Draulans-Noë H A, Wenink A C, Quaegebeur J
Department of Pediatric Cardiology, University Hospital Leiden, The Netherlands.
Pediatr Cardiol. 1990 Jan;11(1):29-35. doi: 10.1007/BF02239544.
A review of 59 anatomical specimens and of the findings in 65 surgically repaired patients with atrioventricular septal defect (AVSD), revealed four patients with a single papillary muscle and 11 with a double-orifice left ventricle. A single papillary muscle of the left ventricle occurred in 1.7% (1 of 59) of the anatomical specimens, and 6% (4 of 65) of the surgical cases. A double orifice of the left ventricle was found in 13.6% (8 of 59) of the anatomical specimens, and 7.7% (6 of 65) of the surgical cases. A single papillary muscle was only seen in cases with a complete defect. Double orifice was associated with partial, complete, or intermediate type of defect, with the highest incidence in the intermediate forms: 40% (4 of 10) of the anatomical specimens and 22% (2 of 9) of the surgical cases. In the anatomical study the specimens, with either single papillary muscle or double-orifice left ventricle, appear to be variants of the same malformation characterized by convergence of chordal insertion and underdevelopment of the left lateral leaflet. Pathology belonging to this spectrum was seen in 15% of our autopsy specimens and 14% of the surgical cases. In the surgical series good operative results were obtained with a conservative approach in cases with a favorable surgical anatomy.
对59个解剖标本以及65例接受手术修复的房室间隔缺损(AVSD)患者的研究结果显示,有4例患者存在单一乳头肌,11例患者存在双孔型左心室。左心室单一乳头肌在解剖标本中占1.7%(59例中的1例),在手术病例中占6%(65例中的4例)。左心室双孔在解剖标本中占13.6%(59例中的8例),在手术病例中占7.7%(65例中的6例)。单一乳头肌仅见于完全缺损病例。双孔与部分、完全或中间型缺损相关,在中间型中发生率最高:在解剖标本中占40%(10例中的4例),在手术病例中占22%(9例中的2例)。在解剖学研究中,具有单一乳头肌或双孔型左心室的标本似乎是同一畸形的变体,其特征为腱索插入汇聚和左侧小叶发育不全。在我们的尸检标本中,15%以及手术病例中14%可见属于该谱系的病理情况。在手术系列中,对于手术解剖结构有利的病例,采用保守方法可获得良好的手术效果。