Atrial giant cell myocarditis: a distinctive clinicopathologic entity.

BACKGROUND: Giant cell myocarditis (GCM) typically causes fulminant heart failure, arrhythmias, or heart block, necessitating aggressive immunosuppression, ventricular assist device insertion, or cardiac transplantation. We describe a novel variant of GCM, primarily involving the atria, that displays distinctive clinical features and follows a more benign course than ventricular GCM. METHODS AND RESULTS: We identified 6 patients (median age 67.5 years, 4 male) with atrial GCM in our pathology consultation practices from 2010 to 2012. Clinical history, imaging, and pathology materials were reviewed. Clinically, 4 patients had atrial fibrillation, 1 had acute heart failure, and 1 had incidental disease at autopsy. Among the 5 living patients, echocardiography revealed severe atrial dilatation (5 cases), mitral/tricuspid regurgitation (5), atrial mural thrombus (3), atrial wall thickening (2), and atrial hypokinesis (2). Ventricular function was preserved in all 5. Histological review of surgically resected atria showed giant cell and lymphocytic infiltrates, lymphocytic myocarditis-like foci, cardiomyocyte necrosis, and cardiomyocyte hypertrophy in all cases. Other features included interstitial fibrosis (5), poorly-formed granulomas (4), eosinophils (4), neutrophils (1), and vasculitis (1). Treatment consisted of steroids and cyclosporine (1), pacemaker placement for sick sinus syndrome (1), and supportive care (3). All 5 living patients returned to baseline exercise tolerance after 6 to 16 weeks of follow-up. CONCLUSIONS: Atrial GCM represents a distinct clinicopathologic entity with a more favorable prognosis than classic ventricular GCM. This disorder should be included in the differential diagnosis of atrial dilatation, particularly when associated with atrial wall thickening. The utility of immunomodulatory therapy for this condition remains unknown.

Left ventricular scar burden specifies the potential for ventricular arrhythmogenesis: an LGE-CMR study.

INTRODUCTION: The extent of left ventricular (LV) scar, characterized by late gadolinium enhancement cardiac MRI (LGE-CMR), has been shown to predict the occurrence of ventricular arrhythmias in implantable cardioverter defibrillator (ICD) recipients. However, the specificity of LGE-CMR for sudden cardiac death (SCD) versus non-SCD is unclear. The aim of this retrospective, observational study was to evaluate this relationship in a cohort of ICD recipients. METHODS AND RESULTS: We included consecutive patients who had undergone LGE-CMR before ICD implantation over a 4-year period (2006-2009). Scar (defined as myocardium with a signal intensity ≥50% of the maximum in scar tissue) was characterized in terms of percent scar and number of transmural LV scar segments in a 17-segment model. The endpoints were appropriate ICD therapy and all-cause mortality. Sixty-four patients (average age 66 ± 11 years, 51 male, median LVEF 30%) were included. During 42 ± 13 months follow-up, appropriate ICD therapy occurred in 28 patients (44%), and 14 patients (22%) died. Number of transmural scar segments (P = 0.005) and percentage LV scar (P = 0.03) were both significantly associated with appropriate ICD therapy. However, neither number of transmural scar segments (P = 0.32) or percent LV scar (P = 0.59) was significantly associated with all-cause mortality. CONCLUSION: In this observational study, in medium-term follow-up, the extent of LV scar characterized by LGE-CMR was strongly associated with the occurrence of spontaneous ventricular arrhythmias but not all-cause mortality. We hypothesize that scar quantification by LGE-CMR may be more specific for SCD than non-SCD, and may prove a valuable tool for the selection of patients for ICD therapy.

The relationship between left ventricular scar and ventricular repolarization in patients with coronary artery disease: insights from late gadolinium enhancement magnetic resonance imaging.

AIMS: The markers of ventricular repolarization corrected QT interval (QTc), QT dispersion (QTD) and Tpeak-to-Tend interval (Tpeak-end) have shown an association with sudden cardiac death (SCD) in the general population. However, their mechanistic relationship with SCD is unclear. The study aim was to evaluate the relationship between QTc, QTD, and Tpeak-end, and the extent and distribution of left ventricular (LV) scar in patients with coronary artery disease at high SCD risk. METHODS AND RESULTS: We included 64 consecutive implantable cardioverter defibrillator (ICD) recipients (66 ± 11 years, 80% male, median left ventricular ejection fraction 30%) who had undergone late gadolinium enhancement cardiac magnetic resonance (CMR) imaging prior to device implantation over 4 years. Scar was quantified using the CMR images and characterized in terms of percent LV scar and number of LV segments with subendocardial/transmural scar. Repolarization parameters were measured on an electrocardiogram performed prior to ICD implantation. After adjustment for potential confounders there was a strong association between the number of limited subendocardial (1-25% transmurality) scar segments and QTc (P = 0.003), QTD (P = 0.002), and Tpeak-end (P = 0.008). However, there was no association between the repolarization parameters and percent LV scar or the amount of transmural scar. During a mean follow-up of 19 ± 10 months 19 (30%) patients received appropriate ICD therapy, but none of the repolarization parameters were associated with its occurrence. CONCLUSION: In this pilot study there was a strong association between limited subendocardial LV scar and prolonged QTc, QTD, and Tpeak-end. However, there was no association between any of these repolarization markers and the delivery of appropriate ICD therapy.

Imaging hypertrophic heart diseases with cardiovascular MR.

The assessment of ventricular hypertrophy is an increasingly common indication for cardiac MR (CMR) in every day clinical practice. CMR is useful to confirm the presence of hypertrophy and to help to define the underlying cause through a combination of a detailed assessment of ventricular function and tissue characterising sequences. As well as being a useful diagnostic tool, some CMR imaging features are of prognostic significance. In this article, we review the typical appearances of common forms of ventricular hypertrophy, focussing principally on left ventricular hypertrophy, and demonstrate the techniques that can be used to differentiate one form of hypertrophy from another.

The relationship between the contralateral collateral supply and myocardial viability on cardiovascular magnetic resonance: can the angiogram predict functional recovery?

BACKGROUND: A collateral circulation which supplies a myocardial territory, subtended by a chronic total occlusion (CTO), may be observed at invasive coronary angiography. The prognostic and protective role of such collateralisation is well demonstrated suggesting that a good collateral circulation may be a predictor of myocardial viability, but current evidence is discrepant. The aim of this study is to assess the relationship between collateralisation from the contralateral epicardial vessels and myocardial viability by cardiovascular magnetic resonance (CMR). METHOD: Consecutive patients with CTO having had both CMR and invasive coronary angiography were retrospectively identified. The collateral circulation was graded with the Cohen and Rentrop classification. CMR images were graded per segment for wall motion (1: normal/hyperkinetic, 2: hypokinetic, 3: akinetic, or 4: dyskinetic) and wall motion score index (WMSI) was calculated. The segmental transmurality of late gadolinium enhancement was scored as 1 (0%), 2 (1-25%), 3 (26-50%), 4 (51-75%) and 5 (76-100%). RESULTS: A good collateral circulation was more likely to supply viable myocardium (p=0.01). There was no relationship between collateral circulation supply and wall motion score index (WMSI), however, increasing transmurality of LGE was significantly associated with higher mean WMSI representing increasing dysfunctional myocardium (p<0.001). CONCLUSION: The presence of collateral coronary circulation at angiography predicts the presence of viability on cardiovascular MRI, with a gradation of greater viability associated with improving Rentrop grade. A collateral circulation at angiography should, therefore, prompt more formal assessment of viability and consideration of revascularisation in order for the patient to obtain the associated functional and prognostic improvement.

The extent of left ventricular scar quantified by late gadolinium enhancement MRI is associated with spontaneous ventricular arrhythmias in patients with coronary artery disease and implantable cardioverter-defibrillators.

BACKGROUND: Characterization of sudden cardiac death (SCD) risk remains a challenge in the application of implantable cardioverter-defibrillator (ICD) therapy. Late gadolinium enhancement cardiac MRI (LGE-CMR) can accurately identify myocardial scar. We performed a retrospective, single-center observational study to evaluate the association between the extent and distribution of left ventricular scar, quantified using LGE-CMR, and the burden of ventricular arrhythmias in patients with coronary artery disease and ICDs. METHODS AND RESULTS: All patients included (2006 to 2009) had undergone LGE-CMR before ICD implantation. Scar (defined as myocardium with a signal intensity ≥50% of the maximum in scar tissue) was characterized in terms of percent scar, scar surface area, and number of transmural left ventricular scar segments. The end point was appropriate ICD therapy. Sixty-four patients (mean age, 66±11 years; male sex, 51) were included. During 19±10 months follow-up, appropriate ICD therapy occurred in 19 (30%) patients. In Cox regression analyses, both percent scar (hazard ratio per 10%, 1.75; 95% CI, 1.09 to 2.81; P=0.02) and number of transmural scar segments (hazard ratio per segment, 1.40; 95% CI, 1.15 to 1.70; P=0.001) were significantly associated with the occurrence of appropriate ICD therapy. CONCLUSIONS: In this pilot study, the extent of myocardial scar characterized by LGE-CMR was significantly associated with the occurrence of spontaneous ventricular arrhythmias. We hypothesize that scar quantification by LGE-CMR may prove a valuable risk stratification tool for the occurrence of ventricular arrhythmias, which may have implications for patient selection for ICD therapy.

The prevalence of coronary arterial abnormalities in pulmonary atresia with intact ventricular septum and their influence on surgical results.

BACKGROUND: The relatively high mortality in patients with pulmonary atresia and intact ventricular septum may be related to the presence of significant coronary arterial anomalies. This retrospective review of cineangiocardiograms was undertaken to further elucidate the types and variety of such coronary arterial abnormalities, and to assess their effect on postoperative survival. MATERIAL AND RESULTS: Details regarding coronary arterial anatomy and abnormalities were assessed in 116 patients. We noted the site and severity of lesions, and the presence of fistulous communications from the right ventricle to the coronary arteries, assessing the proportion of left ventricular myocardium affected by coronary arterial interruptions or significant stenoses, in other words, the amount dependent on coronary circulation from the right ventricle. We also measured diameters of the tricuspid and mitral valves. Fistulas were found in 87 patients (75%), interruptions of major coronary arteries in 40 patients (34%), lack of connections between the coronary arteries and the aorta in 18 patients (16%), and single origin of a coronary artery, with the right coronary artery arising from the left, in 6 patients (5%). We found increased mortality in 47 patients (40%) who had a right ventricular-dependent coronary arterial circulation. The presence of fistulas in itself was not associated with higher mortality, but the presence of coronary arterial interruptions (p = 0.05), and a higher myocardial score (p = 0.0009), were. CONCLUSION: We encountered a higher prevalence of both coronary arterial abnormalities and right ventricular-dependent circulation than previously reported. Awareness of the severity of the coronary arterial abnormalities should assist in planning treatment.