Gender-related differences in patients undergoing mechanical aortic valve replacement with the CarboMedics valve.

AIM: The aim of this paper was to evaluate gender-related differences in patients undergoing mechanical aortic valve replacement with the CarboMedics valve. METHODS: During a 20 year period, 629 patients (median age 60 years) underwent mechanical aortic valve replacement with the CarboMedics valve. Of these, 215 patients were female (34%). The median follow-up for the entire cohort was 10.2 ± 6.2 years. RESULTS: In-hospital mortality for the entire cohort was 9% (male 7.3% vs. female 11.0%, P=0.005). Cox regression analysis revealed redo-surgery (HR=2.35, CI 1.35-4.08), LVEF<30% (HR=2.31, CI 1.36-3.93), age (HR=1.60, CI 1.27-2.02), as well as female gender (HR=2.07, CI 1.28-3.35) as independent predictors of survival. For male gender LVEF<30% (HR=2.47, CI 1.23-4.93) and age (HR=1.75, CI 1.25-2.43) were independent predictors of survival. For female gender, additional CABG (HR=2.15, CI 1.08-4.28), redo surgery (HR=3.64, CI 1.78-7.46) as well as age (HR=1.48, CI 1.06-2.06) were independent predictors of survival. CONCLUSION: Gender per se is an independent risk factor of survival after mechanical aortic valve replacement. Severely impaired LVEF independently predicts survival in males whereas additional CABG and redo surgery do in females. Age affects survival in both sexes. These findings may serve as a basis for further improving gender related outcome.

Results of open heart surgery in Jehovah's Witnesses patients.

AIM: The aim of this paper was to evaluate the results in patients from the religious community of Jehovah's Witnesses (JW) undergoing open heart surgery. METHODS: Between January 1998 and November 2007, 35 patients with a religious background of JW church underwent open heart surgery at the Department of Cardiothoracic Surgery, Medical University of Vienna (Austria). Eighteen patients underwent coronary artery bypass graft (CABG), 11 patients underwent valve surgery and 5 patients underwent combined procedures. One patient underwent isolated ascending aortic replacement. Five patients undergoing CABG were operated without cardiopulmonary bypass (CBP). RESULTS: Mean baseline hematocrit serum levels were 35.8+/-6.3%. The mean decrease of hematocrit serum levels was 20.0+/-21.1% after surgery. The mean decrease of hematocrit serum levels in patients undergoing CABG without CPB was 12.5+/-5.4% and 12.0+/-20.0% in patients after isolated valve replacement. One patient died during the operation. Four patients died in the postoperative period due to anemia. During follow-up, being 34.6+/-34.8 months to date, no cardiovascular related adverse event has been observed. CONCLUSIONS: The decrease of hematocrit serum levels is significantly characterizing the postoperative period of open heart surgery in JW. In patients undergoing CABG without CPB and in patients undergoing isolated valve replacement, decrease of hematocrit serum levels was lowest. Therefore, these techniques should be considered for first choice when appropriate. Furthermore, highly normal preoperative hematocrit serum levels and a meticulous surgical technique remain the mainstay of therapy in these patients.

Double outlet right ventricle: aetiologies and associations.

BACKGROUND: Double outlet right ventricle (DORV), a clinically significant congenital heart defect, occurs in 1-3% of individuals with congenital heart defects. In contrast to other major congenital heart defects, there are no systematic or comprehensive data regarding associations, aetiologies, and pathogenesis of DORV. We analysed reported cases in the medical literature to address these issues. METHODS: We queried the PubMed database using key words "double outlet right ventricle" and "DORV" for case reports, epidemiologic analyses and animal studies with this cardiac anomaly. The anatomic subtype of DORV was classified according to criteria of Van Praagh. RESULTS: Chromosomal abnormalities were present in 61 of the 149 cases of DORV. Trisomies 13 and 18, and del 22q11 were the most commonly associated cytogenetic lesions; different anatomic subtypes of DORV were noted in trisomies 13 and 18 versus del 22q11. DORV was reported in many uncommon or rare non-chromosomal syndromes. Mutations and non-synonymous sequence variants in the CFC1 and CSX genes were the most commonly reported monogenic loci associated with DORV in humans; numerous genes are reported in murine models of DORV. Animal studies implicate maternal diabetes and prenatal exposure to ethanol, retinoids, theophylline, and valproate in DORV teratogenesis. CONCLUSIONS: The large number of genes associated with DORV in both humans and animal models and the different anatomic subtypes seen in specific aetiologies indicate the likelihood of several distinct pathogenetic mechanisms for DORV, including impairment of neural crest derivative migration and impairment of normal cardiac situs and looping.

Tetralogy of Fallot with congenital aortic valvar stenosis: the tetralogy-truncus interrelationship.

Two rare patients are reported with tetralogy of Fallot and congenital aortic valvar stenosis. The anatomic and developmental interrelationship between tetralogy of Fallot and truncus arteriosus is summarized. A study of 100 randomly selected postmortem cases of tetralogy revealed aortic valve pathology in 8%, myxomatous aortic valve leaflets without stenosis in 4%, bicuspid aortic valves without stenosis in 3%, and congenital aortic valvar stenosis in 1%. The frequency of systemic semilunar valve pathology in truncus was much higher (66%): moderate to marked myxomatous change in 44%, mild myxomatous change in 22%, truncal valvar stenosis in 11%, and truncal valvar regurgitation in 15%. Being aware of the tetralogy-truncus interrelationship and knowing that myxomatous aortic valves are prone to premature calcific aortic stenosis and/or regurgitation, physicians should follow the aortic valves of surgically repaired patients with tetralogy of Fallot and truncus arteriosus long term with great care. Timely aortic valvuloplasty or replacement may well prove life-saving in such patients.

Isolated left-sided scimitar vein connecting all left pulmonary veins to the right inferior vena cava.

When the common pulmonary vein fails to develop, the embryonic connections of the pulmonary veins to one or more of the systemic veins almost always persist. Anomalous pulmonary venous connections to the inferior vena cava (IVC) are typically characterized by hypoplasia of the involved pulmonary veins and pulmonary artery, as well as abnormal parenchyma of the involved lung. Such cases have been described as "scimitar syndrome." We report the case of a young female patient in whom all the left pulmonary veins converged into a common vessel that drained into the IVC but who had a normal left pulmonary artery and left lung. Surgical intervention was successful, and our patient is still alive.

A GATA-6 gene heart-region-specific enhancer provides a novel means to mark and probe a discrete component of the mouse cardiac conduction system.

The transcriptional programs that specify the distinct components of the cardiac conduction system are poorly understood, in part due to a paucity of definitive molecular markers. In the present study we show that a cGATA-6 gene enhancer can be used to selectively express transgenes in the atrioventricular (AV) conduction system as it becomes manifest in the developing multichambered mouse heart. Furthermore, our analysis of staged cGATA-6/lacZ embryos revealed that the activity of this heart-region-specific enhancer can be traced back essentially to the outset of the cardiogenic program. We provide evidence that this enhancer reads medial/lateral and anterior/posterior positional information before the heart tube forms and we show that the activity of this enhancer becomes restricted at the heart looping stage to AV myocardial cells that induce endocardial cushion formation. We infer that a deeply-rooted heart-region-specific transcriptional program serves to coordinate AV valve placement and AV conduction system formation. Lastly, we show that cGATA-6/Cre mice can be used to delete floxed genes in the respective subsets of specialized heart cells.