CONGENITAL HEART DISEASE

Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of Fallot repair

A L Knauth^1,2, K Gauvreau¹, A J Powell¹, M J Landzberg^1,2, E P Walsh¹, J E Lock¹, P J del Nido³, T Geva¹

¹ Department of Cardiology, Children’s Hospital Boston, Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA
² Boston Adult Congenital Heart Disease Service, Children’s Hospital Boston, Departments of Pediatrics and Medicine, Harvard Medical School, Boston, Massachusetts, USA
³ Department of Cardiovascular Surgery, Children’s Hospital Boston, Department of Surgery, Harvard Medical School, Boston, Massachusetts, USA

Dr T Geva, Department of Cardiology, Children’s Hospital Boston, 300 Longwood Avenue, Boston, MA 02115, USA; tal.geva{at}cardio.chboston.org

Background: Factors associated with impaired clinical status in a cross-sectional study of patients with repaired tetralogy of Fallot (TOF) have been reported previously.

Objectives: To determine independent predictors of major adverse clinical outcomes late after TOF repair in the same cohort during follow-up evaluated by cardiac magnetic resonance (CMR).

Methods: Clinical status at latest follow-up was ascertained in 88 patients (median time from TOF repair to baseline evaluation 20.7 years; median follow-up from baseline evaluation to most recent follow-up 4.2 years). Major adverse outcomes included (a) death; (b) sustained ventricular tachycardia; and (c) increase in NYHA class to grade III or IV.

Results: 22 major adverse outcomes occurred in 18 patients (20.5%): death in 4, sustained ventricular tachycardia in 8, and increase in NYHA class in 10. Multivariate analysis identified right ventricular (RV) end-diastolic volume Z 7 (odds ratio (OR) = 4.55, 95% confidence interval (CI) 1.10 to 18.8, p = 0.037) and left ventricular (LV) ejection fraction <55% (OR = 8.05, 95% CI 2.14 to 30.2, p = 0.002) as independent predictors of outcome with an area under the receiver operator characteristic curve of 0.850. LV ejection fraction could be replaced by RV ejection fraction <45% in the multivariate model. QRS duration 180 ms also predicted major adverse events but correlated with RV size.

Conclusions: In this cohort, severe RV dilatation and either LV or RV dysfunction assessed by CMR predicted major adverse clinical events. This information may guide risk stratification and therapeutic interventions.

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