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Characterising adequacy or inadequacy of the borderline left ventricle: what tools can we use?
Michelle Kaplinski1, Meryl S Cohen1
1Division of Cardiology,The Children's Hospital of Philadelphia,University of Pennsylvania,Perelman School of Medicine,Philadelphia,Pennsylvania,United States of America.
Insights
Managing borderline left ventricle (LV) in infants is challenging. This review focuses on assessing and treating LV hypoplasia in critical aortic stenosis or arch obstruction cases.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Neonatal Cardiology
Background:
- Borderline left ventricle (LV) represents a spectrum of LV underdevelopment, often linked with other cardiac anomalies.
- Treatment is clear for severe hypoplasia (single ventricle palliation/transplant) or mild forms (no intervention).
- Management of borderline LV in the 'grey zone' remains controversial and institutionally variable.
Purpose of the Study:
- To review the assessment and management strategies for infants with borderline left ventricle.
- To focus on cases associated with critical aortic stenosis or arch obstruction and mitral anomalies.
Main Methods:
- Review of existing literature on borderline left ventricle.
- Discussion of diagnostic and therapeutic approaches for specific congenital heart conditions.
Main Results:
- The spectrum of borderline left ventricle ranges from mild hypoplasia to severe forms like hypoplastic left heart syndrome.
- Associated conditions include critical aortic stenosis, coarctation of the aorta, and various complex heart defects.
- Management decisions are complex for borderline cases not at the extremes of the spectrum.
Conclusions:
- The management of borderline left ventricle in infants with critical aortic stenosis or arch obstruction requires careful consideration.
- Further research and standardized guidelines are needed for the controversial 'grey zone' cases.
- Accurate assessment and tailored interventions are crucial for improving outcomes in these complex pediatric cardiac conditions.
Abstract:
Borderline left ventricle refers to a spectrum of left ventricular underdevelopment, typically associated with other cardiac anomalies. The left ventricle may be mildly hypoplastic, as is sometimes seen accompanying aortic coarctation, or it can be severely hypoplastic, as is seen in hypoplastic left heart syndrome. For patients with a borderline left ventricle that is at either extreme, the treatment decision is relatively straightforward. Those with the most severe form of left ventricle hypoplasia will require single ventricle palliation or cardiac transplantation, whereas those with the mildest form may not need any intervention. It is the management strategy of children that fall within the grey zone of the spectrum, which continues to be controversial and remains variable within and among different institutions. Cardiac diseases with associated left ventricle hypoplasia include critical aortic stenosis, mitral stenosis, coarctation of the aorta, arch hypoplasia, cor triatriatum, unbalanced common atrioventricular canal, Shone's complex, total anomalous pulmonary venous return, and complex conotruncal abnormalities. In this review, we will discuss the assessment and management of infants with borderline left ventricle with critical aortic stenosis or arch obstruction and associated mitral anomalies.
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