Double superior vena cava and left brachiocephalic vein agenesis: a rare systemic vein anomaly and potential source of cardiac implantable electronic device and central venous catheter placement complications.

Abnormal systemic vein development produces anomalous veins, which - in the case of persistent left superior vena cava and/or left brachiocephalic vein - exhibit considerable topographic and morphometric differences in comparison with their usual anatomy. The nature and extent of those developmental anomalies - detected during intravenous procedures, such as cardiac implantable electronic device (CIED) lead insertion or central venous catheter placement - may hinder the procedure itself and/or adversely affect its outcome, both at the stage of cardiac lead advancement through an abnormally shaped vessel and lead positioning within the heart. This may lead to problems in achieving optimal sensing and pacing parameters and in ensuring that the patient cannot feel the pacing impulses. These events accompanied a de novo CIED implantation procedure in the patient with a double superior vena cava and left brachiocephalic vein agenesis, who ultimately required reoperation.

Cardiac Stress in High-Risk Patients Undergoing Major Endovascular Surgery-Focus on Diastolic Function.

OBJECTIVES: The purpose of this study was to determine the relationship between the changes in diastolic function and their association with cardiac biomarkers in the perioperative period in patients undergoing complex endovascular aortic repair. DESIGN: Prospective observational study. SETTING: Single-center academic hospital, central teaching hospital in Warsaw, Poland. PARTICIPANTS: The study comprised 27 high-risk patients scheduled for elective endovascular repair of aortic aneurysm. INTERVENTIONS: Complex endovascular procedure using branched endograft of the thoracoabdominal aorta. Branches of the stent grafts included renal arteries, the superior mesenteric artery, and the celiac trunk. MEASUREMENTS AND MAIN RESULTS: The primary outcome was to evaluate changes in diastolic function parameters assessed with transthoracic echocardiography at two and 24 hours postoperatively. The major secondary outcomes were changes in N-terminal pro-B-type natriuretic peptide (NT-proBNP) and troponin I concentrations, systolic function parameters, hemodynamic parameters at two and 24 hours, length of hospital stay, and 30-day mortality. There was a reduction in e' wave velocity on both the septal and lateral sides at two hours compared with the baseline (p = 0.041 and p = 0.05, respectively). There was an increase in both NT-proBNP and troponin I concentrations after surgery (p = 0.002 and p = 0.034, respectively), with troponin I peaking two hours after surgery and NT-proBNP peaking 24 hours after surgery. CONCLUSIONS: Patients undergoing a branched endovascular aortic repair of a thoracoabdominal aortic aneurysm experience a cardiac insult that manifests with deterioration in diastolic parameters and concomitant increases of troponin and NT-proBNP concentrations. Additional large-scale prospective studies are required to confirm this phenomenon.

Circulating autoantibodies to troponin I in Emery-Dreifuss muscular dystrophy.

The pathogenesis of dilated cardiomyopathy in Emery- Dreifuss muscular dystrophy (EDMD) is still unknown. Autoimmune mechanisms have recently been taken into account. The aim of this investigation was to determine whether the level of circulating antibodies to heart proteins which were previously detected, correlates with disease progression. Troponin I was chosen as the target. Ten patients with EDMD and 10 age-matched normal controls were tested. An enzyme linked immunoassay (ELISA) technique was used to determine the possible relation between the level of anti-troponin I antibodies at diagnosis and at followup. Autoantibodies against troponin I were detected in all EDMD patients. At diagnosis the level was higher in the X-linked EDMD form (X-EDMD), as compared to the autosomal dominant form (AD-EDMD). At follow-up the elevated level of the autoantibodies persisted in all the EDMD cases. However, in the AD-EDMD form, the level was found to be significantly rising with disease progression, in the X-EDMD form, on the other hand, it was declining. No clear-cut relationship between the level of the circulating antibodies and cardiac symptomatology was present. Detection of anti-troponin I antibodies may provide a non-invasive marker of early stages of dilated cardiomyopathy in EDMD.

Evidence for autoimmunity to heart-specific antigens in patients with Emery-Dreifuss muscular dystrophy.

Dilated cardiomyopathy is one of the leading abnormalities in Emery-Dreifuss Muscular Dystrophy (EDMD). The pathogenesis of heart involvement in EDMD is, however, unknown. Autoimmune mechanisms have also to be taken into account. The aim of this study was to search for the presence of anti-heart antibodies in EDMD patients. The anti-heart auto-antibodies were detected in serum of 14 EDMD patients (the X-linked and the AD-Autosomal Dominant form). The control groups comprised 10 patients with Dilated Cardiomyopathy (DCM) and 10 healthy subjects. To screen serum for anti-heart antibodies against ventricular muscle proteins, they were separated by polyacrylamide gel electrophoresis, followed by Western blotting. In EDMD and DCM, IgG anti-heart antibodies against heart ventricular proteins were detected. In both diseases, 85 kD protein appeared to be the most immunogenic. Anti-troponin I (24 kD), anti-tropomyosin (35 kD) and anti-actin (43 kD) reactivity was less intense. There were significant differences in the reactivity of auto-antibodies between both EDMD forms, and also between EDMD and the DCM patients. No clear-cut correlation between the reactivity and frequency of the antibodies and clinical parameters of the EDMD patients was detected. The anti-heart proteins are reliable markers of immune involvement in dilated cardiomyopathy in the course of EDMD. Short- and long-term follow-up may define the role of anti-heart antibodies in predicting the susceptibility at risk of dilated cardiomyopathy in EDMD patients.

Endoscopic study of the morphology of Vieussens valve.

Vieussens valve (Vv) is the ostial valve of the great cardiac vein located near the beginning of the coronary sinus. Knowledge of its anatomy is important for several trans-sinus cardiologic procedures. The frequency of its presence is reported to vary from 65-87%. We documented the post mortem morphology of Vieussens valve in 50 unfixed, intact human coronary sinuses using endoscopy. We believe this is the first study of the anatomy of the coronary sinus and the adjacent venous ostia to employ this technique. Vieussens valve was observed in 78% of specimens. Special attention was given to the shape of the valve leaflets. Five morphological types of Vv were distinguished: single leaflet, flat (16%); single leaflet, concave (20%); double leaflet, flat (8%); double leaflet, concave (32%); and triple leaflet, concave (2%). We found post mortem endoscopy of the coronary sinus to be a good and reliable method of visualizing Vieussens valve.