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I want to get healthier. Close Health Alerts from Harvard Medical School Get helpful tips and guidance for everything from fighting inflammation to finding the best diets for weight loss An under recognized and very often missed subgroup within this broad spectrum concerns patients with left ventricular LV apical aneurysms in the absence of coronary artery disease. We describe a case of HCM with midventricular obstruction and apical aneurysm formation in 3 patients coming from a single family.
This HCM pattern was detected by 2D-echocardiography and confirmed by cardiac magnetic resonance imaging. A cardioverter defibrillator was implanted in one of the patients because of non-sustained ventricular tachycardia detected in h Holter monitoring and an abrupt drop in systolic blood pressure during maximal exercise test.
The defibrillator activated 8 months after implantation by suppression of a ventricular tachycardia providing anti-tachycardia pacing. The patient died due to refractory heart failure 2 years after initial evaluation. The rest of the patients are stable after a 2. The detection of apical aneurysm by echocardiography in HCM patients may be complicated. Ventricular tachycardia arising from the scarred aneurysm wall may often occur predisposing to sudden death.
Peer Review reports. Hypertrophic cardiomyopathy HCM is an extremely heterogeneous disease, in terms of clinical course and phenotypic expression [ 1 — 3 ].
An under recognized and very often missed subgroup within this broad spectrum concerns patients with left ventricular LV apical aneurysms in the absence of coronary artery disease [ 4 — 10 ]. In this report we present a case-series of HCM patients with midventricular obstruction and apical aneurysm formation in a single family.
We studied 3 female patients case-index 1, y; case-index 2, y; case-index 3, y Figure 1 with already diagnosed HCM who were referred to our department for further evaluation.
The patients underwent electrocardiography, echocardiography, h Holter monitoring, treadmill cardiopulmonary exercise test, and cardiac magnetic resonance imaging. Unfortunatelly, genotype analysis was not performed since it is not, at present, available in our laboratory. Coronary arteriography performed in case-index 1 and case-index 2 patients excluded significant atherosclerotic narrowing of the extramural coronary arteries. The distribution of LV hypertrophy assessed by 2-D echo was similar among patients, with predominant thickening in the midseptal and midlateral regions maximum wall thickness 16—18 mm Figure 2 , Additional File 1.
None of the patients showed LV outflow tract obstruction. A formation of a wall-thinning aneurysm involving the LV apex was detected in all 3 patients, a finding also confirmed by means of cardiac magnetic resonance imaging MRI and by cardiac catheterization Figures 3 , 4 and Additionals file 2 , 3. A cardioverter defibrillator was implanted in index-case 1 patient because of non-sustained ventricular tachycardia detected in h Holter monitoring and an abrupt drop in systolic blood pressure during maximal exercise test.
Family tree. Family tree showing hypertrophic cardiomyopathy HCM phenotypic expression through 3 generations. The three female patients we studied case-index 1, y; case-index 2, y; case-index 3, y are represented by black colour filled circles in generations II and III.
SCD: Sudden Cardiac death. Squares represent males and circles females. Black colour filled schemes represent HCM patients. Schemes erased by diagonal lines refer to deceased family members. Two dimensional transthoracic echocardiogram.
Apical four chamber view showing predominant thickening of midseptal and midlateral regions during systole blue arrow and the formation of left ventriculat apical aneurysm arrow head in case index-1 patient. The above data are outlined in Table 3.
Frequency distribution for risk factors in a Hypertrophic Cardiomyopathy population. On the X axis is indexed the number of risk factors and on the Y axis the percent of patients in our cohort presenting this number of risk factors. Data analysis reveals that 74 patients The patients were followed-up for a median period of All patients were alive at the end of follow-up. An implantable cardioverter-defibrillator ICD was implanted in 10 patients for primary prevention of SD.
Among these patients, 3 had 4 risk factors, 2 had 3 risk factors, 4 patients had 2 risk factors, and 1 patient had 1 risk factor. The mean follow-up period after ICD implantation, was The annual incidence of appropriate discharges was One patient had an episode of inappropriate discharge due to sinus tachycardia.
Stored data were reviewed after all discharges. It is well established that in HCM-patients with a history of sustained ventricular tachycardia or documented cardiac arrest the risk of SD is sufficiently high to warrant prophylactic therapy, irrespective of the presence of other risk factors [ 11 , 16 ] Figure 2.
Risk stratification in patents without a history of sustained ventricular tachycardia or documented cardiac arrest is a big clinical challenge and sometimes very difficult.
Among the various clinical and echocardiographic variables considered, the simple model based on a small number of generally accepted risk factors proposed by Elliott et al [ 4 ] is, for the time, the most acceptable. Our study group of patients with HCM is unique by virtue of representing a regional cohort from a distinctive region of Northern Greece. Purpose of this study was the risk stratification of our patients, coming from a representative Northern Greek population, according to the previous referred clinical markers.
Implantable Cardioverter Defibrillator wire in a Hypertrophic Cardiomyopathy patient. An echocardiographic apical four chamber view showing the wire of an Implantable Cardioverter Defibrillator in the right ventricle, passing through tricuspid valve. Defibrillator was implanted in a male HCM patient presenting with previous syncope episodes and positive family history for sudden death.
About half of our patients were referred from other centres for evaluation and treatment, and the other half was firstly diagnosed and evaluated in our clinic. As a consequence of this process, our study does not incorporate a substantial degree of patient selection bias. Survival analysis was not performed in our study, since during the short follow-up period there was no any death among patients, and the objectives of this study was mainly the identification of the risk factors in our cohort.
Although an ABPR to exercise is considered as a prognostic factor only in patients younger than 40 years of age [ 17 ], this marker was also examined in older patients, in order to collect homogeneous data in all patients.
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