CMR Imaging Research Articles

Prediction of recovery after revascularization in chronic Coronary Total Occlusion (CTO) patients. Adenosine or low-dose dobutamine stress with LGE CMR: which is the best combination?

Background Recanalisation of chronic total occlusion (CTO = coronary occlusion > 3 months with a TIMI flow grade 0), is one of the most challenging PCI procedure with specific complications. Eventhough LGE-CMR is routinely used to assess viability, it does not assess ischemic status or inotropic reserve of regions surrounding scar lesions that are particularly at risk of jeopardy in patients with collateralized myocardium. If adenosine stress perfusion (Adeno) has an established high diagnostic accuracy for the detection of CAD, its specific value in the scope of chronic ischemia compared to LGE CMR imaging only or combined to low-dose dobutamine (LDD) is uncertain. The aim of this study was therefore to evaluate which best combination could improve prediction of functional recovery after revascularization of chronic total occlusion (CTO).

Improved high-resolution pediatric vascular imaging with Gadofosveset-Enhanced 3d respiratory navigated IR GRE imaging compared to 3D bSSFP imaging

Background Improved delineation of vascular structures, including coronary arteries and pulmonary veins, is a common indication for CMR imaging in children and requires high spatial resolution given small structure sizes. Currently, pre-contrast 3D, respiratory navigated, T2 prepared, fat saturated imaging with a bSSFP readout (3D bSSFP) is commonly used; however, these images can have limitations including blood pool inhomogeneity and exaggeration of metal artifact. We sought to compare imaging after administration of a blood pool contrast agent, gadofosveset trisodium (GT), with 3D, respiratory navigated, inversion recovery prepared imaging with a gradient echo readout (3D IR GRE) to the standard 3D bSSFP for imaging of pediatric vasculature.

Quantification of the area-at-risk by T1 and T2 mapping CMR at 3T

Background Measuring the area-at-risk (AAR) allows the assessment of myocardial salvage in reperfused STEMI patients. T2weighted CMR has been used to quantify the AAR but is hampered by low signal-to-noise ratio and image artifacts. T1 and T2 mapping CMR may improve upon this. We compared T1 and T2 mapping for quantifying the AAR at 3T. Methods CMR imaging at 3T (Bio-graph mMR, Siemens Healthcare, Erlangen, Germany) was performed in 18 STEMI patients within 10 days of PPCI using T2-mapping and T1-mapping by MOLLI (WIP #699; Siemens Healthcare; UK). Matched short-axis T1 and T2 maps covering the entire left ventricle were analyzed by 2 experienced investigators using 3 analytical methods (in-house macro, ImageJ): manual segmentation, Otsu, and the 2 standard deviation (2SD) thresholding (Fig. 1). Regions-of-interest were drawn in the AAR and remote myocardium. Two investigators analyzed the coronary angiograms to obtain the BARI and APPROACH angiography scores to provide a CMR-independent estimate of the AAR. Results T1 and T2 values were increased within the AAR compared with remote myocardium (mean±SD: T1, 1525 ±116ms vs. 1163±78ms, P<0.001 and T2, 72±7ms vs. 46

Is there value beyond safety in CMR imaging of PM/AICD patients?

Background Pacemaker/ AICD imaging is currently clincally performed in the MRI environment. A vigilant team consisting of Cardiologist, EP staff, and technologists with close patient monitoring and supervision has evolved this heretofore risky endeavor into a procedure with established safety if done properly. However, once safety is established, does such add valuable irrefutable information to merit that risk?

Unusual Cause of STEMI: An Intramyocardial Metastasis

A 66-year-old patient was admitted to the emergency unit for chest pain. The electrocardiogram showed a ST-segment elevation in leads V2-V4 and a slight STsegment depression in lead III. The coronary angiography was normal but CMR imaging and CT scan revealed a tumor surrounding the left anterior artery. Finally, further investigations disclosed a metastasis of squamous cell carcinoma lung cancer.

Unrecognized myocardial infarctions assessed by cardiovascular magnetic resonance are associated with the severity of the stenosis in the supplying coronary artery.

BackgroundA previous study has shown an increased prevalence of late gadolinium enhancement cardiovascular magnetic resonance (LGE CMR) detected unrecognized myocardial infarction (UMI) with increasing extent and severity of coronary artery disease. However, the coronary artery disease was evaluated on a patient level assuming normal coronary anatomy. Therefore, the aims of the present study were to investigate the prevalence of UMI identified by LGE CMR imaging in patients with stable angina pectoris and no known previous myocardial infarction; and to investigate whether presence of UMI is associated with stenotic lesions in the coronary artery supplying the segment of the myocardium in which the UMI is located, using coronary angiography to determine the individual coronary anatomy in each patient.MethodsIn this prospective multicenter study, we included patients with stable angina pectoris and without prior myocardial infarction, scheduled for coronary angiography. A LGE CMR examination was performed prior to the coronary angiography. The study cohort consisted of 235 patients (80 women, 155 men) with a mean age of 64.8 years.ResultsUMIs were found in 25 % of patients. There was a strong association between stenotic lesions (≥70 % stenosis) in a coronary artery and the presence of an UMI in the myocardial segments supplied by the stenotic artery; it was significantly more likely to have an UMI downstream a stenosis ≥ 70 % as compared to < 70 % (OR 5.1, CI 3.1-8.3, p < 0.0001). 56 % of the UMIs were located in the inferior and infero-lateral myocardial segments, despite predominance for stenotic lesions in the left anterior descending artery.ConclusionUMI is common in patients with stable angina and the results indicate that the majority of the UMIs are of ischemic origin due to severe coronary atherosclerosis. In contrast to what is seen in recognized myocardial infarctions, UMIs are predominately located in the inferior and infero-lateral myocardial segments.Trial registrationThe PUMI study is registered at ClinicalTrials.gov (NCT01257282).Electronic supplementary materialThe online version of this article (doi:10.1186/s12968-015-0202-5) contains supplementary material, which is available to authorized users.

Disturbance in Z-Disk Mechanosensitive Proteins Induced by a Persistent Mutant Myopalladin Causes Familial Restrictive Cardiomyopathy

BackgroundFamilial restrictive cardiomyopathy (FRCM) has a poor prognosis due to diastolic dysfunction and restrictive physiology (RP). Myocardial stiffness, with or without fibrosis, underlie RP, but the mechanism(s) of restrictive remodeling is unclear. Myopalladin (MYPN) is a messenger molecule that links structural and gene regulatory molecules via translocation from the Z-disk and I-bands to the nucleus in cardiomyocytes. Expression of N-terminal MYPN peptide results in severe disruption of the sarcomere. ObjectivesThe aim was to study a nonsense MYPN-Q529X mutation previously identified in the FRCM family in an animal model to explore the molecular and pathogenic mechanisms of FRCM. MethodsFunctional (echocardiography, cardiac magnetic resonance [CMR] imaging, electrocardiography), morphohistological, gene expression, and molecular studies were performed in knock-in heterozygote (MypnWT/Q526X) and homozygote mice harboring the human MYPN-Q529X mutation. ResultsEchocardiographic and CMR imaging signs of diastolic dysfunction with preserved systolic function were identified in 12-week-old MypnWT/Q526X mice. Histology revealed interstitial and perivascular fibrosis without overt hypertrophic remodeling. Truncated MypnQ526X protein was found to translocate to the nucleus. Levels of total and nuclear cardiac ankyrin repeat protein (Carp/Ankrd1) and phosphorylation of mitogen-activated protein kinase/extracellular signal–regulated kinase 1/2 (Erk1/2), Erk1/2, Smad2, and Akt were reduced. Up-regulation was evident for muscle LIM protein (Mlp), desmin, and heart failure (natriuretic peptide A [Nppa], Nppb, and myosin heavy chain 6) and fibrosis (transforming growth factor beta 1, alpha–smooth muscle actin, osteopontin, and periostin) markers. ConclusionsHeterozygote MypnWT/Q526X knock-in mice develop RCM due to persistence of mutant MypnQ526X protein in the nucleus. Down-regulation of Carp and up-regulation of Mlp and desmin appear to augment fibrotic restrictive remodeling, and reduced Erk1/2 levels blunt a hypertrophic response in MypnWT/Q526X hearts.

Abstract 17420: First Clinical Experience of Novel MRI Artifact Suppression Sequence in ICD Patients Undergoing Scar Delineation for Ventricular Tachycardia (VT) Ablation

Background: Recent studies have suggested that CMR imaging can be safely performed in selected patients with implanted ICDs. However, metal artifacts significantly impact the evaluation of myocardial fibrosis when using standard late gadolinium enhanced (LGE) protocols. Methods: 10 continuous patients scheduled for VT ablation underwent LGE CMR imaging (1.5T Siemens Avanto) for scar delineation using either the standard clinical late gadolinium enhancement (LGE) sequences (n=5) or a novel MR pulse sequence that used an inversion preparation pulse with a wide inversion bandwidth of 2.4kHz (n=5). Results: Standard LGE MR sequences produced a large central signal void with surrounding hyperintensity due to voxel dephasing. The new wide inversion bandwidth sequence reduced the artifact in the right ventricle from 21±7% to 1.2±1.3% (p&lt;.001). Similarly, the new MRI sequence reduced the artifact in the LV from 44±9% to 4.5±5.7% (p&lt;.001). LV artifact location changed from 100% (inferior), 100% (septal), 100% (lateral) and 100% (anterior) to 0%, 0%, 0% and 60%, respectively. Similarly, the LV artifact present was present in the basal, mid and apical segment in 100%, 100%, and 100% in the old sequence but was reduced to 0%, 60%, and 20% in the new sequence (Fig.1). While the artifact of the standard sequence prevented the reliable predictions of the voltage-defined scar during VT ablation, no significant low voltage area outside the imaged LGE areas were found in the patients imaged with the new MRI sequence. Conclusions: Widening the inversion bandwidth achieves a dramatic reduction in ICD metal artifact during LGE CMR imaging and allows a near completely evaluation of the whole myocardium. This allowed the reliable prediction of low voltage areas during VT ablation despite presence of ICD.

FEATURE TRACKING-BASED STRAIN ANALYSIS PREDICTS THE PRESENCE OF MYOCARDIAL FIBROSIS AMONG PATIENTS WITH NON-ISCHEMIC CARDIOMYOPATHY

Abnormal myocardial deformation has been described in the presence of regional fibrosis, as detected by late gadolinium enhancement (LGE) cardiac magnetic resonance imaging (CMR) in patients with previous infarction, Fabry’s disease and hypertrophic cardiomyopathy. Mid-wall striae pattern fibrosis on LGE imaging has been shown to predict adverse cardiovascular outcomes among patients with non-ischemic cardiomyopathy. However, macroscopic mid-wall fibrosis is a late pathologic finding and requires administration of gadolinium based contrast agent. Non-contrast dependent markers heralding adverse remodeling are desired. In this study we describe a novel strain analysis tool employing feature tracking to predict the presence of mid-wall fibrosis in patients with non-ischemic cardiomyopathy. CMR imaging was performed in 117 patients with idiopathic non-ischemic cardiomyopathy, defined as a left ventricular ejection fraction (LVEF) <55% by CMR with no prior history of myocardial infarction and no CMR findings of ischemic or other specific cardiomyopathy states (ie. hypertrophic cardiomyopathy, amyloidosis, sarcoidosis). Blinded quantitative analysis was performed using a prototype version of a commercial software (cvi42, version 4.1.5 M192, Calgary, Alberta, Canada). Left ventricular regional strain analysis was performed to obtain both radial and circumferential strain of a mid-ventricular short axis slice cine image (Figure 1). Volumetric analysis of sequential short axis cine images was also performed. LGE images were visually scored for the presence of mid-wall striae fibrosis. Mean age was 57.8 +/- 14.5 years and 60 % of patients were male. CMR characteristics are summarized in Table 1. Mid-wall striae fibrosis was identified in 48 patients (41 %). These patients had greater indexed LV end diastolic and systolic volumes and total scar volume with lower LV and RV ejection fraction. Compared to patients without midwall striae fibrosis, radial and circumferential strain values were significantly lower in the mid anteroseptal segments among patients with midwall striae fibrosis despite corresponding anterolateral strain values being similar (Table 1). On multivariate analysis, patients with midwall striae fibrosis had a significantly lower anteroseptal to anterolateral strain ratio (AASR) for both radial (0.61±0.41 vs. 0.37±0.32, p=0.024) and circumferential strain (0.66±0.36 vs. 0.42±0.29, p=0.015). Of note, 15 (22%) patients had a low AASR despite having no midwall striae fibrosis on LGE imaging. AASR is strongly associated with the presence of midwall striae fibrosis in patients with non-ischemic cardiomyopathy. The prognostic utility of this novel imaging marker warrants future study within a prospective multi-centre study.View Large Image Figure ViewerDownload (PPT)

Systematic review and modelling of the cost-effectiveness of cardiac magnetic resonance imaging compared with current existing testing pathways in ischaemic cardiomyopathy.

Systematic review and modelling of the cost-effectiveness of cardiac magnetic resonance imaging compared with current existing testing pathways in ischaemic cardiomyopathy.

The relation between hypointense core, microvascular obstruction and intramyocardial haemorrhage in acute reperfused myocardial infarction assessed by cardiac magnetic resonance imaging.

BackgroundIntramyocardial haemorrhage (IMH) and microvascular obstruction (MVO) represent reperfusion injury after reperfused ST-elevation myocardial infarction (STEMI) with prognostic impact and “hypointense core” (HIC) appearance in T2-weighted images. We aimed to distinguish between IMH and MVO by using T2 *-weighted cardiovascular magnetic resonance imaging (CMR) and analysed influencing factors for IMH development.Methods and resultsA total of 151 patients with acute STEMI underwent CMR after primary angioplasty. T2-STIR sequences were used to identify HIC, late gadolinium enhancement to visualise MVO and T2 *-weighted sequences to detect IMH. IMH+/IMH− patients were compared considering infarct size, myocardial salvage, thrombolysis in myocardial infarction (TIMI) flow, reperfusion time, ventricular volumes, function and pre-interventional medication. Seventy-six patients (50 %) were IMH+, 82 (54 %) demonstrated HIC and 100 (66 %) MVO. IMH was detectable without HIC in 16 %, without MVO in 5 % and HIC without MVO in 6 %. Multivariable analyses revealed that IMH was associated with significant lower left ventricular ejection fraction and myocardial salvage index, larger left ventricular volume and infarct size. Patients with TIMI flow grade ≤1 before angioplasty demonstrated IMH significantly more often.ConclusionsIMH is associated with impaired left ventricular function and higher infarct size. T2 and HIC imaging showed moderate agreement for IMH detection. T2 * imaging might be the preferred CMR imaging method for comprehensive IMH assessment.Key Points• Intramyocardial haemorrhage is a common finding in patients with acute reperfused myocardial-infarction. • T 2 * imaging should be the preferred CMR method for assessment of intramyocardial haemorrhage. • Intramyocardial haemorrhage can be considered as an important influencing factor on patient’s outcome.

Tools for cardiovascular magnetic resonance imaging.

In less than fifteen years, as a non-invasive imaging option, cardiovascular MR has grown from a being a mere curiosity to becoming a widely used clinical tool for evaluating cardiovascular disease. Cardiovascular magnetic resonance imaging (CMRI) is now routinely used to study myocardial structure, cardiac function, macro vascular blood flow, myocardial perfusion, and myocardial viability. For someone entering the field of cardiac MR, this rapid pace of development in the field of CMRI might make it difficult to identify a cohesive starting point. In this brief review, we have attempted to summarize the key cardiovascular imaging techniques that have found widespread clinical acceptance. In particular, we describe the essential cardiac and respiratory gating techniques that form the backbone of all cardiovascular imaging methods. It is followed by four sections that discuss: (I) the gradient echo techniques that are used to assess ventricular function; (II) black-blood turbo spin echo (SE) methods used for morphologic assessment of the heart; (III) phase-contrast based techniques for the assessment of blood flow; and (IV) CMR methods for the assessment of myocardial ischemia and viability. In each section, we briefly summarize technical considerations relevant to the clinical use of these techniques, followed by practical information for its clinical implementation. In each of those four areas, CMRI is considered either as the benchmark imaging modality against which the diagnostic performance of other imaging modalities are compared against, or provides a complementary capability to existing imaging techniques. We have deliberately avoided including cutting-edge CMR imaging techniques practiced at few academic centers, and restricted our discussion to methods that are widely used and are likely to be available in a clinical setting. Our hope is that this review would propel an interested reader toward more comprehensive reviews in the literature.

SHOULD WE ABANDON CARDIA RESYNCHRONIZATION THERAPY IMPLANTATION IN PATIENTS WHO HAVE EXTENSIVE POSTERO LATERAL SCAR ON MAGNETIC RESONANCE IMAGING: A META ANALYSIS

CRT has been shown to reduce morbidity and mortality in select heart failure patients. Upto 30-40% of heart failure patients may not respond to CRT. These, include patients with transmural scar in the posterolateral wall. Therefore, CMR Imaging was felt to be useful tool to asses patients for CRT.

THE ROLE OF CMR IMAGING FOR THE DIFFERENTIAL DIAGNOSIS OF RIGHT-SIDED INTRACARDIAC MASSES: A CASE REPORT

Materials and methods: intracardiac masses are described as abnormal structures inside the heart or immediately concerned to the heart [1] and can be classified as a cardiac tumor, metastasis, ,,thrombus in situ”/,,embolus in transit”, vegetation or iatrogenic material [2, 3]. The precise diagnosis is essential due to the necessity of the expedient well-timed treatment. We report a case of 58 year old woman with recently diagnosed left lung adenocarcinoma, admitted to the emergency department with pulmonary embolism. The case report presented here describes the findings of transthoracical echocardiography that suggested a right atrial mass – thrombus versus embolus. However, the subsequent CMR imaging helped to differentiate a true right atrial mass from a prominent crista terminalis. Conclusions: the cardiovascular magnetic resonance imaging is a valuable diagnostic method for the differentiation of the intracardiac masses when the transthoracical and/or transesophagial echocardiography is inadequate in some clinical cases. This noninvasive, cost-effective imaging technique has a larger field of view and differentiates various conditions of the heart therefore the expedient well-timed treatment could be applied.

CMR imaging in patients with cardiac amyloidosis

CMR imaging in patients with cardiac amyloidosis

Ventricular structure, function, and focal fibrosis in anabolic steroid users: a CMR study

Anabolic steroid (AS) misuse is widespread amongst recreational bodybuilders; however, their effects on the cardiovascular system are uncertain. Our aim was to document the impact of AS use on cardiac structure, function and the presence of focal fibrosis using the gold standard cardiovascular magnetic resonance imaging (CMR). A cross-sectional cohort design was utilised with 21 strength-trained participants who underwent CMR imaging of the heart and speckle-tracking echocardiography. Thirteen participants (30 ± 5 years) taking AS for at least 2 years and currently on a "using"-cycle were compared with age and training-matched controls (n = 8; 29 ± 6 years) who self-reported never having taken AS (NAS). AS users had higher absolute left ventricular (LV) mass (220 ± 45 g) compared to NAS (163 ± 27 g; p < 0.05) but this difference was removed when indexed to fat-free mass. AS had a reduced right ventricular (RV) ejection fraction (AS 51 ± 4 % vs. NAS 59 ± 5 %; p < 0.05) and a significantly lower left ventricular E':A' myocardial tissue velocity ratio [AS 0.99(0.54) vs. NAS 1.78(0.46) p < 0.05] predominantly due to greater tissue velocities with atrial contraction. Peak LV longitudinal strain was lower in AS users (AS -14.2 ± 2.7 % vs. NAS -16.6 ± 1.9 %; p < 0.05). There was no evidence of focal fibrosis in any participant. AS use was associated with significant LV hypertrophy, albeit in-line with greater fat-free mass, reduced LV strain, diastolic function, and reduced RV ejection fraction in male bodybuilders. There was, however, no evidence of focal fibrosis in any AS user.

CMR Imaging With Rapid Visual T1 Assessment Predicts Mortality in Patients Suspected of Cardiac Amyloidosis

This study tested the diagnostic and prognostic utility of a rapid, visual T1 assessment method for identification of cardiac amyloidosis (CA) in a "real-life" referral population undergoing cardiac magnetic resonance for suspected CA. In patients with confirmed CA, delayed-enhancement cardiac magnetic resonance (DE-CMR) frequently shows a diffuse, global hyperenhancement (HE) pattern. However, imaging is often technically challenging, and the prognostic significance of diffuse HE is unclear. Ninety consecutive patients referred for suspected CA and 64 hypertensive patients with left ventricular hypertrophy (LVH) were prospectively enrolled and underwent a modified DE-CMR protocol. After gadolinium administration a method for rapid, visual T1 assessment was used to identify the presence of diffuse HE during the scan, allowing immediate optimization of settings for the conventional DE-CMR that followed. The primary endpoint was all-cause mortality. Among patients with suspected CA, 66% (59 of 90) demonstrated HE, with 81% (48 of 59) of these meeting pre-specified visual T1 assessment criteria for diffuse HE. Among hypertensive LVH patients, 6% (4 of 64) had HE, with none having diffuse HE. During 29 months of follow-up (interquartile range: 12 to 44 months), there were 50 (56%) deaths in patients with suspected CA and 4 (6%) in patients with hypertensive LVH. Multivariable analysis demonstrated that the presence of diffuse HE was the most important predictor of death in the group with suspected CA (hazard ratio: 5.5, 95% confidence interval: 2.7 to 11.0; p < 0.0001) and in the population as a whole (hazard ratio: 6.0, 95% confidence interval 3.0 to 12.1; p < 0.0001). Among 25 patients with myocardial histology obtained during follow-up, the sensitivity, specificity, and accuracy of diffuse HE in the diagnosis of CA were 93%, 70%, and 84%, respectively. Among patients suspected of CA, the presence of diffuse HE by visual T1 assessment accurately identifies patients with histologically-proven CA and is a strong predictor of mortality.

Acute and chronic phase myocardial tissue characteristics in non ST-elevation myocardial infarction in dependence of early versus delayed revascularisation assessed by cardiac magnetic resonance imaging

Background According to current guidelines, an adequate timeframe for revascularisation in the setting of acute non-ST-elevation myocardial infarction (NSTEMI) is considered by current guidelines as being up to 72 hours after symptom onset. Soon after myocardial infarction, changes take place in the affected myocardium that can be visualized and measured by cardiac magnetic resonance imaging (CMR). Aim of the present study was to compare tissue characteristics in patients with NSTEMI that underwent early vs. delayed revascularisation. Therefore, myocardial tissue characteristics were evaluated in the acute and chronic phase of reperfused NSTEMI patients and the findings were correlated to the particular symptom-to-reperfusion time. Methods In 53 patients presenting with NSTEMI, CMR imaging was performed in addition to invasive coronary angiography. The patients were examined on a 1.5 T wholebody scanner using a 32-channel phased-array surface coil. Left-ventricular volumes were assessed by a standard steady-state free-precession sequence. Myocardial edema was evaluated using a 3D T2-weighted blackblood fat-saturated spin-echo sequence. Microvascular obstruction and late gadolinium enhancement were measured by a 3D phase-sensitive inversion-recovery gradient echo sequence about 12 minutes after intravenous administration of gadolinium based contrast agent. CMR imaging was conducted shortly after coronary revascularisation and about 90 days later at follow-up. Results were gained by two experienced readers in consensus.

MOLLI T1 mapping versus T2 W-SPAIR at 3T: myocardial area at risk measurements and the influence of microvascular obstruction

Background Robust CMR imaging is required for the delineation of myocardial area at risk (AAR), so that the success of reperfusion therapies can be evaluated. In this work, we investigate the performance of T1 mapping in assessing AAR one week post-STEMI, and explore the effect of microvascular obstruction (MVO) on T1 relaxation times. Methods CMR imaging was conducted on a Philips 3T Achieva MRI scanner. T2W-weighted spectral attenuated inversion recovery (T2WW-SPAIR), modified look-locker inversion recovery (MOLLI) T1 mapping and late gadolinium enhancement (LGE) sequences were applied as short axis stacks in 10 healthy volunteers and 62 STEMI patients. Receiver operator characteristic (ROC) analysis was applied to calculate a cut-off T1 to to discriminate AAR from normal myocardium. The presence of LGE was used as the positive ROC test state, while healthy myocardium, as measured in volunteers, was used as the negative ROC test state. For comparison with T1 mapping, the AAR was also measured on T2WW images using a threshold signal intensity > 2SD greater than remote. The derived myocardial edema volumes and salvage indices were compared between MVO+ and MVO- groups. Results For T1 mapping, ROC analysis gave a significantly larger area-under-the-curve (AUC) as compared to T2WWSPAIR for delineating myocardial edema (AUC = 0.89

High prevalence of atypical delayed enhancement in alkaptonuria

Background Alkaptonuria is a rare autosomal recessive metabolic disorder with an incidence of 1 case in 250,000 to 1 million live births. This genetic abnormality involves the tyrosine metabolism pathway which results in homogentisic acid accumulation throughout various tissues, including the heart. The purpose of this study is to prospectively determine the prevalence of cardiovascular abnormalities characterized by MRI in patients with alkaptonuria. Methods A group of 57 consecutive adult patients with laboratory confirmed alkaptonuria were referred for evaluation including transthoracic echocardiography and cardiac MRI, which included cine and phase contrast imaging at either 1.5T or 3T. Phase-sensitive inversion-recovery delayed enhancement imaging was performed after gadolinium-DTPA contrast administration. Late gadolinium enhancement patterns were determined by consensus read between two experienced cardiologists using a 17-segment model. Results Overall, 56 completed cine MRI, 53 received gadolinium contrast and 1 subject experienced claustrophobia precluding any imaging. Of the 56 who had CMR imaging, 77% (41 of 56) were male and the average age was 49 ± 12 years old. The prevalence of atypical delayed enhancement was 70% (37 of 53) and predominately involved the right ventricular insertion point (84%, 31 of 37 patients). Other delayed enhancement patterns included midwall (30%, 11 of 37), near aortic root (30%, 11 of 37), focal nodular (11%, 4 of 37), mitral papillary muscle involvement (8%, 3 of 37), and patchy/intermediate (5%, 2 of 37). Involvement of the right ventricular point has been associated with pulmonary hypertension; however, this group of alkaptonuria patients had normal estimated right ventricular systolic pressures measured on echocardiography performed within 1 day (median). The number of segments with atypical delayed enhancement was 2.7 ± 1.4 per patient. The prevalence of any atypical delayed enhancement trended higher for females vs. males (86% vs. 62%, p = NS) and was not associated with age. One subject had subendocardial delayed enhancement in a coronary artery distribution consistent with myocardial infarction. Conclusions Myocardial fibrosis based on late gadolinium enhancement abnormalities, especially involving the right ventricle insertion site, are common in patients with alkaptonuria despite not having pulmonary hypertension or cardiac structural abnormalities and suggests myocardial involvement due to this metabolic disorder. Further study is required to determine the etiology and clinical significance.