Abnormal origin of the right pulmonary artery from the ascending aorta. Echocardiographic diagnosis apropos of 2 cases

Anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital malformation. Formerly identified at angiography and during surgery, it can now be diagnosed by two-dimensional echocardiography, as was done in the two cases reported here. The first case was a neonate in heart failure in whom echocardiography revealed the anomalous origin of the right pulmonary artery; this diagnosis was confirmed at post-mortem examination. The second case was a 4-month old infant in heart failure; echocardiography displayed the malformation which was confirmed at angiography and surgery. In both cases echocardiography showed discontinuity between the pulmonary trunk and the right pulmonary artery and continuity between the pulmonary trunk and the left pulmonary artery. There also was continuity between the right pulmonary artery and the ascending aorta. Doppler ultrasound showed a systolic and a diastolic flow in the right pulmonary artery and only a systolic flow in the left pulmonary artery. A colour-coded doppler examination in one case confirmed the anomalous origin of the right pulmonary artery and the absence of any other abnormality. Two-dimensional echocardiography combined with colour-coded doppler ultrasound therefore is the method of choice to diagnose anomalous origin of the right pulmonary artery from the ascending aorta.     

Retrograde pulmonary vein wedge angiography: A new technique

A new technique of pulmonary vein wedge angiography to delineate pulmonary artery anatomy is described. Conventional pulmonary vein wedge angiography requires an interatrial communication—natural or created by a transseptal puncture. In retrograde pulmonary vein wedge angiography, the left atrium and pulmonary veins are entered from arterial route using a specially designed catheter. This technique can be specially utilized for patients in whom there is no interatrial communication.     

Anomalous left coronary artery originating from the pulmonary artery. Report on 15 cases.

Fifteen infants and children with the diagnosis of anomalous left coronary artery from the pulmonary trunk have been encountered at the Children's Hospital Medical Center, Boston, Massachusetts from 1958 to 1973. After thorough clinical and laboratory evaluation, they have been treated by anticongestive measures. Nine patients have had ligation of the anomalous left coronary artery at its entrance into the pulmonary artery; one patient has undergone coronary bypass surgery. The lelctrocardiogram proved to be the most helpful diagnostic clinical laboratory test, Vectorcardiograms are valuable not only in diagnosis but also in the follow-up of the patients from the prognostic point of view. The most sensitive tool for the definitive diagnosis is an aortic rool angiogram; we have no false negatives or false positives with this method. The twelve patients with complete cardiac catheterization data could be divided into three groups, according to the pressure and magnitude of the left-to-right shunt at the pulmonary level. All patients with an appreciable le?T-TO-RIGHT SHUNT SURVIVED. Patients in whom no left-to-right shunt could be demonstrated by angiography died. Half of the patients with only small left-to-right shunt survived; The results of surgical and medical treatment, were identical within the three groups. Medical management in infancy, according to coronary care principles, with definitive surgical correction at a later age, is the preferred treatment. Ligation of the anomalous left coronary artery is recommended in severely symptomatic infants with documented left-to-right shunt at the pulmonary artery level, who do not respond to medical management.     

Patterns of anomalous pulmonary venous connection/drainage in hypoplastic left heart syndrome: diagnostic role of Doppler color flow mapping and surgical implications.

Differentiation between anomalous connection and anomalous drainage of the pulmonary veins in hypoplastic left heart syndrome is important before either the Norwood procedure or heart transplantation is performed. To determine the prevalence of echocardiographically detected anomalous connection or drainage, or both, of pulmonary veins in patients with this syndrome, preoperative two-dimensional echocardiographic and Doppler color flow mapping studies of 317 patients who underwent the stage I Norwood procedure were reviewed. The term "connection" was used to describe the precise anatomic attachment of the pulmonary veins and the term "drainage" to describe the physiologic end point of pulmonary venous flow. Twenty patients (6.3%) had anomalous connection or drainage, or both, of the pulmonary veins by preoperative echocardiographic and Doppler examination. The subcostal and suprasternal scans best showed the anatomic details of the pulmonary veins. All these patterns were confirmed intraoperatively and could be grouped as follows: 1) partial anomalous connection and drainage (two patients); 2) total anomalous connection and drainage (eight patients); 3) normal connection with total anomalous drainage (eight patients); and 4) normal connection with partial anomalous drainage (two patients). The advantage of adding Doppler color flow mapping to two-dimensional echocardiography and conventional Doppler study was clearly demonstrated in the detection of small accessory vertical veins, their course and the presence or absence of obstruction. Doppler color flow mapping was especially helpful in detecting anomalous drainage of the right pulmonary veins to the right of the superior attachment of the septum primum.     

The role of transesophageal echocardiography in the management of patients with acute and chronic pulmonary thromboembolism

Acute and chronic pulmonary thromboembolism carry high mortality. The role of transesophageal echocardiography (TEE), however, has not been well delineated in patients with suspected pulmonary thromboembolism. The aim of the present study was to demonstrate the value of Tee in patients with various clinical manifestations of pulmonary thromboembolic disease. Twelve patients--ten males and two females, age 47-85 years--are presented in whom central pulmonary thromboembolism was found by TEE. Six patients were referred for breathlessness and had moderate to severe pulmonary hypertension (PH) with (3) or without (1) right atrial thrombus or had right heart dilatation (1) or right ventricular myxoma (1) on transthoracic echocardiography (TTE). Thrombolysis (2), surgery (2), and heparin (2) treatment was performed without angiography. All but one patient recovered. Six patients had severe PH by TTE, one of them had a right atrial thrombus. Angiography was done in five patients in whom surgery was considered. Pulmonary thromboendarterectomy was successfully performed in two patients, it was contraindicated in two patients for advanced age or severe left ventricular dysfunction, both patients died during follow-up, and two patients were waiting for surgery. In conclusion: TEE has a definite role in the management of patients with acute pulmonary thromboembolism or in pulmonary embolism associated with right-sided intracardiac masses and in the selection of patients with PH for pulmonary thromboendarterectomy.     

应用再植技术治疗起源异常左冠状动脉的效果分析

目的:分析和讨论应用冠状动脉再植技术治疗先天性起源于肺动脉的左冠状动脉异常(ALCAPA)的外科治疗效果和经验。方法:回顾分析2008年4月~2013年4月26例应用冠状动脉再植技术治疗先天性ALCAPA患儿的临床资料。其中男14例,女12例;年龄4月~6岁。术前均经超声心动图,CT和或心血管造影检查明确诊断。结果:术前左室射血分数(LVEF)20~80(48±18)%,心胸比0.54~0.77(0.64±0.07)。并发二尖瓣关闭不全(MI)轻度以上21例,其中中度以上17例。左心室心尖部室壁瘤1例,房间隔缺损1例。同期行二尖瓣成形11例,室壁瘤切除1例,房间隔缺损修补1例。本组患儿手术均顺利完成,无手术死亡。体外循环时间91~238(150±37)min,心肌阻断时间64~200(126±36)min。随访1~61(29±19)月,患儿无远期死亡。最后一次随诊LVEF35~75(64±11)%,心胸比0.48~0.65(0.57±0.05),较术前明显改善。MI中度以下24例。结论:冠状动脉再植技术手术效果良好,对于中度以上MI应积极治疗。     

The noninvasive assessment of anomalous origin of the left coronary artery from the pulmonary artery

We present a patient with anomalous origin of the left main coronary artery from the pulmonary artery. We correlate the findings of echocardiography and myocardial imaging with angiography, and discuss the value of the noninvasive techniques in the diagnosis and in the followup of such patients.     

Left main coronary artery compression from pulmonary artery enlargement due to pulmonary hypertension: A contemporary review and argument for percutaneous revascularization

Extrinsic compression of the left main coronary artery by an enlarged pulmonary artery is an increasingly recognized and potentially reversible cause of angina and left ventricular dysfunction in patients with pulmonary hypertension. The diagnosis of extrinsic left main coronary artery compression requires a high index of suspicion and should be considered in patients with severe pulmonary hypertension who experience angina. Coronary angiography with intravascular ultrasound is the gold standard for diagnosis of this condition, though cardiac computed tomography and magnetic resonance angiography allow for noninvasive means of screening. The optimal treatment is debatable, but percutaneous coronary intervention appears to be a feasible, safe, and effective treatment option for patients with extrinsic compression of the left main coronary artery from pulmonary artery enlargement. Given the high risk of postoperative right ventricular failure and mortality observed with surgical revascularization in these patients, we recommend that physicians recognize percutaneous coronary intervention as the preferred revascularization strategy for selected patients with extrinsic compression of the left main coronary artery due to pulmonary hypertension. © 2010 Wiley‐Liss, Inc.     

Communication between right pulmonary artery and left atrium

The 12th case of a direct communication between the right pulmonary artery and the left atrium is reported, with special emphasis on the clinical and hemodynamic findings. The anatomic differences in the 12 cases are categorized into three general types: an anomalous vessel connecting the posterior aspect of the right pulmonary artery and draining into the left atrium with (1) normal pulmonary venous connections or (2) the pulmonary vein draining into the anomalous vessel; and (3) an anomalous communication draining into the left atrium in place of the absent right lower pulmonary vein. In patients with this anomaly, surgery should result in complete cure; early intervention is recommended because the mortality rate is higher for patients who are older at operation and because there is an increased chance that cerebral and Systemic emboli will occur if surgery is not performed.     

Anomalous origin of left coronary artery from pulmonary artery associated with pulmonary hypertension

This is a report on a 10-year-old child with anomalous origin of left coronary artery (LCA) from pulmonary artery (ALCAPA), severe pulmonary hypertension (PH), old myocardial infarction and poor intercoronary collateralization. It discusses the echocardiographic pitfalls in this particular setting and introduces a new echocardiographic view (posterior pulmonary cusp view) for visualization of the anomalous origin of LCA from the posterior pulmonary cusp (PC) in patients with ALCAPA from the PC of the pulmonary artery. We describe three echocardiographic pitfalls that can mislead the echocardiographer and two helpful hints that guide the clinician to the correct diagnosis.The survival of this child shows that limited size of left ventricular myocardial infarction and severe mitral regurgitation in early infancy can result in a life-saving pulmonary hypertension which preserves viability and function of left ventricle despite lack of intercoronary collateral arteries. After one year follow-up, she is doing well on medical treatment.     

Single left pulmonary vein with normal pulmonary venous drainage: Association with partial anomalous pulmonary venous return

An unusual case of a single left pulmonary vein draining the left lung and joining the left atrium without venous obstruction is reported. This anomaly occurred in association with partial anomalous pulmonary venous return from the upper lobe of the right lung to the superior vena cava. A similar case has been previously reported from our laboratory. The diagnosis of a single left pulmonary vein can be made by routine chest roentgenography because of the charácteristic radiologic appearance of this anomaly. It is important to distinguish this benign entity from more ominous pulmonary radiodensities.     

Partial anomalous origin of the left pulmonary artery

The pulmonary sling or aberrant left pulmonary artery has an incidence of 3% to 6% of all anomalies of the aortic arch system. We report a boy with coexistence of a normal and an anomalous left pulmonary artery. Associated congenital anomalies were coarctation, mitral stenosis, and imperforate anus. Cardiac catheterization and angiocardiography at the age of 5 months demonstrated an inconspicuous pulmonary trunk dividing into left and right branches. There was a normal right pulmonary artery, and a main left pulmonary artery supplying the left upper lobe, lingula, and anterior segments of the left lower lobe. In addition, an anomalous left lower lobe artery originated from the proximal right pulmonary artery, passed inferior to the tracheal bifurcation to the left supplying posterior segments of the left lower lobe. Chest X-rays and bronchoscopy could not detect any malformation of the trachea and pulmonary lobulation. There are only three reports of partial anomalous origin of the left pulmonary artery, and only one had a similar course of the accessory artery. This report is the first to present selective angiography and echocardiographic findings of the partial anomalous left pulmonary artery. The development of the partial anomalous pulmonary artery can be explained by the plexiform nature of the primary pulmonary vascular bed and can help to understand the embryology of the pulmonary arteries.     

Use of balloon occlusion to improve visualization of anomalous pulmonary venous return in an adult with cor triatriatum

Cor triatriatum, although rare in adults, is often associated with anomalous pulmonary venous return. Accurate diagnosis of the pulmonary venous return and its anatomic connections is essential in planning proper surgical correction. This case illustrates the advantage of selective pulmonary artery angiography in obtaining accurate detail of the pulmonary venous anatomy from digital subtraction levophase images. Selective right and left pulmonary angiography was performed using a balloon catheter to simultaneously occlude the opposite pulmonary artery. This technique was well tolerated and greatly enhanced visualization of the anomalous pulmonary venous connections, allowing proper planning for corrective surgery.     

Partial anomalous pulmonary venous return: case report and review of the literature.

Isolated partial anomalous pulmonary venous return (APVR) is an uncommon finding. A patient with isolated APVR had pulmonary hypertension without demonstrable left-to-right shunting prior to anticoagulant treatment of pulmonary emboli. After anticoagulant therapy, with a fall in pulmonary pressures and resistance to near-normal levels, left-to-right shunting was then detected by oximetry and angiography. The anomaly was visualized on electron beam angiography and confirmed by conventional angiography after anticoagulant therapy. Contrary to the expected obligatory drainage of highly saturated blood associated with APVR, lack of detectable shunting was thought to be due to the obstruction of regional blood due to thromboembolism.     

Echo Doppler detection of external compression of the vertical vein causing obstruction in total anomalous pulmonary venous connection

External obstruction of the pulmonary venous return was detected by two-dimensional (2-D) and pulsed Doppler echocardiography and was confirmed by angiography and at surgery in two infants with isolated supracardiac total anomalous pulmonary venous connection (TAPVC) to the left vertical vein. In both infants, the left vertical vein ascended behind the left pulmonary artery and anterior to the left main stem bronchus. This arrangement produces mechanical obstruction to both vertical vein flow and pulmonary venous drainage. Early detection and surgical correction of obstructed pulmonary venous return are necessary to prevent severe right-sided heart failure and death. Doppler echocardiography is recommended when evaluating infants with TAPVC for possible anatomic or mechanical obstruction to pulmonary venous return.     

Partial anomalous pulmonary venous connection and pulmonary arterial hypertension

Background and objective: Isolated partial anomalous pulmonary venous connection (PAPVC) has been implicated as a cause of pulmonary arterial hypertension (PAH); however this condition is often overlooked in the diagnostic work up of patients with PH. We studied the prevalence of PAH both in patients with isolated PAPVC or associated with other congenital heart diseases (CHD) such as atrial septal defect (ASD). We also aimed to identify factors related to the presence of PAH in these patients. Methods: We retrospectively analyzed data from the Adult CHD database at the Cleveland Clinic, USA between October 2005–2010. We included all patients diagnosed with PAPVC with or without other CHD. We excluded all patients with previous corrective surgeries. Results: We identified 14 (2.5%) patients with PAPVC. Group I included patients with PAPVC (with or without patent foramen ovale (PFO)). Group II included patients with PAPVC associated with other CHD. PAH was seen in six (6/14, 42.8%) patients, two (2/7, 28.5%) in group I and four (4/7, 57.1%) in group II (P = 0.3). The mean pulmonary artery pressure in all patients (n = 14) was 29.5 ± 13.8 mm Hg. Group I had a mean PAP of 23.6 ± 6.6 mm Hg as compared to 33.7 ± 16.5 mm Hg for group II (P = 0.34). The two patients in group I with PAH had either two anomalous pulmonary veins or a condition (sickle cell disease) that could potentially explain the haemodynamic findings. Conclusions: Patients with PAPVC (with or without PFO) in the absence of other CHD had normal pulmonary arterial pressure (PAP) unless they have two pulmonary veins with anomalous return or associated conditions known to cause PAH.     

Mixed variety of total anomalous pulmonary venous connection: diagnosis by 2D echocardiography and Doppler colour flow imaging.

Of the many types of total anomalous pulmonary venous connection, mixed type is the least common. Its accurate non-invasive diagnosis by echocardiography poses a diagnostic challenge. We report our experience of echocardiography in 21 infants with mixed type of total anomalous pulmonary venous connection aged 25 days to one and half years. Multiple windows were used to identify individual pulmonary veins and various sites of drainage. Cardiac catheterisation and angiography were performed for 17 cases. In 11 of 21 cases, the left upper pulmonary vein was seen draining into vertical vein and the left lower and right-sided pulmonary veins were draining into the coronary sinus. Cardiac and supracardiac combinations of other types were seen in eight more cases. Both drainage sites were supracardiac in one case and supracardiac and infracardiac in another. On comparing echocardiographic findings with those obtained at cardiac catheterisation and/or surgery (carried out in 18 cases), there were three instances of error. In two cases (echocardiography performed without the use of colour flow imaging) the second site of drainage could not be defined. These patients were catheterised as all four pulmonary veins were not delineated by echo. The third error occurred in a case where although two sites of drainage were picked up by echo but at surgery, the right lower pulmonary vein was noted to have a double connection, both to coronary sinus and to vertical vein. We conclude that mixed type of total anomalous pulmonary venous connection can be accurately diagnosed by echocardiography and Doppler colour flow imaging. The diagnostic errors are rare and would not alter the surgical management.     

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。     

Atypical Bland-White-Garland syndrome: a 58-year-old woman with stenosis of the pulmonary origin of the left coronary artery

The anomalous origin of the left coronary artery from the main pulmonary trunk (also known as Bland-White-Garland syndrome) is a rare congenital malformation that occurs in 0.4% of patients with cardiac anomalies. We present an adult case (a 58-year-old woman) of atypical Bland-White-Garland syndrome. The patient displayed a stenosis at the ostium of the anomalous origin of the left coronary artery and an aortopulmonary fistula. Using conventional angiography, it was not possible to differentiate between an anomalous origin of the pulmonary coronary artery and total stenosis of the left main coronary artery in combination with a pulmonary fistula. However, transesophageal echocardiography (TEE) succeeded in making this differential diagnosis. CONCLUSION: If there is subtotal or total occlusion, TEE can be used for detection of coronary vessel morphology, particularly in cases of coronary anomalies.     

Echocardiographic diagnosis of anomalous origin of the left coronary artery from the pulmonary artery

The objective of this study was to analyze echocardiographic characteristics of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) and to explore the diagnostic value of transthoracic echocardiography. The echocardiographic characteristics of 8 patients hospitalized with ALCAPA from 2000 to 2005 were analyzed retrospectively, including the results of real time three-dimensional echocardiography in one case, and compared with angiographic results. Eight cases included 6 older type patients and 2 infant type patients. Echocardiography showed abnormal vessel inserting into pulmonary artery (PA), continuous shunt into PA and intercoronary collateral signals within the ventricular septum in all cases and bifurcate structure of the abnormal vessel with retrograde filling in 4 cases. The morphological and functional changes and valvular regurgitation induced by insufficient myocardial perfusion were also evaluated. In former 4 patients, 2 cases were misdiagnosed as right coronary artery-PA fistula and the other 2 cases were given an uncertain diagnosis of anomalous origin of the coronary artery because of the visualization of the echo-free linear structure which apparently arose from the aorta resembling a normal left coronary artery. The latter 4 patients were correctly diagnosed by excluding the aforementioned interference. The diagnosis of ALCAPA was confirmed by angiocardiography in all patients and by intraoperative findings in 4 patients. Based on the apprehension of ultrasonic features and the enhancement of diagnostic alertness, the echocardiography can evaluate ALCAPA accurately and give more information than angiography. It may be the first diagnostic choice.