Carrel Thierry, Sharipov Islamjan, Jalilov Adham, Sromicki Juri, Vogt Paul Robert
Department for Cardiac Surgery, University Hospital Zürich, Zurich, Switzerland.
Clinic for Cardiac Surgery, Republican Specialized Center for Cardiology, Tashkent, Uzbekistan.
Front Cardiovasc Med. 2022 Sep 14;9:920614. doi: 10.3389/fcvm.2022.920614. eCollection 2022.
While aneurysm of the aortic root, interrupted aortic arch, and aortic coarctation are the most frequent aortic diseases in adolescents and younger adults, there are a number of rare pathologies of the thoracic aorta that need individualized treatment.
We present a small case series of unusual aortic pathologies in patients presenting with a broad spectrum of symptoms: tiredness, dysphagia, dyspnea, arterial hypertension, renal failure, and claudication. (1). Segmental agenesia of the descending aorta. (2). Balanced double aortic arch with complete vascular ring. (3). Right descending aortic arch, left lusorian artery with Kommerell diverticulum. (4). Large patent ductus (2.5 cm) and ventricular septal defect. (5). Aneurysm of the aortic arch in the presence of tuberous sclerosis. (6). Pseudo-aneurysm of the proximal descending aorta following coarctation patch plasty. (7). Supravalvular aortic stenosis combined with severe aortic valve stenosis. (8). Pseudo-aneurysm following ascendens-to-descendens bypass because of recurrent coarctation. (9). Takayasu arteriitis with severe stenosis in the thoraco-abdominal aorta.
The following procedures were performed, using individualized cardiopulmonary bypass, canulation and cerebral protection strategies. (1). Ascending to supraceliac extra-anatomic bypass. (2). Division of the ductus ligament and of the anterior aortic arch distally to the left subclavian artery. (3). Excision of the Kommerell diverticulum and translocation of the left subclavian artery. (4). Patch closure of the aorto-pulmonary window (patent ductus) and closure of the ventricular septal defect. (5). Complete aortic arch replacement combined with debranching of the supra-aortic vessels. (6). Graft interposition of the proximal descending aorta. (7). Enlargement of the ascending aorta and aortic valve replacement. (8). Exclusion of the pseudo-aneurysm, end-to-side graft interposition. (9). Ascending aorta to infrarenal aorta or ascending to bi-iliac artery bypass (planned). All patients were operated on without operative mortality. One patient died in-hospital from pulmonary complications one week after surgery. One patient is still awaiting surgery. All other patients recovered very well from the operation and did not show any residual symptoms.
Rare pathologies of the thoracic aorta in younger patients may cause a broad spectrum of unusual symptoms; in some of them, diagnosis is delayed. Cross-sectional imaging is mandatory for optimal operative planning Surgical treatment can be performed with very satisfying results. The prognosis of these patients is usually favorable following surgery.
虽然主动脉根部瘤、主动脉弓中断和主动脉缩窄是青少年和年轻成年人中最常见的主动脉疾病，但胸主动脉还有许多罕见病变需要个体化治疗。
我们报告了一组小病例系列，这些患者患有各种不寻常的主动脉病变，症状广泛：疲劳、吞咽困难、呼吸困难、动脉高血压、肾衰竭和跛行。（1）降主动脉节段性发育不全。（2）平衡型双主动脉弓伴完整血管环。（3）右位降主动脉弓、左迷走动脉伴Kommerell憩室。（4）大型动脉导管未闭（2.5厘米）和室间隔缺损。（5）结节性硬化症合并主动脉弓瘤。（6）缩窄修补术后降主动脉近端假性动脉瘤。（7）主动脉瓣上狭窄合并严重主动脉瓣狭窄。（8）因复发性缩窄行升主动脉至降主动脉旁路术后假性动脉瘤。（9）高安动脉炎伴胸腹主动脉严重狭窄。
采用个体化体外循环、插管和脑保护策略进行了以下手术。（1）升主动脉至腹腔干上解剖外旁路术。（2）切断动脉导管韧带和左锁骨下动脉远端的主动脉弓前部。（3）切除Kommerell憩室并移位左锁骨下动脉。（4）修补主肺动脉窗（动脉导管未闭）并关闭室间隔缺损。（5）全主动脉弓置换并离断主动脉弓上血管。（6）降主动脉近端植入人工血管。（7）扩大升主动脉并置换主动脉瓣。（8）排除假性动脉瘤，端侧植入人工血管。（9）升主动脉至肾下腹主动脉或升主动脉至双髂动脉旁路术（计划中）。所有患者均接受手术，无手术死亡。1例患者术后1周因肺部并发症死于医院。1例患者仍在等待手术。所有其他患者术后恢复良好，未出现任何残留症状。
年轻患者胸主动脉的罕见病变可能导致广泛的不寻常症状；其中一些患者诊断延迟。横断面成像对于优化手术规划至关重要。手术治疗可取得非常满意的效果。这些患者术后预后通常良好。
