Sleeper M M, Kusiak C M, Shofer F S, O'Donnell P, Bryan C, Ponder K P, Haskins M E
Section of Cardiology, Department of Clinical Studies, University of Pennsylvania School of Veterinary Medicine, Philadelphia, PA, USA.
J Inherit Metab Dis. 2008 Jun;31(3):424-31. doi: 10.1007/s10545-008-0821-1. Epub 2008 May 23.
The purpose of this study was to define the cardiovascular abnormalities present in young and adult cats affected with the lysosomal storage diseases mucopolysaccharidosis (MPS) I and MPS VI.
Eighteen cats affected with MPS I and 10 cats affected with MPS VI were evaluated by physical examination, electrocardiography and echocardiography. Electrocardiography (ECG) was performed on all MPS I and 9 of the MPS VI cats. Twelve unaffected cats underwent complete examinations for comparison purposes.
No cardiovascular abnormalities were noted on physical examination. Measured ECG intervals were normal in affected cats; however, sinus arrhythmia was noted more frequently than in the unaffected cats. Significant echocardiographic abnormalities included aortic valve thickening, regurgitation and aortic root dilation. Significant mitral valve thickening was also noted. The severity of changes increased in older affected cats.
As affected animals increased in age, more cardiac abnormalities were found with increasing severity. Significant lesions included the mitral and aortic valves and ascending aorta, but myocardial changes were not recognized. MPS I and MPS VI cats have similar cardiovascular findings to those seen in children and constitute important models for testing new MPS therapies.
本研究旨在明确患有溶酶体贮积病黏多糖贮积症（MPS）I型和MPS VI型的幼年及成年猫所存在的心血管异常情况。
对18只患有MPS I型的猫和10只患有MPS VI型的猫进行体格检查、心电图检查和超声心动图检查。对所有患有MPS I型的猫以及9只患有MPS VI型的猫进行了心电图（ECG）检查。为作比较，对12只未受影响的猫进行了全面检查。
体格检查未发现心血管异常。受影响猫的心电图测量间期正常；然而，窦性心律失常的出现频率高于未受影响的猫。显著的超声心动图异常包括主动脉瓣增厚、反流和主动脉根部扩张。还发现二尖瓣显著增厚。随着受影响猫年龄增长，变化的严重程度增加。
随着受影响动物年龄增长，发现的心脏异常越来越多，且严重程度不断增加。显著病变包括二尖瓣、主动脉瓣和升主动脉，但未发现心肌变化。患有MPS I型和MPS VI型的猫具有与儿童相似的心血管表现，是测试新型MPS疗法的重要模型。
