Ventricular assist device as a bridge to transplant, and extracorporeal membrane oxygenation for primary graft failure in a child with hemophilia A.

OBJECTIVE: To report the management of hemophilia in a patient with dilated cardiomyopathy during application of the Berlin-Heart biventricular assist. DESIGN: Case report. INTERVENTIONS AND RESULTS: The Berlin-Heart biventricular assist was successfully used as a bridge to heart transplantation (178 days) in a child with hemophilia A; post-transplant extracorporeal membrane oxygenation was implanted until the patient recovered from primary graft failure. Anticoagulant therapy varied as a function of patient status: 1) postoperative bleeding was treated by partial replacement of deficient factors (VII and VIII) and hemoderivatives in order to maintain factor VIII at 50% of normal levels; 2) once the bleeding had stopped, the effect of the hemophilia itself maintained the degree of anticoagulation required by the ventricular assist device; and 3) transplant surgery was followed by complete replacement of factor VIII and intravenous heparinization (a simple way of preventing clot formation in the device and to ensure proper scarring of surgical wounds). CONCLUSIONS: Selection of anticoagulant therapy as a function of patient status in terms of bleeding and surgical-wound scarring progress is vital for the proper functioning of support techniques (Berlin-Heart biventricular assist and extracorporeal membrane oxygenation) in hemophiliac patients. Collagen dressings placed on surgical wounds achieved good functional and aesthetic results, as well as mechanically isolating the scars from the exterior.

[Carotid intima-media thickness in heterozygous familial hypercholesterolemia: associated factors and changes following 1 year of treatment]. / Grosor de la íntima-media carotídea en la hipercolesterolemia familiar heterocigótica: factores asociados y variación al año de tratamiento.

BACKGROUND AND OBJECTIVE: To evaluate the factors associated with intima-media thickness (IMT) and carotid plaques in patients with familial hypercholesterolemia (FH) and to assess the evolution following 1 year of treatment. PATIENTS AND METHOD: Subjects (n=201) diagnosed as having probable or definite FH according to the MED-PED criteria had a clinical history, physical examination, blood chemistry and lipid profile determined. Ultrasound was used to measure the IMT in both common carotid arteries and to assess the presence of plaques. The measurements were repeated in 123 of the patients after 1 year of treatment. RESULTS: Mean age (standard deviation) was 47.8 (13.2) years; 46.3% were males; 11.4% had a clinical history of cardiovascular disease (CVD); 15.9% were hypertensive; 26.4% were smokers; mean plasma total cholesterol concentration was 362 (49)mg/dL; 66.2% had received previous treatment with statins. In the multivariate analysis, baseline IMT was directly associated with age and history of CVD and inversely related to the years of treatment with statins. The presence of carotid plaques was associated directly with the cholesterol-years index and inversely with years of treatment with statins. The mean LDL-cholesterol reduction at 1 year of treatment was 50%. Neither the IMT nor the percentage of patients with plaques changed significantly following 1 year of treatment. CONCLUSIONS: Intensive hypocholesterolemic treatment in our patients with probable or definite FH did not increase significantly either the carotid IMT or the prevalence of plaques.