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Abstract：
OBJECTIVE: According to current international guidelines, hypertrophic cardiomyopathy (HCM) patients should be managed in specialized units. However, there is lack of data on the impact of the creation of these units in the management of HCM patients. Our goal was to assess the impact of the creation of an Inherited Inherited Diseases Cardiac Unit (ICDU) in the current management of patients with HCM.
METHODS: We analyzed 114 consecutive patients (62.6±8 years old, 70.2% males) with HCM. Variables related to optimal management of HCM patients and their family study were recorded, as well as guidance on the risk of sudden death. We analyzed whether patients were assessed by the ICDU or at a general cardiology consultation (GCC).
RESULTS: 50 patients were assessed in the IDCU and 64 in the GCC. Familial screening was more frequent in patients assessed by the IDCU (45.3% vs. 4%; p<0.01), requesting more genetic studies of the index case (70.3% vs. 14%; p<0.01) and cardiac magnetic resonance (53.1% vs. 18%; p<0.01). Sudden death risk score was performed more frequently in patients after the creation of an IDCU (67.2% vs. 28%; p<0.01). Treatment with beta-blockers was similar in both groups (72% vs. 78.1%; p=0.24). An implantable cardiac defibrillator was indicated similarly in both groups (12.5% in ICDU and 6% in GC; p=0.24).
CONCLUSIONS: The implementation of an IDCU improved the quality of the medical care for HCM patients by performing a better study of the patients and their families.
METHODS: We analyzed 114 consecutive patients (62.6±8 years old, 70.2% males) with HCM. Variables related to optimal management of HCM patients and their family study were recorded, as well as guidance on the risk of sudden death. We analyzed whether patients were assessed by the ICDU or at a general cardiology consultation (GCC).
RESULTS: 50 patients were assessed in the IDCU and 64 in the GCC. Familial screening was more frequent in patients assessed by the IDCU (45.3% vs. 4%; p<0.01), requesting more genetic studies of the index case (70.3% vs. 14%; p<0.01) and cardiac magnetic resonance (53.1% vs. 18%; p<0.01). Sudden death risk score was performed more frequently in patients after the creation of an IDCU (67.2% vs. 28%; p<0.01). Treatment with beta-blockers was similar in both groups (72% vs. 78.1%; p=0.24). An implantable cardiac defibrillator was indicated similarly in both groups (12.5% in ICDU and 6% in GC; p=0.24).
CONCLUSIONS: The implementation of an IDCU improved the quality of the medical care for HCM patients by performing a better study of the patients and their families.
摘要：
目标：根据当前的国际准则，肥厚型心肌病(HCM)患者应在专门的单位进行治疗.然而,缺乏关于创建这些单元对HCM患者管理的影响的数据.我们的目标是评估在当前HCM患者管理中创建遗传性遗传性疾病心脏单元（ICDU）的影响。
方法：我们分析了114例连续患者（62.6±8岁，70.2％的男性）患有HCM。记录与HCM患者及其家庭研究的最佳管理相关的变量，以及猝死风险的指导。我们分析了患者是否通过ICDU或一般心脏病学会诊（GCC）进行评估。
结果：在IDCU中评估了50例患者，在GCC中评估了64例患者。在IDCU评估的患者中，家族性筛查更为频繁(45.3%vs.4%；p<0.01)，要求对索引病例进行更多的遗传研究(70.3%与14％；p<0.01）和心脏磁共振（53.1％vs.18%；p<0.01)。在创建IDCU后，患者更频繁地进行猝死风险评分(67.2%vs.28%；p<0.01)。两组的β受体阻滞剂治疗相似(72%vs.78.1%；p=0.24）。两组患者均采用植入式心脏除颤器（ICDU为12.5％，GC为6％；p=0.24）。
结论：通过对患者及其家属进行更好的研究，IDCU的实施提高了HCM患者的医疗质量。
方法：我们分析了114例连续患者（62.6±8岁，70.2％的男性）患有HCM。记录与HCM患者及其家庭研究的最佳管理相关的变量，以及猝死风险的指导。我们分析了患者是否通过ICDU或一般心脏病学会诊（GCC）进行评估。
结果：在IDCU中评估了50例患者，在GCC中评估了64例患者。在IDCU评估的患者中，家族性筛查更为频繁(45.3%vs.4%；p<0.01)，要求对索引病例进行更多的遗传研究(70.3%与14％；p<0.01）和心脏磁共振（53.1％vs.18%；p<0.01)。在创建IDCU后，患者更频繁地进行猝死风险评分(67.2%vs.28%；p<0.01)。两组的β受体阻滞剂治疗相似(72%vs.78.1%；p=0.24）。两组患者均采用植入式心脏除颤器（ICDU为12.5％，GC为6％；p=0.24）。
结论：通过对患者及其家属进行更好的研究，IDCU的实施提高了HCM患者的医疗质量。
