Ochronotic heart disease leading to severe aortic valve and coronary artery stenosis

Velez Ana K.; Gaughan Natalie A.; Thomas Rosmi P.; Schena Stefano

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Ochronotic heart disease leading to severe aortic valve and coronary artery stenosis

机译：Oronolotic心脏病导致严重主动脉瓣和冠状动脉狭窄

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Cardiac ochronosis is a rare disease, estimated to affect 1 in 250,000 persons. While there is extensive evidence of the musculoskeletal alterations of the disease, cardiac involvement has not been widely studied and most information we currently have derives from case reports and case series. We report the case of a 64-year old patient with a known history of alkaptonuria who presented with dyspnea and weight loss. On evaluation, he was found to have severe aortic stenosis, coronary artery disease, and interventricular septal hypertrophy. Surgery revealed extensive ochronotic pigment deposition affecting the cardiac septum, both internal thoracic arteries, the native coronary arteries, and the aortic valve. Ochronotic heart disease is an often disregarded presentation of alkaptonuria. More information is needed on the course of the disease, as well as long-term outcomes after valve replacement surgery and/or coronary artery bypass grafting in patients with alkaptonuria.

机译：心脏性赭石症是一种罕见的疾病，估计每25万人中就有1人患病。虽然有大量证据表明该病的肌肉骨骼改变，但心脏受累尚未得到广泛研究，我们目前掌握的大多数信息来自病例报告和病例系列。我们报告一例64岁患者，已知有碱尿病史，表现为呼吸困难和体重减轻。经评估，他患有严重的主动脉狭窄、冠状动脉疾病和室间隔肥厚。手术发现广泛的赭石色素沉积影响心脏中隔、胸内动脉、固有冠状动脉和主动脉瓣。赭石性心脏病是一种经常被忽视的碱丙酮尿症。需要更多关于阿尔卡普顿尿患者的病程，以及瓣膜置换术和/或冠状动脉旁路移植术后的长期结果的信息。

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《Journal of cardiac surgery.》 |2021年第9期|共4页
作者
Velez Ana K.; Gaughan Natalie A.; Thomas Rosmi P.; Schena Stefano;
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作者单位

Johns Hopkins Univ Sch Med Dept Surg Div Cardiac Surg Baltimore MD 21205 USA;

Florida Atlantic Univ Coll Med Boca Raton FL 33431 USA;

Philadelphia Coll Osteopath Med Philadelphia PA USA;

Johns Hopkins Univ Sch Med Dept Surg Div Cardiac Surg Baltimore MD 21205 USA;

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正文语种 eng
中图分类心脏血管和淋巴系外科学;
关键词
alkaptonuria; aortic valve stenosis; cardiovascular ochronosis; cardiovascular pathology; coronary artery disease;

机译：alkaptonuria;主动脉瓣狭窄;心血管ochronosis;心血管病理学;冠状动脉疾病;

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2. Predicting asymptomatic coronary artery stenosis by aortic arch plaque in acute ischemic cerebrovascular disease: beyond the cervicocephalic atherosclerosis? [J] . Xin Ma, Qi Kong, Chen Wang, 中华医学杂志（英文版） . 2019,第008期
3. Novel treatment for critical aortic stenosis with severe aortic root calcification and coronary disease: Combined left internal thoracic artery graft to left anterior descending coronary artery and deployment of transcatheter aortic valve implantation valve under direct vision [J] . Nick Curzen, Paul Diprose, Wendy George, The journal of thoracic and cardiovascular surgery . 2013,第6期

机译：重度主动脉钙化和冠状动脉疾病的严重主动脉瓣狭窄的新治疗方法：将左胸内动脉移植到冠状动脉左前降支并在直视下部署经导管主动脉瓣膜植入瓣膜
4. Coronary Artery Disease and Symptomatic Severe Aortic Valve Stenosis: Clinical Outcomes after Transcatheter Aortic Valve Implantation [J] . Jennifer Mancio, Ricardo Fontes-Carvalho, Marco Oliveira, Frontiers in Cardiovascular Medicine . 2015,第2期

机译：冠状动脉疾病和有症状的严重主动脉瓣狭窄：经导管主动脉瓣植入后的临床结果
5. Coronary Hemodynamics in Patients With?Severe Aortic Stenosis and Coronary Artery Disease Undergoing Transcatheter Aortic Valve Replacement [J] . Yousif Ahmad, Matthias G?tberg, Christopher Cook, JACC. Cardiovascular interventions . 2018,第20期

机译：患者患者冠状动脉血流动力学患者严重主动脉狭窄和冠状动脉疾病经历转膜主动脉瓣膜置换
6. Aortic Valve Replacement or Heart Transplantation in Patients with Aortic Stenosis and Severe Left Ventricular Dysfunction [C] . Czer L.S.C., Gol S., Soukiasian H., International Congress on Coronary Artery Disease. . 2013

机译：主动脉狭窄和严重左心室功能障碍患者的主动脉瓣替代或心脏移植
7. Middle Aortic Syndrome and Renal Artery Stenosis: Disease beyond the Arch [D] . Rumman, Rawan K. 2016

机译：中间主动脉综合征和肾动脉狭窄：疾病超越拱门
8. Coronary Artery Disease and Symptomatic Severe Aortic Valve Stenosis: Clinical Outcomes after Transcatheter Aortic Valve Implantation [O] . Jennifer Mancio, Ricardo Fontes-Carvalho, Marco Oliveira, 2015

机译：冠状动脉疾病和有症状的严重主动脉瓣狭窄：经导管主动脉瓣植入后的临床结果
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机译：冠状动脉狭窄引起亚临床，缺血性冠状动脉疾病的小型猪静息和高持续性+ G亚型的局部冠状动脉血流量

Ochronotic heart disease leading to severe aortic valve and coronary artery stenosis

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