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 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.     

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.     

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.     

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.     

经左房顶部矫治心上型完全性肺静脉异位引流的体会

目的探讨经左房顶部径路矫治心上型完全性肺静脉异位引流的体会。方法2005年1月至2008年1月经左房顶部径路矫治心上型完全性肺静脉异位引流5例,女性3例,男性2例,年龄2-13（7．00±3．85）岁,在全麻体外循环下经左房顶部径路进行矫治。结果均治愈出院,随访0．5～2．5年,生长发育良好,无心律失常和吻合口狭窄。结论经左房顶部径路矫治心上型完全性肺静脉异位引流,手术方便,显露好,减少心律失常和吻合口狭窄的发生。     

Stenting of the vertical vein in obstructed total anomalous pulmonary venous return as rescue procedure in a neonate.

A newborn girl with progressive respiratory distress and cyanosis was found to have severely obstructed supracardiac total pulmonary venous return (TAPVR). Stenting of the stenosis provided immediate and effective relief of the obstruction until corrective surgery was performed. Catheter intervention should be considered in high-risk infants with obstructed supracardiac TAPVR as part of the strategy for preoperative cardiovascular stabilization.     

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.     

Hypoplastic left heart syndrome with premature closure of foramen ovale: Report of an unusual type of totally anomalous pulmonary venous return

Summary We report on a case of hypoplastic left heart syndrome (HLHS), associated with premature closure of the foramen ovale and an unusual type of totally anomalous pulmonary venous return. The existence of an anomalous connection of the right upper pulmonary vein to the superior vena cava-right atrial (SVC-RA) junction and the existence of the anomalous intrapulmonary venous channel between right upper and lower pulmonary vein allowed all the pulmonary blood to drain into the SVC-RA junction, whereas she had only partially anomalous pulmonary venous connection. The several intrapulmonary venous channels helped to delay the progression of pulmonary venous obstruction. Chromosomal analysis revealed that the patient had XO Turner syndrome. We conclude that all infants with HLHS should be carefully evaluated for the existence of anomalous pulmonary venous return. Two-dimensional Doppler echocardiography is one of the most useful techniques for evaluating such anomalies.     

经胸和经食道超声心动图评价成人部分型肺静脉异位连接

目的通过经胸和经食道超声心动图对研究对象进行全面检查,探讨部分型肺静脉异位连接（partial anomalous pulmonary venous connection,PAPVC）的解剖学特征及其与手术的相关性。方法对2008年6月至2011年7月36例（男12例,女24例,年龄15—59岁,中位年龄37岁）经外科手术证实的先天性PAPVC患者的临床资料进行回顾性分析,所有患者均行经胸超声心动图检查。27例进一步行经食道超声心动图检查,所得诊断结果与外科手术诊断结果进行比较。结果36例PAPVC均为右肺静脉异位连接,其中两条右肺静脉21例（58．3％）,单纯右上肺静脉11例（30．5％）,单纯右下肺静脉4例（11．1％）。合并静脉窦型缺损33例（91．6％）。经胸及（或）经食道超声心动图联合检查,与手术结果对比,正确诊断PAPVC31例（86．1％）。结论超声心动图在对PAPVC的诊断中起到重要作用,为外科术前提供足够的信息,可作为PAPVC诊断的首选检查方法。     

Multiple ventricular septal defects associated to anomalous venous returns,mitral valve disease,myocardium hypertrophy,and right outflow obstruction: a multimodality imaging assessment

We report a rare congenital heart disease characterized by multiple ventricular septal defects associated to anomalous systemic and pulmonary venous returns, marked apical myocardial hypertrophy of both ventricles and of right outflow, and hypoplastic mitral anulus. Multimodality imaging is mandatory to assess anatomical details.     

Echocardiographic evaluation of total anomalous pulmonary venous connection: Comparison of obstructed and unobstructed type

This study aims to compare the differences between obstructed and unobstructed total anomalous pulmonary venous connection (TAPVC) using echocardiography, and to evaluate the clinical and echocardiographic parameters associated with pulmonary venous obstruction (PVO).We conducted a retrospective study of 70 patients with TAPVC between 2014 and 2019. The morphologic and hemodynamic echocardiographic parameters of patients were observed and measured, and the parameters between obstructed and unobstructed TAPVC were compared. The clinical and echocardiographic parameter differences between the two groups were used for ROC curve analysis.Obstructed TAPVC was found in 30 (42.9%) of 70 patients. Between obstructed and unobstructed TAPVC, there were significant differences in atrial septal defect size, pulmonary artery maximum velocity (PA V_max ), peak E velocity of mitral valve, left ventricular fractional shortening, left ventricular ejection fraction, stroke volume and the incidence of patent ductus arteriosus, but there was no significant difference in birth weight. The first diagnosis age of obstructed TAPVC was earlier than unobstructed type. The ROC curve analysis for the first diagnosis age showed the sensitivity and specificity were 76.7%, 80% respectively. The ROC curve analysis for the PA V_max showed the sensitivity and specificity were 88.5%, 67.6% respectively.Patients with TAPVC had a high incidence of PVO. The presence of PVO can affect the size of atrial septal defect and the closure of the ductus arteriosus, cause significant changes in PA V_max, peak E velocity of mitral valve, left ventricular fractional shortening, left ventricular ejection fraction, stroke volume, lead to earlier symptoms and earlier first diagnosis age. The first diagnosis age and PA V_max were excellent values since they associated with PVO.     

A Case of an Anomalous Superior Vena Cava with Anomalous Pulmonary Veins—When Two Wrongs Do not Make a Right

Intravenous agitated saline injection is useful in identifying right‐to‐left shunting at the atrial or intrapulmonary level. Anomalous systemic venous drainage to the left atrium is a rare but easily correctable cause of right‐to‐left shunting which, if left undiagnosed, may have serious consequences, including meningitis and pyogenic brain abscesses. This case illustrates an unusual cause of right‐to‐left shunting and the utility of venous microbubble injection in its diagnosis. (Echocardiography 2011;28:E39‐E41)     

Abnormal venous connection between the left upper pulmonary vein and the left brachiocephalic vein,associated with rheumatic combined valvular heart disease

Summary A case of partial anomalous pulmonary venous return (PAPVR) associated with mitral stenosis and aortic regurgitation is described. The diagnostic clue was radiocardiography using radioiodide serum albumin (RISA), our routine procedure before cardiac catheterization. The abnormal vessel connected with both the left upper pulmonary vein (PV) and the left brachiocephalic vein, without a stenotic lesion. Aortic valve replacement, open mitral commissurotomy, and simple ligation of the anomalous vein were successfully performed.     

53岁心内型完全性肺静脉异位引流手术1例及文献回顾

完全性肺静脉异位引流(total anomalous pulmonary venous connection, TAPVC)是一种罕见的紫绀型先天性心脏病,缺氧和充血性心力衰竭可导致明显症状,通常患者在出生后几个月内需要手术矫正,成人病例在临床上比较少见,尤其是生存至中老年更是罕见。我们成功为1例53岁心内型TAPVC合并房间隔缺损、肺动脉高压患者实施手术。     

53岁心内型完全性肺静脉异位引流手术1例及文献回顾

完全性肺静脉异位引流(total anomalous pulmonary venous connection, TAPVC)是一种罕见的紫绀型先天性心脏病,缺氧和充血性心力衰竭可导致明显症状,通常患者在出生后几个月内需要手术矫正,成人病例在临床上比较少见,尤其是生存至中老年更是罕见。我们成功为1例53岁心内型TAPVC合并房间隔缺损、肺动脉高压患者实施手术。     

Two‐ and four‐dimensional echocardiography with high‐definition flow imaging and spatiotemporal image correlation in the diagnosis of fetal isolated partial anomalous pulmonary venous connection

Partial anomalous pulmonary venous connection (PAPVC) is a rare malformation. We describe a case of PAPVC, in which the left pulmonary veins coursed to the left innominate vein through a vertical vein and finally drained into the right superior vena cava; the right pulmonary veins were connected to the left atrium. Tracing the origin and destination of abnormal vessels presented at the three‐vessel and trachea view is useful for the diagnosis. Four‐dimensional echocardiography with high‐definition flow imaging and spatiotemporal image correlation facilitates the identification of the drainage of fetal pulmonary veins, which should be considered as a complementary modality in obstetric ultrasonic examination when cardiac abnormalities are suspected.