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Background Sirolimus constitutes a safe and effective treatment for cardiac manifestations of tuberous sclerosis complex (TSC) in children but only four cases describing prenatal treatment of rhabdomyomas with mTOR inhibitors have been published. Case In this case, sirolimus was initiated at 26 weeks´ gestation in a pregnant woman with TSC with a fetus with a large rabdomyoma conditioning severe arrythmia. There was a significant reduction in the tumor size with ongoing treatment and a partial reversion of the arrythmia. Conclusion m-TOR inhibitors can be considered for severe cases of fetal rhabdomyomas with poor prognosis given its potencial benefits.

Objectives to develop appropriate tools to assess midwives' attitudes and behaviour in relation to decision making involving risk. Design a questionnaire and series of vignettes were developed and testes to explore midwives' intrapartum decision making in relation to their attitudes towards risk. An innovative online computer package was developed specifically for use in the STORK Study which enabled the programme to be very tightly controlled with limited functions accessible to participants. Testing a pilot study was conducted with over 50 midwives and nurses to ensure face and content validity of the vignettes and questionnaire. Initially designed to be a paper‐based study, rigorous piloting highlighted the many difficulties in presenting it in that particular format. The solution to this problem was to develop the study as a secure online package. Findings online data collection provided the researchers with a greater degree of control of the data collection process, not achievable using traditional paper survey methods. Another example of this control is the immediate entry of data from participants' responses to a background database which automatically stores and backs up data this means that no additional time is required for data entry. The cost of employing an information technology professional was easily offset by the financial savings made through the limited use of stationery and postage. Key conclusions although the development and testing of the research tools for the STORK Study was labour and time intensive, ultimately a questionnaire and vignette package was produced that had been rigorously tested by over 50 midwives and nurses. The researchers are confident in the reliability of the questionnaire and vignettes, as well as the validity of the data collected. The use of an online survey is clearly indicated when the population has readily available internet access, and where controlling the process of data collection is required, as such control cannot be achieved in traditional survey and questionnaire implementation.

to explore midwives' intrapartum referral decisions in relation to their dispositional attitude towards risk.a web-based correlation study examined the association between midwife's personality (personal risk tendency), place of work (location), years of experience and the timing of their decisions to make referrals (referral score) in a series of fictitious case scenarios (vignettes).102 midwives providing labour care in both consultant-led units (CLU) and community maternity units (CMU) from four Scottish health board areas.a correlational analysis was carried out to test the association between total risk scores and midwives' referral scores. Between-group comparisons were also conducted for experienced vs inexperienced midwives, midwives practising in CLU vs CMU settings and between the four health board areas.despite being presented with identical information in the vignettes, midwives made a wide range of referral decisions. There was no association between referral scores and measures of risk, personality or years of experience. No statistically significant difference between the referral scores of midwives working in CLUs or CMUs was observed. However, a statistically significant difference did emerge between the four health board areas, with midwives from one area making referrals at a significantly earlier stage. The maternity services in this area had experienced several high profile adverse events prior to this study; this may have influenced their referral behaviour (the availability heuristic), in terms of making more cautious decisions.there was no evidence that variability in the range of referral decisions was due to personality factors, risk propensity, experience or whether the midwife worked in a CLU or CMU. Local factors such as recent adverse events may significantly influence subsequent referral behaviour. Further research is required to identify why the midwives showed so much unexplained variability in their responses to the vignettes.

Traumatic experiences associated with cardiac surgery (CS) can result in traumatic memories and posttraumatic stress disorder (PTSD). Because it is known that subjects who develop PTSD often show sustained reductions in circulating cortisol concentrations, we performed a prospective, randomized study to examine whether exogenously administered stress doses of hydrocortisone during the perioperative period of CS reduces the long-term incidence of chronic stress and PTSD symptoms.Patients (n = 91) were prospectively randomized to receive either stress doses of hydrocortisone or standard treatment during the perioperative period of CS. Of 48 available patients at 6 months after CS, 26 had received stress doses of hydrocortisone and 22 standard treatment. Traumatic memories and PTSD symptoms were diagnosed with previously validated questionnaires.As compared with patients after standard therapy, patients from the hydrocortisone group had significantly lower chronic stress symptom scores (p.05). There was no significant difference regarding the number or type of traumatic memories between the hydrocortisone and the standard treatment groups.Stress doses of hydrocortisone in patients undergoing CS are associated with a lower intensity of chronic stress and PTSD symptoms at 6 months after CS.

BACKGROUND The study evaluates clinical results and hemodynamic parameters one year after implantation of a stentless quadrileaflet mitral valve (QMV). METHODS Since August 1997 28 patients received the QMV, patient age was 69 +/- 8 years; 13 had predominant mitral stenosis and 15 incompetence, preoperative NYHA functional class was III or IV and cardiac index 1.8 +/- 0.6 L/min/m2. RESULTS Surgery was performed using a conventional (25) or a minimally invasive approach (3). 20 patients received a medium and 8 a large-size prosthesis, crossclamp time was 58 +/- 19 min. Additional procedures were myocardial revascularization in four, tricuspid repair in two, and left-atrial radiofrequency ablation to restore sinus rhythm in six patients. Perioperative mortality (1) was not valve-related. All other patients were discharged on time. At postoperative, 6-, and 12-months follow-up mean transvalvular pressure gradients were 4.2 +/- 1.5 / 4 +/- 0.9/ 3.8 +/- 1.4 mmHg and mitral valve orifice area index was 1.5 +/- 0.3 / 1.6 +/- 0.3 / 1.6 +/- 0.4, NYHA class was I or II. CONCLUSIONS The QMV is well suited for mitral valve replacement. The anulo-ventricular continuity is preserved and the QMV function resembles native mitral valve function. If its performance is maintained in the long term the QMV may be the mitral prosthesis of choice.

Mitral valves, including the papillary muscles, were harvested from freshly slaughtered sheep and implanted in 10 recipient growing sheep. A strip of ovine pericardium was used to reinforce the anulus and a patch of expanded polytetrafluoroethylene was used to cover the donor papillary muscle. In four valves the natural chordae tendineae at the anterior cusp were supported by two pairs of 5-0 expanded polytetrafluoroethylene sutures. At the beginning of the experiment two animals died early of valve incompetence. Another animal had to be put to death because of paraplegia after extracorporeal circulation and one died on postoperative day 41 of endocarditis. Six sheep were electively put to death, five after 153 ± 8 days and one after 43 days. At the time of follow-up, a complete hemodynamic study including echocardiography and stress testing was performed. Heart rate at rest was 91 ± 14 beats/min, left ventricular systolic pressure 96 ± 20 mm Hg, left ventricular end-diastolic pressure 11 ± 5 mm Hg, cardiac output 2.04 ± 0.40 L/min, and the mean transvalvular pressure gradient was 4.9 ± 2.8 mm Hg as measured by cardiac catheterization. Echocardiography showed excellent movement of all valve components. However, minor regurgitation was evident in one case. Gross morphologic characteristics showed good healing at the anulus and host papillary muscle; in one case there was a rupture of one small first-order chorda. On radiography no signs of calcification were evident. Light microscopy revealed minor to moderate collagen degeneration in all valve components. However, on electron microscopy the presence of numerous fibroblasts within the long-term implants indicated the biosynthesis of new collagen. In summary, allograft mitral valve replacement seems to be a promising procedure if the chordae tendineae are reinforced by expanded polytetrafluoroethylene sutures. (J THORAC CARDIOVASC SURG 1996;111:595-604)

The in vivo echocardiographic behavior of a new mitral allograft design that incorporates ePTFE patches and chordae to enhance durability has been evaluated in sheep. A strip of pericardium was sewn around the anulus margin and an ePTFE patch of 0.4 mm thickness placed over the truncated papillary muscle tip. In four out of ten cases, 5/0 ePTFE sutures were used additionally to reinforce the native chordae of the anterior leaflet. The allografts were cold-stored at 4 degrees C after antibiotic treatment and implanted in weanling sheep. Valvular function was first examined intraoperatively by transesophageal echocardiography and again after a mean follow up of 153 +/- 8 days by epicardial echo. In all six long term survivors, valve motion was unimpaired and similar to that of the natural mitral valve. There was sufficient coaptation and concentric opening action of the leaflets. Ventricular contraction was enhanced by maintaining the annulo-ventricular connection of the native valve. Color and pulsed Doppler revealed a physiologic biphasic flow pattern and only minor regurgitation in one case. Mean transvalvular pressure gradient was 3.3 +/- 2.1 mmHg on average which correlates well with values obtained from subsequent cardiac catheterization. In conclusion, this new design of a mitral allograft shows excellent in vivo echocardiographic behavior after five months of implantation. Therefore, human allografts of this type could be recommended for clinical application.

It was examined whether the performance of right hemisphere patients in the comprehension of cartoons is predominantly influenced by disturbed processing of affective material or by cognitive deficits. Cartoons differing in their cognitive demand were presented to groups of right hemisphere patients, aphasics and normal controls. Subjects were required to identify the cartoon in a multiple choice set of four pictures. Additionally, subjects were asked to rate the funniness on a three-point scale. The results indicate that the impairment of right hemisphere patients is caused by a combination of visuo-perceptive and cognitive deficits rather than by an inability to identify the affective component of cartoons.

This study analyzed early clinical results after stentless mitral valve (Quadrileaflet Mitral Valve (QMV)/Quattro) implantation. A total of 28 patients have received a Quattro valve since August 1997. Patient age was 69+/-8 years; the underlying disease was mitral incompetence (14) and stenosis (13). Preoperative New York Heart Association functional class was 3.2+/-0.4 and cardiac index was 1.8+/-0.6. Mean cross-clamp duration was 58+/-19 minutes. Twenty patients received a 28-mm prosthesis, and eight patients received a 30-mm prosthesis. Four patients had myocardial revascularization, two had tricuspid valve repair, and six had radiofrequency ablation therapy to restore sinus rhythm. Mortality (1) was nonvalve related. Reoperation was necessary in two patients for posterior paravalvular leakage (1) and for functional stenosis (1). Echocardiography showed satisfactory hemodynamic function. By stentless mitral valve implantation the annuloventricular continuity is preserved to stabilize left ventricular function. The Quattro valve resembles native mitral valve function and is well suited for mitral valve replacement.