背景:四瓣肺动脉瓣(QPV)是一种罕见的先天性异常。简单QPV主要在2000年以前的尸检时被诊断出来,QPV的频率约为0.02%-0.41%。QPV最初在2000年后使用经胸超声心动图(TTE)诊断,在2009年后使用对比计算机断层扫描(CT)或心脏磁共振成像(CMR)诊断。使用TTE获得肺动脉瓣的横截面视图是困难的。我们的目的是回顾关于发病率的论文,胚胎学,诊断,相关的先天性心脏异常,QPV患者的预后,并进一步与四瓣主动脉瓣(QAV)患者进行比较。
方法:我们在12个月大的婴儿中诊断为QPV轻度狭窄。从左高胸骨旁短轴视图开始,换能器上方有轻微的角度,获得了QPV的短轴视图。
结果:在尸检确诊的QPV病例中,Hurwitz的b型具有三个相等的尖点和一个较小的尖点占主导地位,而Hurwitz的a型有四个相等的尖点在临床诊断病例中占主导地位。QPV患者的先天性心脏异常和瓣膜狭窄比QAV患者更常见。QAV患者的冠状动脉异常和感染性心内膜炎比QPV患者更常见。PR的发生率在a型QPV中比在b型QPV中更常见。就主动脉瓣反流(AR)的发生率而言,a型QAV和b型QAV之间没有差异。假设QPV是罗斯手术的风险因素。然而,QPV已在某些患者中用作自体移植物。
结论:在QPV和QAV之间,在频率上发现了各种差异,诊断方法,瓣膜形态,阀门功能,相关的先天性心脏病,和感染性心内膜炎的频率。
Quadricuspid pulmonary valve (QPV) is a rare congenital anomaly. Simple QPV had been mainly diagnosed at the time of autopsy before 2000, and the frequency rates of QPV are approximately 0.02%-0.41%. QPV was initially diagnosed using transthoracic echocardiography (TTE) after 2000 and with contrast computed tomography (CT) or cardiac magnetic resonance imaging (CMR) after 2009. Obtaining the cross-sectional view of the pulmonary valve using TTE is difficult. We aimed to review the papers regarding the incidence, embryology, diagnosis, associated congenital heart anomalies, and prognosis in patients with QPV, and furthermore to compare with those in patients with quadricuspid aortic valve (QAV).
We diagnosed QPV with mild stenosis in a 12-month-old infant. With a slight angulation of the transducer superiorly from the left high parasternal short-axis view, a short-axis view of QPV was obtained.
In QPV cases diagnosed at autopsy, Hurwitz\'s type-b with three equal cusps and one smaller cusp is dominant, whereas Hurwitz\'s type-a with four equal cusps is dominant in clinically diagnosed cases. Congenital heart anomaly and valvular stenosis are more frequent in patients with QPV than in patients with QAV. Coronary artery anomalies and infectious endocarditis are more frequent in patients with QAV than in patients with QPV. The incidence of PR is more common in type-a QPV than in type-b QPV. There is no difference between type-a QAV and type-b QAV with respect to the incidence of aortic regurgitation (AR). It is assumed that QPV is a risk factor for a Ross operation. However, QPVs have been used as autografts in certain patients.
Between QPV and QAV, various differences were found in frequency rates, diagnostic methods, valve morphology, valve function, associated congenital heart diseases, and frequencies of infectious endocarditis.