Dervanian P, Macé L, Folliguet T A, di Virgilio A, Grinda J M, Fuzellier J F, De Geeter B, Morville P, Neveux J Y
Département de Chirurgie Cardiovasculaire et Cardiaque Pédiatrique, Hôpital Marie Lannelongue and Paris Sud University, 133 avenue de la Résistance, 92350 Le Plessis Robinson, France.
Pediatr Cardiol. 1998 Jul-Aug;19(4):369-73. doi: 10.1007/s002469900327.
The prognosis of Marfan syndrome in both adult and pediatric patients is primarily related to the cardiovascular complications. In infantile Marfan syndrome, although involvement of the mitral valve is the most frequently encountered cardiovascular lesion, the aortic root can be more worrisome because of its excessive dilatation, leading to aortic insufficiency or dissection. If the role of elective surgery is relatively well defined for adult patients, it is still debated during childhood. We report two patients, aged 22 months and 5 years, each presenting an aortic root aneurysm related to Marfan syndrome, and each treated with the Bentall procedure without specific age-related mortality or morbidity. These two patients experienced normal growth and were free of any complication for a follow-up period of 8 and 2 years, respectively. More than an absolute value of the aortic root dimension, it is the conjunction of the rate of progression of the aortic root dilatation, the degree and the duration of the aortic valve regurgitation, and its resulting left ventricular dysfunction that must be taken into consideration in choosing the surgical option.
成人和儿童马方综合征的预后主要与心血管并发症相关。在婴儿型马方综合征中，虽然二尖瓣受累是最常见的心血管病变，但主动脉根部因其过度扩张更令人担忧，可导致主动脉瓣关闭不全或夹层。对于成年患者，择期手术的作用相对明确，但在儿童期仍存在争议。我们报告了两名患者，年龄分别为22个月和5岁，均患有与马方综合征相关的主动脉根部瘤，均接受了Bentall手术，且均未出现与年龄相关的特定死亡率或发病率。这两名患者生长正常，分别随访8年和2年，均无任何并发症。在选择手术方案时，除了主动脉根部尺寸的绝对值外，还必须考虑主动脉根部扩张的进展速度、主动脉瓣反流的程度和持续时间及其导致的左心室功能障碍等因素。
