A unique pattern of delayed enhancement of a large cardiac fibroma on magnetic resonance imaging.

MRI is a valuable noninvasive tool that helps in predicting the type of cardiac tumors and guiding management decisions. Several reports have described the appearance of cardiac fibromas on MRI, which typically show hyperenhancement on myocardial delayed enhancement (MDE) imaging, with or without a dark core. This report demonstrates the unique appearance of a large solitary ventricular septal cardiac fibroma in a 5-month-old patient on MDE imaging, with two discrete dark cores, each surrounded by a hyperenhancing pseudocapsule.

Novel contouring method for optimizing MRI flow quantification in patients with aortic valve disease.

Optimizing MRI aortic flow quantification is crucial for accurate assessment of valvular disease severity. In this study, we sought to evaluate the accuracy of a novel method of contouring systolic aortic forward flow in comparison to standard contouring methods at various aortic levels. The study included a cohort of patients with native aortic valve (AoV) disease and a small control group referred to cardiac MRI over a 1-year period. Inclusion criteria included aortic flow quantification at aortic valve and one additional level, and no or trace mitral regurgitation (MR) documented both by the MRI AND an echocardiogram done within a year. In addition to flow quantification with standard contouring (SC), a novel Selective Systolic Contouring (SSC) method was performed at aortic valve level, contouring the area demarcated by the AoV leaflets in systole. The bias in each technique's estimate of aortic forward flow was calculated as the mean difference between aortic forward flow and left ventricular stroke volume (LV SV). 98 patients (mean age 56, 71% male) were included: 33 with tricuspid and 65 with congenitally abnormal (bicuspid or unicuspid) AoV. All methods tended to underestimate aortic forward flow, but the bias was smallest with the SSC method (p < 0.001). Therefore, SSC yielded the lowest estimates of mitral regurgitant volume (4.8 ml) and regurgitant fraction (3.9%) (p < 0.05). SSC at AoV level better approximates LV SV in our cohort, and may provide more accurate quantitative assessment of both aortic and mitral valve function.

Computed tomographic angiography identification of intramural segments in anomalous coronary arteries with interarterial course.

Certain coronary anomalies are associated with high risk features. We sought to determine the diagnostic accuracy of coronary computed tomographic angiography (CTA) in determining high-risk features, particularly intramural segments. Anomalous coronary arteries can be associated with adverse clinical events. Anomalous coronaries that course between the great vessels (interarterial) have been associated with sudden death. High-risk features of interarterial vessels described in the literature include; a slit-like orifice, acute angle of origin, and intramural segments (within the wall of the aorta). Although computed tomography (CT) findings of acute angle and slit like orifice have been described previously no prior evaluations regarding CT identification of an intramural segment have been reported. An intramural segment has distinct surgical management implications. All interarterial anomalous coronary arteries do not have an intramural segment. Since October 2004, 15 patients were diagnosed by CTA as having an anomalous coronary artery with an interarterial course, which were then confirmed by intraoperative examination of their coronary origins and course during aortic root/coronary artery surgery. The CTA images were retrospectively analyzed for the presence of high-risk features by a radiologist blinded to the surgical findings. Comparison of these findings was made to the findings at surgery. The anomalous coronary was the right coronary artery in 10 patients and the left coronary artery in 5. Eleven patients had an intramural segment identified at surgery. Pre-operative coronary CTA showed that all patients with an intramural course of the anomalous artery, had slit-like orifice, an acute angle of origin (mean 18.4 ± 3.4°), and an elliptical shaped cross-section throughout the intramural segment of the anomalous vessel. The average vessel height/width ratio for anomalous coronary vessels without an intramural segment was 1.03; compared to a ratio of 2.19 for anomalous vessels with an intramural segment (P = 0.003). Coronary CTA can identify an intramural segment of an anomalous interarterial coronary artery by its elliptical shape. Identifying an intramural segment has important clinical and surgical implications.

Methods to access the surgically excluded cavotricuspid isthmus for complete ablation of typical atrial flutter in patients with congenital heart defects.

BACKGROUND: Cavotricuspid isthmus (CVTI)-dependent flutter in postoperative congenital heart disease patients is common and difficult to treat. OBJECTIVE: The purpose of this study was to evaluate techniques for accessing excluded portions of the CVTI after Fontan or atrial switch procedures and completely ablating flutter. METHODS: Patients who had undergone Fontan or atrial switch procedures and had CVTI-dependent flutter requiring ablation between 1990 and 2007 were identified. Flutters induced, methods for accessing the CVTI, use of intracardiac echocardiography, complications, and success rates were noted. RESULTS: Sixteen patients (44% males, mean age at ablation 28 years) were identified: 14 prior Fontan and 2 Mustard repair, with a total of 19 ablation procedures. In 13 (81%) of 16 patients, access to the entire CVTI could not be achieved via a systemic venous route. The excluded CVTI was accessed by retrograde transaortic approach in 6 and by anterograde transconduit puncture in 1 patient, with termination and lack of reinducibility of CVTI-dependent flutter achieved in all cases. One patient developed high-grade AV block requiring pacemaker therapy. Follow-up data (range 1-89 months, mean 29 months) were available for 18 of 19 procedures. CVTI atrial flutter recurred in 1 of 7 patients involving access to the pulmonary venous side. CONCLUSION: Even when surgical procedures exclude a portion of the CVTI, complete ablation of "typical" atrial flutter, including documentation of bidirectional block, can be achieved by novel approaches targeting the surgically excluded arrhythmogenic atrial tissue.

Successful transcatheter closure of an aorto-left atrial fistula.

Aorto-left atrial fistula is a rare entity in which the integrity of the aortic root bordering the left atrium is disrupted. The clinical presentation is highly variable, depending predominantly on the size of the fistula and the pressure difference between the aorta and the left atrium. Surgical repair was the standard treatment. Recently, however, there have been reports of successful transcatheter closure. We report a 32-year-old male with Shone's syndrome who had multiple prior surgical procedures including aortic and mitral valve replacements. He presented with an aorto-left atrial fistula that was successfully closed percutaneously using an Amplatzer atrial septal defect device.