High proportion of patients with bleeding of unknown cause in persons with a mild-to-moderate bleeding tendency: Results from the Vienna Bleeding Biobank (VIBB).

INTRODUCTION: Data on clinical characteristics and the prevalence of underlying coagulopathies in patients with mild-to-moderate bleeding disorders (MBDs) are scarce. AIM: We established the Vienna Bleeding Biobank (VIBB) to characterize and thoroughly investigate Austrian patients with MBDs. RESULTS: Four hundred eighteen patients (female = 345, 82.5%) were included. A platelet function defect (PFD) was diagnosed in 26 (6.2%) and a possible PFD in 30 (7.2%) patients. Eight patients (1.9%) were diagnosed with von Willebrand disease (VWD) (type 1 n = 6; type 2 n = 2), and 29 patients had low VWF (30-50 IU/dL). Deficiencies in factor VIII, IX, XI or XIII were found in 11 (2.6%), 3 (0.7%), 3 (0.7%) and 1 patient(s), 2 patients had dysfibrinogenaemia, and further 2 had possible PFD and FXI deficiency. Probable causal mutations were detected in 8 of 11 patients with FVIII deficiency, 2 of 3 patients with FIX deficiency and 2 of 8 patients with VWD. Three hundred three patients (72.5%) had normal results in the coagulation assays and were categorized as patients with bleeding of unknown cause (BUC). The bleeding score did not differ between patients with and without established diagnosis. A diagnosis of a bleeding disorder was more frequently made in men than in women (49.3% vs 22.9%). Male sex (OR 3.55, 95% CI: 2.02-6.22; P < .001) and blood group 0 (OR 1.86, 95% CI: 1.17-2.94; P = .008) were independently associated with diagnosis of a bleeding disorder. CONCLUSION: The high rate of patients with BUC despite in-depth haemostatic assessment underlines the incompleteness of available routine laboratory tests. Males with MBDs were more likely to be diagnosed with an established bleeding disorder than females.

Long-term outcome of primary Raynaud's phenomenon and its conversion to connective tissue disease: a 12-year retrospective patient analysis.

OBJECTIVES: To determine the frequency of development of connective tissue disease (CTD) in patients considered to have idiopathic Raynaud's phenomenon (RP) > 10 years. PATIENTS AND METHODS: Based on initial evaluation, 113 women and 29 men were divided into either 'primary RP' (n=109) or 'possible secondary RP' (n=33) groups and were re-evaluated after a median follow-up period of 12.4 years. RESULTS: Overall 20 patients (14.1%) progressed to a definite CTD; 10 from 'primary RP' (9.2%) and 10 from possible secondary RP' (30.3%). Initial presence of antinuclear antibodies, thickening of fingers, higher age at onset of RP, and female sex seemed to be important determinants for a possible transition to a CTD. CONCLUSION: In RP-patients, who are at risk of development of a CTD, serial clinical and laboratory controls are warranted for the decision to initiate therapy early.

Retrieval-oriented design of clinical research forms.

Computerized clinical forms are subject to a wide variety of different requirements. They have to allow detailed documentation and must be user-friendly. State-of-the-art applications for design permit clinicians themselves to create their own forms as needed, with the various variables presented in different ways depending on their intended use. Often, however, only aspects of clinical documentation are considered, with no thought being given to subsequent data retrieval. This article presents guidelines for the retrieval-oriented design of clinical forms. It discusses where anticipatory measures for structuring forms are easier to accomplish than complex data linkage at the time of retrieval and analysis.

Growth of the pulmonary autograft after the Ross operation in childhood.

OBJECTIVE: Excellent hemodynamic performance has been demonstrated after aortic valve replacement using the autologous pulmonary valve as described by D. Ross. However, in the pediatric population there is concern in regard to growth of the autograft and late dilatation in the systemic circulation. METHODS: Since 1991, 30 children (mean age, 11.3+/-3.1 years) had aortic valve replacement with the pulmonary autograft as a root replacement. All children had yearly clinical and echocardiographic follow-up. RESULTS: There were no perioperative deaths; one child died late in a car accident. At the last follow-up (mean follow-up, 4.3+/-2.6 years), all patients were in NYHA class I. There was one early reoperation, in which the autograft had to be reconstructed due to a leaflet perforation. There were no major valve related events. All children showed normal somatic growth. The annulus diameter increased significantly from 18+/-2 at surgery to 20+/-3.5 mm at the latest follow-up (P<0.004). The sinus also increased significantly in diameter from 29+/-4 at surgery to 34+/-2 mm at the last follow-up (P<0.001). This increase in autograft size, both for the annulus and the sinus, paralleled the increase in body surface area with no evidence for unproportional dilatation. Hemodynamic measurements demonstrated physiological peak gradients of 6.8+/-2.9 mmHg and no or trivial aortic insufficiency in 95% of this rapidly growing patient population. CONCLUSION: These data demonstrate growth of the pulmonary autograft parallel to somatic growth without undue dilatation in the systemic circulation. The hemodynamics are excellent with regard to physiological gradients and no increase in aortic insufficiency.

Mid-term follow up of mitral valve reconstruction due to active infective endocarditis.

BACKGROUND AND AIM OF THE STUDY: Mitral valve reconstruction in patients with acute endocarditis (AE) is a challenging operation which prompts the surgeon into immediate action. This report summarizes the mid-term results of 22 patients who required mitral valve reconstruction due to AE. METHODS: Mean patient age was 46 years (range: 20-79 years); mean follow up was 46 months (range: 1-90 months). Preoperatively, >70% of patients had severe mitral regurgitation and were in NYHA functional class III. Surgical techniques used were annuloplasty (n = 16; 10 with Carpentier ring, five Wooler-Kay and one Frater); suture closure of the perforation (n = 1), patch closure of the perforation (n = 5), leaflet resection with primary closure (n = 2), leaflet resection with patch closure (n = 8), and chordal transfer (n = 3). Additional surgery included CABG (n = 3) and De Vega plasty (n = 4). Aortic valve replacement or reconstruction (n = 9) included one mechanical valve, one bioprosthesis, one reconstruction and six homografts. Patients were followed up annually in our outpatient department and/or by questionnaires. RESULTS: Two patients died perioperatively due to either low output syndrome or uncontrolled sepsis. There were three reoperations; two of these were successful, and one patient subsequently died. In addition, one patient died six years after operation due to prostatic cancer, and one seven years later due to progressive heart failure. At the last follow up, 15 patients were in NYHA class I (68%) and five in class II (23%); no or only mild mitral insufficiency was seen on transthoracic echocardiography (91%). The estimated survival rate at 60 months was 87 +/- 12.7%, and 12 patients were followed up for >60 months. No incidence of recurrent valve infection occurred. CONCLUSION: Mitral valve reconstruction in patients with AE shows a low incidence of valve-related complications with promising postoperative functional results and mid-term survival. On this basis, mitral valve reconstruction for mitral insufficiency secondary to AE may be recommended as a valve salvage treatment, when it is technically possible.

[6 years clinical experience with the TEKNA bivalve heart valve prosthesis]. / Sechs Jahre klinische Erfahrung mit TEKNA-Doppelflügel-Herzklappenprothese.

OBJECTIVE: The revised Edwards-Duromedics valve was introduced onto the market in June 1990. In September 1993, the producer changed the name of the valve to TEKNA, which is a low profile, bileaflet, mechanical prosthesis with a self-irrigating hinge mechanism. The results of a prospective follow-up study after valve replacement with this prosthesis are presented. METHODS: From November 1990 to December 1996, 173 TEKNA prostheses were implanted in 161 patients (73 men, 88 women), either in the aortic (AVR; 48.4%), mitral (MVR; 44.1%), or aortic and mitral positions (DVR; 7.5%). Patient ages ranged from 17 to 83 years (mean age 59.2 +/- 8.7 years). Follow-up was 97.5% complete, comprising 433.15 patient-years for a mean follow-up of 34.8 +/- 23 months. RESULTS: Overall operative (30-day) mortality was 4.97%. Fifteen patients died in the late period, corresponding to a linearized late mortality rate of 3.5%/pt-yr. Actuarial survival at six years was 75.3% +/- 6.2% overall. The actuarial freedom from complications was calculated as follows (linearized rates in parentheses): valve-related mortality 91.3% +/- 5.1% (0.92%/pt-yr); thromboembolism 94.2% +/- 2.5% (1.62%/pt-yr); valve thrombosis 98.6 +/- 1.0% (0.46%/pt-yr); bleeding events 85.9% +/- 4.7% (2.77%/pt-yr); prosthetic valve endocarditis 98.6 +/- 0.9% (0.46%/pt-yr); nonstructural dysfunction 93.2 +/- 3.2% (1.38%/pt-yr); reoperation 87.1 +/- 5.3% (2.08%/pt-yr). Functional class according to NYHA was improved from preoperatively 72.05% in III or IV to 6-year postoperative 94.2% in I or II. CONCLUSION: The clinical performance of the TEKNA valve is quite satisfactory, with low incidence of valve-related complications. No structural deterioration has been observed. Further, the patients physical ability and quality of life were significantly improved.

Long-term results of heart valve replacement with the Edwards Duromedics bileaflet prosthesis: a prospective ten-year clinical follow-up.

OBJECTIVE: The Edwards Duromedics valve (Baxter Healthcare Corp., Edwards Division, Santa Ana, Calif.) was designed with a self-irrigating hinge mechanism to reduce thromboembolic complications. After good initial clinical results, distribution was suspended in 1988 after reports of valve fracture after 20,000 valves had been implanted. The manufacturer conducted extensive studies to improve the Edwards Duromedics and reintroduced a modified version, which is available as Edwards Tekna. The purpose of the study was the evaluation of long-term results of the original Edwards Duromedics that might be important for the current version, the Edwards Tekna valve. METHODS: A prospective clinical 10-year follow-up was performed of 508 patients who underwent valve replacement with the Edwards Duromedics valve in the aortic (n = 268), mitral (n = 183), and aortic and mitral (n = 56) position. RESULTS: The perioperative mortality rate was 6.9%; follow-up was 98% complete, comprising 3648 patient-years for a mean follow-up of 86 months (range: 33 to 144 months). The actuarial freedom from complications at the 10-year follow-up and the incidence rate (percent per patient-year) were as follows: late mortality rate, 69.2% +/- 2.4% (3.5% per patient-year); thromboembolism, 90.7% +/- 1.6% (0.96% per patient-year); anticoagulation-related hemorrhage, 87.7% +/- 1.7% (1.34% per patient-year); prosthetic valve endocarditis, 96.7% +/- 0.09% (0.38% per patient-year); valve-related mortality rate, 89.3% +/- 1.6% (1.21% per patient-year); valve failure, 86.2% +/- 1.85% (1.54% per patient-year); and valve-related morbidity and mortality rate, 71.1% +/- 2.3% (3.2% per patient-year). Three leaflet escapes were observed (one lethal, two successful reoperations; 99.1% +/- 0.05% freedom, 0.08% per patient-year). All patients functionally improved (86% in New York Heart Association classes I and II), and incidence of anemia was insignificant. CONCLUSIONS: These results confirm that the Edwards Duromedics valve shows excellent performance concerning thromboembolism, hemolysis, and functional improvement and will serve as a reference for the last version, the Edwards Tekna valve, where comparable long-term data are currently not available.