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Abstract：
Boxer arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease that may result in sudden death or heart failure.
To prospectively study the natural history of Boxer ARVC.
72 dogs (49 ARVC, 23 controls).
Boxers >1 year of age were recruited for annual reevaluation. CONTROLS were defined as being ≥6 years of age and having <50 ventricular premature complex (VPCs)/24 h. ARVC was defined as ≥300 VPCs/24 h in the absence of other disease. Dogs were genotyped for the striatin deletion when possible. Descriptive statistics were determined for age; VPC number; annual change in VPC number; and left ventricular (LV) echocardiographic dimensions. Survival time was calculated.
Controls: median age of 7 years (range, 6-10); number of VPCs 12 (range, 4-32). Median time in study of 6 years (range, 2-9). Seventeen of 23 were genotyped (5 positive, 12 negative). ARVC: median age of diagnosis of 6 (range, 1-11). Median time in study 5 years (range, 3-8). A total of 33% were syncopal and 43/49 were genotyped (36 positive, 7 negative). Yearly change in VPCs was 46 (range, -7,699 to 33,524). Annual percentage change in LV dimensions was 0, and change in fractional shortening (FS%) was 2%. Two dogs had FS% <20%. Although ARVC dogs died suddenly, there was no difference in survival time between groups. ARVC median age of survival was 11 years, and for controls was 10 years.
Arrhythmogenic right ventricular cardiomyopathy is a disease of middle age and frequently is associated with the striatin deletion. Syncope occurs in approximately 1/3 of affected dogs; systolic dysfunction is uncommon. The prognosis in many affected dogs is good.
To prospectively study the natural history of Boxer ARVC.
72 dogs (49 ARVC, 23 controls).
Boxers >1 year of age were recruited for annual reevaluation. CONTROLS were defined as being ≥6 years of age and having <50 ventricular premature complex (VPCs)/24 h. ARVC was defined as ≥300 VPCs/24 h in the absence of other disease. Dogs were genotyped for the striatin deletion when possible. Descriptive statistics were determined for age; VPC number; annual change in VPC number; and left ventricular (LV) echocardiographic dimensions. Survival time was calculated.
Controls: median age of 7 years (range, 6-10); number of VPCs 12 (range, 4-32). Median time in study of 6 years (range, 2-9). Seventeen of 23 were genotyped (5 positive, 12 negative). ARVC: median age of diagnosis of 6 (range, 1-11). Median time in study 5 years (range, 3-8). A total of 33% were syncopal and 43/49 were genotyped (36 positive, 7 negative). Yearly change in VPCs was 46 (range, -7,699 to 33,524). Annual percentage change in LV dimensions was 0, and change in fractional shortening (FS%) was 2%. Two dogs had FS% <20%. Although ARVC dogs died suddenly, there was no difference in survival time between groups. ARVC median age of survival was 11 years, and for controls was 10 years.
Arrhythmogenic right ventricular cardiomyopathy is a disease of middle age and frequently is associated with the striatin deletion. Syncope occurs in approximately 1/3 of affected dogs; systolic dysfunction is uncommon. The prognosis in many affected dogs is good.
摘要：
Boxer心律失常性右心室心肌病（ARVC）是一种可能导致猝死或心力衰竭的疾病。
前瞻性研究BoxerARVC的自然史。
72只狗(49只ARVC,23个控件）。
Boxers>招募1岁的人进行年度重新评估。控制定义为年龄≥6岁，室性早搏（VPCs）<50/24小时。在没有其他疾病的情况下，ARVC定义为≥300VPCs/24小时。在可能的情况下,对狗进行纹状体蛋白缺失的基因分型。确定年龄的描述性统计；VPC数量；VPC数量的年度变化；和左心室（LV）超声心动图尺寸。计算生存时间。
对照组：年龄中位数为7岁（范围，6-10)；VPC数量12(范围，4-32).研究的中位时间为6年(范围，2-9).23人中有17人进行了基因分型(5人呈阳性，12负）。ARVC：诊断年龄中位数为6岁（范围，1-11).研究中值时间5年(范围，3-8).共有33%为晕厥，43/49为基因分型(36阳性，7负）。VPC的年度变化为46(范围，-7699至33524）。LV尺寸的年度百分比变化为0，缩短分数的变化（FS%）为2％。两只狗的FS%<20%。虽然ARVC狗突然死亡，组间生存时间无差异。ARVC中位生存年龄为11岁,对于对照组来说是10年。
致心律失常性右心室心肌病是一种中年疾病，常伴有纹状体蛋白缺失。晕厥发生在大约1/3的受影响的狗中;收缩功能障碍并不常见。许多受影响的狗的预后良好。
前瞻性研究BoxerARVC的自然史。
72只狗(49只ARVC,23个控件）。
Boxers>招募1岁的人进行年度重新评估。控制定义为年龄≥6岁，室性早搏（VPCs）<50/24小时。在没有其他疾病的情况下，ARVC定义为≥300VPCs/24小时。在可能的情况下,对狗进行纹状体蛋白缺失的基因分型。确定年龄的描述性统计；VPC数量；VPC数量的年度变化；和左心室（LV）超声心动图尺寸。计算生存时间。
对照组：年龄中位数为7岁（范围，6-10)；VPC数量12(范围，4-32).研究的中位时间为6年(范围，2-9).23人中有17人进行了基因分型(5人呈阳性，12负）。ARVC：诊断年龄中位数为6岁（范围，1-11).研究中值时间5年(范围，3-8).共有33%为晕厥，43/49为基因分型(36阳性，7负）。VPC的年度变化为46(范围，-7699至33524）。LV尺寸的年度百分比变化为0，缩短分数的变化（FS%）为2％。两只狗的FS%<20%。虽然ARVC狗突然死亡，组间生存时间无差异。ARVC中位生存年龄为11岁,对于对照组来说是10年。
致心律失常性右心室心肌病是一种中年疾病，常伴有纹状体蛋白缺失。晕厥发生在大约1/3的受影响的狗中;收缩功能障碍并不常见。许多受影响的狗的预后良好。
