Abid Dorra, Daoud Emna, Ben Kahla Sahar, Mallek Souad, Abid Leila, Fourati Hela, Mnif Zeineb, Kammoun Samir
Cardiology Department, Hedi Chaker Hospital, Sfax, Tunisia.
Department of Radiology, Hedi Chaker Hospital, Sfax, Tunisia.
Cardiovasc J Afr. 2015 Jul 23;26(4):e6-8. doi: 10.5830/CVJA-2015-005.
Persistent truncus arteriosus (PTA) is a rare conotruncal defect, defined as a single arterial vessel arising from the heart, which gives origin to the systemic, pulmonary and coronary circulations. It has an extremely poor prognosis and carries a high mortality rate during the early years of life unless surgically repaired. A few known cases have been reported of patients reaching maturity, and exceptionally, patients suffering from this disease having lived into the fourth decade. The purpose of this report was to present a new case of PTA type 1, diagnosed by echocardiography and MRI, in a 41-year-old woman, with the peculiarity of long survival into adult life. She had also experienced a full-term pregnancy and delivery of a normal infant three years prior to her diagnosis. Pulmonary vascular disease made her condition inoperable but she was doing well with medical management after a follow up of 15 months. Based on this work, we concluded that pulmonary arterial hypertension is deleterious for life in some cardiovascular diseases, but in others, allows survival, as occurred in these patients with PTA. The patient's clinical course and anatomical findings are reported, along with factors that may have contributed to her longevity.
永存动脉干（PTA）是一种罕见的圆锥动脉干畸形，定义为起源于心脏的单一动脉血管，它为体循环、肺循环和冠状动脉循环供血。其预后极差，若不进行手术修复，在生命早期死亡率很高。已有少数已知病例报道患者存活至成年，极少数情况下，患有这种疾病的患者活到了四十多岁。本报告的目的是介绍一例通过超声心动图和磁共振成像（MRI）诊断出的1型PTA新病例，该病例为一名41岁女性，其特殊之处在于成年后存活时间较长。在她被诊断出该病的三年前，她还经历了一次足月妊娠并产下一名正常婴儿。肺血管疾病使她的病情无法进行手术治疗，但经过15个月的随访，她通过药物治疗情况良好。基于这项工作，我们得出结论，肺动脉高压在某些心血管疾病中对生命有害，但在其他疾病中，如这些患有PTA的患者，却能使患者存活。报告了该患者的临床病程和解剖学发现，以及可能有助于她长寿的因素。
