Fetal Echocardiography in Predicting Postnatal Outcome in Borderline Left Ventricle

Abstract

Objectives Prenatal prediction of postnatal univentricular versus biventricular circulation in patients with borderline left ventricle (bLV) remains challenging. This study investigated prenatal fetal echocardiographic parameters and postnatal outcome of patients with a prenatally diagnosed bLV.

Methods We report a retrospective study of bLV patients at four prenatal centers with a follow-up of one year. BLV was defined as z-scores of the left ventricle (LV) between –2 and –4. Single-ventricle palliation (SVP), biventricular repair (BVR), and no surgical or catheter-based intervention served as the dependent outcome. Prenatal ultrasound parameters were used as independent variables. Cut-off values from receiver operating characteristic curves (ROC) were determined for significant discrimination between outcomes.

Results A total of 54 patients were diagnosed with bLV from 2010 to 2018. All were live births. Out of the entire cohort, 8 (15 %) received SVP, 34 (63 %) BVR, and 12 (22 %) no intervention. There was no significant difference with regard to genetic or extracardiac anomalies. There were significantly more patients with endocardial fibroelastosis (EFE) in the SVP group compared to the BVR group (80 % vs. 10 %), (p < 0.001). Apex-forming LV (100 % vs. 70 %) and lack of retrograde arch flow (20 % vs. 80 %) were associated with no intervention (p < 0.001). With respect to BVR vs. SVP, the LV sphericity index provided the highest specificity (91.7 %) using a cutoff value of ≤ 0.5.

Conclusion The majority of bLV patients maintained biventricular circulation. EFE, retrograde arch flow, and LV sphericity can be helpful parameters for counseling parents and further prospective studies can be developed.

Zusammenfassung

Ziel Die pränatale Vorhersage eines postnatal univentrikulären versus biventrikulären Kreislaufs bei Föten mit grenzwertigem linkem Ventrikel (bLV, “borderline left ventricle”) bleibt eine Herausforderung. Diese Studie untersuchte pränatale fetale echokardiografische Parameter und den postnatalen Outcome von Patienten mit einer pränatal diagnostizierten bLV.

Methoden Wir berichten über eine retrospektive Studie von Föten mit bLV an 4 Pränatalzentren mit einer Nachverfolgung von einem Jahr. BLV wurde definiert als z-Scores des linken Ventrikels (LV) zwischen –2 und –4. Als abhängiger Outcome dienten die Einzelventrikel-Palliation (SVP), die biventrikuläre Korrektur (BVR) und keine chirurgische oder Katheter-basierte Intervention. Die pränatalen Ultraschallparameter wurden als unabhängige Variablen verwendet. Die Cut-off-Werte aus den Receiver-Operating-Characteristic (ROC) -Kurven wurden für eine signifikante Diskriminierung zwischen den Outcomes ermittelt.

Ergebnisse Von 2010–2018 wurden insgesamt 54 Patienten mit bLV diagnostiziert. Alle waren Lebendgeburten. Von der gesamten Kohorte erhielten 8 (15 %) SVP, 34 (63 %) BVR und 12 (22 %) keine Intervention. Es gab keinen signifikanten Unterschied in Bezug auf genetische oder extrakardiale Anomalien. Es gab signifikant mehr Patienten mit endokardialer Fibroelastose (EFE) in der SVP- im Vergleich zu BVR-Gruppe (80 % vs. 10 %; p < 0,001). Ein an der Herzspitzenbildung beteiligter LV (100 % vs. 70 %) und fehlender retrograder Bogenfluss (20 % vs. 80 %) waren mit keiner Intervention assoziiert (p < 0,001). Für die Differenzierung von BVR vs. SVP lieferte der LV-Sphärizitätsindex bei einem Cut-off-Wert von ≤ 0,5 die höchste Spezifität (91,7 %).

Schlussfolgerung Die Mehrheit der bLV-Patienten hielt einen biventrikulären Kreislauf aufrecht. EFE, retrograder Bogenfluss und LV-Sphärizität können hilfreiche Parameter bei der Beratung der Eltern und der Entwicklung weiterer prospektiver Studien sein.

Key words

congenital heart disease - borderline left ventricle - fetal echocardiography - hypoplastic left heart complex - prenatal diagnosis

Publication History

Received: 01 November 2020

Accepted: 19 May 2021

Article published online:
05 July 2021

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