Heart failure resulting from giant left atrial synovial sarcoma metastasis.

Synovial sarcoma metastasis affecting the heart and infiltrating the mitral valve is a very rare pathology. We report the case of a 44-year-old male treated with chemotherapy for atypical synovial sarcoma of the oral mucosa who presented to our clinic after cardiac decompensation with a presumptive diagnosis of myxoma of the left atrium. A large necrotic tumour positive for CK 22, EMA, CD 99 and BCL-2 but negative for translocation in COBRA-FISH analysis by break-apart probe could be excised and revealed a very rare subtype of synovial sarcoma metastasis arising from the endocard of the left atrium. The tumour was resected and the mitral valve reconstructed through ring annuloplasty.

[Thrombotic microangiopathy after extracorporeal circulation: important differential diagnosis]. / Thrombotische Mikroangiopathien nach extrakorporaler Zirkulation : Wichtige Differenzialdiagnose.

Thrombotic microangiopathies are characterized by platelet activation, endothelial damage, hemolysis and microvascular occlusion. This group of diseases is primary represented by thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Patients present with microangiopathic hemolytic anemia and thrombocytopenia as well as occlusion-related organ ischemia to a variable degree. A deficiency of the metalloprotease ADAMTS-13 is a major risk for acute disease manifestation as this is a regulator of unusually large von Willebrand factor (vWF) multimers, which are extremely adhesive and secreted by endothelial cells. In classical TTP an ADAMTS-13 activity below 5% is specific, whereas in other forms of thrombotic microangiopathies activity of ADAMTS-13 ranges from very low to normal. Symptoms of different forms of thrombotic microangiopathy are frequently overlapping and a clear classification according to clinical criteria is often difficult. Due to a high mortality, particularly of TTP, immediate diagnosis and therapy are essential. In this article two cases of thombotic microangiopathy after cardiac surgery are reported. After exclusion of TTP and HUS as well as other etiologies of thrombotic microangiopathy a relationship between the use of extracorporeal circulation and the pathogenesis of thrombotic microangiopathy is assumed.

Clinical outcome after composite grafting of calcified left anterior descending arteries.

AIM: The treatment of a severely calcified left anterior descending coronary artery (LAD) remains a challenge in cardiac surgery. The objective of this retrospective study was to assess the early clinical outcome obtained from LAD reconstruction, which was performed using a combination of both, saphenous vein and the left internal thoracic artery (LITA) as a composite graft, in order to achieve complete revascularization. METHODS: Between January 1998 and December 2003, 71 patients, 13 female and 58 male, with a mean age of 67+/-9.12 years were retrospectively analyzed. All patients suffered from a severe 3-vessel disease with a diffusely calcified LAD. The design of the reconstruction consisted in a long arteriotomy of the LAD grafted with a matched segment of the saphenous vein using the plaque exclusion technique to avoid endarterectomy. The LITA was then anastomosed to the saphenous vein in an end-to-end configuration. With this design, the saphenous vein was used first as a ''patch'' reconstruction of the LAD and second as an elongation for the LITA. The clinical outcome was assessed by mailed questionnaires or by telephone interview with the responsible cardiologist or general practitioner. RESULTS: The follow-up was 100%, comprising a mean follow-up time of 17+/-11.8 months. Overall mortality was 7% (N=5/71). Four cardiac deaths and 1 non cardiac-related death were registered. The in-hospital mortality was 2.8% (2/71); 2 cardiac-related deaths and one non cardiac-related death were observed after 30 days (4.2%). Postoperative myocardial infarction without heart failure was seen in 4 patients (5.4%). In addition, an episode of transitory cerebral ischemia was observed in 1 patient (1.4%). No further postoperative complications occurred. At the time of evaluation, 67% of the patients were in functional class CCS 0 and 33% in functional class CCS I to II. CONCLUSIONS: Composite graft reconstruction without endarterectomy is an alternative treatment option for severely calcified LADs with a good early clinical outcome.

Molecular differentiation of ischemic and valvular heart disease by liquid chromatography/fourier transform ion cyclotron resonance mass spectrometry.

Proteomic patterns of myocardial tissue in different etiologies of heart failure were investigated using a direct analytical approach with High Performance Liquid Chromatography (HPLC)/Fourier Transform Ion Cyclotron Resonance Mass Spectrometry (FT-ICR MS). Right atrial appendages from 20 patients, 10 with hemodynamically significant isolated aortic valve disease and 10 with symptomatic coronary artery disease were collected during elective cardiac surgery. After preparation of tissue samples and tryptic digestion of proteins, the peptide mixture was HPLC-separated and on-line analyzed by electrospray FT-ICR MS. Data obtained from HPLC / FT-ICR MS runs were compared for classification. To extract the classification features, the selection of best individual features was applied and the "nearest mean classifier" was used for the classification of test samples and the sample projection onto classification patterns. The pattern distribution characteristics of aortic and coronary diseases were clearly different. No interference between samples of both disease categories was registered, even if the distribution of unsupervised classified test samples were closer. Samples representing aortic valve disease showed a closer accumulation pattern of spots compared to the samples representing coronary disease, which indicated a more specific protein classification. Through selective identification of specific peptides and protein patterns with FTMS, valvular and coronary heart disease is for the first time clearly distinguished at molecular level. The described methodology could also be feasible in search for specific biomarkers in plasma or serum for diagnostic purposes.

[Current evidence-based situation in coronary revascularization--CABG vs. PCI and diabetes?]. / Aktuelle evidenzbasierte Situation in der Koronarrevaskularisation--CABG vs. PCI und Diabetes?

A variety of randomized, controlled trials comparing percutaneous coronary intervention (PCI) with coronary artery bypass grafting (CABG) found similar results in mortality but significant differences in number of reinterventions in favor of CABG. This work gives an overview about the relevance and limitations of these studies in line with newly published large scale observational studies, which reveal significantly lower mortality-rates in CABG patients. Emphasis is placed on the special situation in the diabetic patient.

Protecting the endothelial integrity of internal thoracic arteries.

BACKGROUND: Previous functional studies on human internal thoracic arteries, comparing the effect of the traditional harvesting method (occlusion with a clip) with a method leaving the artery perfused, revealed considerably impaired endothelial function associated with enhanced contractility after clipping. We have now investigated whether these observations could be correlated (1) with plasma markers of endothelial dysfunction, and (2) with structural changes in the endothelial layer. METHODS: 32 patients were randomly distributed into groups of clipped and perfused arteries. Arterial blood samples were obtained from both the artery and extracorporeal circulation to determine sP-selectin, sE-selectin, sL-selectin, and thrombomodulin using enzyme-linked immunosorbent assay. Arteries from three patients were examined by scanning electron microscopy. RESULTS: Concentrations of sP-selectin and thrombomodulin were significantly higher in plasma from clipped arteries compared to perfused arteries, whereas sE-selectin and sL-selectin concentrations were similar within the groups. Scanning electron microscopy revealed significant structural changes and loss of endothelial cells in clipped arteries. CONCLUSION: Biochemical and structural results support our findings that leaving the internal thoracic artery perfused preserves endothelial function in the arterial graft.

[Ambulatory long-term management of a left ventricular assist device. Current modality in terminal heart failure]. / Ambulante Langzeitbetreuung mit einem Linksherzunterstützungssystem. Eine zeitgemässe Modalität in der Behandlung der terminalen Herzinsuffizienz.

We report about the first use and successful outpatient therapy with an implantable pulsatile left ventricular assist device (LVAD, Novacor) in a patient with a combined dilative and ischemic cardiomyopathy in Switzerland. The patient, a 51 year old man (112 kg, 191 cm, blood type A) developed end-stage heart failure (New York Heart Association class (NYHA) IV) while he was awaiting orthotopic heart transplantation. Due to his profession as an independent graphic-designer the patient was afraid of a long-term temporary disablement with consecutive bankruptcy. Therefore we decided and performed the implantation of a Novacor-LVAD as a bridge to transplantation with the possibility to outpatient therapy in a favourable course. The patient was supported by the device for more than five months. His cardiac functional status returned to NYHA class I, and the patient was discharged 5 weeks after implantation. He returned into his normal life and started working at 100% again. Furthermore the LVAD enabled the patient to participate in almost all activities. Five months (151 days) after implantation a donor organ became available and the patient underwent orthotopic heart transplantation. The use and successful outpatient therapy with an implantable pulsatile left ventricular assist device in our patient has proved to be save, reliable, life-saving, quality of life improving and could be an important alternative for the economic burden in heart failure therapy.

FK506 does not affect cardiac contractility and adrenergic response in vitro.

FK506 (tacrolimus) is a new immunosuppressant being used in cardiac allograft transplantation. While cyclosporine A has been shown to exert an acute negative inotropic effect on isolated heart muscle preparations, little is known of the inotropic influence of FK506. The Ca(2+) release channel of human skeletal muscle and cardiac muscle is associated with FK506 binding proteins (FKBP), FKBP12 and FKBP12.6, respectively. FKBPs can be dissociated by treatment with FK506. As a consequence of FK506 exposure, isolated skeletal muscle and cardiac muscle ryanodine receptors show altered gating characteristics. Therefore, we analyzed the direct inotropic effect of FK506 exposure to isolated, intact heart muscle preparations from the human and rabbits. Experiments were performed on isolated, electrically stimulated right atrial auricular muscle strips obtained from human myocardium during elective open heart surgery and on intact right ventricular trabeculae from rabbit hearts. The human preparations were exposed to concentrations of 8 x 10(-9), 8 x 10(-8) and 8 x 10(-6) M FK506 followed by a cumulative dose-response curve with isoprenaline as a non-selective beta-adrenoceptor agonist. Our data suggest that FK506 does not exert any positive or negative inotropic effect in either human or rabbit myocardium.

[Reverse remodeling by surgery--fact or fiction?]. / "Reverse remodeling" durch Chirurgie induzierbar?

Mortality of chronic heart failure in industrial countries remains unacceptably high despite advances in medical therapy. Heart transplantation, the gold standard in the treatment of end-stage heart failure is reserved for only a few patients because of the shortage of donor hearts. Surgical alternatives to transplantation include dynamic cardiomyoplasty (CMP), mitral valve reconstruction, left ventricular reduction surgery (PLVR) and ventricular assist devices (VAD). Improved survival and objective physiologic improvement have not been documented for CMP in the treatment of dilative cardiomyopathy. Mitral valve reconstruction on the other hand shows promising results. PLVR is an innovative procedure in which the heart is surgically reduced in size and cardiac function is dramatically improved immediately after surgery. The presence of long-term effects is still unknown. VAD have been shown to be extremely effective as a short- and long-term "bridge" to heart transplantation. They are not approved for permanent support. A randomized trial in the U.S. is underway to compare the efficacy of these devices with the efficacy of medical therapy in NYHA functional class IV patients in quality of life, survival and costs.

FP-receptor mediated trophic effects of prostanoids in rat ventricular cardiomyocytes.

The aim of this study was to characterize the receptor subtype involved in cardiac effects of prostanoids. For this purpose we determined in neonatal and adult rat cardiomyocytes effects of prostanoids on inositol phosphate (InsP)-formation (assessed as accumulation of total [(3)H]-InsP's in myo-[(3)H]-inositol pre-labelled cells) and on rate of protein synthesis (assessed as [(3)H]-phenylalanine incorporation), and on contractile force in left ventricular strips of the rat heart. For comparison, effects of prostanoids on InsP-formation and contractile force were determined in rat thoracic aorta, a classical TP-receptor containing tissue. Prostanoid increased InsP-formation and rate of protein synthesis in neonatal as well as adult rat cardiomyocytes; the order of potency was in neonatal (PGF(2alpha)>PGD(2)> or =PGE(2)> or =U 46619>PGE(1)) and adult (PGF(2alpha)>PGD(2)> or =PGE(2)>U 46619) rat cardiomyocytes well comparable. Moreover, in electrically driven left ventricular strips PGF(2alpha) caused positive inotropic effects (pD(2) 7.5) whereas U 46619 (up to 1 microM) was uneffective. In contrast, in rat thoracic aorta U 46619 was about 100 times more potent than PGF(2alpha) in increasing InsP-formation and contractile force. The TP-receptor antagonist SQ 29548 only weakly antagonized prostanoid-induced increases in rate of protein synthesis (pK(B) about 6) in rat cardiomyocytes but was very potent (pK(B) about 8-9) in antagonizing prostanoid-induced increases in InsP-formation and contractile force in rat aorta. We conclude that, in cardiomyocytes of neonatal and adult rats, the prostanoid-receptor mediating increases in InsP-formation and rate of protein synthesis is a FP-receptor. Moreover, stimulation of these cardiac FP-receptors can mediate increases in contractile force.

Aberrant right pulmonary artery and double outlet ventricle: one-stage repair.

We report a 13-month-old male child with anomalous origin of the right pulmonary artery from the ascending aorta and a double outlet right ventricle. Aortic wall was used for elongation of the pulmonary artery and side-to-end connection to the pulmonary trunk. Special emphasis is made on this particular operative technique for strictly laterally originating right pulmonary artery that requires no prosthetic material, avoids extreme stretching, and may enable normal growth potential.

[Reverse remodeling by surgery - fact or fiction?]. / "Reverse remodeling" durch Chirurgie induzierbar?

Mortality of chronic heart failure in industrial countries remains unacceptably high despite advances in medical therapy. Heart transplantation, the gold standart in the treatment of end-stage heart failure is reserved for only a few patients because of the shortage of donor hearts. Surgical alternatives to transplantation include dynamic cardiomyoplasty (CMP), mitral valve reconstruction, left ventricular reduction surgery (PLVR) and ventricular assist devices (VAD). Improved survival and objective physiologic improvement have not been documented for CMP in the treatment of dilative cardiomyopathy. Mitral valve reconstruction of the other hand shows promising results. PLVR is an innovative procedure in which the heart is surgically reduced in size and cardiac function is dramatically improved immediately after surgery. The presence of long-term effects is still unknown. VAD have been shown to be extremely effective as a short- and long-term "bridge" to heart transplantation. They are not approved for permanent support. A randomized trial in the U. S. is underway to compare the efficacy of these devices with the efficacy of medical therapy in NYHA functional class IV patients in quality of life, survival and costs.

Effects of diagnostic application of monoclonal antibody on survival in melanoma patients.

Follow-up data of more than 300 melanoma patients were reviewed in which an immunoscintigraphy study had been performed as part of a routine staging protocol. All patients had received a commercially available 99mTc-labeled F(ab')2 fragment specific to a high-molecular-weight melanoma-associated antigen (HMW-MAA). Survival data of patients that had received one injection (group I) were compared with those receiving two or more injections (group II). For better comparison, patients were matched according to the following risk factors: (i) age, (ii) sex, (iii) tumor level according to Clark, (iv) tumor depth according to Breslow, (v) location of primary tumor, and (vi) metastases [and if positive, (vii) location of metastases]. Twenty-eight patient pairs were established that showed no statistically significant differences comparing the above-mentioned risk factors. In addition, no significant difference was found comparing the interval between primary diagnosis of disease and first injection of monoclonal antibody (MAb) in both groups. In contrast, survival time between first diagnosis of melanoma was significantly prolonged in group II (multiple injections) as compared to group I (single injection). This was true for survival time between primary diagnosis as well as from first injection of MAb until death or closing time of study. Our data suggest, that multiple injections of melanoma-specific F(ab')2 fragment have a beneficial effect on median survival in melanoma patients.