1/52. Multiple aneurysmal dysplasia of pulmonary valvular sinuses with congenital focal aplasia of the annulus.We report the case of a 22-year-old woman in whom a focal aplasia of the pulmonary valve annulus associated with a multiple aneurysmal compensatory dysplasia was found. This patient had been followed since the age of seven years for a congenital asymmetric hypertrophic cardiomyopathy. Cardiac Doppler analysis showed significant pulmonary valvular insufficiency. The patient underwent heart transplant surgery because of total and refractory cardiac insufficiency. Pathological examination of the explanted heart (at the European Homograft Bank) enabled us to describe this valvular malformation. To date, this anomaly has not been described in the literature. Whether it is a malformation discovered fortuitously, or whether it is responsible in part for some of the signs associated with congenital hypertrophic cardiomyopathy is unclear. However, its role might be disclosed by a rigorous investigation of the patient's family.- - - - - - - - - - ranking = 1keywords = cardiomyopathy, dysplasia (Clic here for more details about this article) |
2/52. Ablation of ventricular tachycardia by isolating the critical site in a patient with arrhythmogenic right ventricular cardiomyopathy.We describe a patient with arrhythmogenic right ventricular cardiomyopathy in whom ventricular tachycardia (VT) was ablated by isolating a relatively large area of the critical site using catheter ablation. Endocardial mapping showed abnormal fragmented electrograms with delayed potential (DP) from an entire area of the aneurysm. Pace mappings from the aneurysm produced a QRS morphology identical to that of clinical VT. After catheter ablation was performed at the exit site of the VT critical area, programmed stimulation inside the aneurysm captured the DP but not the QRS complexes. These data suggest that VT can be ablated successfully by isolation of the critical area.- - - - - - - - - - ranking = 283.15783800512keywords = right ventricular cardiomyopathy, arrhythmogenic, ventricular cardiomyopathy, arrhythmogenic right, cardiomyopathy (Clic here for more details about this article) |
3/52. Evolution of left ventricular involvement in arrhythmogenic right ventricular cardiomyopathy.Left ventricular (LV) involvement in arrhythmogenic right ventricular cardiomyopathy (ARVC) is fairly well known, but the evolution of LV involvement during long-term follow-up has not been well documented. We describe such evolution in a patient followed for 9 years. Evolution was confirmed by a progressive perfusion defect of the LV wall in myocardial scintigrams and by the development of LV asynergy with ventricular aneurysm formation in left ventriculograms. As the right ventricle progressively enlarged, we concluded that ARVC is a diffuse and progressive myocardial disease that affects both ventricles.- - - - - - - - - - ranking = 283.15783800512keywords = right ventricular cardiomyopathy, arrhythmogenic, ventricular cardiomyopathy, arrhythmogenic right, cardiomyopathy (Clic here for more details about this article) |
4/52. Apical aneurysm and left ventricular hypertrophy.A 59-year-old woman presented with an embolic transient ischemic attack and a history of controlled hypertension for 16 years. Both echocardiogram and MRI showed severe biventricular hypertrophy and an apical aneurysm with a thrombus. The occurrence of an apical aneurysm in the presence of cardiac hypertrophy is a rare finding and has been described in patients with hypertrophic cardiomyopathy. However, it has not been reported in patients with systemic arterial hypertension. In this patient the lack of a relationship between the severity of the hypertrophy and the levels of blood pressure, together with the presence of histologic disorganization of myocardial cardiac muscle cells by endomyocardial biopsy suggested the diagnosis of hypertrophic cardiomyopathy.- - - - - - - - - - ranking = 0.97381187994353keywords = cardiomyopathy (Clic here for more details about this article) |
5/52. glycogen storage disease associated with left ventricular aneurysm in an elderly patient.glycogen storage disease (GSD) types II, III, IV, and V may be associated with cardiomyopathy, but, with the exception of type III GSD, adult cases are extremely rare. A 62-year-old man was found to have GSD and a concomitant left ventricular aneurysm. He had been comparatively well until the age of 62 years, although he had suffered a cerebral infarction at the age of 35 years. The damage caused by glycogen deposition was strictly confined to the myocardium. Left ventriculography revealed a left ventricular aneurysm in the apex. The serial change on electrocardiogram, as well as the findings of the echocardiogram and of cardiac catheterization, resembled those of the dilated phase of hypertrophic cardiomyopathy. However, a left ventricular endomyocardial biopsy specimen revealed central vacuolar degeneration of myocytes with depositions of glycogen. The GSD type remains unknown in the present patient, because the activity of debranching enzyme (type III) measured from the skeletal muscle specimen was normal, whereas that of acid maltase (type II) was slightly low. It is possible that there is a specific malfunction of the acid maltase of the myocardium in the present patient.- - - - - - - - - - ranking = 0.97381187994353keywords = cardiomyopathy (Clic here for more details about this article) |
6/52. aneurysm of the left atrial appendage.A 43-year-old woman underwent excision of an aneurysm of the left atrial appendage, which had been causing cerebrovascular embolic episodes. We attribute the aneurysm to congenital dysplasia of the musculi pectinati in the left atrial appendage and of the bands of atrial muscle from which they arise. In appendix I, we draw attention to the morphologically similar arrangements of inner and outer bands that emanate from a common transverse interatrial band and yield morphologically similar medial, descending, and ascending palm-leaf arrangements of musculi pectinati. In addition, we observe that the strap-like arrangements of musculi in both atria connect the outer band with the para-annular segment of the inner band. In appendix II, we briefly review the literature concerning musculi pectinati and related bands.- - - - - - - - - - ranking = 0.005237624011293keywords = dysplasia (Clic here for more details about this article) |
7/52. Hypertrophic cardiomyopathy with midventricular obstruction and apical aneurysm: a case report.A 71-year-old woman had hypertrophic cardiomyopathy associated with midventricular obstruction and an apical aneurysm in the left ventricle. She had had abnormal electrocardiograms for more than 30 years and for the past year had been suffering from occasional attacks of dizziness and low systemic blood pressure. Holter 24-h electrocardiographic monitoring revealed ventricular paroxysmal contractions (676/day) with nonsustained ventricular tachycardia. Doppler echocardiography revealed paradoxical jet flow from the apical aneurysm to the left ventricular outflow during early diastole. magnetic resonance imaging depicted midventricular hypertrophy and a dyskinetic thin apical wall, which were confirmed by angiography. Coronary angiograms showed no narrowing of the major extramural coronary arteries, but there was compression of aberrant coronary arteries apparently feeding the hypertrophic portion of the left ventricular wall. Stress thallium-201 myocardial imaging showed a persistent severe defect in the left ventricular apex. A hemodynamic study revealed low cardiac output and an intraventricular pressure gradient (approximately 90 mmHg) between the left ventricular apical high-pressure chamber and the subaortic low-pressure chamber. The present case represents a rare combination of hypertrophic cardiomyopathy, midventricular obstruction, and an apical aneurysm in an elderly woman. myocardial ischemia may have played an important role in the genesis of the apical aneurysm.- - - - - - - - - - ranking = 2.9214356398306keywords = cardiomyopathy (Clic here for more details about this article) |
8/52. prenatal diagnosis of ventricular aneurysm: a report of two cases and a review.Ventricular aneurysms have rarely been reported prenatally. Their prognosis is variable depending on factors such as early detection, their relative size in comparison to the ventricular cavity, growth on follow-up, and signs of cardiac failure. In view of the fact that it may be useful to report on additional cases in order to make available further information on aetiology, prognosis and neonatal management, we hereby report on two cases of ventricular aneurysm with good mid-term prognosis. One case of apical aneurysm of the left ventricle was associated with a muscular ventricular septal defect, the features of which are compatible with a fetal myocardial infarction and ventricular septal rupture in absence of coronary artery anomalies as demonstrated by catheterisation. Another case of sub-tricuspidal aneurysm of the right ventricle associated with mitral prolapse appears to be a component of diffuse connective dysplasia. Despite the early gestational age at diagnosis, the large size of the aneurysm and the associated defect, both the infants were asymptomatic in infancy prompting a conservative management.- - - - - - - - - - ranking = 0.005237624011293keywords = dysplasia (Clic here for more details about this article) |
9/52. Noncompaction of ventricular myocardium, complete atrioventricular block and minor congenital heart abnormalities: case report of an unusual coexistence.Ventricular noncompaction is a rare cardiomyopathy characterized by numerous, prominent ventricular trabeculations and deep intertrabecular recesses and is caused by a disorder of endomyocardial morphogenesis. It can occur in patients with other congenital anomalies, such as various ventricular obstructive lesions, or in isolated form in the absence of associated anomalies. patients with this unique disease may present with heart failure, systemic emboli, and ventricular arrhythmias. In this case report, we present an unusual coexistence of ventricular noncompaction and atrial septal aneurysm and mitral valve cleft in a young patient who presented with complete AV-block.- - - - - - - - - - ranking = 0.48690593997177keywords = cardiomyopathy (Clic here for more details about this article) |
10/52. Perforated aneurysms of left side valves during active infective endocarditis complicating hypertrophic obstructive cardiomyopathy.The most frequent site of vegetative lesion in patients with hypertrophic cardiomyopathy is anterior mitral leaflet, due to chronic endocardial trauma arising from systolic anterior motion. We describe three cases of serious infective endocarditis complicated lesions (vegetation, aneurysm and perforation) on aortic and mitral valves, in patients with obstructive hypertrophic cardiomyopathy. In particular, we observed how severe valvular damage and dysfunction, combined with particular hemodynamic conditions, are followed by adverse clinical outcome. We performed transthoracic echocardiogram and transoesophageal echocardiography studies to define morphologic and hemodynamic features of infection, deciding the proper therapy and we planned the echocardiographic follow-up.- - - - - - - - - - ranking = 2.9214356398306keywords = cardiomyopathy (Clic here for more details about this article) |
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