Diffuse cardiac fibrosis quantification in early systemic sclerosis by magnetic resonance imaging and correlation with skin fibrosis.

PURPOSE: To evaluate the utility of cardiac magnetic resonance (CMR) T1 mapping in early systemic sclerosis (SSc) and its association with skin score. METHODS: Twenty-four consecutive patients with early SSc referred for cardiovascular evaluation and 12 controls without SSc were evaluated. All patients underwent cine, T1 mapping, and late gadolinium enhanced (LGE) CMR imaging. T1 mapping indices were compared between SSc patients and controls (extracellular volume fraction [ECV], gadolinium partition coefficient [λ], pre-contrast T1, and post-contrast T1). The association between T1 mapping parameters and the modified Rodnan skin score (mRSS) was determined. RESULTS: There were no significant differences in cardiac structure/function between SSc patients and controls on cine imaging, and 8/24 (33%) SSc patients had evidence of LGE (i.e., focal myocardial fibrosis). Of the T1 mapping parameters (indices indicative of diffuse myocardial fibrosis), ECV differentiated SSc patients from controls the best, followed by λ, even when the eight SSc patients with LGE were excluded. ECV had a sensitivity and specificity of 75% and 75% for diffuse myocardial fibrosis (optimal abnormal cut-off value of >27% [area under ROC curve=0.85]). In the 16 patients without evidence of LGE, each of the 4 CMR T1 mapping parameters (ECV, λ, Pre-T1 and Post-T1) correlated with mRSS (R=0.51-0.65, P=0.007-0.043), indicating a correlation between SSc cardiac and skin fibrosis. CONCLUSIONS: The four T1 mapping indices are significantly correlated with mRSS in patients with early SSc. Quantification of diffuse myocardial fibrosis using ECV should be considered as a marker for cardiac involvement in SSc clinical studies.

"No culprit" ST-elevation myocardial infarction: role of cardiac magnetic resonance imaging.

Patients with presumed ST-elevation myocardial infarction (STEMI) have no clear culprit artery in approximately 10-15% of cases. We examined the value of cardiac magnetic resonance (CMR) for diagnosis in patients with "no culprit" STEMI. Data from a comprehensive prospective registry of STEMI patients were reviewed from March 2003 to December 2009. "No culprit" patients were followed for diagnosis and clinical outcome. CMR was performed at the discretion of the attending cardiologist. Of 2728 consecutive presumed STEMI patients, 412 (15%) had no clear culprit artery. Of these, 202 (49%) had abnormal cardiac biomarkers with a definitive diagnosis in 157 (78%). Diagnoses in this group included myocardial infarction without a culprit lesion (24%), myopericarditis (22%), and stress cardiomyopathy (21%). In 210 (51%) patients with normal biomarkers, only 84 (40%) received a definitive diagnosis. Diagnoses in this group included myopericarditis (27%), noncardiac causes (21%), and cardiomyopathy (14%). CMR was performed in 123 (30%) "no culprit" patients. Patients who had CMR were more likely to have a definitive diagnosis than those who did not (95/123 [77%] vs. 144/289 [50%]; P=0.01). In particular, "no culprit" patients with abnormal biomarkers were more likely to have a definitive diagnosis with CMR. CMR led to a diagnosis different from the presumptive clinical diagnosis in 53% of all cases. CMR is a valuable diagnostic tool to improve diagnostic accuracy in patients with "no culprit" STEMI.

Concurrent basilar artery double fenestration with aneurysm and vertebral artery dissection: case report and literature review of rare cerebrovascular abnormalities.

Many disorders can cause aneurysm and/or dissection of the cerebral arteries, including fibromuscular dysplasia (FMD), connective tissue disorders, cerebral vasculitis, infection, and vascular malformations. Arterial fenestration is a rare congenital finding that can also cause aneurysms, and can rarely dissect and bleed. Treatment of aneurysm and dissection with subarachnoid hemorrhage can be very complicated, and requires case-by-case analysis of the risks and benefits of antithrombotic therapy. To the authors' knowledge, no case of double fenestration of the basilar artery has been reported. This report presents a case of concurring vertebral artery dissection and double fenestration of the basilar artery with aneurysm. The fenestration and FMD are considered possible main contributing causes of this presentation. A literature review of cerebrovascular fenestration and FMD is provided and the relationship between the 2 is considered. Lastly, the use of antithrombotic therapy in the setting of subarachnoid hemorrhage, dissection, and stent placement is discussed.

Complicated cerebral venous sinus thrombosis with intracranial hemorrhage and mastoiditis.

Cerebral venous sinus thrombosis (CVST) is a rare cause of stroke, occurring when a blood clot forms in any of the brain venous sinuses. Symptoms include neurological deficits, headache, seizures, and coma. There are many predisposing factors for CVST including prothrombotic conditions, oral contraceptives, pregnancy/puerperium, malignancy, infection, and head injury. Cerebral venous sinus thrombosis has no identifiable underlying etiology in about 12.5% to 33% of the cases. Diagnosis has become easier with newer imaging techniques, such as magnetic resonance venography. The treatment options for CVST include symptomatic treatment, anticoagulation (AC), thrombolysis, and thrombectomy. Controversy exists over the efficacy and safety of AC in patients with CVST with concurrent intracranial hemorrhage (ICH). We present a complex case of CVST with ICH and mastoiditis as well as provide a literature review about CVST.