Author + information
- Clinton Cochran,
- Lindsey Gakenheimer-Smith,
- Sunkyung Yu,
- Ray Lowery,
- Jimmy Lu,
- Maryam Ghadimi Mahani,
- Prachi Agarwal and
- Adam Dorfman
In repaired tetralogy of Fallot (rTOF), pulmonary insufficiency and volume overload result in varying degrees of right ventricular (RV) dilation. A subset of patients are diagnosed at initial cardiac magnetic resonance (CMR) with a massively dilated RV, far beyond published pulmonary valve replacement (PVR) criteria; these RVs are unlikely to return to normal size after PVR and patients may be at greater risk of poor outcomes. This study aims to identify risk factors for massive RV dilation at initial CMR.
A nested case-control study was conducted in all patients with rTOF with massive RV dilation (end-diastolic volume [EDV] ≥200 ml/m2, based on published data for distribution of EDV in this population) on initial CMR from 10/2007-3/2015. Patients were matched with rTOF controls with EDV<200 mL/m2 by age at first CMR, gender, and type of repair. Multivariable logistic regression was used to identify anatomic, clinical and surgical risk factors associated with massive RV dilation.
Of 387 rTOF patients identified, 39 subjects (median age 21.9 years, interquartile range [IQR] 15.5-31.0, 72% male, median RV EDV 227 mL/m2, IQR 213-250) met inclusion criteria and were compared to 73 controls (median RV EDV 155 ml/m2, IQR 130-169). In univariate analysis, age at repair and non-Caucasian race were significantly associated with massive RV dilation (both p=0.01). Use of diuretics at the time of first CMR trended toward significance (p=0.07). In multivariable analysis, repair at >6 months of age (adjusted odds ratio [AOR] 2.95, 95% confidence interval [CI] 1.14 - 7.61, p=0.03) and non-Caucasian race (AOR = 7.57, 95% CI 1.73-33.2, p=0.01) were independently associated with massive RV dilation. Era of repair, time since repair, history of systemic to pulmonary shunt palliation, genetic anomaly and additional cardiac lesions did not differ between groups.
Age > 6 months at repair and non-Caucasian race are independently associated with massively dilated RV >200 ml/m2 at first CMR in rTOF patients. Earlier referral for CMR in the presence of these risk factors may be indicated. The potential role of healthcare disparities in these findings requires further investigation.
Poster Hall, Hall A/B
Sunday, March 11, 2018, 3:45 p.m.-4:30 p.m.
Session Title: Outcomes: Tetralogy of Fallot, Ebstein, Fontan and PDA
Abstract Category: 10. Congenital Heart Disease: Pediatric
Presentation Number: 1255-366
- 2018 American College of Cardiology Foundation