Kawahira Y, Kishimoto H, Kawata H, Ikawa S, Ueda H, Nakajima T, Kayatani F, Inamura N, Nakada T
Department of Cardiovascular Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Izumi, Osaka 590-02, Japan.
Pediatr Cardiol. 1997 Mar-Apr;18(2):107-11. doi: 10.1007/s002469900126.
The objective of the study was to examine the relation between the morphology of the common atrioventricular valve and regurgitation of the valve in patients with right atrial isomerism. We examined seven consecutive patients with documented right atrial isomerism who subsequently underwent postmortem examination during a 10-year period. The degree of regurgitation and the diameters of the common valve were evaluated via cineangiography, and the site of regurgitation was evaluated by echocardiography. The morphology of the common atrioventricular valve was assessed further at autopsy. Cineangiography revealed valve diameters ranging from 14.8 to 27.8 mm (mean 20.9 mm). Valvar regurgitation was revealed within 2 months of birth in all patients. Regurgitation abruptly worsened in three patients after placement of a Blalock-Taussig shunt or a central shunt and postintubation. Autopsies revealed that the common atrioventricular valve consisted of four leaflets in five patients, and three leaflets in two. The anterior leaflets were large and protruding in all patients, and the lateral leaflets were thickened in six. All patients had a mass consisting of the left lateral leaflets and chordae with direct attachment of the chordae to the ventricular muscle (the right lateral leaflet was attached to the ventricular muscle and immobile in one patient). The lateral leaflets clung to the ventricular wall and exhibited poor movement in six patients. Leaflets with poor mobility corresponded to the regurgitant valvar site as assessed by echocardiography in six patients; and the regurgitation in three patients with acute deterioration occurred at the valvar side with poor mobility. It is concluded that the common atrioventricular valve in patients with right atrial isomerism has morphologic characteristics that may be associated with valvar regurgitation and malignant potential for abrupt deterioration after replacement of systemic-pulmonary shunting.
本研究的目的是探讨右心房异构患者共同房室瓣形态与瓣膜反流之间的关系。我们检查了7例连续记录有右心房异构的患者，这些患者在10年期间随后接受了尸检。通过心血管造影评估反流程度和共同瓣膜直径，通过超声心动图评估反流部位。在尸检时进一步评估共同房室瓣的形态。心血管造影显示瓣膜直径范围为14.8至27.8毫米（平均20.9毫米）。所有患者在出生后2个月内均发现瓣膜反流。3例患者在放置Blalock-Taussig分流术或中心分流术及插管后反流突然加重。尸检显示，5例患者的共同房室瓣由四个瓣叶组成，2例由三个瓣叶组成。所有患者的前瓣叶均大且突出，6例患者的外侧瓣叶增厚。所有患者均有一个由左外侧瓣叶和腱索组成的肿块，腱索直接附着于心室肌（1例患者的右外侧瓣叶附着于心室肌且固定不动）。6例患者的外侧瓣叶紧贴心室壁且活动不良。6例患者中，活动不良的瓣叶与超声心动图评估的反流瓣膜部位相对应；3例急性病情恶化患者的反流发生在活动不良的瓣膜侧。结论是，右心房异构患者的共同房室瓣具有形态学特征，可能与瓣膜反流以及体肺分流置换术后突然恶化的恶性潜能有关。
