The distribution and prognosis of anomalous coronary arteries identified by cardiovascular magnetic resonance: 15 year experience from two tertiary centres.

BACKGROUND: Aberrant coronary arteries represent a diverse group of congenital disorders. Post-mortem studies reveal a high risk of exercise-related sudden cardiac death in those with an anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS) with an inter-arterial course. There is little documentation of lifetime history and long-term follow-up of patients with coronary artery anomalies. METHODS: Patients with anomalous coronary arteries undergoing cardiovascular magnetic resonance over a 15-year period were identified and classified by anatomy and course. Medical records were reviewed for major adverse cardiovascular events (MACE). Revascularisation or myocardial infarction counted only if occurring in the distribution of the anomalous artery. RESULTS: Consecutive patients with coronary artery anomalies were retrospectively identified (n = 172). Median follow-up time was 4.3 years (IQR 2.5-7.8, maximum 15.6). 116 patients had ACAOS of which 64 (55%) had an inter-arterial course (IAC) and 52 (45%) did not. During follow up 110 ACAOS patients were alive, 5 died and 1 lost to follow-up.ACAOS patients experienced 58 MACE events (5 cardiovascular deaths, 5 PCI, 24 CABG and 24 had myocardial infarction). 47 MACE events occurred in ACAOS with IAC and 11 in those without (p < 0.0001), the statistical difference driven by surgical revascularisation and myocardial infarction. CONCLUSIONS: In life, patients with an anomalous coronary artery originating from the opposite sinus of Valsalva taking an IAC have higher rates of both myocardial infarction and surgical revascularisation during long-term follow up, compared to those without IAC.

Assessment of coronary stent deployment using computer enhanced x-ray images-validation against intravascular ultrasound and best practice recommendations.

OBJECTIVE: To investigate the accuracy of stent measurements using coronary x-ray angiograms with a computer based stent enhancement algorithm applied (StentBoost, SB). To derive recommendations for best practice when using such systems. BACKGROUND: Computer enhancement algorithms allow better visualization of intracoronary stents to assist in ensuring adequate stent deployment. Factors that affect the accuracy of measurements taken on such systems are yet to be fully understood. METHODS: We analysed stent deployment of 43 stents in 33 patients measuring minimum stent diameter and cross sectional area (CSA) using intravascular ultrasound (IVUS), SB enhanced x-ray images, and quantitative coronary angiography (QCA). We investigated if the use of two projections and method of calibration influenced correlation between IVUS and SB measurements. RESULTS: Using two views and performing calibration via the guide catheter improved agreement between SB and IVUS measurements. For example, minimum stent diameter assessed with SB using one view and balloon markers for calibration produced a correlation coefficient, r, of 0.21, whereas using two views and the guide catheter for calibration increased agreement to r = 0.62. Relative measures of stent deployment, such as the ratio of minimum to maximum CSA, produced good correlation between IVUS and SB (r = 0.74). CONCLUSIONS: When using the SB system, two projection angles should be used to image the stent. For absolute measurements, the guide catheter should be used for calibration purposes. Relative measures of stent size, which are probably sufficient for assessment of deployment, also give good agreement with similar measures on IVUS, and require no calibration.

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.

Cardiovascular magnetic resonance of myocardial edema using a short inversion time inversion recovery (STIR) black-blood technique: diagnostic accuracy of visual and semi-quantitative assessment.

BACKGROUND: The short inversion time inversion recovery (STIR) black-blood technique has been used to visualize myocardial edema, and thus to differentiate acute from chronic myocardial lesions. However, some cardiovascular magnetic resonance (CMR) groups have reported variable image quality, and hence the diagnostic value of STIR in routine clinical practice has been put into question. The aim of our study was to analyze image quality and diagnostic performance of STIR using a set of pulse sequence parameters dedicated to edema detection, and to discuss possible factors that influence image quality. We hypothesized that STIR imaging is an accurate and robust way of detecting myocardial edema in non-selected patients with acute myocardial infarction. METHODS: Forty-six consecutive patients with acute myocardial infarction underwent CMR (day 4.5, +/- 1.6) including STIR for the assessment of myocardial edema and late gadolinium enhancement (LGE) for quantification of myocardial necrosis. Thirty of these patients underwent a follow-up CMR at approximately six months (195 +/- 39 days). Both STIR and LGE images were evaluated separately on a segmental basis for image quality as well as for presence and extent of myocardial hyper-intensity, with both visual and semi-quantitative (threshold-based) analysis. LGE was used as a reference standard for localization and extent of myocardial necrosis (acute) or scar (chronic). RESULTS: Image quality of STIR images was rated as diagnostic in 99.5% of cases. At the acute stage, the sensitivity and specificity of STIR to detect infarcted segments on visual assessment was 95% and 78% respectively, and on semi-quantitative assessment was 99% and 83%, respectively. STIR differentiated acutely from chronically infarcted segments with a sensitivity of 95% by both methods and with a specificity of 99% by visual assessment and 97% by semi-quantitative assessment. The extent of hyper-intense areas on acute STIR images was 85% larger than those on LGE images, with a larger myocardial salvage index in reperfused than in non-reperfused infarcts (p = 0.035). CONCLUSIONS: STIR with appropriate pulse sequence settings is accurate in detecting acute myocardial infarction (MI) and distinguishing acute from chronic MI with both visual and semi-quantitative analysis. Due to its unique technical characteristics, STIR should be regarded as an edema-weighted rather than a purely T2-weighted technique.

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.

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.

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.

The lipoprotein lipase gene serine 447 stop variant influences hypertension-induced left ventricular hypertrophy and risk of coronary heart disease.

LVH [LV (left ventricular) hypertrophy] is an independent risk factor for CHD (coronary heart disease). During LVH, the preferred cardiac energy substrate switches from FAs (fatty acids) to glucose. LPL (lipoprotein lipase) is the key enzyme in triacylglycerol (triglyceride) hydrolysis and supplies FAs to the heart. To investigate whether substrate utilization influences cardiac growth and CHD risk, we examined the association between the functional LPL S447X (rs328) variant and hypertension-induced LV growth and CHD risk. LPL-X447 has been shown to be more hydrolytically efficient and would therefore release more free FAs than LPL-S477. In a cohort of 190 hypertensive subjects, LPL X447 was associated with a greater LV mass index [85.2 (1.7) in S/S compared with 91.1 (3.4) in S/X+X/X; P=0.01], but no such association was seen in normotensive controls (n=60). X447 allele frequency was higher in hypertensives with than those without LVH {0.14 [95% CI (confidence interval), 0.08-0.19] compared with 0.07 (95% CI, 0.05-0.10) respectively; odds ratio, 2.52 (95% CI, 1.17-5.40), P=0.02}. The association of LPL S447X with CHD risk was then examined in a prospective study of healthy middle-aged U.K. men (n=2716). In normotensive individuals, compared with S447 homozygotes, X447 carriers were protected from CHD risk [HR (hazard ratio), 0.48 (95% CI, 0.23-1.00); P=0.05], whereas, in the hypertensives, X447 carriers had increased risk [HR, 1.54 (95% CI, 1.13-2.09) for S/S (P=0.006) and 2.30 (95% CI, 1.53-3.45) for X447+ (P<0.0001)] and had a significant interaction with hypertension in CHD risk determination (P=0.007). In conclusion, hypertensive LPL X447 carriers have increased risk of LVH and CHD, suggesting that altered FA delivery constitutes a mechanism through which LVH and CHD are associated in hypertensive subjects.

Assessing Coronary Blood Flow Physiology in the Cardiac Catheterisation Laboratory.

BACKGROUND: Contemporary management of coronary disease focuses on the treatment of stenoses in the major epicardial vessels. However, myocardial blood flow is known to be contingent on a range of factors in addition to the patency of the epicardial vessels. These include anatomical and physiological factors such as the extent of myocardium supplied by the vessel, systemic blood pressure, the natural variation in vascular tone in response to physiological needs which allows for coronary autoregulation and pathological factors such as the presence of downstream obstruction to flow due to disease of the small coronary vessels or myocardium. The assessment of clinical effectiveness and adequacy of coronary revascularisation requires the ability to comprehensively and accurately assess and measure myocardial perfusion. CONCLUSION: In this article, we review the current methods of evaluating coronary blood flow and myocardial perfusion in the cardiac catheterisation laboratory.

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.

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.

Role of MRI in clinical cardiology.

Rapid progress has been made in cardiac MRI (CMRI) over the past decade, which has firmly established it as a reliable and clinically important technique for assessment of cardiac structure, function, perfusion, and myocardial viability. Its versatility and accuracy is unmatched by any other individual imaging modality. CMRI is non-invasive and has high spatial resolution and avoids use of potentially nephrotoxic contrast agent or radiation. It has been extensively studied against other established non-invasive imaging modalities and has been shown to be superior in many scenarios, particularly with respect to assessment of cardiac and great vessel morphology and left ventricular function. Furthermore, its clinical use continues to expand with increasing experience and proliferation of CMRI centres. As worldwide prevalence of cardiovascular disease continues to rise, CMRI provides opportunity for improved and cost-effective non-invasive assessment. Continued progress in CMRI technology promises to further widen its clinical application in coronary imaging, myocardial perfusion, comprehensive assessment of valves, and plaque characterisation.

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.

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.

Is Intra-Aortic Balloon Pump Counterpulsation Sufficient to Treat Patients in Cardiogenic Shock, Undergoing Primary Percutaneous Coronary Intervention.

BACKGROUND: A high number of patients do not survive primary percutaneous coronary intervention (PCI) complicated by cardiogenic shock (CS), even when assisted with intra-aortic balloon pump (IABP) counterpulsation. There is no accepted consensus on who may most benefit from IABP counterpulsation, although previous retrospective studies have reported predictors of survival for patients undergoing PCI and cardiac surgery. To date, a risk model for emergency primary PCI patients has not been ascertained. The objective of this study was to identify independent predictors for in-hospital survival, to create a standardized risk model to predict patients who may require IABP insertion during primary PCI. METHOD: Retrospective data were from 165 patients who had undergone primary PCI with IABP due to CS complicating acute myocardial infarction (AMI), from September 2007 to 2010, and underwent logistic regression analysis, to evaluate the incremental risk factors associated with survival. RESULTS: The overall in-hospital mortality was 32.1% (53 patients). The incremental independent predictors for in-hospital survival were: patient age of less than 60 years (OR: 0.303, 95% CI: 0.11 - 0.83, P < 0.02) and the use of IABP support alone, as opposed to in adjunction with inotropic support (OR: 3.177, 95% CI: 1.159 - 8.708, P < 0.025). CONCLUSION: This study illustrated an age of less than 60 years, and the use of IABP alone, to be independent predictors of in-hospital survival in patients with CS complicating AMI who undergo primary PCI assisted by IABP. No specific risk model could be determined.

Quantification of pulmonary autograft characteristics using magnetic resonance imaging.

BACKGROUND AND AIM OF THE STUDY: The diameters and distensibility of the native pulmonary root and their effect on pulmonary autograft performance were examined pre- and postoperatively using cardiac ultrasound and magnetic resonance imaging (MRI). METHODS: Eight patients undergoing the Ross procedure were prospectively involved. The diameters of the native aortic, native pulmonary and autograft roots were measured at the level of the annulus, sinus, sinotubular junction and in the main root using MRI through the cardiac cycle. Ultrasound was also used to estimate the degree of regurgitation, both pre- and postoperatively. RESULTS: The pulmonary root implanted into the systemic circulation increased in size but decreased in distensibility significantly at the sinus, sinotubular junction and main root, but not at the annulus. Postoperatively, the pulmonary autograft annulus showed a similar size and distensibility to that of the native aortic annulus. A wide range of aortic annular sizes (22-30 mm) produced clinically competent valves postoperatively. All undersized pulmonary valves showed only trivial regurgitation postoperatively. Although there was no clear correlation between root shape and valve insufficiency, two patients with mild and moderate autograft regurgitation both had divergent pulmonary roots (diameter at sinotubular junction > annulus diameter) preoperatively. CONCLUSION: The pulmonary autograft using the root replacement technique functioned well in all but one case. The shape of the native pulmonary root may be a determinant of early autograft regurgitation, as well as the diameter and the size mismatch between the great arteries.

A comparison of left ventricular mass between two-dimensional echocardiography, using fundamental and tissue harmonic imaging, and cardiac MRI in patients with hypertension.

PURPOSE: To compare left ventricular mass (LVM) as measured by two-dimensional (2D) echocardiography using two different calculation methods: truncated ellipse (TE) and area length (AL), in both fundamental and tissue harmonic imaging frequencies, to LVM as measured by, the current gold standard, cardiac magnetic resonance imaging (MRI). Turbo gradient echo (TGE) pulse sequence was utilized for MRI. MATERIALS AND METHODS: Thirty-two subjects with history of hypertension were recruited. The images were acquired, contours were traced and the LVM was calculated for all four different echocardiography methods as well as for the cardiac MRI method. The intra-observer variabilities were calculated. The four different echocardiography methods were compared to cardiac MRI using the method described by Bland and Altman. RESULTS: Twenty-five subjects had adequate paired data sets. The mean LVM as measured by cardiac MRI was 162+/-55 g and for the four different echocardiography methods were: fundamental AL 165+/-55 g, harmonic AL 168+/-53 g, fundamental TE 148+/-50 g, harmonic TE 149+/-45 g. The intra-observer variability for cardiac MRI method, expressed as bias +/- 1 standard deviation of the difference (S.D.D.), was 2.3+/-9.2 g and for the four different echocardiography methods were: fundamental TE 0.4+/-26.8 g, fundamental AL 0.6+/-27.0 g, harmonic TE 6.7+/-21.8 g, harmonic AL 6.4+/-22.9 g. The mean LVM for the AL method was closest to the cardiac MRI technique, while TE underestimated LVM. The 95% limits of agreement were consistently wide for all the 2D echocardiography modalities when compared with the cardiac MRI technique. CONCLUSION: The intra-observer variability in measurements of 2D echocardiographic LVM, together with the wide limits of agreement when compared to the gold standard (cardiac MRI) are sufficiently large to make serial estimates of LVM, of single patients or small groups of subjects, by 2D echocardiography, unreliable.

Does the use of additional X-ray beam filtration during cine acquisition reduce clinical image quality and effective dose in cardiac interventional imaging?

The impact of spectral filtration in digital ('cine') acquisition was investigated using a flat panel cardiac interventional X-ray imaging system. A 0.1-mm copper (Cu) and 1.0-mm aluminium (Al) filter added to the standard acquisition mode created the filtered mode for comparison. Image sequences of 35 patients were acquired, a double-blind subjective image quality assessment was completed and dose-area product (DAP) rates were calculated. Entrance surface dose (ESD) and effective dose (E) rates were determined for 20- and 30-cm phantoms. Phantom ESD fell by 28 and 41 % and E by 1 and 0.7 %, for the 20- and 30-cm phantoms, respectively, when using the filtration. Patient DAP rates fell by 43 % with no statistically significant difference in clinical image quality. Adding 0.1-mm Cu and 1.0-mm Al filtration in acquisition substantially reduces patient ESD and DAP, with no significant change in E or clinical image quality.

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.