Congenital absence of the circumflex coronary artery. Clinical and cinearteriographic observations

Two patients found to have congenital absence of the circumflex coronary artery are reported. The clinical, electrocardiographic, vectorcardiographic, and cineangiographic findings are presented. No distinctive clinical or laboratory abnormalities were found except for the unusual anatomical posterior origin of the ostium of the main left coronary artery.     

Tetralogy of Fallot with absence of the pulmonary valve and the left branch of the pulmonary artery: echocardiographic diagnosis]

The absence of the pulmonary valve and the left pulmonary artery were recognized by cross-sectional and Doppler echocardiography in one patient with tetralogy of Fallot. The right oblique subxiphoid view was the best approach for visualization of these anatomical abnormalities. The diagnosis was confirmed at catheterization and at operation. It is suggested that premature closure of the ductus arteriosus may be responsible for the failure in the development of both the pulmonary valve and the left pulmonary artery.     

单纯性单侧肺动脉缺如的临床特点及回顾性分析

目的:提高对单纯性单侧肺动脉缺如(UAPA)的认识和早期诊治水平。方法:结合我院2009年经肺血管造影检查明确诊断的1例单纯性单侧肺动脉缺如的临床资料,和国内1999年至2009年文献检索到的13例单纯性单侧肺动脉缺如病例的详细资料,对UAPA的临床特点进行回顾性总结分析。结果:我院患者以胸闷憋气症状就诊,曾于外院误诊为"肺栓塞",行多排CT肺动脉造影(CTPA)、X线肺血管造影(CPA)最终确诊单纯性UAPA。总结14例病例后发现单纯性UAPA病例以成年人多见(10/14),女性8例,男性6例,左侧缺如6例,右侧缺如8例,主要症状为:咳嗽、咳痰、反复肺部感染(9/14),活动后胸闷、气促、喘息(9/14),咯血(3/14),最终以选择性肺血管造影检查和多排螺旋CT肺血管造影确诊病例为最多,分别为6例,5例。在8例有治疗资料中,对症治疗4例(4/14),手术治疗4例(4/14)。结论:UAPA是一种罕见的先天性肺血管畸形,单纯性UAPA更为少见,症状以咳嗽、咳痰、咯血、反复肺部感染、胸闷及气促为主。目前X线肺血管造影仍然是诊断UAPA的金标准。近几年多排螺旋CT肺血管造影、核磁共振(MRI)肺血管造影正逐渐成为诊断UAPA的重要手段之一。目前单纯UAPA的治疗方法主要以对症治疗为主,外科治疗可根据病情选择患侧肺动脉重建、选择性肺侧支血管栓塞和结扎、患侧全肺或肺叶切除术。     

Comprehensive management of branch pulmonary artery stenosis

Branch pulmonary artery stenosis may occur as an isolated finding or in conjunction with other cardiac malformations; it may be congenital or acquired postoperatively. Transcatheter management with balloon pulmonary angioplasty (BPA) and/or endovascular stenting is generally considered the procedure of choice for most patients. With the introduction of high-pressure balloons, the results of BPA have improved significantly (75%). Stent implantation has increased the success rate to over 90%. However, BPA continues to be the initial procedure of choice for distal stenoses, or those associated with branching points, and in small children or infants. Stent implantation is preferred over BPA for central or proximal stenoses, and those due to kinking or tenting, external compression, intimal flaps, failed balloon dilation, and early postoperative procedures. Certain lesions, such as supravalvar pulmonary stenosis, or stenosis at the branch pulmonary arteries bifurcation, are better dealt with at surgery. High-risk situations for the development of postoperative pulmonary artery (PA) stenosis exist in small infants with hypoplastic branch PAs after a conotruncal procedure, after duct ligation, after shunt insertion, after PA band placement, or after pulmonary arteriotomies or anastomoses of any kind. A combined collaborative transcatheter and surgical approach is essential for many patients with PA stenosis, particularly in complex forms of tetralogy of Fallot.     

Voluminous fistula between the right coronary artery and a branch of the pulmonary artery causing myocardial ischemia

A 76-year-old female patient was referred to our institution because of typical chest pain. A continuous murmur was audible at the lower sternal border. A transthoracic echocardiogram showed non-dilated right and left ventricles with mild left ventricular inferior wall hypokinesia and an exercise stress test was positive for myocardial ischemia. A coronary angiogram showed no signs of atherosclerotic coronary artery disease, but it revealed a voluminous fistula between the proximal segment of the right coronary artery and a branch of the pulmonary artery, which was percutaneously closed using 3 embolization coils. Such late presentation of a voluminous coronary fistula is extremely rare and, to the best of our knowledge, very few case reports like this have been published.     

Percutaneous closure of complex fistula between the internal mammary artery and a lobar branch of a pulmonary artery

A through review of the literature identified only 20 reported cases of fistula involving the internal mammary (internal thoracic) artery and a lobar branch of a pulmonary artery. Surgical closure was frequently done to avoid complications associated with this anomaly. We report the first patient in whom percutaneous treatment was accomplished with a combined technique involving an Amplatzer Duct Occluder device and coils.     

Juxtaposition of atrial appendages: angiocardiographic observations

The angiographic features of four cases of left-sided juxtaposition and one case of right-sided juxtaposition of the atrial appendages are described. The anomalous appendage was cephalad and dorsal to the normal position of the left atrial appendage and was larger than normal during atrial diastole; the appendage located on the anatomically correct side was caudad and its diastolic size was less than normal. The four cases of left-sided juxtaposition were all associated with transposition of the great vessels. Right-sided juxtaposition was detected inadvertently in a patient with a congenital obstructive lesion of the mitral valve. Left-sided juxtaposition can be demonstrated angiocardiographically by opacification of the right atrium, whereas right-sided juxtaposition can be seen by contrast injection into any cardiac chamber but the left ventricle, with certain exceptions.