Porter Inna, Vacek James
University of Kansas Hospital, Rainbow Boulevard, Kansas City, KS, USA.
J Med Case Rep. 2008 May 30;2:184. doi: 10.1186/1752-1947-2-184.
Single ventricle and truncus arteriosus are both rare congenital cardiac syndromes with limited survival. Their occurrence together is extremely uncommon and prolonged survival is exceptionally rare. We present the case of a patient who had both of these defects with survival to age 45.
We describe the vase of a 45-year-old man with the unusual occurrence of two very rare congenital cardiac defects. He was found to have both truncus arteriosus and single ventricle with long survival. His history, clinical course, and anatomic findings are discussed along with the factors which may have contributed to his longevity, which is unique in the medical literature. His management reflected the state of medical knowledge at the time when he presented, and although alternate approaches may have been utilized if the patient presented today, this case does indicate the efficacy of the management options available at the time and place of the patient's contacts with the medical care system in Belarus. We discuss the findings, frequency, classification, and management of both of these congenital defects.
This case demonstrates that patients with very complex congenital cardiac disease may survive to adulthood, presenting challenges in both medical and surgical treatment. As the management of these patients is constantly evolving, and interventional techniques are improving, patients such as this with prolonged survival will be more common, with each case providing insights to future treatment. Challenges in management may include prior care provided in health care systems with limited resources.
单心室和共同动脉干均为罕见的先天性心脏综合征,患者存活率有限。这两种疾病同时出现极为罕见,能长期存活的情况更是格外稀少。我们报告一例同时患有这两种缺陷且存活至45岁的患者病例。
我们描述了一名45岁男性的病例,他患有两种极为罕见的先天性心脏缺陷,这种情况不同寻常。他被发现同时患有共同动脉干和单心室,且存活时间较长。我们讨论了他的病史、临床病程、解剖学发现以及可能促成其长寿的因素,这在医学文献中是独一无二的。他的治疗反映了他就诊时的医学知识水平,尽管如果该患者现在就诊可能会采用其他方法,但这个病例确实表明了当时患者在白俄罗斯与医疗保健系统接触时可用的治疗方案的有效性。我们讨论了这两种先天性缺陷的发现、发生率、分类和治疗。
该病例表明,患有非常复杂的先天性心脏病的患者可能存活至成年,这在医学和外科治疗方面都带来了挑战。随着对这些患者的治疗不断发展,介入技术不断改进,像这样长期存活的患者将会更加常见,每个病例都能为未来的治疗提供见解。管理方面的挑战可能包括在资源有限的医疗保健系统中提供的前期治疗。