warfarin and Tetralogy-of-Fallot

Although the pulmonary position has been preferred for the placement of homografts or bioprosthetic valves, the limited longevity of these implants makes reoperation almost unavoidable. Good durability of mechanical valves in the pulmonary position has been reported. The mid-term outcomes were analyzed of patients who received an On-X mechanical heart valve for pulmonary valve replacement (PVR) with a relatively low International Normalized Ratio (INR).. Between February 2008 and February 2011, mechanical On-X valves (size range: 19 to 25 mm) were implanted for PVR in six patients. Tetralogy of Fallot (ToF) was the most common diagnosis (n = 6). Postoperatively, warfarin was used to maintain the INR at 1.5-2.0. The surgical results and follow up were reviewed retrospectively, with valve failure, thrombosis, embolism, bleeding, reoperation and death being defined as end-points.. There was no intraoperative or in-hospital mortality. The mean age of patients at the time of PVR was 31 +/- 19.7 years (range: 16-61 years). In patients who underwent repair of ToF (n = 4) the average duration between PVR and previous right ventricular outflow tract (RVOT) reconstruction was 8.75 +/- 3.3 years (range: 5-12 years). The mean follow up period was 3.13 +/- 1.31 years (range: 2-5 years). There was no 30-day mortality, late death, thromboembolism, major bleeding event or valve dysfunction, and no patient needed reoperation. All survivors were categorized as NYHA class I or II.. A three-year experience with the On-X bileaflet mechanical valve in the pulmonary position demonstrated excellent mid-term durability, with no thromboembolisms or bleeding events when the INR was maintained at 1.5-2.0. For patients who require PVR, either as primary procedure or reoperation, the On-X mechanical heart valve might represent an appropriate option.

Topics: Adolescent; Adult; Aneurysm; Anticoagulants; Aortic Valve; Female; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; International Normalized Ratio; Male; Middle Aged; Postoperative Care; Prosthesis Design; Pulmonary Artery; Pulmonary Valve; Pulmonary Valve Insufficiency; Pulmonary Valve Stenosis; Tetralogy of Fallot; Treatment Outcome; Warfarin

A 2-year-old girl with tetralogy of Fallot presented with diffuse cranial infarct after cardiac angiography. Heterozygosity for factor V Leiden and prothrombin 20210A mutations were detected. The authors suggest that if thrombosis develops in patients with congenital heart disease, genetic risk factors should be evaluated.

Topics: Child, Preschool; Factor V; Female; Genetic Predisposition to Disease; Heparin, Low-Molecular-Weight; Humans; Mutation; Prothrombin; Stroke; Tetralogy of Fallot; Warfarin

Tetralogy of Fallot (TOF) is the commonest form of cyanotic congenital heart defect after infancy [Brickner ME, Hillis LD, Lange RA. Congenital Heart Disease in Adults-Second of Two Parts. NEJM 2000; 342(5):334-342.]. There are few studies assessing the risk of surgical correction in adult patients and long-term survival into the fourth decade of life is rare. The case history is described of a 61-year old female presenting with probable viral myocarditis. Subsequent investigations revealed an underlying diagnosis of tetralogy of Fallot. The patient remains asymptomatic despite persistent hypoxia. Potential factors contributing to longevity in this case are relatively good pulmonary blood flow via large branch pulmonary arteries, and the possible gradual development of right ventricular outflow tract obstruction over a long time period.

Topics: Adrenergic beta-Antagonists; Anticoagulants; Cardiac Catheterization; Diagnosis, Differential; Diuretics; Drug Therapy, Combination; Echocardiography, Doppler, Color; Electrocardiography; Female; Humans; Middle Aged; Radiography, Thoracic; Tetralogy of Fallot; Warfarin

Topics: Acenocoumarol; Adolescent; Angiography; Anticoagulants; Arteriosclerosis Obliterans; Blood Vessels; Child; Drug Therapy; Embolism; Geriatrics; Heparin; Humans; Postoperative Care; Tetralogy of Fallot; Thromboangiitis Obliterans; Thrombosis; Vascular Surgical Procedures; Warfarin