Journal of the American Society of Echocardiography
Clinical InvestigationFetal and Pediatric EchocardiographyImpact of Right Ventricular Geometry and Left Ventricular Hypertrophy on Right Ventricular Mechanics and Clinical Outcomes in Hypoplastic Left Heart Syndrome
Section snippets
Methods
The institutional research ethics board approved this study with waiver of informed consent. We retrospectively identified children from the Hospital for Sick Children in Toronto with HLHS from our institutional database between 2008 and 2010.12 For this study, classic HLHS was defined as usual atrial arrangement, atrioventricular and ventricular-arterial concordance, and an LV deemed too small to support the systemic circulation leading to single-ventricle palliation.21 Patients at all stages
Results
Forty-eight infants and children with HLHS (median age, 0.75 years; range, 0-14 years; interquartile range, 3.62 years; mean, 2.2 years) were retrospectively analyzed. Table 1 shows their clinical characteristics, prevalence and severity of TR, and different anatomical variants of HLHS separately for apical bulging versus nonapical bulging. There was no significant difference in clinical parameters between these groups. All patients underwent a Blalock- Taussig- shunt or hybrid procedure, and
Discussion
The major findings of the study are (1) the definition of an “apical bulging” HLHS phenotype, which is related to apicolateral hypertrophy of the hypoplastic LV; (2) the association of this phenotype with more pronounced regional dysfunction and lower global RV strain compared with the nonapical bulging phenotype; and (3) the discovery that transplant or death were related to reduced RV function in the apical bulging group.
Conclusion
Apical bulging of the RV in HLHS is present in association with LV lateral wall and apical hypertrophy. Apical bulging is associated with reduced basal septal and RV global longitudinal strain, possibly due to the impact of abnormal interventricular interaction with a hypertrophied residual LV. The combination of apical bulging with low RV strain appears to be associated with transplant or death. Indeed, transplant or death occurred in most cases in patients with reduced RV strain associated
Acknowledgments
The study was supported by a research grant from the national health organization of North Norway “Helse Nord” (grant no. HNF1342-17).
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