Coffin-Lowry syndrome is an X-linked recessive syndrome of mental retardation, characteristic facies and skeletal anomalies. In one patient with the syndrome, we observed early recurrent episodes of congestive heart failure with intercurrent normalization and the late development of mitral insufficiency due to annular dilation and congenital abnormalities of the valve apparatus. This unusual course of cardiac involvement, the non-adaptation of the left ventricular contractility to the aggravation of the mitral insufficiency and the postoperative persistence of the ventricular dysfunction, underline the possible role of an associated primary myocardial disease. This clinical observation demonstrates clearly that a mitral valve malformation can occur in patients with the syndrome, but also the role of a dilated cardiomyopathy, which can be secondary to the mitral regurgitation, but is more likely a myocardial disorder occurring as part of the syndrome.
for the Vascular Quality Initiative, Rochester and Minneapolis, Minnesota; Salt Lake City, Utah; Boston and Worcester, Massachusetts; Ann Arbor, Michigan; and Hanover, New HampshireBackground: Blood transfusions are associated with adverse events. We examined perioperative transfusion practices and associated complications following open vascular procedures nationwide in the Vascular Quality Initiative (VQI). Methods: Adults undergoing open abdominal aortic aneurysm repair (OAR) and lower extremity arterial bypass (Bypass) within VQI (2003e2016) were identified. All emergent cases, patients with preoperative hemoglobin <7 g/dL, preoperative hospitalization >1 day, or a return to operating room during the index hospitalization were excluded. Units of red blood cells transfused were the primary outcome. Secondary outcomes were postoperative myocardial infarction (MI) and death. Patient, center, and procedural factors were evaluated. Multivariable mixed effects negative binomial regression and multivariable logistic regression were performed. Results: We identified 24,131 procedures (OAR 3885, 16.1%; Bypass 20,246, 83.9%) among 22,532 patients (10.1% had >1 procedure). Overall, 37.5% of OAR and 19.5% of Bypass were transfused. Transfusion rates varied across estimated blood loss quartiles and across various preoperative hemoglobin levels. The overall rate of postoperative MI and death was 4.0% and 1.8% for OAR, and 2.2% and 0.7% for Bypass, respectively. In univariate and multivariable
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