Carrel Thierry, Berdat Pascal, Pavlovic Mladen, Sukhanov Sergei, Englberger Lars, Pfammatter Jean-Pierre
Clinic for Cardiovascular Surgery, University Hospital Berne, CH-3010 Berne, Switzerland.
Eur J Cardiothorac Surg. 2003 Aug;24(2):249-54. doi: 10.1016/s1010-7940(03)00302-6.
Dilatation of the aortic root is a well-known cardiovascular manifestation in children and adult patients with connective tissue disease (e.g. Marfan syndrome). Dilatation of the ascending aorta is extremely rare and may be associated with bicuspid aortic valve. This report evaluates the incidence of dilatative aortic root and ascending aortic pathology in patients younger than 18 years and analyzes the results obtained after repair and replacement strategies.
Between 1/1995 and 12/2002, a total of 752 operations on the thoracic aorta were performed in adult and pediatric patients. We present our experience with a group of 26 patients <18 years of age, who required isolated surgery of the aortic root and/or ascending aorta because of a dilatative lesion. Fifteen patients had isolated aortic root dilatation (13 of them suffered from Marfan syndrome), eight patients presented with an idiopathic dilatation of the ascending aorta and three patients had dilatation in association with a bicuspid aortic valve. Mean age was 10 +/- 4.8 years (4-18 years). Repair of the aortic root with preservation of the aortic valve (Yacoub, David or selective sinus repair) was performed in nine patients, replacement using a homograft was performed in five patients, composite graft with mechanical prosthesis in two patients, with biological prosthesis in one patient and Ross operation was performed in one case. Isolated supracoronary graft replacement was performed in eight patients.
Two patients died during hospitalization: a 10-year old girl developed respiratory failure on the 2nd postoperative day and autopsy revealed Ehlers-Danlos syndrome with a massive intrapulmonary emphysema. A 14-year-old Marfan patient with severely depressed preoperative LV function died from low cardiac output following composite-graft, mitral and tricuspid valve repair. One patient required aortic valve replacement 7 days after an aortic valve sparing root repair. There was no additional perioperative morbidity. In the long-term, two patients died from rupture of the thoracic aorta, both following minor non-cardiovascular surgical procedures. Both had normal sized descending and abdominal aorta.
Repair of the aortic root and/or ascending aorta in children and adolescent patients can be performed with acceptable early and late results. While the presence of severe comorbidity may adversely affect early outcome, long-term survival was mainly determined by rupture of the descending aorta.
主动脉根部扩张是儿童和成年结缔组织病患者（如马凡综合征）中一种众所周知的心血管表现。升主动脉扩张极为罕见，可能与二叶式主动脉瓣有关。本报告评估了18岁以下患者中扩张性主动脉根部和升主动脉病变的发生率，并分析了修复和置换策略后的结果。
1995年1月至2002年12月期间，成年和儿科患者共进行了752例胸主动脉手术。我们介绍了一组26例年龄小于18岁的患者的经验，这些患者因扩张性病变需要单独进行主动脉根部和/或升主动脉手术。15例患者有孤立性主动脉根部扩张（其中13例患有马凡综合征），8例患者表现为升主动脉特发性扩张，3例患者的扩张与二叶式主动脉瓣有关。平均年龄为10±4.8岁（4 - 18岁）。9例患者采用保留主动脉瓣的主动脉根部修复术（亚库布术式、大卫术式或选择性窦部修复术），5例患者采用同种异体移植物置换术，2例患者采用带机械瓣膜的复合移植物置换术，1例患者采用生物瓣膜置换术，1例患者进行了罗斯手术。8例患者进行了孤立性冠状动脉上移植物置换术。
2例患者在住院期间死亡：一名10岁女孩术后第2天出现呼吸衰竭，尸检显示埃勒斯 - 当洛综合征伴大量肺内气肿。一名术前左心室功能严重受损的14岁马凡综合征患者在进行复合移植物、二尖瓣和三尖瓣修复后因心输出量低死亡。一名患者在保留主动脉瓣的根部修复术后7天需要进行主动脉瓣置换。围手术期无其他并发症。长期来看，2例患者死于胸主动脉破裂，均在进行小型非心血管手术后。二者降主动脉和腹主动脉大小正常。
儿童和青少年患者的主动脉根部和/或升主动脉修复术可取得可接受的早期和晚期结果。虽然严重合并症的存在可能对早期结果产生不利影响，但长期生存主要取决于降主动脉破裂。
