Obstructive Left Heart Disease in Neonates With a Borderline Left Ventricle: Diagnostic Challenges to Choosing the Best Outcome

In most newborns with left heart obstruction, the choice between a single-ventricle or biventricular management pathway is clear. However, in some neonates with a borderline left ventricle, this decision is difficult. Existing criteria do not reliably identify neonates who will have a good long-term outlook after biventricular repair (BVR). The objective of this study was prospective assessment of the outcome after BVR for newborns in whom the left ventricle (LV) was considered borderline by an expert group. This study was a prospective follow-up evaluation of neonates with obstructive left heart disease related to a borderline LV who underwent biventricular management between January 2005 and April 2011. Of 154 neonates who required intervention for left heart obstruction, 13 (7.8 %) met the echocardiographic (echo) inclusion criteria. At the first and last echo, the z-scores were respectively -1.76 +/- A 1.37 and -0.66 +/- A 1.47 (p = 0.013) for the mitral valve, -1.02 +/- A 1.57 and -0.23 +/- A 1.78 (p = 0.056) for the aortic valve, and 13.77 +/- A 5.8 and 20.85 +/- A 8.9 ml/m(2) (p = 0.006) for the LV end-diastolic volume. At this writing, all 12 survivors are clinically well. However, LV diastolic dysfunction and pulmonary artery hypertension was present in 5 (36 %) of 12 patients. Endocardial fibroelastosis (EFE) was detected in five patients at the last follow-up echo, but only in two patients preoperatively. Cardiac magnetic resonance imaging did not confirm EFE in any of assessed patients. The study authors could not reliably predict the outcome after BVR for neonates with left heart obstruction and a borderline LV. The presence of EFE with consequent diastolic dysfunction is more important than LV volume in determining the outcome. Prospective identification of EFE remains challenging.