The Role of Multimodality Imaging in Pediatric Cardiomyopathies

Abstract

Cardiomyopathies are a heterogeneous group of myocardial diseases representing the first cause of heart transplantation in children. Diagnosing and classifying the different phenotypes can be challenging, particularly in this age group, where cardiomyopathies are often overlooked until the onset of severe symptoms. Cardiovascular imaging is crucial in the diagnostic pathway, from screening to classification and follow-up assessment. Several imaging modalities have been proven to be helpful in this field, with echocardiography undoubtedly representing the first imaging approach due to its low cost, lack of radiation, and wide availability. However, particularly in this clinical context, echocardiography may not be able to differentiate from cardiomyopathies with similar phenotypes and is often complemented with cardiovascular magnetic resonance. The latter allows a radiation-free differentiation between different phenotypes with unique myocardial tissue characterization, thus identifying the presence and extent of myocardial fibrosis. Nuclear imaging and computed tomography have a complementary role, although they are less used in daily clinical practice due to the concern related to the use of radiation in pediatric patients. However, these modalities may have some advantages in evaluating children with cardiomyopathies. This paper aims to review the strengths and limitations of each imaging modality in evaluating pediatric patients with suspected or known cardiomyopathies.

Pediatric dilated cardiomyopathy: a review of current clinical approaches and pathogenesis

“…However, given the imminent development of pediatric DCM with these progressive diseases, measures to prevent or delay adverse cardiac remodeling must be considered. Strain analysis is another technique that has shown promise on echocardiogram and CMR in detecting maladaptive physiology before symptoms present ( 20 ). Additionally, while CMR is not often necessary in the initial diagnosis of pediatric DCM, it can provide insight into the etiology, including myocarditis, inflammation, myocardial edema, and iron overload ( 21 ).…”

Myocardial Functional Imaging in Pediatric Nuclear Cardiology

“…In particular, in hypertrophic cardiomyopathy (HCM), myocardial ischemia has been suggested to contribute to the pathophysiology of the disease and appears to be related to decreased subendocardial perfusion in the hypertrophied segments, compression of intramural small vessels, and myocardial bridging. MPI can contribute as a reliable noninvasive method for the detection of myocardial ischemia, adding to risk stratification and treatment [14,15]. -Heart transplantation: One of the long-term complications of heart transplantation is the development of cardiac allograft vasculopathy.…”

“…Cardiomyopathies: For the assessment of the extent of myocardial damage in pediatric patients with cardiomyopathies, myocardial perfusion SPECT offers important information regarding cardiac pump function and myocardial function [ 6 , 14 ]. In particular, in hypertrophic cardiomyopathy (HCM), myocardial ischemia has been suggested to contribute to the pathophysiology of the disease and appears to be related to decreased subendocardial perfusion in the hypertrophied segments, compression of intramural small vessels, and myocardial bridging.…”

How Imaging Techniques Improve Ventricular Arrhythmia Ablation: A Multimodality-Based Approach

“…Even if it allows the differentiation of subforms like hypertrophic phenotype vs. dilated phenotype, it does not allow accurate differentiation regarding the etiology and substrate localization. Indeed, in the hypertrophic phenotype, one can identify pure subsets of hypertrophic cardiomyopathy (genetically inherited forms), Fabry's disease, amyloidosis, etc., whose correct identification is based on clinical, laboratory, genetic and, in some cases, cardiac nuclear essays [22,23].…”