purpose of this study was to describe the clinical characteristics and outcomes in middle-aged patients with Fontan palliation.

METHODS

All adults with Fontan palliation were divided into 'middle-aged' group (>40 years of age at baseline encounter), and 'young adult' group. Clinical characteristics were assessed across multiple domains (atrial arrhythmias, heart failure, and hepatorenal function). Clinical outcomes were assessed as composite outcome of death/heart transplant.

RESULTS

Of 455 adults (median age 26 years), 63 (14 %) were middle-aged adults while 392 (86 %) were young adults. Compared to the young adult group, the middle-aged group had a higher prevalence of atrial arrhythmias, greater neurohormonal activation, and worse hepatorenal function. Of 63 patients in the middle-aged group, 26 (41 %) patients died, and 4 (6 %) patients underwent heart transplant. The middle-aged group had a higher 5-year cumulative incidence of death/transplant (45 % versus 21 %, p < 0.001), and 10-year cumulative incidence of death/transplant (64 % versus 29 %, p < 0.001) compared to the young adult group. Higher pulmonary artery wedge pressure (hazard ratio 1.18 [1.09-1.27]), and higher model for end-stage liver disease excluding international normalized ratio score (hazard ratio 1.07, [1.03-1.14]), were associated with death/transplant.

CONCLUSIONS

About 14 % of adults with Fontan palliation were middle-aged, and these patients had higher prevalence of cardiovascular comorbidities, hepatorenal dysfunction, and mortality. These data underscore the need for better risk stratification strategies to identify patients at risk for adverse outcomes and allow for early referral for transplant evaluation in this emerging subpopulation.

CLINICAL SUMMARY: The purpose of this study was to describe the clinical characteristics (comorbidities and end-organ dysfunction) and outcomes (death/transplant) in middle-aged patients with Fontan palliation. Of 455 patients (median age 26 years), 63 (14 %) were middle-aged adults while 392 (86 %) were young adults. Compared to the young adult group, the middle-aged group had a higher prevalence of atrial arrhythmias, greater neurohormonal activation, worse hepatorenal function, and higher risk of death/transplant. The correlates of death/transplant were higher pulmonary artery wedge pressure and hepatorenal dysfunction. These data underscore the need for better risk stratification in this emerging population.