Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders caused by collagen synthesis defects. EDS type Ⅳ, or vascular EDS, is caused by loss-of-function mutations in the type Ⅲ pro-collagen ...Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders caused by collagen synthesis defects. EDS type Ⅳ, or vascular EDS, is caused by loss-of-function mutations in the type Ⅲ pro-collagen gene (COL3A1 ). Common complications of EDS type IV include gastrointestinal bleeding and bowel perforations, posing diagnostic and therapeutic dilemmas for both surgeons and gastroenterologists. Here, we describe a complicated case of EDS type Ⅳ in a 35-year-old caucasian female who presented with overt gastro-intestinal bleeding. The patient had a prior history of spontaneous colonic perforation, and an uncomplicated upper endoscopy was performed. A careful ileoscopy was terminated early due to tachycardia and severe ab-dominal pain, and a subsequent computed tomography scan confirmed the diagnosis of ileal perforation. The patient was managed conservatively, and demonstrated daily improvement. At the time of hospital discharge, no further episodes of gastrointestinal blood loss had occurred. This case highlights the benefit of conservative management for EDS patients with gastrointestinal hemorrhage. It is recommended that surgical treatmentshould be reserved for patients who fail conservative treatment or in cases of hemodynamic instability. Finally, this case demonstrates the necessity for a higher threshold of operative or endoscopic interventions in EDS type Ⅳ patients.展开更多
目的:评价改良迷宫术同期进行瓣膜手术的电生理效果及对心脏功能的远期影响。方法:24 例慢性房颤伴有二尖瓣疾病施行改良迷宫和二尖瓣手术的患者进行12导联心电图、心内电生理、动态心电图和超声心动图检查,平均随访(92.7±11.9)个...目的:评价改良迷宫术同期进行瓣膜手术的电生理效果及对心脏功能的远期影响。方法:24 例慢性房颤伴有二尖瓣疾病施行改良迷宫和二尖瓣手术的患者进行12导联心电图、心内电生理、动态心电图和超声心动图检查,平均随访(92.7±11.9)个月。结果:(1)术后3个月90%恢复窦性心律,术后1年以上100%恢复窦性心律。(2)除1例Ⅰ度房室传导阻滞外,窦房结及房室结功能检查均正常。(3)除高位右房外心房各部位有效不应期均显著延长,在心房各部位猝发和程控刺激均不能诱发房扑和房颤。(4)电生理检查有正常的心房激动和房室同步顺序。(5)动态心电图显示有良好的心率变时性反应和运动耐力。(6)随访期中再住院率为4%。(7)超声心动图显示随访期左房长径较术后明显减小[(5.52±1.22) cm vs (6.77±1.36) cm, P<0.01];随访期左房容积明显小于术后[(91.97±52.64) cm3vs(155.35±88.86) cm3, P<0.001];随访期右房长径明显小于术后[(4.72±0.85) cm vs (5.77±1.18) cm,P<0.05]。(8)左心室收缩功能正常,平均EF值为(56.00±19.75)%,平均FS值为(32.86±9.53)%。结论:在改良迷宫同时进行瓣膜手术均能安全有效的消除房颤维持窦律减小心房容积,恢复正常的房室同步传导和左心功能。展开更多
文摘Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders caused by collagen synthesis defects. EDS type Ⅳ, or vascular EDS, is caused by loss-of-function mutations in the type Ⅲ pro-collagen gene (COL3A1 ). Common complications of EDS type IV include gastrointestinal bleeding and bowel perforations, posing diagnostic and therapeutic dilemmas for both surgeons and gastroenterologists. Here, we describe a complicated case of EDS type Ⅳ in a 35-year-old caucasian female who presented with overt gastro-intestinal bleeding. The patient had a prior history of spontaneous colonic perforation, and an uncomplicated upper endoscopy was performed. A careful ileoscopy was terminated early due to tachycardia and severe ab-dominal pain, and a subsequent computed tomography scan confirmed the diagnosis of ileal perforation. The patient was managed conservatively, and demonstrated daily improvement. At the time of hospital discharge, no further episodes of gastrointestinal blood loss had occurred. This case highlights the benefit of conservative management for EDS patients with gastrointestinal hemorrhage. It is recommended that surgical treatmentshould be reserved for patients who fail conservative treatment or in cases of hemodynamic instability. Finally, this case demonstrates the necessity for a higher threshold of operative or endoscopic interventions in EDS type Ⅳ patients.
文摘目的:评价改良迷宫术同期进行瓣膜手术的电生理效果及对心脏功能的远期影响。方法:24 例慢性房颤伴有二尖瓣疾病施行改良迷宫和二尖瓣手术的患者进行12导联心电图、心内电生理、动态心电图和超声心动图检查,平均随访(92.7±11.9)个月。结果:(1)术后3个月90%恢复窦性心律,术后1年以上100%恢复窦性心律。(2)除1例Ⅰ度房室传导阻滞外,窦房结及房室结功能检查均正常。(3)除高位右房外心房各部位有效不应期均显著延长,在心房各部位猝发和程控刺激均不能诱发房扑和房颤。(4)电生理检查有正常的心房激动和房室同步顺序。(5)动态心电图显示有良好的心率变时性反应和运动耐力。(6)随访期中再住院率为4%。(7)超声心动图显示随访期左房长径较术后明显减小[(5.52±1.22) cm vs (6.77±1.36) cm, P<0.01];随访期左房容积明显小于术后[(91.97±52.64) cm3vs(155.35±88.86) cm3, P<0.001];随访期右房长径明显小于术后[(4.72±0.85) cm vs (5.77±1.18) cm,P<0.05]。(8)左心室收缩功能正常,平均EF值为(56.00±19.75)%,平均FS值为(32.86±9.53)%。结论:在改良迷宫同时进行瓣膜手术均能安全有效的消除房颤维持窦律减小心房容积,恢复正常的房室同步传导和左心功能。
