Key result
In infants with borderline left hearts, absence of aortic coarctation repair was associated with a higher risk of death, transplantation, or single-ventricle conversion (HR 54.3; 95% CI 6.3-47.1).
Why the study?
CMR may augment 2D echocardiography in decision-making for biventricular repair in borderline hypoplastic left hearts, but the relationship between modalities and imaging predictors of management and survival needed evaluation.
Does the use of CMR-derived variables, such as ascending aortic flow, improve patient selection and predict transplant-free survival for biventricular repair in infants with borderline hypoplastic left hearts?
Cohort (n=67)
Does the use of CMR-derived variables, such as ascending aortic flow, improve patient selection and predict transplant-free survival for biventricular repair in infants with borderline hypoplastic left hearts?
CMR-derived ascending aortic flow is a crucial integrative index for assigning infants with borderline hypoplastic left hearts to biventricular repair, allowing successful repair in many patients who would have been precluded by historical 2D echocardiographic criteria.
May support biventricular repair when coarctation is addressed; leaves open CMR's role in prospective selection.
Cardiac magnetic resonance imaging (CMR) may augment 2-dimensional (2D) echocardiography in decision-making for biventricular repair in borderline hypoplastic left hearts. This study evaluates: 1) the relationship between 2D echocardiography and CMR; 2) imaging variables affecting assignment to biventricular vs non-biventricular management; and 3) variables affecting transplant-free biventricular survival. We reviewed clinical, echocardiographic, and CMR data in 67 infants, including CMR-determined ascending aortic (AAo) flow and comparable left ventricular end-diastolic volume indexed (LVEDVi) by 2D-echocardiography and CMR. Treatment assignment to biventricular repair was either direct (BV, n = 45) or with a bridging hybrid procedure (H1-BV, n = 12). Echocardiographic LVEDVi was <20 mL/m2 in 83% of biventricular repair infants and underestimated CMR-LVEDVi by 16.8 mL/m2. AAo flows had no/weak correlation with aortic and mitral valve z-scores or LVEDVi. AAo flows differed between BV, H1-BV, and single-ventricle groups (median): 2.1, 1.7, and 0.7 L/min/m2, respectively. Important variables for treatment assignment were presence of endocardial fibroelastosis, AAo flow, and mitral valve z-score. Biventricular repair was achieved in 54. The median follow-up was 8.0 (0.1-16.4) years. Transplant-free biventricular survival was 96%, 82%, and 77% at 1, 5, and 10 years, respectively. Patients without aortic coarctation repair were at higher risk of death, transplantation, or single-ventricle conversion (HR: 54.3; 95% CI: 6.3-47.1; P < 0.001) during follow-up. AAo flow had a smaller nonlinear effect with hazard ratio increasing at lower flows. Historical 2D echocardiographic criteria would have precluded many patients from successful biventricular repair. AAo flow, an integrative index of left heart performance, was important in assigning patients to a biventricular circulation and affected survival. Biventricular survival was strongly associated with the need for aortic coarctation repair.
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Kang et al. (2022) conducted a cohort in borderline hypoplastic left hearts (n=67). Absence of aortic coarctation repair vs. Aortic coarctation repair was evaluated on death, transplantation, or single-ventricle conversion (HR 54.3, 95% CI 6.3-47.1, p=<0.001). In infants with borderline left hearts, absence of aortic coarctation repair was associated with a higher risk of death, transplantation, or single-ventricle conversion (HR 54.3; 95% CI 6.3-47.1).
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