The neo-aortic valve in patients with hypoplastic left heart syndrome is largely preserved: a serial follow-up CMR study.

BACKGROUND

In hypoplastic left heart syndrome (HLHS) patients, neo-aortic valve regurgitation can negatively impact right ventricular (RV) function. We assessed neo-aortic valve function and RV volumetric parameters by analysing serial cardiovascular magnetic resonance (CMR) studies in HLHS patients after completion of total cavopulmonary connection (TCPC).

METHODS

Consecutive CMR examinations of 80 patients (female: 22) with two (n = 80) or three (n = 45) examinations each were retrospectively analysed. RV volumetry was performed using short-axis cine images. RV end-diastolic and end-systolic volumes normalised to body surface area (BSA, RVEDVi, RVESVi), ejection fraction (RVEF) and stroke volume (RVSV) were measured. Neo-aortic flow, regurgitant fraction (RF) and peak velocity were quantified from phase-contrast cine images.

RESULTS

Median neo-aortic regurgitation was mild at all three examinations (RF <20%) and there was no significant increase in RF over time (p > 0.05). None of the patients had significant neo-aortic valve stenosis (peak velocity >3 m/s). RF correlated with RVESVi and RVEF at the second examination. At the third examination, RF correlated with RVESVi and RVEDVi even in patients with RF <15% (RVESVi: r = 0.40, p = 0.001; RVEDVi: r = 0.34, p = 0.031).

CONCLUSION

Assessment of serial CMR studies in HLHS patients after TCPC completion demonstrates a preserved neo-aortic valve function. Nevertheless, thorough follow-up is mandatory as even mild neo-aortic dysfunction might impact RV size and function over a longer term.