Apical hypertrophic cardiomyopathy (ApHCM) is an HCM variant, affecting frequently males in midlife. It is characterized by apical obliteration and persistent diastolic contraction, often resulting ...in microvascular ischaemia. We report five cases of ApHCM, with evidence of intramyocardial calcification on echocardiogram. On cardiac magnetic imaging (MRI), a hypointense component at early gadolinium enhancement (EGE) sequences, compatible with calcium, and a deep layer, with hyperintensity at late gadolinium enhancement (LGE) sequences, referable to fibrosis, suggest an endomyocardial fibrosis (EMF) diagnosis. EMF pathologic hallmark is endocardium and myocardium scarring, evolving to dystrophic calcification. It is found only in few ApHCM patients. Our series is the largest one described until now. Analysing patients' history, coexistent inflammatory triggers were evident in all of them, so their co‐morbidities could represent a further cause of small vessel disease, in the context of ischaemic microvascular stress due to hypertrophy, leading to fibrosis and dystrophic calcification. This series could demonstrate the relation between apical fibrosis/calcification and microvascular ischaemia due to hypertrophy and inflammatory triggers.
Patients with a short QT syndrome (SQTS) are at risk of sudden cardiac death (SCD). It is not known whether abbreviation of cardiac repolarization alters mechanical function in SQTS. Controversies ...persist regarding whether the U wave is a purely electrical or mechanoelectrical phenomenon.
The present study uses echocardiographic measurements to discriminate between the hypotheses for the origin of the U wave.
Diagnostic work-up including echocardiography and electrocardiogram was performed in 5 SQTS patients (39 +/- 19 years old) from 2 unrelated families with a history of SCD and 5 age-matched and gender-matched control subjects.
QT intervals were 268 +/- 18 ms (QTc 285 +/- 28 ms) in SQTS versus 386 +/- 20 ms (QTc 420 +/- 22 ms) in control subjects (P < .005). In SQTS patients, the end of the T wave preceded aortic valve closure by 111 +/- 30 ms versus -12 +/- 11 ms in control subjects (P < .005). The interval from aortic valve closure to the beginning of the U wave was 8 +/- 4 ms in patients and 15 +/- 11 ms in control subjects (P = .25). Thus, the inscription of the U wave in SQTS patients coincided with aortic valve closure and isovolumic relaxation, supporting the hypothesis that the U wave is related to mechanical stretch.
Our data show for the first time a significant dissociation between the ventricular repolarization and the end of mechanical systole in SQTS patients. Coincidence of the U wave with termination of mechanical systole provides support for the mechanoelectrical hypothesis for the origin of the U wave.
Background Cardiac magnetic resonance (CMR) imaging with late gadolinium enhancement (LGE) can identify areas of myocardial fibrosis in vivo in patients with hypertrophic cardiomyopathy (HCM). The ...aim of this study was to examine the association between clinical-morphological variables, risk factor for sudden death, and LGE findings in a consecutive, unselected population of HCM patients. Methods From January 2005 to August 2009, 124 HCM patients (53 ± 17 years, 86 men) were prospectively evaluated with CMR examination, assessing left ventricular (LV) hypertrophy, function, and LGE. Results In univariate analysis, patients were divided into tertiles according to the number of segments positive for LGE (first tertile, 0.3 ± 0.4; second tertile, 2.2 ± 0.4; third tertile, 5.2 ± 1.9 segments). Male gender ( P = .05), maximum LV wall thickness ( P = .002), nonsustained ventricular tachycardia ( P = .001), ejection fraction <50% ( P = .02), LV mass ( P = .02), left atrium dilation ( P = .04), perfusion defects ( P ≤ .001), and telesystolic volume ( P = .04) were all positively related with the number of segments of LGE. In multivariable analysis, male gender ( P = .007), maximum LV wall thickness ( P = .006), LV mass ( P = .031), and perfusion alterations ( P = .017) were independent predictors of LGE extent. Conclusions Our study shows an independent association, even at multivariate analysis, between the entity of LGE and maximum LV wall thickness, mass, and perfusion defects in patients with HCM. Whether the presence and the extent of LGE translates into clinical events later on awaits further long-term follow-up studies.
A 59-year-old man underwent an echocardiography study after myocardial infarction and it showed a thin, mobile mass attached to the aortic valve. A diagnosis of Lambl’s excrescence (LE) was ...suspected. Coronary occlusion as a consequence of embolism of LE’s material could not be excluded and the patient underwent surgical excision. Histology confirmed the diagnosis; however a differential diagnosis with papillary fibroelastoma could not be established because both of these structures are histologically indistinguishable. A brief survey of the literature is presented. Evidence-based recommendations for treatment have not been established yet.
Anomalous fibromuscular bands in the left atrium were already described in the 19
th
century. Recently, the greater attention to the anatomy of the left atrium and the technological improvement have ...made their finding more frequent. Here, we present six cases, out of approximately 30,000 unselected echocardiograms, in which the use of the three-dimensional echo allowed a better definition of their anatomy, course, and motility.
Abstract
Introduction
Apical Hypertrophic Cardiomyopathy (ApHCM) characterized by persistent diastolic apical contraction results in dynamic apical small-vessel obstruction with microvascular ...ischemia. Of note, endomyocardial fibrosis (EMF) and calcification are described only in few patients.
Case presentation
We report 5 cases of ApHCM with apical intramyocardial calcification. They presented characteristic ECG pattern and fibrosis at echocardiogram. All patients presented a preserved ejection fraction (EF), except for one patient with mild reduced EF. Global longitudinal strain was reduced in 3 patients. Diastolic dysfunction was evidenced in 3 patients. Right ventricle involvement was detected in one patient only.On cardiac magnetic resonance, a superficial hypo-intense component, compatible with calcium and a deep layer featured by late gadolinium enhancement (LGE) related to fibrotic tissue, were revealed. LGE was present in all of patients in the apex. One patient presented an apical aneurysm, with high ESC-SCD risk score and ICD implantation.
Conclusion
EMF pathologic hallmarks were the endocardium and myocardium scarring, evolving to dystrophic calcification. In clinical practice, only a minority of ApHCM patients develops EMF and calcifications. Our clinical series is the largest one in literature. Analyzing patients’ history, a microvascular inflammatory trigger was evident in all of them, particularly severe chronic kidney disease, diabetes, high degree obesity, malaria infection, peripheral microangiopathy and form of thalassemia. This series could demonstrate the pathophysiological relation between apical fibrosis, calcification and microvascular ischemia due to hypertrophy and inflammatory conditions coexistence. A broader case series could evaluate any correlation with their long-term outcome and management strategies.
We studied left atrial function in 55 patients undergoing electrical (n = 23) or chemical (intravenous administration of propafenone, n = 32) attempts at cardioversion from atrial fibrillation. ...Chemical attempts at cardioversion revealed a significant increase in spontaneous echo contrast and a significant decrease in left atrial appendage Doppler flow, even in patients who did not have successful conversion to sinus rhythm.
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