Toscano Alessandra, Pasquini Luciano, Iacobelli Roberta, Di Donato Roberto M, Raimondi Francesca, Carotti Adriano, Di Ciommo Vincenzo, Sanders Stephen P
Department of Cardiology, Bambino Gesù Pediatric Hospital, Rome, Italy.
J Thorac Cardiovasc Surg. 2009 Mar;137(3):538-42. doi: 10.1016/j.jtcvs.2008.08.023.
Congenital mitral ring is a rare subtype of congenital mitral stenosis. Our objective is to review the anatomic findings and surgical results of this lesion and to identify early predictors of outcome.
Clinical reports, echocardiographic studies, cardiac catheterizations, surgical reports, and follow-up data of all patients with mitral ring diagnosed at the Bambino Gesù Hospital were retrospectively reviewed.
Between January of 1987 and June of 2007, a mitral ring was diagnosed in 25 patients (13 male) with a mean age at diagnosis of 36 months. The ring was identified in a single neonate but seemed to develop and progress during infancy. All but 1 patient had associated cardiac anomalies. We identified 2 distinct subtypes: "intramitral ring" in 18 of 25 patients (72%), associated with complex valve pathology and a worse outcome, and "supramitral ring" in 7 of 25 patients (18%), in whom the mitral apparatus was usually normal and the outcome was better. The ring was surgically removed in 13 of 25 patients (52%) (mean Doppler gradient 15 +/- 4 mm Hg). The gradient decreased in 9 of 13 patients (mean diastolic transmitral gradient <or= 5 mm Hg) and has remained stable during a mean follow-up of 90 months. The mean diastolic gradient remained high (>8 mm Hg) in the other 4 patients, all with intramitral ring. All 4 patients underwent repeat mitral valvuloplasty. There were no operative or late deaths.
Two types of congenital mitral ring, with diverse valve pathology and outcome, were identified. Rarely seen in neonates, the ring develops and often progresses during infancy. Recognition is important because surgical results are better than for other forms of congenital mitral stenosis.
先天性二尖瓣环是先天性二尖瓣狭窄的一种罕见亚型。我们的目的是回顾该病变的解剖学发现和手术结果，并确定预后的早期预测因素。
对在 Bambino Gesù 医院诊断为二尖瓣环的所有患者的临床报告、超声心动图研究、心导管检查、手术报告和随访数据进行回顾性分析。
在 1987 年 1 月至 2007 年 6 月期间，25 例患者（13 例男性）被诊断为二尖瓣环，诊断时的平均年龄为 36 个月。该环在 1 例新生儿中被发现，但似乎在婴儿期发展和进展。除 1 例患者外，所有患者均伴有心脏异常。我们确定了 2 种不同的亚型：25 例患者中有 18 例（72%）为“二尖瓣内环”，与复杂的瓣膜病变相关且预后较差；25 例患者中有 7 例（18%）为“二尖瓣上环”，其二尖瓣装置通常正常且预后较好。25 例患者中有 13 例（52%）接受了手术切除（平均多普勒压差为 15±4 mmHg）。13 例患者中有 9 例压差降低（平均舒张期二尖瓣压差≤5 mmHg），在平均 90 个月的随访期间保持稳定。另外 4 例患者的平均舒张期压差仍然很高（>8 mmHg），均为二尖瓣内环患者。所有 4 例患者均接受了再次二尖瓣成形术。无手术或晚期死亡病例。
确定了两种类型的先天性二尖瓣环，其瓣膜病变和预后各不相同。二尖瓣环在新生儿中很少见，在婴儿期发展且常进展。认识到这一点很重要，因为手术结果优于其他形式的先天性二尖瓣狭窄。
