Cases reported "Cardiomyopathies"

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1/21. Echocardiographic evidence of atrial myopathy in amyloidosis: a case report.

    Cardiac involvement occurs in up to 50% of patients with primary amyloidosis. Diffuse amyloid deposits lead to impairment of myocardial systolic and diastolic function. Due to the severe left ventricular diastolic abnormality, left atrial contribution to left ventricular stroke volume remains critical. We report a case of primary amyloidosis where we assessed non-invasively left atrial systolic function.
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2/21. Giant hydatid cyst in the interventricular septum of a pregnant woman.

    A 25-year-old woman, pregnant for 38 weeks, was admitted to our clinic with dyspnea. Transthoracic echocardiography revealed a large cyst that originated from the left side of the interventricular septum, decreasing left ventricular volume and almost entirely obstructing the left ventricular outflow tract. Cardiac magnetic resonance imaging confirmed a grade 1 hydatid cyst, which measured 61 x 59 x 66 mm. The cyst was excised after cesarean section. Extirpation required the creation of a small septal defect, which we closed without a patch, by suturing the septum directly to the left ventricular wall. The patient was discharged without symptoms. The case reported here is of particular interest not only because a hydatid cyst is rarely seen in the interventricular septum, but because a giant hydatid cyst obstructing the left ventricular outflow tract is very rare in pregnancy.
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3/21. Normalization of left ventricular parameters following combined pimobendan and carvedilol treatment in a case of unclassified cardiomyopathy with longstanding refractory status.

    A 58-year-old man with a 64-month history of unclassified cardiomyopathy developed congestive heart failure (CHF) and had been dependent on long-term intravenous positive inotropes. Combined pimobendan and carvedilol administration resulted in marked symptomatic improvement from new york Heart association functional class IV to I. Echocardiograms showed improvement of left ventricular (LV) ejection fraction from 15 to 48%, and LV end-diastolic diameter from 6.7 to 4.9 cm. This mode of therapy not only improved LV contractile function but also normalized LV volume, which was an unusual clinical course compared with the general course of advanced CHF.
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4/21. Transient increase in wall thickness of the left ventricular apex during recovery from "ampulla" cardiomyopathy.

    "Ampulla" cardiomyopathy is a syndrome characterized by transient abnormal left ventricular wall motion with hypokinesia around the apical area and hyperkinesia at the basal area, without any detectable coronary lesion. Two cases of transient wall thickening of the left ventricular apex during recovery from "ampulla" cardiomyopathy are described. Apical wall thickening was documented by left ventriculography, echocardiography, and thallium (201Tl) single-photon emission computed tomography (SPECT) during the recovery phase. The thickness of the apical wall subsequently returned to normal. Both patients underwent provocation tests. Coronary spasms were positive. This transient increase in left ventricular apical volume may have been caused by myocardial inflammation secondary to "ampulla" cardiomyopathy.
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5/21. Successful use of levosimendan in a patient with peripartum cardiomyopathy.

    This case represents the first reported use of levosimendan, a calcium-sensitizing drug with additional vasodilation properties, for treatment of a woman with peripartum cardiomyopathy. Levosimendan induced a steady decline of increased pulmonary capillary wedge pressure, followed by a definitive increase in cardiac stroke volume. The patient recovered from this serious episode of heart failure, and she ultimately regained left ventricular function. IMPLICATIONS: Acute heart failure is a life-threatening event that only rarely occurs during childbirth. We report a case of a woman who experienced cardiovascular collapse during vaginal delivery. We determined that she met current diagnostic criteria for peripartum cardiomyopathy, and we successfully treated her with levosimendan, a calcium-sensitizing drug with additional vasodilation properties.
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6/21. A puzzling intrauterine death: non-compaction of the fetal ventricular myocardium presenting with reversed end-diastolic flow velocity in the umbilical arteries.

    BACKGROUND: Absent or reversed end-diastolic flow velocity (ARED) in the umbilical arteries is typically associated with severe intrauterine growth restriction (IUGR), although there may be other causes, such as aneuploidy. CASE: A healthy 30-year-old gravida 2, para 0 woman with an uneventful pregnancy and normal prenatal screening test results presented with sudden cessation of fetal movement at 26 weeks' gestation. Ultrasound demonstrated unexplained ARED with normal fetal growth and normal amniotic fluid volume. The fetus died during transfer to the labour and delivery unit. The cause of fetal demise was initially assumed to be feto-maternal hemorrhage, but postmortem examination revealed non-compaction of the ventricular myocardium. CONCLUSION: Intrinsic myocardial disease should be considered in the differential diagnosis when ARED is discovered in the umbilical arteries, especially when fetal growth is normal. Postmortem examination should be performed even if a prior 18- to 20-week anatomical ultrasound has been reported as normal.
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7/21. AL cardiac amyloidosis and arterial thromboembolic events.

    OBJECTIVE: To study the prevalence and characteristics of arterial thromboembolic events (ATEE) in the course of AL amyloidosis. methods: We report the case of a non-anticoagulated patient with AL amyloidosis restrictive cardiomyopathy who developed acute lower limb ischaemia. We then prospectively determined the prevalence of ATEE in all patients with AL amyloidosis who were evaluated in our institution for autologous peripheral stem cell transplantation. RESULTS: Nine out of 15 non-anticoagulated patients (60%) developed ATEE: ischaemic stroke (3), transient cerebral ischaemic attack (2), multiple peripheral arterial emboli (1), bilateral iliac artery thrombosis (1), bilateral optic nerve ischaemia (1), and mesenteric ischaemia (1). Haemodynamic stasis seemed to play a leading role in the pathophysiology of ATEE, in that all patients were on sinus rhythm and only one had a thrombus on echocardiography. We identified possible contributing factors to ATEE occurrence: concomitant treatments with oestroprogestogen regimen, thalidomide, granulocyte-macrophage colony-stimulating factor (GM-CSF) and extracellular volume disturbances related to the cytapheresis procedure. CONCLUSION: We report on an unusual frequency of ATEE among patients with AL cardiac amyloidosis. Despite its theoretical risks, anticoagulation should be discussed for patients with amyloid cardiomyopathy.
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8/21. Ultrastructural abnormalities of mitochondria and deficiency of myocardial cytochrome c oxidase in a patient with ventricular tachycardia.

    A 30-year-old woman presented with life-threatening ventricular tachycardia without overt heart disease. Ultrastructural investigation of endomyocardial biopsy disclosed abnormally structured and often enlarged mitochondria. Morphometry revealed the ratio of volume density of mitochondria to myofibrils to be markedly increased to 0.667 as compared with five controls (mean: 0.46; range: 0.445-0.479). Investigation of mitochondrial respiratory chain enzymes revealed a 90% reduction in activity of cytochrome c oxidase. Our data suggest that mitochondrial cardiomyopathy may induce malignant ventricular arrhythmias.
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9/21. rupture of the heart in a burn patient: a case report of free wall rupture of the left ventricle.

    A 63-year-old male was burned when in a state of alcoholic intoxication, he fell head first into a bathtub filled with hot water and received burns covering 40 per cent BSA. Despite surgical wound debridement, a severe sepsis continued and a sudden cardiac arrest occurred. autopsy revealed a large volume of blood in the pericardial space. The heart was ruptured through a myocardial abscess in the posterior wall of the left ventricle. The principle cause of his death was attributed to acute cardiac tamponade following cardiac rupture through a myocardial abscess.
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10/21. MR imaging of apical hypertrophic cardiomyopathy with left ventricular endomyocardial calcification.

    A rare case of apical hypertrophic cardiomyopathy with massive left ventricular endomyocardial calcification was studied with MR. The extent of the hypertrophy was clearly demonstrated, enabling accurate estimations of myocardial mass and volumes. Furthermore, an apical calcification was found and its volume estimated. In this case, MR gave the same information that was available by echocardiography, CT, and cardiac catheterization without the disadvantages of these methods.
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