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Long-term survival of a patient with single atrium and single ventricular heart: a case report

机译:单心房和单心室患者的长期生存:一例报告

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Uni-ventricular heart is rare and accounts for 1-2%of all congenital cardiac malformations. Without correctivesurgery, it has a fatal course in the neonatal period or inearly infancy and survival into adult life is unusual [1].Majority of patients with uni-ventricular heart have toundergo surgical procedures during infancy or childhoodto ensure a balanced pulmonary and systemic blood flow.Although, for a long time, Fontan-type procedures havebeen performed as corrective surgical treatment, it is alsoassociated with significant mortality and morbidity dueto complications such as atrial arrhythmia, venouscongestion, protein-losing enteropathy, thromboembolismand ventricular failure [2]. However, rarely some patientslive almost a normal life without any surgical interventionsdue to a balanced native hemodynamic status. Onlyaround 10 cases have been reported worldwide whosurvived into late adulthood; age up to 62 years in oneand up to 50 years in the rest [3]. We did not find anyreports from Sri Lanka of long survival of such patients.
机译:单心室心脏很少见,占所有先天性心脏畸形的1-2%。如果不进行矫正手术,它在新生儿期或婴儿早期就具有致命的病程,并且成年后存活是不寻常的[1]。大多数单心室患者必须在婴儿期或儿童期接受手术,以确保平衡的肺和全身血流尽管长期以来,丰坦型手术已作为矫正外科手术治疗,但由于房性心律不齐,静脉充血,蛋白丢失性肠病,血栓栓塞性心室衰竭等并发症,它也伴随着显着的死亡率和发病率[2]。然而,由于平衡的天然血液动力学状态,很少有患者在没有任何手术干预的情况下几乎过着正常的生活。全世界仅报告了约10例存活至成年晚期的病例。年龄最高可达62岁,其余年龄最高可达50岁[3]。我们没有发现斯里兰卡此类患者长期存活的任何报道。

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