The First Report of Calcified Amorphous Tumor Associated with Infective Endocarditis: A Case Report and Review of Literature.

BACKGROUND Calcified amorphous tumor (CAT) of the heart is a rare non-neoplastic intracardiac mass, which is composed of calcium deposition surrounded by amorphous fibrous tissue. The clinical presentation of cardiac CAT resembles that of other cardiac tumors or vegetation, though there is no previous report of a CAT complicated with infective endocarditis. CASE REPORT A 67-year-old male with a history of end stage renal failure and gastric cancer who was on adjuvant chemotherapy presented with a cardiac mass. The mass was resected and diagnosed as CAT pathologically. Two separate sets of blood cultures were positive for Enterococcus faecalis, thus, the patient was diagnosed with infective endocarditis. Antibiotic treatment was continued for 6 weeks after surgery, and the patient recovered uneventfully. However, he died from a complication of his gastric cancer 5 months later. CONCLUSIONS This is the first report of CAT associated with infective endocarditis. Blood cultures should be obtained to differentiate infective endocarditis or CAT with infectious endocarditis from CAT alone, because CAT with infective endocarditis may present atypically and may be more likely to require antibiotic treatment along with surgery.

Complete response of hepatocellular carcinoma with right atrium and pulmonary metastases treated by combined treatments (a possible treatment effect of natural killer cell): A case report and literature review.

RATIONALE: Hepatocellular carcinomas (HCCs) with metastases to the right atrium (RA) and lungs are rare, with a poor prognosis. Furthermore, the treatment outcomes in patients with advanced HCCs remain unsatisfactory. PATIENT CONCERNS: A 46-year-old man presented to our hospital for dyspnea on exertion and abdominal pain. DIAGNOSES: HCC and extra-hepatic metastases to the lung and RA. INTERVENTIONS: Multidisciplinary treatment including radiotherapy (RT), transarterial chemoembolization (TACE), and sorafenib. During a follow-up evaluation computed tomography, he experienced a radio-contrast-induced anaphylaxis. After the event, treatment such as RT, TACE, and sorafenib were continued. OUTCOMES: His tumor burden decreased, finally leading to a complete response as per the modified Response Evaluation Criteria in Solid Tumors. The patient is still alive, 30 months after the episode. Subsequent blood tests showed increased natural killer (NK) cell activity, which was significantly higher than that seen in other age-matched HCC patients with an identical stage of the tumor, receiving sorafenib. This suggests that the increase in NK cells induced by anaphylaxis influenced the tumor burden. LESSONS: We report here a rare case of long-term survival of an HCC patient with multiple metastases treated with multidisciplinary modalities, in which high NK cell activity was observed after a radio-contrast-induced anaphylactic reaction during follow-up investigations.

Thrombotic thrombocytopenic purpura and cardiac papillary fibroelastoma: a 'unique coexistence'.

Thrombotic thrombocytopenic purpura (TTP), a complex thrombotic microangiopathy, remains an evolving enigma. A 49-year-old African-American woman presented with acute left hemiplegia, an ischemic cerebrovascular accident involving the right middle cerebral artery. Sequential appearance of thrombocytopenia and evidence of microangiopathic haemolysis led to the diagnosis of acquired idiopathic autoimmune TTP. This was managed with plasma exchange (PEX) and steroids. Early haematologic relapse within a month was managed with the addition of rituximab attaining sustained remission. The patient presented 3 years later with acute confusion and expressive aphasia due to multiple infarcts involving the left parieto-occipital cortex. Transoesophageal echocardiography demonstrated a pedunculated 6 mm mitral valvular mass consistent with a papillary fibroelastoma. Anticoagulation was instituted and the patient was continued on therapeutic oral warfarin. A haematologic relapse of TTP eventually emerged and was managed with PEX, steroids and rituximab. This vignette demonstrates several dilemmas in the clinical presentation, diagnosis and management of TTP in current day practice. Rituximab has adjuvant benefits to PEX and is being investigated as potential first-line therapy. Monitoring ADAMTS13 activity and inhibitor titre, as in our case, prove to have prognostic significance. Cardiac fibroelastomas are rare benign cardiac tumours usually arising from valvular endocardium with thromboembolic potential. One of the proposed mechanisms of origin of these masses is organizing thrombi in the setting of endocardial injury and inflammation questioning a possible link to thrombotic microangiopathy. To the best of our knowledge, this is the first report of this unique coexistence.

Left ventricular cardiac fibroma in a child presenting with ventricular tachycardia.

Cardiac tumors in children are rare. Although most are histologically benign, they can be associated with life-threatening arrhythmias and sudden death. We report a 7-year-old boy, with a first episode of symptomatic tachycardia, who was found to have a left ventricular (LV) fibroma. He had a normal echocardiogram prior to an electrophysiology study, which revealed a sustained monomorphic ventricular tachycardia and a radio-opacity near the LV apex. These findings prompted a cardiac MRI, which demonstrated a discrete mass on his LV apex and free wall. Our case emphasizes that structural heart disease should be aggressively pursued in children presenting with ventricular tachycardia.

The diverse clinical manifestations of tuberous sclerosis complex: a review.

Tuberous sclerosis complex (TSC) is an autosomal dominant multisystem neurocutaneous disorder. TSC results in hamartomatous lesions primarily involving the skin, central nervous system, kidneys, eyes, heart, and lungs. The clinical findings and severity of TSC are highly variable. Recent advances in our understanding of the complexities of the TSC1 and TSC2 genes are making genotype-phenotype correlations possible. While managing seizures, cognitive dysfunction, and behavioral abnormalities are the primary responsibility of the neurologist, familiarity with all aspects of this disease helps provide better comprehensive care for affected individuals.

Evaluation of the effects of intramyocardial injection of DNA expressing vascular endothelial growth factor (VEGF) in a myocardial infarction model in the rat--angiogenesis and angioma formation.

OBJECTIVES: The effects of direct intramyocardial injection of the plasmid encoding vascular endothelial growth factor (phVEGF165) in the border zone of myocardial infarct tissue in rat hearts were investigated. BACKGROUND: Controversy exists concerning the ability of VEGF to induce angiogenesis and enhance coronary flow in the myocardium. METHODS: Sprague-Dawley rats received a ligation of the left coronary artery to induce myocardial infarction (MI). At 33.1 +/- 6.5 days, the rats were injected with phVEGF165 at one location and control plasmid at a second location (500 microg DNA, n = 24) or saline (n = 16). After 33.1 +/- 5.7 days, the hearts were excised for macroscopic and histologic analysis. Regional blood flow ratios were measured in 18 rats by radioactive microspheres. RESULTS: phVEGF165-treated sites showed macroscopic angioma-like structures at the injection site while control DNA and saline injection sites did not. By histology, 21/24 phVEGF165-treated hearts showed increased focal epicardial blood vessel density and angioma-like formation. Quantitative morphometric evaluation in 20 phVEGF165-treated hearts revealed 44.4 +/- 10.5 vascular structures per field in phVEGF165-treated hearts versus 21.4 +/- 4.7 in control DNA injection sites (p < 0.05). Regional myocardial blood flow ratios between the injection site and noninfarcted area did not demonstrate any difference between phVEGF,165-treated hearts (0.9 +/- 0.2) and saline-treated hearts (0.7 +/- 0.1). CONCLUSIONS: Injection of DNA for VEGF in the border zone of MI in rat hearts induced angiogenesis. Angioma formation at the injection sites did not appear to contribute to regional myocardial blood flow, which may be a limitation of gene therapy for this application.

Cardiopulmonary bypass and cell-saver technique in combined oncologic and cardiovascular surgery.

The purpose of this study was to work out an adequate operative technique for patients with malignant tumors who also need open heart surgery or procedures on major blood vessels. We had 8 such patients. In 6 of them, a tumor (3 cases hypernephroid cancer and 3 cases retroperitoneal sarcoma) had grown through the inferior vena cava (IVC) up to the right atrium. Two patients had lung cancer together with severe coronary artery disease. All of these patients were operated on using a heart-lung machine (HLM) and cell saver (CS). In 6 patients the intravascular portion of the tumor was extracted as much as possible through a right atrium approach (in 3 cases a nephrectomy was performed). Two patients had a one-stage coronary artery bypass graft (CABG) and a lobectomy. All of the patients had uneventful postoperative periods and were alive when checked on 1 year after the procedures. During cytological investigation after each operation, tumor cells were found only on the internal surface of the HLM arterial filters with 20 microns holes. We suggest that special cardiovascular devices such as the HLM and CS might be used in borderline situations in oncology without increasing the risk of hematogenous tumor dissemination.

Clofilium-induced block of delayed rectifier type K+ current in atrial tumor cells (AT-1 cells).

Atrial tumor myocytes derived from transgenic mice (AT-1 cells) have been shown to express mRNAs encoding cardiac K+ channels and display a cardiac electrophysiological phenotype. The major K+ current is the rapid component of the delayed rectifier (I(kr)). The purpose of the study was to characterize the mode of action of a class III anti-arrhythmic agent (C3A), clofilium, in these cells to elucidate further the mechanism and functional consequence of block. We show that clofilium blocks this I(kr) in a dose-dependent manner with IC50 of 1.25 microM. Clofilium induced block was irreversible for higher concentrations (> or = 50 microM) and partially reversible for lower doses. The reversal potential for this current was -77.5+/-1.5 mV. The block is voltage dependent and the drug probably binds the channel in the open state.

[Transformation of skeletal muscle by progressive sequential stimulation with a view toward its use as a myocardial substitute]. / Transformation d'un muscle squelettique par stimulation séquentielle progressive en vue de son utilisation comme substitut myocardique.

Progressive sequential stimulation of a skeletal muscle using trains of 30 Hz impulses with increasing frequencies from 20/min. to 80/min. within 3 months, allowed us to obtain in goats a transformation of the fast twitch glycolytic muscular fibers into fatigue resistant slow twitch oxidative muscular fibers. The conditioned muscle can be used in the treatment of various myocardial lesions or to reinforce cardiac contractility in severe cardiac insufficiencies. The first clinical case successfully operated upon is reported.

Primary and secondary heart tumors in mice maintained on various diets.

Two mice of the TM strain, maintained on Purina chow supplemented with refined coin oil plus free fatty acids, in one case, and with refined corn oil without free fatty acids, in the other case, developed primary heart rhabdomyosarcomas. The tumors metastasized to the lungs and the kidney, in the first case, and to the lungs alone in the second case. One mouse of the TM strain, on the diet supplemented with corn oil plus free fatty acids, developed a fibrosarcoma behind the right anterior extremity. Metastases from the tumor were found in the liver, lungs and myocardium. Another mouse of the C57 Bl. strain, injected subcutaneously, in the interscapular region, at the age of 7 days, with a single dose of 30 mug of Delestrogen, developed, at the point of injection, a large rhabdomyosarcoma. At the age of 5333 days, when the animal was killed, metastases from the tumor were found in the intercostal muscles and in the heart. Three mice, one of the TM strain, on a diet supplemented with corn oil and cholesterol, and two of the C57 Br. strain, on a diet supplemented with raw egg yolk (rich in cholesterol) had organized blood clots in the atria (in two mice) and one extending into the ventricle.