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International Consortium for Multimodality Phenotyping in Adults With Non-compaction
Recruiting
I'm InterestedTrial ID: NCT04424030
Purpose
Non-compaction cardiomyopathy (NCCM) is a heterogeneous, poorly understood disorder
characterized by a prominent inner layer of loose myocardial tissue, and associated with
heart failure, stroke, severe rhythm irregularities and death. For a growing population
diagnosed with NCCM there is a need for better risk stratification to appropriately allocate
(or safely withhold) these impactful preventive measures. The goal of this international
consortium is to improve care of patients with non-compaction cardiomyopathy. We hypothesize
that comprehensive analysis of clinical, genetic, structural and functional information will
improve risk stratification. In addition, we hypothesize that detailed structural analysis
will allow for differentiation of pathological and benign patterns of non-compaction. In a
large cohort of adult patients with suspected NCCM we will perform in-depth phenotyping,
including clinical information, pedigree data, genetics, echocardiography and MRI, and follow
patients for up to 3 years. We will apply machine-learning based analytics to develop
predictive models and compare their performance to currently used models and treatment
criteria. Secondly, in a subset of patients we will perform high-resolution cardiac CT for
detailed structural characterization of the myocardial wall. We will investigate associations
between myocardial structure and regional contractile function, as assessed by echo and MRI.
The aim of this proposal is to identify a structural signature associated with pathological
non-compaction and improve developed risk prediction models. Discovery of pathological
structural signatures through innovative imaging techniques, in relation to myocardial
contractility, will advance our understanding of NCCM.
Official Title
International Consortium for Multimodality Phenotyping in Adults With Non-compaction
Stanford Investigator(s)
Koen Nieman
Professor of Medicine (Cardiovascular Medicine) and of Radiology (CV Imaging)
Eligibility
Inclusion Criteria:
- ≥18 years old
- Hypertrabeculation of the left ventricle fulfilling the echo-based Jenni criteria of
NCCM
- Clinical cardiac MRI examination performed or planned
Exclusion Criteria (general cohort):
- Complex congenital disease (including transposition great arteries, tetralogy of
Fallot, tricuspid atresia, truncus arteriosis, single ventricle, hypoplastic left
heart, pulmonary atresia, double-outlet RV), neuromuscular disorders or isolated RV
non-compaction
- Inability to provide informed consent
- Contra-indications to MRI, which apply if the clinical cardiac MRI has not yet been
performed at the time of study enrollment: permanent pacemakers/ICDs, MRI contrast
medium allergy, significant arrhythmia with highly irregular RR intervals, severe
dyspnea with inability to lay flat/breath hold, inability to communicate with the MRI
technician or follow commands for any reason (psychosis, agitation, etc.), other
site-specific contra-indications to clinical MRI of the heart.
Exclusion Criteria (cardiac CT examination):
- Age <21 years
- Decompensated heart failure, or otherwise clinically unstable
- BMI>40 kg/m2
- Pregnancy (or cannot be ruled out)
- Known iodine contrast medium allergy
- Kidney dysfunction: eGFR<45 ml/min
- Thyroid disease: toxic multinodular goiter, Graves' disease, Hashimoto's thyroiditis
Intervention(s):
diagnostic test: Echocardiography
diagnostic test: Magnetic resonance imaging (MRI)
diagnostic test: Computed tomography (CT)
Recruiting
I'm InterestedContact Information
Stanford University
School of Medicine
300 Pasteur Drive
Stanford,
CA
94305
Matthew Wheeler, MD
650-725-7622