Viveiros Monteiro André, Fiarresga António, Cacela Duarte, de Sousa Lídia, Ramos Ruben, Galrinho Ana, Branco Luísa, Cruz Ferreira Rui
Cardiology Department, Hospital of Santa Marta, Lisbon, Portugal.
Cardiology Department, Hospital of Santa Marta, Lisbon, Portugal.
Rev Port Cardiol. 2016 Sep;35(9):499.e1-4. doi: 10.1016/j.repc.2015.11.027. Epub 2016 Aug 6.
Acromegaly is a rare disease, mostly caused by a growth hormone (GH)-secreting benign pituitary tumor, with an increased production of GH and insulin-like growth factor 1 (IGF-1). Cardiovascular complications are common and are associated with cardiomyocyte apoptosis and concentric cardiac hypertrophy. Suppression of GH and IGF-1 appears to improve cardiac function only in the short term, with little or no decrease in left ventricular mass or improvement in cardiac function after prolonged treatment. Alcohol septal ablation (ASA) has emerged as a minimally invasive alternative to septal myectomy, with significant improvement in symptoms, gradients and left ventricular remodeling. In this report, we describe the case of a 73-year-old woman with acromegaly due to a pituitary adenoma diagnosed and treated surgically at the age of 38 but with recurrence and reoperation at the age of 50. She was referred to our cardiology department due to a three-month history of progressively worsening exercise-induced dyspnea and orthopnea under optimal medical therapy. Echocardiography and magnetic resonance imaging revealed severe basal hypertrophy of the interventricular septum (19 mm), dynamic left ventricular outflow tract obstruction with a gradient of 70 mmHg at rest and 120 mmHg with Valsalva maneuver, and systolic anterior movement (SAM). Genetic testing excluded the most frequent forms of familial hypertrophic cardiomyopathy. ASA was performed with injection of 2 cc of alcohol in the first septal branch of the left coronary artery, without complications. At one-year reassessment, significant clinical and echocardiographic improvement was noted, with disappearance of SAM. To our knowledge, there have been no previously reported cases of ASA in hypertrophic cardiomyopathy due to acromegaly. We report a case of successful ASA in acromegalic cardiomyopathy.
肢端肥大症是一种罕见疾病，主要由分泌生长激素（GH）的垂体良性肿瘤引起，伴有GH和胰岛素样生长因子1（IGF-1）分泌增加。心血管并发症很常见，与心肌细胞凋亡和向心性心脏肥大有关。抑制GH和IGF-1似乎仅在短期内能改善心脏功能，长期治疗后左心室质量几乎没有减少或心脏功能几乎没有改善。酒精间隔消融术（ASA）已成为间隔肌切除术的一种微创替代方法，在症状、压力阶差和左心室重塑方面有显著改善。在本报告中，我们描述了一名73岁女性的病例，她因垂体腺瘤在38岁时被诊断出患有肢端肥大症并接受了手术治疗，但在50岁时复发并再次手术。在最佳药物治疗下，她因进行性加重的运动性呼吸困难和端坐呼吸3个月病史被转诊至我们的心脏病科。超声心动图和磁共振成像显示室间隔严重基底肥厚（19毫米），动态左心室流出道梗阻，静息时压力阶差为70 mmHg，瓦尔萨尔瓦动作时为120 mmHg，以及收缩期前向运动（SAM）。基因检测排除了最常见的家族性肥厚型心肌病类型。在左冠状动脉第一间隔支注射2 cc酒精进行了ASA，无并发症。在一年的复查中，观察到临床和超声心动图有显著改善，SAM消失。据我们所知，此前尚无因肢端肥大症导致的肥厚型心肌病行ASA的报道。我们报告了一例肢端肥大性心肌病成功行ASA的病例。
