Cardiac Magnetic Resonance Derived Left Ventricular Eccentricity Index and Right Ventricular Mass Measurements

Meindina G Haarman1, Iris Coenraad1, Quint A J Hagdorn1

  • 1Center for Congenital Heart Diseases, Department of Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, 9700 RB Groningen, The Netherlands.

Insights

Cardiac MRI measures of right ventricular function and remodeling predict survival in pediatric idiopathic/heritable pulmonary arterial hypertension (PAH). These findings can aid risk stratification for children with PAH.

Area of Science:

  • Cardiology
  • Pediatric Imaging
  • Pulmonary Hypertension

Background:

  • Pulmonary arterial hypertension (PAH) significantly impacts right ventricular (RV) function and remodeling, crucial for patient outcomes.
  • Risk stratification in pediatric PAH requires reliable noninvasive prognosticators.
  • The prognostic utility of RV characteristics from cardiac magnetic resonance (CMR) in pediatric PAH is underexplored.

Purpose of the Study:

  • To identify CMR-derived morphometric and functional RV characteristics that predict outcomes in children with PAH.
  • To assess the prognostic value of specific RV parameters in pediatric patients with idiopathic/heritable PAH (IPAH/HPAH) and PAH associated with congenital heart disease (PAH-CHD).

Main Methods:

  • Retrospective analysis of CMR data from 38 children with severe PAH (IPAH/HPAH or PAH-CHD).
  • Evaluated RV ejection fraction (RVEF), indexed RV mass (RVMi), RV/LV mass ratio (RVM/LVM-ratio), and left ventricular eccentricity index (LVEI).
  • Correlated these parameters with transplant-free survival.

Main Results:

  • In children with IPAH/HPAH, RVEF, RVMi, RVM/LVM-ratio, and LVEI were significantly correlated with transplant-free survival.
  • These correlations were not observed in the PAH-CHD subgroup.
  • Patients presented with severe PAH, indicated by WHO Functional Class, elevated NT-proBNP, and high PAP/PVRi.

Conclusions:

  • CMR-derived RV function and remodeling metrics (LVEI, RVMi, RVM/LVM-ratio, RVEF) are prognostic for transplant-free survival in pediatric IPAH/HPAH.
  • These imaging parameters hold potential for inclusion in risk stratification scores for pediatric PAH management.

Related Concept Videos

Aortic Regurgitation II: Clinical Features and Diagnostic Tests01:22

Aortic Regurgitation II: Clinical Features and Diagnostic Tests

Aortic valve regurgitation (AR) occurs when the aortic valve fails to close properly, allowing blood to flow backward from the aorta into the left ventricle. This backflow can result in two distinct clinical presentations: acute and chronic AR, each characterized by its own set of symptoms and physical findings.Acute Aortic RegurgitationAcute AR presents with a sudden onset of severe symptoms. Patients typically experience profound dyspnea (shortness of breath), chest pain, and signs of left...
36
Mitral Stenosis II: Clinical features and Diagnostic Tests01:23

Mitral Stenosis II: Clinical features and Diagnostic Tests

Mitral stenosis is a heart condition in which the mitral valve, which allows blood to flow from the left atrium to the left ventricle, becomes narrowed or stenotic. This narrowing hinders blood flow and leads to clinical symptoms requiring specific medical evaluations and management strategies. The following overview outlines the clinical symptoms, assessments, diagnostic findings, prevention methods, and treatments for mitral stenosis.Clinical ManifestationsDyspnea (shortness of breath): This...
26
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
17
Mitral Stenosis I: Introduction01:22

Mitral Stenosis I: Introduction

Mitral Valve Stenosis (MVS) is a heart condition where the mitral valve narrows, impeding blood circulation from the left atrium to the left ventricle. The etiology and pathophysiology of this condition are multifaceted, leading to a cascade of cardiovascular complications.Causes of Mitral Valve StenosisRheumatic Heart Disease: It is the main cause of mitral valve stenosis, particularly in developing nations. This condition arises from rheumatic fever, an inflammatory illness resulting from...
19