Saccheri María Cristina, Cianciulli Tomás Francisco, Challapa Licidio Wilde, Lax Jorge A, Beck Martín A, Morita Luis A, Gagliardi Juan A
Echocardiography Laboratory, Division of Cardiology, Hospital General de Agudos del Gobierno de la Ciudad de Buenos Aires "Dr. Cosme Argerich, Buenos Aires, Argentina.
Researcher of the Ministry of Health of the Government of the City of Buenos Aires, Buenos Aires, Argentina.
Echocardiography. 2018 May;35(5):643-650. doi: 10.1111/echo.13829. Epub 2018 Feb 19.
Fabry disease (FD) and hypertrophic cardiomyopathy (HCM) are two diseases with a different pathophysiology, both cause left ventricular hypertrophy (LVH) and myocardial fibrosis. Although remodeling and systolic dysfunction of the left atrium (LA) are associated with atrial fibrillation and stroke in HCM, changes in the size and function of the LA have not been well studied in FD with LVH.
The following groups were studied prospectively, and their respective findings compared: 19 patients with non-obstructive HCM (Group I), 20 patients with a diagnosis of Fabry cardiomyopathy (Group II), and 20 normal subjects matched for sex and age (Group III). Left ventricular mass index was measured using Devereux' formula, left atrial volume with Simpson's biplane method and left atrial mechanical function, including strain and strain rate, was measured using the speckle tracking technique. Strain and strain rate of the reservoir were measured during the three phases: reservoir (SR S), passive conduit (SR E) and atrial contraction (SR A).
Patients with HCM had a larger left atrial volume than patients with FD (48.16 ± 14.3 mL/m vs 38.9 ± 14.9 mL/m respectively, P < .001), but in both disorders there was a severe decrease in left atrial function: reservoir strain in the apical four-chamber view: 17.47% in HCM vs 22.5% in FD, P = .24), strain rate in the apical chamber view: SR A: -0.80/seconds in HCM vs -1.04/seconds in FD (P = .88), SR S: 0.69/seconds in HCM vs 0.93 in FD (P = .12), SR E: -0.80 seconds in HCM vs -0.97/seconds in FD (P = .18).
In this echocardiographic study we used speckle tracking to assess left atrial mechanical function and showed that FD is associated to an atrial cardiomyopathy, affecting the three phasic functions of the LA. Although in patients with HCM left atrial volume is larger than in patients with FD, both disorders exhibit severe decrease in left atrial function. These findings should be considered, given the potentially serious complications that can occur with the two diseases.
法布里病（FD）和肥厚型心肌病（HCM）是两种病理生理学不同的疾病，二者均可导致左心室肥厚（LVH）和心肌纤维化。尽管左心房（LA）的重塑和收缩功能障碍与HCM中的心房颤动和中风有关，但在伴有LVH的FD中，LA大小和功能的变化尚未得到充分研究。
对以下几组进行前瞻性研究，并比较各自的研究结果：19例非梗阻性HCM患者（I组）、20例诊断为法布里心肌病的患者（II组）和20例年龄和性别匹配的正常受试者（III组）。使用Devereux公式测量左心室质量指数，采用Simpson双平面法测量左心房容积，并使用斑点追踪技术测量左心房机械功能，包括应变和应变率。在三个阶段测量储存期的应变和应变率：储存期（SR S）、被动管道期（SR E）和心房收缩期（SR A）。
HCM患者的左心房容积大于FD患者（分别为48.16±14.3 mL/m和38.9±14.9 mL/m，P<0.001），但在这两种疾病中，左心房功能均严重下降：心尖四腔视图中的储存期应变：HCM为17.47%，FD为22.5%，P = 0.24），心尖腔视图中的应变率：SR A：HCM为-0.80/秒，FD为-1.04/秒（P = 0.88），SR S：HCM为0.69/秒，FD为0.93（P = 0.12），SR E：HCM为-0.80秒，FD为-0.97/秒（P = 0.18）。
在这项超声心动图研究中，我们使用斑点追踪技术评估左心房机械功能，结果表明FD与心房心肌病相关，影响LA的三个阶段性功能。尽管HCM患者的左心房容积大于FD患者，但这两种疾病均表现出左心房功能严重下降。考虑到这两种疾病可能发生的潜在严重并发症，这些发现应予以重视。
