Under-recognized complications in patients with paroxysmal nocturnal haemoglobinuria: raised pulmonary pressure and reduced right ventricular function.

Pulmonary hypertension is becoming a recognized complication of the hereditary and acquired haemolytic anaemias, associated with a poor prognosis. Recently we reported that patients with paroxysmal nocturnal haemoglobinuria (PNH) have high levels of N-terminal pro-brain natriuretic peptide (NT-proBNP), a biomarker associated with both right and left ventricular dysfunction and cardiac dysfunction. In the current study we evaluated a cohort of patients (N = 29) with haemolytic PNH for elevated pulmonary artery systolic pressure and cardiac function by Doppler-echocardiography. Of the 29 patients, eight were further studied using cardiac magnetic resonance imaging (MRI), as well as two additional patients (number of patients studied using cardiac MRI = 10). Plasma from the first cohort (N = 29) demonstrated intravascular haemolysis associated with a 12-fold increase in median nitric oxide (NO) consumption when compared with healthy volunteers (P < 0·001). Doppler echocardiography demonstrated normal left ventricular function and elevated pulmonary artery systolic pressure in 41% of patients. Cardiac MRI from the second cohort (N = 10) demonstrated depressed right ventricular function in 80% of PNH patients tested, and 60% had findings suggestive of subclinical small pulmonary emboli. Together, these data suggest a high prevalence of haemolysis-associated NO scavenging, Doppler-estimated systolic pulmonary hypertension, and depressed right ventricular function in patients with PNH.

The role of multi-modality imaging for sinus of Valsalva aneurysms.

Sinus of Valsalva aneurysms (SVAs) are uncommon but important entities. They are most often congenital in origin, resulting from incomplete fusion of the aortic media to the aortic valve annulus. Less frequently, they may be acquired, usually secondary to infective endocarditis. Unruptured aneurysms may be clinically silent and diagnosed incidentally, but can also produce symptoms as a consequence of mass effect on related structures. Rupture may present with sudden hemodynamic collapse but can have a more insidious onset depending upon the site and size of the perforation. Early diagnosis is imperative and can usually be made reliably by transthoracic echocardiography. However, transesophageal echocardiography may sometimes be required for confirmation. Cardiovascular magnetic resonance imaging (CMRI) and multi-detector computed tomography are being increasingly utilized for evaluation of SVAs and can offer valuable complimentary information. CMRI in particular enables a comprehensive assessment of anatomy, function and flow in a single sitting. Surgical repair forms the mainstay of treatment for both ruptured and unruptured aneurysms and has low complication rates. This article provides an overview of the pathological and clinical aspects of SVAs and discusses in detail the role of advanced imaging modalities in their evaluation.

Heart-type fatty acid-binding protein predicts long-term mortality and re-infarction in consecutive patients with suspected acute coronary syndrome who are troponin-negative.

OBJECTIVES: The purpose of this study was to establish the prognostic value of measuring heart fatty acid-binding protein (H-FABP) in patients with suspected acute coronary syndrome (ACS) (in particular, low- to intermediate-risk patients), in addition to troponin measured with the latest third-generation troponin assay. BACKGROUND: We have previously shown that H-FABP is a useful prognostic marker in patients with proven ACS. METHODS: Patients (n = 1,080) consecutively admitted to the hospital with suspected ACS were recruited over 46 weeks. Siemens Advia Ultra-TnI (Siemens Healthcare Diagnostics, Newbury, United Kingdom) and Randox Evidence H-FABP (Randox Laboratories, Ltd., Co., Antrim, United Kingdom) were analyzed on samples collected 12 to 24 h from symptom onset. After exclusion of patients with ST-segment elevation and new left bundle branch block, 955 patients were included in the analysis. RESULTS: The primary outcome measure of death or readmission with myocardial infarction after a minimum follow-up period of 12 months (median 18 months) occurred in 96 of 955 patients (10.1%). The H-FABP concentration was an independent predictor of death or myocardial infarction, after multivariate adjustment. Patients with H-FABP concentrations >6.48 microg/l had significantly increased risk of adverse events (adjusted hazard ratio: 2.62, 95% confidence interval: 1.30 to 5.28, p = 0.007). Among troponin-negative patients (which constituted 79.2% of the cohort), the aforementioned cutoff of 6.48 microg/l identified patients at very high risk for adverse outcomes independent of patient age and serum creatinine. CONCLUSIONS: We have demonstrated that the prognostic value of elevated H-FABP is additive to troponin in low- and intermediate-risk patients with suspected ACS. Other studies suggest that our observations reflect the value of H-FABP as a marker of myocardial ischemia, even in the absence of frank necrosis.

Sinus of valsalva aneurysms: assessment with cardiovascular MRI.

OBJECTIVE: The aim of this article is to present the role of cardiovascular MRI in the assessment of sinus of Valsalva aneurysms. An imaging protocol is described, along with a systematic approach to interpret MR findings and a synopsis of key findings. CONCLUSION: Radiologists should have a systematic approach to the assessment and evaluation of sinus of Valsalva aneurysms to facilitate optimal patient management.

Aortocoronary bypass graft fistula after surgical treatment of circumflex coronary artery fistula: a unique variation of a rare condition successfully treated with percutaneous embolization.

Multiple coronary artery fistulae are rare, complications can be life-threatening, and with large or symptomatic fistulae, intervention is mandatory. Both surgical and percutaneous interventions are well-described. We believe this is the first report of the embolization of an acquired fistula following initial surgical treatment of multiple congenital fistulae.

The potential role of MRI in veterinary clinical cardiology.

Over the last decade, magnetic resonance imaging (MRI) has become established as a useful referral diagnostic method in veterinary medicine that is widely used in small animal brain and spinal diseases, aural, nasal and orbital disorders, planning soft tissue surgery, oncology and small animal and equine orthopaedics. The use of MRI in these disciplines has grown due to its unparalleled capability to image soft tissue structures. This has been exploited in human cardiology where, despite the inherent difficulties in imaging a moving, contractile structure, cardiac MRI (CMRI) has become the optimal technique for the morphological assessment and quantification of ventricular function. Both CMRI hardware and software systems have developed rapidly in the last 10 years but although several preliminary veterinary CMRI studies have been reported, the technique's growth has been limited and is currently used primarily in clinical research. A review of published studies is presented with a description of CMRI technology and the potential of CMRI is discussed along with some of the reasons for its limited usage.

Complete left pericardial defect: evaluation with supine and decubitus dual source CT.

We present a case of complete left pericardial defect which was evaluated with retrospectively gated dual source CT. Imaging findings included right heart chamber dilatation, extreme levoposition and excessive cardiac mobility which was demonstrated by repeat imaging in the left lateral decubitus position. Cardiac CT is an excellent means of evaluating pericardial disease owing to its high spatial resolution. Decubitus imaging helps confidently distinguish partial from complete forms of pericardial defect.

Delayed enhancement imaging: standardised segmental assessment of myocardial viability in patients with ST-elevation myocardial infarction.

OBJECTIVE: To prospectively compare a selective short axis slice positioning method (selective 3-of-5) used in combination with a single long-axis slice, to the conventional short axis multi-slice technique in the assessment of myocardial viability. MATERIALS AND METHODS: Thirty-one patients with recent or chronic ST segment elevation myocardial infarct (STEMI) were recruited to undergo delayed enhancement (DE) cardiac magnetic resonance imaging (CMR). All patients underwent both methods of DE imaging, with subsequent review of both sets of data by two experienced observers. Sensitivity and specificity, as well as intra and interobserver reproducibility for both techniques were assessed. RESULTS: There was good agreement between the selective 3-of-5 and the conventional multi-slice method for the assessment of viability, with no significant difference in results for sensitivity or reproducibility between the techniques. CONCLUSION: In patients with STEMI, a selective 3-of-5 short axis slice acquisition used in combination with a single vertical long-axis slice can be utilised to produce a standard American Heart Association (AHA) 17-segment model for the assessment of myocardial viability.

Myocardial T1 mapping: application to patients with acute and chronic myocardial infarction.

T(1) maps obtained with modified Look-Locker inversion recovery (MOLLI) can be used to measure myocardial T(1). We aimed to evaluate the potential of MOLLI T(1) mapping for the assessment of acute and chronic myocardial infarction (MI). A total of 24 patients with a first MI underwent MRI within 8 days and after 6 months. T(1) mapping was performed at baseline and at selected intervals between 2-20 min following administration of gadopentetate dimeglumine (Gd-DTPA). Delayed-enhancement (DE) imaging served as the reference standard for delineation of the infarct zone. On T(1) maps the myocardial T(1) relaxation time was assessed in hyperenhanced areas, hypoenhanced infarct cores, and remote myocardium. The planimetric size of myocardial areas with standardized T(1) threshold values was measured. Acute and chronic MI exhibited different T(1) changes. Precontrast threshold T(1) maps detected segmental abnormalities caused by acute MI with 96% sensitivity and 91% specificity. Agreement between measurements of infarct size from T(1) mapping and DE imaging was higher in chronic than in acute infarcts. Precontrast T(1) maps enable the detection of acute MI. Acute and chronic MI show different patterns of T(1) changes. Standardized T(1) thresholds provide the potential to dichotomously identify areas of infarction.

Relationship between central sympathetic drive and magnetic resonance imaging-determined left ventricular mass in essential hypertension.

BACKGROUND: Sympathetic activation has been implicated in the development of left ventricular hypertrophy (LVH). However, the relationship between sympathetic activation and LV mass (LVM) has not been clearly defined across a range of arterial pressure measurements. The present study was planned to determine that relationship, using cardiac magnetic resonance imaging to accurately quantify LVM, in hypertensive patients with and without LVH and in normal subjects. METHODS AND RESULTS: Twenty-four patients with uncomplicated and untreated essential hypertension (LVH[-]) were compared with 25 patients with essential hypertension and left ventricular hypertrophy (LVH[+]) and 24 normal control subjects. Resting muscle sympathetic nerve activity was quantified as multiunit bursts and single units. Cardiac magnetic resonance imaging-determined LVM was indexed to body surface area (LVM index); in the LVH[-] group, LVM index was 67+/-2.1 g/m2, a value between those of the LVH[+] (91+/-3.4 g/m2) and normal control (57+/-2.2 g/m2) groups, respectively. The sympathetic activity in the LVH[-] group (53+/-1.3 bursts per 100 cardiac beats and 63+/-1.6 impulses per 100 cardiac beats) was between (at least P<0.001) those of the LVH[+] (66+/-1.7 bursts per 100 cardiac beats and 77+/-2.2 impulses per 100 cardiac beats) and normal control (39+/-3.0 bursts per 100 cardiac beats and 45+/-3.4 impulses per 100 cardiac beats) groups. Significant positive correlation existed between sympathetic activity and LVM index in the LVH[-] and LVH[+] groups (at least r=0.76, P<0.0001) but not in the normal control group. However, no consistent relationship existed between arterial blood pressure and sympathetic activity or LVM index. CONCLUSIONS: These findings further support the hypothesis that central sympathetic activation is associated with the development of LVH in human hypertension.

Do flat detector cardiac X-ray systems convey advantages over image-intensifier-based systems? Study comparing X-ray dose and image quality.

The recent introduction of "flat-panel detector" (FD)-based cardiac catheterisation laboratories should offer improvements in image quality and/or dose efficiency over X-ray systems of conventional design. We compared three X-ray systems, one image-intensifier (II)-based system (system A), and two FD-based designs (systems B and C), assessing their image quality and dose efficiency. Phantom measurements were performed to assess dose rates in fluoroscopy and cine acquisition. Phantom dose rates were broadly similar for all systems, with all systems classified as offering "low" dose rates in fluoroscopy on standard phantoms. Patient X-ray dose rate and subjective image quality was assessed for 90 patients. Dose area product (DAP) rates were similar for all systems, except system C, which had a lower DAP rate in fluoroscopy. In terms of subjective image quality, the order of preference was (best to worst): system C, system A, system B. This study indicates that the use of an FD detector does not infer an automatic improvement in image quality or dose efficiency over II based designs. Specification and configuration of all of the components in the X-ray system contribute to the dose levels used and image quality achieved.

Myocardial T1 mapping for detection of left ventricular myocardial fibrosis in chronic aortic regurgitation: pilot study.

OBJECTIVE: The aim of this study was to identify diffuse myocardial fibrosis secondary to chronic aortic regurgitation by comparing the T1 relaxation times of left ventricular myocardium in a pilot patient group with a previously established normal range of times. SUBJECTS AND METHODS: Eight patients with chronic aortic regurgitation and normal coronary arteries awaiting surgical valve replacement underwent a comprehensive MRI examination that included assessment of left ventricular function, severity of valvular regurgitation, and presence of overt myocardial scar evidenced by delayed enhancement. For each patient, myocardial T1 relaxation times determined with a modified Look-Locker technique before and after contrast administration were compared with values previously established for 15 healthy volunteers. RESULTS: There was no statistical difference (p > 0.05) in slice-averaged myocardial T1 relaxation times either before or after gadolinium administration in the patient group compared with the normal range of times. Segmental averaged T1 relaxation times in segments with abnormal wall motion did, however, show statistically significant differences from healthy controls 10, 15, and 20 minutes after administration of gadolinium (510 vs 476 milliseconds, p = 0.001; 532 vs 501 milliseconds, p = 0.002; 560 vs 516 milliseconds, p = 0.001, respectively). Two of the aortic regurgitation patients also had focal areas of myocardial delayed enhancement. CONCLUSION: Segment-based myocardial T1 mapping has the potential for showing differences between relaxation times in aortic regurgitation and in normal hearts, suggesting the existence of a diffuse myocardial fibrotic process.

The assessment of left ventricular hypertrophy in hypertension.

The presence of left ventricular hypertrophy (LVH) in hypertension, as detected by the electrocardiogram or echocardiography, is associated with an increased risk of mortality and morbidity several times above and beyond the risk of hypertension alone. The LIFE (Losartan Intervention For Endpoint reduction in hypertension) study confirmed that pharmacological agents, which reduce LVH, confer further reduction in morbidity and mortality. This makes the identification of patients with LVH all the more important. In this article we describe the various methods available to diagnose the presence of LVH in patients with hypertension, and consider their strengths and their place in clinical practice and in research.

Human myocardium: single-breath-hold MR T1 mapping with high spatial resolution--reproducibility study.

A prospective study approved by the local ethics committee was performed to establish the normal range and reproducibility of myocardial T1 values as assessed with single-breath-hold T1 mapping with high spatial resolution. With a 1.5-T magnetic resonance (MR) imaging system, baseline and contrast material-enhanced modified Look-Locker inversion recovery, or MOLLI, imaging was performed in 15 healthy volunteers who had given written informed consent. Image quality scores and myocardial T1 values were derived for standard short-axis segments and sections. Results were compared with those from a second MR imaging study performed on the same day (baseline only) and those from a third study performed on a different day (baseline and contrast enhanced; eight volunteers). Intra- and interobserver agreement were determined. Myocardial T1 maps were obtained rapidly in a reproducible fashion. A normal range for baseline and postcontrast myocardial T1 was established (baseline mean T1 in short-axis sections, 980 msec +/- 53 [standard deviation]; 95% confidence interval: 964, 997; number of sections, 43). This technique could enable direct quantification of changes in tissue characteristics in ischemic and inflammatory myocardial diseases.

The reproducibility of left ventricular volume and mass measurements: a comparison between dual-inversion-recovery black-blood sequence and SSFP.

The aim of this study was to compare a dual-inversion-recovery black-blood (BB) magnetic resonance imaging (MRI) sequence with steady-state free precession (SSFP) for the assessment of left ventricular parameters. The improved endocardial border definition seen with SSFP was not observed at the epicardial border. Improvements in segmentation at the left ventricular epicardial border have been observed with this black-blood sequence. Left ventricular (LV) mass and LV end-diastolic volume (EDV) measurements as well as inter-observer and intra-observer variability were compared between images acquired with a dual inversion BB and SSFP sequence. The mean+/-1 standard deviation (SD) for LV EDV was 178.3+/-52.7 ml measured with SSFP and 158.8+/-62.2 ml with BB. This difference was not statistically significant (p=0.22). For SSFP, the mean value of LV mass was 124.0+/-27.0 g and 147.5+/-37.4 g for BB, a statistically significant difference (p<0.0001). The dual-inversion-recovery BB imaging showed improved reproducibility for LV mass measurements compared with SSFP and improved spatial resolution. For studies requiring LV mass measurements, the dual-inversion-recovery BB sequence offers improved spatial resolution and improved reproducibility to SSFP.

MR imaging of cardiac tumors.

Magnetic resonance (MR) imaging is an important tool in the evaluation of cardiac neoplasms. T1-weighted, T2-weighted, and gadolinium-enhanced sequences are used for anatomic definition and tissue characterization, whereas cine gradient-echo imaging is used to assess functional effects. Recent improvements in pulse sequences for cardiac MR imaging have led to superior image quality, with reduced motion artifact and improved signal-to-noise ratio and tissue contrast. Although there is some overlap in the MR imaging appearances of cardiac tumors, particularly of primary malignancies, differences in characteristic locations and features should allow confident differentiation between benign and malignant tumors. Indicators of malignancy at MR imaging are invasive behavior, involvement of the right side of the heart or the pericardium, tissue inhomogeneity, diameter greater than 5 cm, and enhancement after administration of gadolinium contrast material (as a result of higher tissue vascularity). Concomitant pericardial or pleural effusions are rare in benign processes but occur in about 50% of cases of malignant tumors. MR imaging offers improved resolution, a larger field of view, and superior soft-tissue contrast compared with those of echocardiography, suggesting that knowledge of the MR imaging features of cardiac neoplasms is important for accurate diagnosis and management.

Coronary artery disease: myocardial perfusion MR imaging with sensitivity encoding versus conventional angiography.

PURPOSE: To evaluate the technical performance of sensitivity encoding (SENSE)-accelerated myocardial perfusion magnetic resonance (MR) imaging and prospectively assess the diagnostic accuracy of this examination for depiction of significant coronary artery disease (CAD). MATERIALS AND METHODS: All 102 subjects provided written informed consent, and the local ethics committee approved the study. A saturation-recovery segmented k-space gradient-echo pulse sequence was combined with SENSE to allow dynamic acquisition of myocardial perfusion data on four parallel short-axis MR image sections at every heartbeat. This technique was evaluated in 10 healthy volunteers and in 92 patients scheduled to undergo conventional coronary angiography. Gadopentetate dimeglumine was peripherally injected at rest and during adenosine-induced stress. The maximal upslope of the signal intensity-time profiles was plotted for 16 myocardial segments defined on three MR image sections, and a myocardial perfusion reserve index (MPRI) between stress and rest, normalized to the input function from the blood pool of the most basal section, was calculated. Areas under receiver operating characteristic curves (AUCs) were used to assess the diagnostic performance of cardiac MR imaging for depiction of greater than 70% CAD seen at coronary angiography, the reference standard. RESULTS: In volunteers, the mean myocardial enhancement was 2.1 +/- 1.2 (standard deviation), with homogeneous signal intensity distribution across the segments. The diagnostic accuracy of MPRI measurements was high (AUC, 0.908; sensitivity, 88% [52 of 59 patients]; specificity, 82% [27 of 33 patients]). Diagnostic performance was similar among separate analyses of the three coronary territories and among separate analyses of data in the patients with diabetes mellitus, left ventricular hypertrophy, or myocardial infarction. CONCLUSION: Multisection myocardial perfusion MR imaging with SENSE is feasible and has high diagnostic accuracy in the detection of CAD.

Assessment of regional left ventricular function: accuracy and reproducibility of positioning standard short-axis sections in cardiac MR imaging.

The assessment of regional left ventricular (LV) function with cardiac magnetic resonance (MR) cine techniques requires a standardized section positioning. A simple selective short-axis method for selective positioning of three short-axis sections (basal, midcavity, apical) was tested for its accuracy, compared with accepted criteria, in 21 volunteers (mean age, 32 years +/- 11) and in 23 patients with myocardial infarction (mean age, 56 years +/- 12). Reproducibility of section positioning and of regional LV parameters was tested in the volunteers. Among the six accuracy criteria defined for standard sections, the selective short-axis approach had an average accuracy of 90.9% in volunteers and 87.7% in patients, compared with 92.1% and 90.6%, respectively, for a multisection approach covering the whole LV. There was very good reproducibility of the selected intersection gap (r = 0.89, P < .001) and of measured midcavity end-diastolic diameters in vertical (r = 0.83, P < .001) and horizontal (r = 0.85, P < .001) long-axis orientations. The proposed method produces standardized short-axis section positions that meet the recommendations for cardiac imaging. The study was approved by the local ethics committee, and all subjects gave written informed consent.

Assessment of non-ST-segment elevation acute coronary syndromes with cardiac magnetic resonance imaging.

OBJECTIVES: The goal of this study was to determine: 1) if the presence of significant coronary stenosis in patients presenting with non-ST-segment elevation acute coronary syndromes (NSTE-ACS) can be predicted by cardiac magnetic resonance (CMR) imaging; and 2) if the analysis of several CMR methods improves its diagnostic yield compared with analysis of individual methods. BACKGROUND: With modern acquisition techniques, several CMR methods for the assessment of coronary artery disease (CAD) can be combined in a single noninvasive scanning session. Such a multicomponent CMR examination has not previously been applied to a large patient population, in particular those with a high prevalence of CAD in an acute situation. METHODS: Sixty-eight patients presenting with NSTE-ACS underwent CMR imaging of myocardial function, perfusion (rest and adenosine-stress), viability (by late contrast enhancement), and coronary artery anatomy. Visual analysis of CMR was carried out. First, all CMR data were reviewed in combination ("comprehensive analysis"). In further separate analyses, each CMR method was analyzed individually. The ability of CMR to detect coronary stenosis >/=70% on X-ray angiography was determined. RESULTS: Comprehensive CMR analysis yielded a sensitivity of 96% and a specificity of 83% to predict the presence of significant coronary stenosis and was more accurate than analysis of any individual CMR method; CMR was significantly more sensitive and accurate than the Thrombolysis In Myocardial Infarction risk score (p < 0.001). CONCLUSIONS: Cardiac magnetic resonance imaging accurately predicts the presence of significant CAD in patients with NSTE-ACS. In this study, a comprehensive analysis of several CMR methods improved the accuracy of the test.