Impact of Right Ventricular Geometry and Left Ventricular Hypertrophy on Right Ventricular Mechanics and Clinical Outcomes in Hypoplastic Left Heart Syndrome


Rösner A, Bharucha T, James A, Mertens L, Friedberg MK.
J Am Soc Echocardiogr. 2019 Jul 24. pii: S0894-7317(19)30767-9. doi: 10.1016/j.echo.2019.06.003. [Epub ahead of print] PMID: 31351794
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Take Home Points:

  • In patients with hypoplastic left heart syndrome, right ventricular function is a key determinant of long-term transplant free survival.
  • The impact of geometry of the systemic right ventricle and hypoplastic left ventricle on right ventricular mechanics is poorly understood.
  • Right ventricular apical bulging resulting from the hypertrophied and hypoplastic left ventricle negatively impact both regional and global right ventricular systolic function (figure 2 and 3).
  • Although left ventricular hypertrophy is often seen in the setting of mitral stenosis and aortic atresia, apical bulging was present in all anatomic subtypes.
  • There was suggestion of association between reduced right ventricular strain and death or transplant specifically in the subset with apical bulging (figure 6).


Commentary from Dr. Shaji Menon (Salt Lake City), section editor of Pediatric Cardiology Journal Watch: This is a retrospective cohort study of 48 hypoplastic left heart syndrome patient from the Hospital for Sick Children, Toronto with the median age of 2.2 years.  Right ventricle apical bulging defined as a sigmoid shaped septum with the right ventricle leftward apical segment contiguous with the left ventricle lateral wall.  Regional and global right ventricular strain were measured using speckle tracking echocardiography and comparisons were made between those with apical bulging and dose without apical bulging. RV global (–7.3% 6 2.8% vs –11.2% 6 4.4%; P = .001), basal septal (–3.8% 6 3.2% vs –11.4% 6 5.8%; P = .0001) and apicolateral (–5.1% 6 3.5% vs –8.0% 6 5.8%, P = .001) longitudinal strain were lower in patients with versus without apical bulging, respectively. Apical bulging was equally prevalent in all HLHS anatomical variants.

Figure 1:

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