Serial Magnetic Resonance Imaging in Hypoplastic Left Heart Syndrome Gives Valuable Insight Into Ventricular and Vascular Adaptation

• Published in: Journal of the American College of Cardiology Vol. 61; no. 5; pp. 561 - 570
• Main Authors: Bellsham-Revell, Hannah R., MBBS, Tibby, Shane M., MBChB, MSc, Bell, Aaron J., MBChB, Witter, Thomas, RN, Simpson, John, Beerbaum, Philipp, MD, Anderson, David, MD, Austin, Conal B, Greil, Gerald F., MD, Razavi, Reza, MD
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 05-02-2013; Elsevier; Elsevier Limited; Elsevier Biomedical
• Subjects: Adaptation, Physiological - physiology; Biological and medical sciences; Cardiology; Cardiology. Vascular system; Cardiovascular; Catheters; Clinical Research; Cohort Studies; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Electrocardiography; Female; Heart; hemi-Fontan operation; Humans; hypoplastic left heart syndrome; Hypoplastic Left Heart Syndrome - diagnosis; Hypoplastic Left Heart Syndrome - physiopathology; Internal Medicine; magnetic resonance imaging; Magnetic Resonance Imaging, Cine - methods; Male; Medical sciences; NMR; Norwood procedure; Nuclear magnetic resonance; remodeling of systemic right ventricle; Veins & arteries; Ventricular Function, Left - physiology; Ventricular Function, Right - physiology; Ventricular Remodeling - physiology; magnetic resonance imaging; RPA; hypoplastic left heart syndrome; ejection fraction; RV; left ventricle; MRI; TCPC; total cavopulmonary connection; indexed end-diastolic volume; HR; LV; hemi-Fontan; SSFP; iSV; tricuspid regurgitation; EDV; end-diastolic volume; 3-dimensional; hemi-Fontan operation; left pulmonary artery; EF; heart rate; indexed end-systolic volume; ESV; end-systolic volume; remodeling of systemic right ventricle; HLHS; iESV; Norwood procedure; steady-state free precession; 3D; right pulmonary artery; sensitivity encoding; right ventricle; LPA; SENSE; HF; iEDV; TR; indexed stroke volume; Heart disease; Blood vessel; Medical imagery; Left Heart Hypoplasia; Cardiovascular disease; Circulatory system; Cardiology; Congenital disease; Knowledge; Nuclear magnetic resonance imaging; Syndrome; Adaptation
• ISSN: 0735-1097; 1558-3597
• DOI: 10.1016/j.jacc.2012.11.016

Objectives This study sought to investigate changes in magnetic resonance imaging (MRI) ventricular volumes and vascular dimensions before hemi-Fontan (HF) and before total cavopulmonary connection (TCPC) in children with hypoplastic left heart syndrome (HLHS). Background The systemic right ventricle (RV) in HLHS is subject to significant changes in volume loading throughout the surgical stages of palliation, particularly after the HF. Methods Fifty-eight patients had paired pre-HF and pre-TCPC MRI for assessment of changes of RV volumes, neoaortic flow, and vascular dimensions. Results Comparison of pre-HF and pre-TCPC MRI results showed a decrease of indexed RV end-diastolic volume and end-systolic volume (98 ml/m2 to 87 ml/m2 and 50 ml/m2 to 36 ml/m2 , respectively) with stroke volume remaining constant (49 ml/m2 vs. 51 ml/m2 ), leading to an increased RV ejection fraction (51% vs. 59%). These findings persisted after excluding the 3 patients who underwent tricuspid valve repair as part of their HF procedure. Indexed RV end-diastolic volume plotted against neoaortic stroke volume demonstrated a Frank-Starling–like curve that shifted upward after HF. The indexed distal left and right cross-sectional pulmonary artery areas were reduced after HF. Conclusions In HLHS, serial MRI shows the adaptation of the systemic RV after HF with volume reduction in the context of a preserved stroke volume and an increased ejection fraction. The staged palliation in HLHS may be a risk factor particularly for reduced left pulmonary artery growth in itself as no factors investigated in this study were found to significantly impact on this.