The Role of Cardiovascular Magnetic Resonance in ARVC

Grigoratos, Chrysanthos; Aquaro, Giovanni Donato

doi:10.1007/s11886-021-01488-1

Cited by 3 publications

(3 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…9 Several other diseases such as advanced left heart failure, pulmonary and tricuspid valve regurgitation, and arrhythmogenic right ventricular cardiomyopathy (ARVC) may lead to right heart dilatation in the absence of L-R shunts. 10,11 Patients with suspected L-R shunts typically undergo phase-contrast MRI to compare the flow between the pulmonary and systemic circulation (Qp and Qs respectively). 9 Phase-contrast acquisitions require extensive planning leading to prolonged examination times, reduced patient comfort, and limited scanner efficiency.…”

mentioning

confidence: 99%

“…In addition, MRI is considered useful in adults with nonspecific findings such as murmurs and RV dilatation, potentially caused by shunts, that are otherwise difficult to diagnose by echocardiography 9 . Several other diseases such as advanced left heart failure, pulmonary and tricuspid valve regurgitation, and arrhythmogenic right ventricular cardiomyopathy (ARVC) may lead to right heart dilatation in the absence of L‐R shunts 10,11 . Patients with suspected L‐R shunts typically undergo phase‐contrast MRI to compare the flow between the pulmonary and systemic circulation (Qp and Qs respectively) 9 .…”

mentioning

confidence: 99%

See 1 more Smart Citation

Right/Left Ventricular Blood Pool T2 Ratio as an Innovative Cardiac MRI Screening Tool for the Identification of Left‐to‐Right Shunts in Patients With Right Ventricular Disease

Emrich

Bordonaro

Schoepf

et al. 2021

Magnetic Resonance Imaging

View full text Add to dashboard Cite

Background Left‐to‐right (L‐R) shunts are characterized by a pathological connection between high‐ and low‐pressure systems, leading to a mixing of oxygen‐rich blood with low oxygenated blood. They are typically diagnosed by phase‐contrast cardiac magnetic resonance imaging (MRI) which requires extensive planning. T2 is sensitive to blood oxygenation and may be able to detect oxygenation differences between the left (LV) and right ventricles (RV) caused by L‐R shunts. Purpose To test the feasibility of routine T2 mapping to detect L‐R shunts. Study Type Retrospective. Population Patients with known L‐R shunts (N = 27), patients with RV disease without L‐R shunts (N = 21), and healthy volunteers (HV; N = 52). Field Strength/Sequence 1.5 and 3 T/balanced steady‐state free‐precession (bSSFP) sequence (cine imaging), T2‐prepared bSSFP sequence (T2 mapping), and velocity sensitized gradient echo sequence (phase‐contrast MRI). Assessment Aortic (Qs) and pulmonary (Qp) flow was measured by phase‐contrast imaging, and the Qp/Qs ratio was calculated as a measure of shunt severity. T2 maps were used to measure T2 in the RV and LV and the RV/LV T2 ratio was calculated. Cine imaging was used to calculate RV end‐diastolic volume index (RV‐EDVi). Statistical Tests Wilcoxon test, paired t‐tests, Spearmen correlation coefficient, receiver operating curve (ROC) analysis. Significance level P < 0.05. Results The Qp/Qs and T2 ratios in L‐R shunt patients (1.84 ± 0.84 and 0.89 ± 0.07) were significantly higher compared to those in patients with RV disease (1.01 ± 0.03 and 0.72 ± 0.10) and in HV (1.04 ± 0.04 and 0.71 ± 0.09). A T2 ratio of >0.78 showed a sensitivity, specificity, and negative predictive value of 100%, 73.9%, and 100%, respectively, for the detection of L‐R shunts. The T2 ratio was strongly correlated with the severity of the shunt (r = 0.83). Data Conclusion RV/LV T2 ratio is an imaging biomarker that may be able to detect or rule‐out L‐R shunts. Such a diagnostic tool may prevent unnecessary phase‐contrast acquisitions in cases with RV dilatation of unknown etiology. Level of Evidence 3 Technical Efficacy Stage 2

show abstract

mentioning

confidence: 99%

mentioning

confidence: 99%

Right/Left Ventricular Blood Pool T2 Ratio as an Innovative Cardiac MRI Screening Tool for the Identification of Left‐to‐Right Shunts in Patients With Right Ventricular Disease

Emrich

Bordonaro

Schoepf

et al. 2021

Magnetic Resonance Imaging

View full text Add to dashboard Cite

show abstract

“…However, its progression can cause patchy scar formation [ 87 ] and thereby increase stiffness and impair contractility of the myocardium. In ACM patients, cardiac fibrosis can be determined by CMR imaging using LGE [ 88 , 89 ] or by EMB with classical histology [ 90 ]. In addition, it can be investigated ex vivo after HTx or autopsy [ 91 , 92 ].…”

Section: Pathogenesismentioning

confidence: 99%

Insights Into Genetics and Pathophysiology of Arrhythmogenic Cardiomyopathy

Gerull¹,

Brodehl

2021

Curr Heart Fail Rep

View full text Add to dashboard Cite

Purpose of Review Arrhythmogenic cardiomyopathy (ACM) is a genetic disease characterized by life-threatening ventricular arrhythmias and sudden cardiac death (SCD) in apparently healthy young adults. Mutations in genes encoding for cellular junctions can be found in about half of the patients. However, disease onset and severity, risk of arrhythmias, and outcome are highly variable and drug-targeted treatment is currently unavailable. Recent Findings This review focuses on advances in clinical risk stratification, genetic etiology, and pathophysiological concepts. The desmosome is the central part of the disease, but other intercalated disc and associated structural proteins not only broaden the genetic spectrum but also provide novel molecular and cellular insights into the pathogenesis of ACM. Signaling pathways and the role of inflammation will be discussed and targets for novel therapeutic approaches outlined. Summary Genetic discoveries and experimental-driven preclinical research contributed significantly to the understanding of ACM towards mutation- and pathway-specific personalized medicine.

show abstract