Unusual form of bilateral partial anomalous pulmonary venous drainage

A 23-year-old man with known valvular pulmonary stenosis was found to have a very unusual form of bilateral partial anomalous pulmonary venous drainage. The venous drainage from the upper parts of both lungs was anomalous, draining from the left lung via a persistent left-sided superior vena cava and from the right lung directly into the right atrial/right superior vena caval junction. The venous drainage from the lower parts of both lungs was normal. The embryological pathogenesis of the defect is discussed.     

Unusual pulmonary venous flow profile in a patient with mitral valve perforation secondary to bacterial endocarditis

Pulmonary venous flow patterns have been well described in the literature to assess severity of mitral regurgitation (MR) and the degree of diastolic dysfunction. We report a case of posterior mitral leaflet perforation due to bacterial endocarditis causing an alteration of pulmonary venous flow, not previously described in the literature. This pulmonary venous flow pattern is unique in that it reflects dynamic changes in left atrial pressure in the background of severe MR.     

完全性肺静脉异位引流的外科治疗

目的总结完全性肺静脉异位引流（total anomalous pulmonary venous drainage,TAPVD）的外科治疗经验。方法TAPVD27例（心上型15例,心内型12例）均在体外循环下手术。心上型6例采用传统双心房联合切口,9例采用心上法,后者经上腔静脉与升主动脉之间显露左心房与共同静脉干行-一侧吻合：心内型切开房间隔缺损与冠状静脉开口之间的残余房间隔壁,自体心包将肺静脉开口与冠状静脉窦隔入左心房。结果早期死亡2例,其他病人痊愈出院,术后随访3个月-4年,恢复良好,无吻合口狭窄发生,出院后无因心、肺衰竭死亡病人。结论TAPVD早期诊断极为重要,手术治疗的关键在于左心房与肺总静脉吻合口应足够大,避免狭窄的发生。     

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.     

Transcatheter therapy of partial anomalous pulmonary venous connection with dual drainage and coarctation of the aorta in a single patient

A 12-year-old boy was found to have aortic coarctation and a partial anomalous pulmonary venous connection. Historically, multiple cardiac pathologies, such as in the present case, required a surgical approach. We describe transcatheter treatment of the coarctation with a stent and occlusion of the partial anomalous pulmonary venous connection with an Amplatzer vascular plug in a single patient without complications.     

Evaluation of total anomalous pulmonary venous drainage with cross-sectional colour-flow Doppler echocardiography

Three patients with total anomalous pulmonary venous drainage(TAPVD) were studied by real-time cross-sectional colour-flowDoppler echocardiography. Serial suprasternal, parasternal andsubcostal scans were obtained. In all cases surgical or angiocardiographicconfirmation was available. Two patients had supracardiac drainage(to the left vertical vein or to the right superior vena cava)and one patient had infracardiac drainage. An abnormal forwardflow in the left innominate vein and vertical vein was visualizedin those patients with supracardiac TAPVD. Abnormal venous flowwas also imaged in one patient with mixed drainage. In the patientwith infradiaphragmatic TAPVD characteristic flow signals wereidentified in the inferior vena cava (retrograde flow) and inthe descending aorta and anomalous pulmonary venous channel(forward flow). In all patients the patterns of pulmonary venousflow allowed us to distinguish TAPVD from contiguous structuresand to validate two-dimensional cross-sectional imaging.     

Partial anomalous pulmonary venous drainage and intact atrial septum with mitral stenosis: the paradox of a small shunt

Clinical, hemodynamic, and angiographic features of 10 patients with partial anomalous pulmonary venous drainage, intact atrial septum, and rheumatic mitral stenosis have been presented. Seventeen patients with this combination of anomalies reported in the literature have also been reviewed. The clinical diagnosis of mitral stenosis was possible in each of our 10 cases. Partial anomalous pulmonary venous drainage and intact atrial septum in addition to mitral stenosis was clinically suspected in only four patients. The findings suggesting additional presence of partial anomalous pulmonary venous drainage and intact atrial septum in a patient with mitral stenosis include: wide variable splitting of the second sound, pulmonary ejection systolic murmur with or without a thrill, and radiological evidence of unilateral increase in pulmonary vascularity or unilateral hilar pulsations. Hemodynamic findings were characterized by a relatively modest left to right shunt (Qp/Qs:2.2 +/- 1.4). Elevated pulmonary vascular resistance was found in the anomalously draining as well as the normally draining lung segments (9.1 +/- 4.9 and 6.5 +/- 3.4 units, respectively, t = 1.32;NS). The mechanism of the unexpected high resistance in the anomalously draining lung segments permitting only a small left to right shunt is discussed.     

Partial anomalous pulmonary venous connection associated with Lutembacher's syndrome

Lutembacher's syndrome is a rare combination of congenital atrial septal defect and mitral stenosis (almost always rheumatic). The hemodynamic effect of this combination makes the clinical diagnosis difficult due to a wide variation of clinical presentation. Echocardiography and cardiac catheterization are useful tools for proper diagnosis and planning of the therapeutic strategy. We present a 28-year-old female with Lutembacher's syndrome associated with partial anomalous pulmonary venous connection (PAPVC), which was surgically corrected. To our knowledge, this combination has never been reported in the literature.     

Successful transcatheter occlusion of an anomalous pulmonary vein with dual drainage to the left atrium

We describe a case of a scimitar syndrome “variant” where dual drainage existed from the right upper and middle pulmonary veins to the inferior vena cava and left atrium. Device closure of the anomalous vein at the level of the connection to the IVC was successful in achieving diversion of pulmonary venous flow to the left atrium. Vigilance during work‐up of anomalous pulmonary venous drainage (whether isolated or associated with other cardiac defects that may be amenable to device closure) is important to define the presence of dual connections to the left atrium, in which case a less‐invasive transcatheter approach may be feasible. © 2015 Wiley Periodicals, Inc.     

Echocardiographic diagnosis of partial anomalous pulmonary venous connection from right upper lobe to the coronary sinus

A 36-year-old woman was admitted because of an enlarged right heart. Echocardiographic examination revealed an abnormal vessel connecting to the dilated coronary sinus. The abnormal vessel traveled in the direction from the right axillary to the left epigastric region. Partial anomalous pulmonary venous connection (PAPVC) from the right upper lobe to the coronary sinus was initially considered as a possible diagnosis by echocardiography. At surgery, diagnosis of an isolated PAPVC of the right upper pulmonary vein to the coronary sinus was confirmed.     

Severe aortic regurgitation and partial anomalous pulmonary venous connection in a Turner syndrome patient

Turner syndrome (TS) is one of the most common sex chromosome diseases. Short stature (if untreated) and ovarian dysgenesis (streak ovary) are two typical clinical manifestations of these patients. A variety of cardiovascular abnormalities has been found associated with TS. We report a 29-year-old TS patient with severe aortic regurgitation, bicuspid aortic valve (BAV) and partial anomalous pulmonary venous connection (PAPVC). We discuss the diagnostic and surgical management of cardiovascular complications in TS patients.     

The case of the missing pulmonary vein: A focused update on anomalous pulmonary venous connection in congenital cardiovascular disease

Partial anomalous pulmonary venous connection is defined by one or more of the pulmonary veins draining to the heart into a location other than the left atrium. Depending on the location of the anomalous venous connection, they can be categorized as supracardiac, infracardiac, cardiac, and mixed types. In some cases, there is no hemodynamic consequence; in others, it can result in tricuspid regurgitation, right heart dilation, and pulmonary hypertension. Frequently, the reason for referral can be asymptomatic right heart dilation of unknown significance. Diagnosis is often difficult by transthoracic echocardiogram unless there is a high index of suspicion, and the appropriate views are obtained. Cardiac CT (computed tomography) or cardiac MRI (magnetic resonance imaging) can provide more precise anatomic detail as needed. The current article reviews the etiology and pathophysiology of partial anomalous pulmonary venous connection, and also reviews the current knowledge on their treatment.     

Right lung cancer with partial anomalous pulmonary venous return and absent right upper lobe

When a vascular shunt is present, major lung resection may cause acute right heart failure. We report a case of right lung cancer with ipsilateral partial anomalous pulmonary venous return (PAPVR) and absent right upper lobe managed by pneumonectomy. A 48-year-old former smoker was diagnosed as right lung cancer; chest computed tomography (CT) revealed an anomalous right pulmonary vein draining into inferior vena cava. Bronchus of right upper lobe was not found in fiberoptic bronchoscopy. Right pneumonectomy was performed. Pathological examination revealed a T2aN2M0 squamous lung cancer. The patient tolerated well without notable descending of arterial oxygen pressure.     

Pulmonary valve vegetations and flail leaflet detected by cross-sectional echocardiography in infective endocarditis: A case report

A case report is presented of a patient with subacute bacterialendocarditis affecting the pulmonary valve. There was no pasthistory of valvular heart disease or narcotic addiction andno other valve was involved. Serial echocardiograms demonstratedthe vegetations, but only cross-sectional echocardiography showedthe flail movement of the leaflet and the size and shape ofthe vegetations.     

The echocardiographic diagnosis of totally anomalous pulmonary venous connection in the fetus

BACKGROUND—Infants with isolated totally anomalous pulmonary venous return often present severely decompensated, such that they are at high risk for surgical repair. On the other hand, if surgical repair can be safely accomplished, the outlook is usually good. Thus prenatal diagnosis would be expected to improve the prognosis for the affected child.
OBJECTIVE—To describe the features of isolated totally anomalous pulmonary venous drainage in the fetus.
DESIGN—Four fetuses with isolated totally anomalous pulmonary venous connection were identified and the echocardiographic images reviewed. Measurements of the atrial and ventricular chambers and both great arteries were made and compared with normal values.
SETTING—Referral centre for fetal echocardiography.
RESULTS—There were two cases of drainage to the coronary sinus, one to the right superior vena cava, and one to the inferior vena cava. Right heart dilatation relative to left heart structures was a feature of two cases early on, and became evident in some ratios late in pregnancy in the remaining two.
CONCLUSIONS—Ventricular and great arterial disproportion in the fetus can indicate a diagnosis of totally anomalous pulmonary venous connection above the diaphragm. However, in the presence of an atrial septal defect or with infradiaphragmatic drainage, right heart dilatation may not occur until late in pregnancy. The diagnosis of totally anomalous pulmonary venous drainage in fetal life can only be reliably excluded by direct examination of pulmonary venous blood flow entering the left atrium on colour or pulsed flow mapping.


Keywords: congenital heart disease; fetus; total anomalous pulmonary venous drainage; echocardiography     

Stent implantation as a stabilization technique in supracardiac total anomalous pulmonary venous connection.

A 6-week-old male presented in-extremis with obstructed supra-cardiac total anomalous pulmonary venous connection. Balloon dilation and stent implantation in the stenotic segment of the vertical vein relieved the obstruction until the patient was stable enough to undergo surgical repair. An unusual form of extrinsic vertical vein compression was found at surgery.