Left Vertical Vein Research Articles

Persistent left vertical vein: An unusual cause of pulmonary hypertension and cirrhosis in a patient with hypertrophic obstructive cardiomyopathy.

The persistent vertical (levoatriocardinal) vein is an abnormal, persistent connection between the pulmonary venous system and the systemic venous system (1). This variation accompanies total pulmonary venous return anomaly and rarely with pathologies such as atrial septal defect, ventricular septal defect, and mitral stenosis (1–3). Its association with hypertrophic obstructive cardiomyopathy (HOCM) has been shown in only one case (4). In this case report, we have presented the interventional treatment of both the pathologies in the same session.

Meandering vessel on a chest radiograph in a cyanotic neonate: rare presentation of intrapulmonary course of right vertical vein in patients with supracardiac total anomalous pulmonary venous connection.

In most cases of supracardiac TAPVC typically there is confluence of pulmonary veins behind the heart on left side, constituting a left vertical vein which joins the left brachiocephalic vein thus establishing systemic communication (1).

Successful Treatment of a Case of Partial Anomalous Pulmonary Venous Connection (PAPVC) with Interventional Transcatheter Closure

BackgroundPartially anomalous pulmonary venous connection (PAPVC) is a rare type of congenital cardiovascular disease in which part of the pulmonary veins connects to the right atrium or its tributaries. Traditionally, when patients have evidence of blood drainage pathway, multiple malformations, and/or significant left‐to‐right shunt, treatment has always required a hypothermia by‐pass thoracotomy under general anesthesia. Invasive surgical treatment of these conditions in the elderly patients leads to high risk for complications and slow recovery. The authors report the successful treatment of a PAPVC patient with interventional transcatheter to eliminate the left‐to‐right shunt, decrease overloads of right heart and further avoid the sinoatrial node dysfunction by surgery.MethodsA 55‐year‐old female patient was admitted due to a complaint of intermittent chest tightness, chest pain, shortness of breath, one year of radiating pain to the right back and deterioration in the two months prior. Computed tomography angiography (CTA) revealed upper left pulmonary vein drainage into left brachiocephalic vein and dilation of right heart; therefore, a PAPVC was suspected. Ultrasound indicated decrease of left ventricular diastole function ( III) moderate mitral regurgitation, with no evidence of an intracardiac shunt. Digital subtraction angiography (DSA) revealed a contrast drainage from left upper pulmonary vein into left subclavian vein via an ascending vertical vein, and some contrast flowed into left ventricle. Amplatzer device of 16mm was deployed via a long 10 French sheath and precisely released into the bifurcation of the left brachiocephalic vein and vertical vein to block the persisting vertical vein. Contrast injected into left subclavian vein showed successful occlusion of vertical vein. Contrast drain into right atrium through left brachiocephalic vein and superior vena cava.ResultsThe PAPVC patient was found with the upper left pulmonary vein drainage into left subclavian vein via an ascending vertical vein, the pulmonary vein drainage into left atrium and a partial left‐to‐right shunt. After carefully reviewed the results of examination, the vertical vein was occluded with interventional transcatheter. There was no arrhythmia, intraoperative and postoperative complaints. The symptoms of chest tightness and chest pain were resolved completely. Patient was discharged from the hospital three days after surgery. Ultrasound examination two weeks later showed normal diastolic function of left ventricle, no mitral regurgitation, and no vertical vein shunt. Patient had no complaints during postoperative check at 2 months. Follow up ultrasound at 3 months after surgery showed no evidence of pulmonary artery hypertension.ConclusionsFor the elderly and PAPVC with many complications, minimally invasive surgery such as catheter intervention is an alternative approach. A case of PAPVC patient was successfully treatment with an Amplatzer ductal occlusion device to obstruct the vertical vein between the pulmonary circulation and systemic circulation instead of using a thoracotomy surgery. This device appears to be an appropriate choice of intervention in such unusual cases requiring venous occlusion.This abstract is from the Experimental Biology 2018 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.

Unusual Form of Partial Pulmonary Venous Connection

Success in congenital heart surgery has increased with improvement in surgical technique, anesthesia, and perioperative care. Transthoracic echocardiography (TTE) is regarded as an initial screening and diagnostic method in patients, but it may be limited by lack of an adequate acoustic window and more importantly suboptimal depiction of the extracardiac vasculature. Computed tomography angiography is an important alternative because of its wide availability; short acquisition time can also provide useful information about all anatomical structures. In this article, we present a case of bilateral partial anomalous pulmonary venous connection in which TTE is inadequate to show the left vertical vein.

Prenatal diagnoses of an uncommon isolated obstructed supracardiac total anomalous pulmonary venous connection

Introduction:Total anomalous pulmonary venous connection is an uncommon congenital heart disease. Four types are described based on the site of pulmonary venous drainage: supracardiac, cardiac, infradiaphragmathic, and mixed connection. In most cases of supracardiac type, the common venous confluence drains through an ascending left vertical vein into the brachiocephalic vein, right superior vena cava, and then into the right atrium. Anomalous connection of the pulmonary venous confluence directly to the right SVC, especially the obstructed form is an unusual and severe supracardiac variant. The prenatal diagnosis is challenging.Patient concerns:We present a case report of a fetus diagnosed with TAPVC at 23 gestational weeks.Diagnosis interventions:The 4-chamber view showed a small left atrium, with a “smooth” posterior wall and the absence of pulmonary vein connection. This is the first case of prenatally diagnosed isolated, obstructed supracardiac type with drainage directly into the right superior vena cava.Conclusion:The obstetrician and fetal cardiologist should be cautious at the direct and indirect echocardiographic signs of this condition. A prenatal diagnose of isolated, obstructed form is important for adequate planning of delivery and postnatal surgery in a tertiary center.

Abstract 12818: First Experience of Three-dimensional Rotational Angiography Image Integration With Electroanatomical Mapping System to Guide Catheter Ablation of Atrial Fibrillation

Introduction: Rotational angiography of the left atrium with three-dimensional (3D) reconstruction (3D-ATG) represents a modern method enabling to create computed tomography (CT) like 3D images on a standard X-ray machine. Recently, electroanatomical mapping system can import 3D images reconstructed by 3D-ATG. Hypothesis: We assessed the hypothesis that atrial fibrillation (AF) ablation using 3D-ATG would be feasible with a significant reduction in effective dose without compromising image quality compared with 3D CT image. Methods: 3D-ATG was performed by using the Philips Allura Xper FD 10 system operated at a low-frame pulsed fluoroscopy (7.5 frames per second). Effective radiation dose was calculated from dose area product (DAP) measurements in 103 patients (mean age of 65 years, 71% men) undergoing AF ablation guided by 3D-ATG. Organ dose was measured at 37 points with a radiophotoluminescence glass dosimeter inserted in the position of an anthropomorphic Rando Phantom. Effective dose was calculated by multiplying organ dose by tissue weighting factor. The DAP to effective dose conversion factor was calculated by measurement of DAP at the same time of radiation exposure. Effective dose at CT examination was estimated from dose length product and conversion factor of 0.014. Left atrial dimensions and vertical ostial pulmonary vein (PV) diameters for each imaging method were compared. Results: The DAP to effective dose conversion factor of 3D-ATG was 2.4x10 -4 mSv/mGy•cm 2 by using the Philips Allura Xper FD 10 system in our hospital. Mean DAP for all patients was 7777±1488mGy•cm 2 for rotational angiography of the left atrium. The corresponding effective radiation doses for 3D-ATG were 1.9±0.4mSv. The effective doses for CT examinations were 13.6±4.2mSv (p<0.001). The correlations of left atrial dimension were r=0.73 for sagittal plane, 0.76 for coronal, and 0.80 for vertical (p<0.005). The correlation coefficient between 3D-ATG and 3D-CT for the ostial PV diameters was r=0.72 for left superior PV, 0.88 for left inferior PV, 0.60 for right superior PV, and 0.65 for right inferior PV (p<0.005). Conclusions: AF ablation using 3D-ATG is possible with a significant reduction in effective dose without compromising image quality.

Surgical treatment of infra-cardiac total anomalous pulmonary venous connection in 13 neonates

Objective To evaluate the effectiveness of urgent surgical correction for infra-cardiac total anomalous pulmonary venous connection(TAPVC) in neonates. Methods From October 2009 to January 2015, 13 patients with infra-cardiac TAPVC received surgical correction.The age of patients ranged from 6 to 28 days[(15.08±7.42)days], the body weight ranged from 2.5 to 4.8 kg[(3.34±0.67) kg]. A median sternotomy was performed.Continuous cardiopulmonary bypass using bicaval cannulation with aortic cross-clamping and mild systemic hypothermia were used in all patients.Bi-atrial incision technique making anastomosis between the posterior left atrial wall and the vertical vein were employed in 3 cases.In the other 10 cases, the heart was elevated upward and to the right to expose the anomalous descending vertical vein to facilitate the anastomosis between the posterolateral left atrial wall and vertical vein. Results Emergent or subemergent operations were performed in all patients without surgical death.In the early stage after operation, delayed sternal closure was employed in 11 patients, pulmonary hypertension crisis occurred in 3 patients, small atrial septal defect was kept open for hemodynamic stabilization in 6 patients, temporary cardiac pacemaker for proper heart rate in 3 patients.All patients were uneventfully discharged except 2 patients died of severe low cardiac output syndrome.During the period of follow up, 2 patients presented with recurrent pulmonary infection and signs of pulmonary venous obstruction including the increased flow speed at the site of anastomosis.One of 2 patients was lost of follow up and the other patient was uneventfully discharged after the second operation.The rest 9 patients received postoperative follow up for 1 month to 5 years, echocardiography, X ray chest radiography, and electrocardiogram were performed during this period.As a result, all children had good cardiac function and with sinus rhythm on electrocardiogram and apparently reduced pulmonary congestion on radiography.No obstructive pulmonary venous return was observed on echocardiography. Conclusion Corrective operation for infra-cardiac TAPVC in neonate on urgent basis may provide favorite outcomes. Key words: Anomalous pulmonary venous connection; Cardiac surgical procedures; Congenital heart disease; Neonate

Successful endovascular repair of an unusual right-to-left shunt presenting with cerebral ischemia

Ischemic stroke due to congenital cardiopulmonary vasculature anomalies is rare in adults. We report a 54-year-old man with a stroke due to a unique right-to-left shunt who underwent successful endovascular treatment. This patient developed acute onset of right arm weakness with facial droop and aphasia which improved after intravenous thrombolysis. An MRI showed acute cerebral ischemia in the left middle cerebral artery and left posterior cerebral artery distribution. The patient developed recurrent stroke symptoms during agitated saline injection while undergoing a transthoracic echocardiogram which showed right-to-left shunting. Chest CT scan and conventional angiography revealed near occlusion of the superior vena cava. Head and upper limb venous return drained via a large left vertical vein into an anomalous left pulmonary vein into the left atrium. He underwent endovascular surgery to relieve the superior vena cava obstruction and to occlude the source of right-to-left shunt. While rare, congenital cardiopulmonary vascular anomalies may result in ischemic stroke in adults. CT angiography may be necessary to evaluate cardiopulmonary vasculature when right-to-left shunting is discovered on echocardiography in the setting of ischemic stroke. With large right-to-left shunts, agitated saline should be avoided.

Percutaneous closure of an anomalous vertical vein

We report the case of a 39-year-old male with a history of untreated arterial hypertension. He presented to a community hospital with pulmonary edema and anasarca, and was transferred to our heart center for further investigation and therapy. Echocardiography revealed the enlargement of all four heart chambers including a severely compromised systolic function of both ventricles. Coronary angiography ruled out coronary artery disease. Computer tomography of the thorax was performed to exclude pulmonary embolism. Surprisingly, an anomalous left vertical vein was found, connecting left upper pulmonary veins to the left innominate vein as well as to the left atrium (Fig. 1a). Magnetic resonance tomography confirmed the diagnosis of this anatomical large left partial anomalous pulmonary venous connection (PAPVC) with a pulmonary stroke volume of 86 ml and a systemic stroke volume of 68 ml. The resulting left-to-right shunt was relatively small with a pulmonary to systemic cardiac index (Qp:Qs) of just 1.26 and a shunt stroke volume in the vertical vein of 12 ml. Furthermore, a mild coarctation of the aorta and a minimal late enhancement of the left ventricle could be demonstrated, the latter finding was consistent with hypertensive heart disease. Right heart catheterization revealed moderate pulmonary hypertension with a mean pulmonary pressure of 34 mmHg and a mean pulmonary capillary wedge pressure of 29 mmHg. We decided to close the vertical vein percutaneously. After retrograde intubation of the vertical vein, repetitive angiograms at various heights of the vein were performed to define size and target zone of the closure device. Thereby, a further connection to the right atrium via the accessory hemiazygos vein was found, explaining the missing 6 ml of calculated shunt stroke volume (Fig. 2a). Afterwards, this finding of a strictly posterior originating additional connection could be confirmed by re-evaluation of the initial CT scan (Fig. 1b, c). We decided to use an 18-mm Amplatzer vascular plug II (St. Jude Medical, St. Paul, MN, USA) to occlude both anomalous connections. After positioning the Amplatzer TorqVue 2 Delivery Sheath (St. Jude Medical) in the left atrium, the vascular plug II was advanced. While pull-back of the sheath the plug was positioned in the target zone, which was the distal vertical vein at the orifice of the accessory hemiazygos vein, just above the orifice of the left pulmonary veins. After deployment of the device, there was no more washout into the left innominate vein: neither from the accessory hemiazygos vein nor from pulmonary veins (Fig. 2b), and a pulmonary angiogram confirmed successful closure of the anomalous vertical vein also. Fetal venous circulation is very variable and complex. During normal development of the human fetal venous system the left supracardinal vein connects the accessory hemiazygos vein and the left atrium with the left innominate vein. Normally, this vein occludes before birth. However, in cases of left PAPVC, one or more left pulmonary veins can drain into the left innominate vein via a Electronic supplementary material The online version of this article (doi:10.1007/s00392-013-0597-7) contains supplementary material, which is available to authorized users.

Novel catheter‐interventional strategy for intracardiac connecting of total anomalous pulmonary venous return in newborns with hypoplastic left heart‐syndrome prior to hybrid approach

Total anomalous pulmonary venous return (TAPVR) associated with hypoplastic left heart-syndrome (HLHS) is a rare condition, and from the therapeutical point of view associated with a high Aristotle score and thus increased mortality. We report two newborns with HLHS, one with a supracardiac type of TAPVR and mildly obstructed left vertical vein, and the other with a supracardiac type of TAPVR in association with cor triatriatum and severely obstructed left-sided vertical vein. In both patients, radio frequency perforation from the pulmonary venous confluence to the systemic venous atrium was performed with consecutive gradual balloon dilatation, followed by stent placement in one. Hybrid stage I, and comprehensive stage II were successfully performed thereafter. Currently, both are awaiting their Fontan completion. Transcatheter intracardiac connecting of supracardiac type of TAPVR in newborns with HLHS is feasible and might render these children suitable candidates for further hybrid approach.

Right to left shunting through communications between the left superior intercostal vein tributaries and the left atrium: A potential cause of paradoxical embolism

ObjectivesTo investigate the role of collateral venous pathways between the left brachiocephalic vein (LBV) and the left atrium through an arcade comprising the left superior intercostal vein (LSICV), left vertical vein (LVV), and pulmonary veins as a potential cause of paradoxical embolism. MethodsA retrospective search was performed to find symptomatic patients with negative work up for paradoxical emboli whose chest CT or MR angiography by left arm contrast injection showed a visible right to left shunt through the LSICV/LVV collateral pathway (symptomatic group). We also evaluated the characteristics of this collateral pathway in 150 chest CT angiographies from general referrals (comparison group). ResultsWe found 7 symptomatic patients. Initial presentations included neurological symptoms in all patients and episodes of hypoxemia in three patients. Communications between the LBV and left atrium through the LSICV/LVV pathway was seen is all 7 cases and confirmed by transesophageal echocardiography in two. An abnormal LBV was seen in 6 cases (absence in one, stenosis in 5). Moderate superior vena cava stenosis was seen in one and acute right subclavian vein thrombosis in one. Six patients had past history of an upper extremity central line placement. In the comparison group, LSICV was seen in 73 (48%), connecting to the hemiazygos vein in 41, to a LVV in 19 and to the left atrium in 7. ConclusionLSICV/LVV collaterals are common and can be a potential route for paradoxical emboli especially when stenosis of a draining vein such as LBV exists.

Tc-99m MAA Findings in Dilated Cardiomyopathy With Partial Anomalous Venous Connections

Tc-99m MAA showed asymmetric uptake in the lung field in a 21-year-old man with dilated cardiomyopathy. CT revealed partial anomalous venous connections in the left upper lobe. Angiogram of the left pulmonary upper lobe showed all the contrast material drained into the left vertical vein. The possible cause of relative increase in the left upper lobe blood flow is that right pulmonary blood flow is slowed by the high pressure in the left atrium due to dilated cardiomyopathy, whereas the flow from the left upper lobe drains into the superior vena cava which has less pressure than left atrium.

Partial anomalous pulmonary venous return: more common from the left or right lung?

Dear editor, I read with great interest ‘‘Anatomical study of partial anomalous pulmonary venous return with special references to the bronchial vein: supplementary report’’ (Okamoto et al. 2008). They describe an anomalous right upper lobe vein draining into the superior vena cava. They note that this is the most frequent type of partial anomalous pulmonary venous return. While previous studies corroborate this statement (Brody 1942; Snellen et al. 1968), a more recent study examining the prevalence of partial anomalous pulmonary venous return using computed tomography provides conflicting data. In this study, 79% had an anomalous left upper lobe vein draining into a persistent left vertical vein, 17% had a right upper lobe vein draining into the superior vena cava, and 3% had a right lower lobe vein draining into the suprahepatic inferior vena cava (Haramati et al. 2003). This difference in prevalence may partially be due to easier detection of left upper lobe anomalous veins than right upper lobe anomalous veins with computed tomography (Haramati et al. 2003). However, the patient population studied may also be a factor. Prior studies reporting partial anomalous pulmonary venous return were mostly autopsy series or surgical reports of a young population with cardiac defects. These data may not apply to the adult population. The recent study utilizing computed tomography was performed in an adult population, and suggests that the characteristics of partial anomalous pulmonary venous return may differ in pediatric and adult populations.

Imaging Features of the Total Anomalous Pulmonary Venous Connection to the Portal Vein Shown by Multidetector Computed Tomography Angiography

A 2-month-old girl with ventriculoseptal defect and pulmonary hypertension (PHT) presented with respiratory distress and coughing. Peroral cyanosis, tachypnea, dyspnea, growth retardation, systolic murmur, crepitant rales in both lungs, and hepatomegaly were discovered at the physical examination. A chest roentgenogram showed a normal-sized heart and mild venous congestion. Echocardiography exhibited a small perimembranous ventriculoseptal defect. The pulmonary veins were draining into the portal vein through a vertical vein. Angiography showed drainage of the pulmonary veins into a portal vein via a vertical vein. The superior vena cava and right atrium were filling via an innominate vein. Multidetector computed tomography (MDCT) angiography showed that the right and the left pulmonary veins were joined at the anterior side of the proximal descending aorta, forming the vertical vein. The latter connected to the portal confluens, with the splenic vein and superior mesenteric vein below the diaphragm (Figs. 1 and 2). There also was hepatomegaly, but no congenital anomaly of the pulmonary arteries and lungs was observed. Despite conservative therapy, the girl died in 2 weeks because of congestive heart failure and pneumonia. The infradiaphragmatic total anomalous pulmonary venous connection (TAPVC) is almost always obstructed or narrowed when it passes through the diaphragm. However, no obstruction was observed in the reported case. Because no congenital anomaly was found in the lungs or pulmonary arteries and because the pulmonary venous return was not partial, ‘‘scimitar syndrome’’ was easily excluded. The snowman figure seen on the chest radiographs is due to enlargement of the left vertical vein and superior vena cava and characteristic of type 1 TAPVC. In contrast, other types of TAPVC show nonspecific roentgenographic findings. Nonobstructive types include cardiomegaly with right atrial and right ventricular enlargement, an enlarged pulmonary vein, and increased pulmonary flow. Obstructive types demonstrate

Third Left Pulmonary Vein with Abnormal Return Associated with Arteriovenous Fistula

Anomalies of pulmonary veins are uncommon and vary widely in their anatomic spectrum and clinical presentation. A 20-year-old woman with complaints of effort-induced dyspnea and easy fatigability was diagnosed with a third left pulmonary vein with abnormal return and arteriovenous fistula accompanied by a secundum atrial septal defect (ASD). Complete surgical repair was performed by ASD closure with a pericardial patch and triple ligation of the left vertical vein and associated third pulmonary vein. The patient was discharged on the seventh postoperative day in good health. Her last control examination was performed in the second postoperative year, revealing normal echocardiographic findings with an excellent clinical course.

Computed tomography of partial anomalous pulmonary venous connection in adults.

To systematically describe the imaging features and clinical correlates of a partial anomalous pulmonary venous connection diagnosed on computed tomography (CT) in adults. Twenty-nine adults with a partial anomalous pulmonary venous connection on CT were retrospectively identified. There were 19 women and 10 men, with a mean age of 53 (range: 19-83) years. Four cases were identified by review of 1825 consecutive chest CT reports from July 2000-July 2001, and 25 cases were culled from chest radiology teaching files at 3 institutions. Inclusion criteria were availability of CT images and medical charts. Chest radiographs (25 of 29 cases) were reviewed for mediastinal contour abnormalities, heart size, and pulmonary vascular pattern. Chest CT scans were reviewed for location, size, and drainage site of the anomalous vein; presence or absence of a pulmonary vein in the normal location; cardiac size and configuration; and pulmonary vasculature. Charts were reviewed for evidence of pulmonary and cardiovascular disease, history of congenital heart disease, and results of other cardiac imaging. The prevalence of a partial anomalous pulmonary venous connection was 0.2% (4 of 1825 chest CT reports). Seventy-nine percent (23 of 29 patients) had an anomalous left upper lobe vein connecting to a persistent left vertical vein, only 5% (1 of 23 patients) of whom had a left upper lobe vein in the normal location. Seventeen percent (5 of 29 patients) had an anomalous right upper lobe vein draining into the superior vena cava, 60% (3 of 5 patients) of whom also had a right upper lobe pulmonary vein in the normal location. One patient (3%) had an anomalous right lower lobe vein draining into the suprahepatic inferior vena cava. Chest radiographic findings were abnormal left mediastinal contour in 64% (15 of 25 patients), abnormal right mediastinal contour in 8% (2 of 25 patients), and cardiomegaly in 24% (6 of 25 patients). Computed tomography findings were cardiomegaly in 48% (14 of 29 patients), right atrial enlargement in 31% (9 of 29 patients), right ventricular enlargement in 31% (9 of 29 patients), and pulmonary artery enlargement in 14% (4 of 29 patients). Pulmonary or cardiovascular symptoms were present in 69% (20 of 29 patients), 55% (11 of 20 patients) of whom had specific alternative diagnoses (excluding congestive heart failure and pulmonary hypertension) to explain the symptoms. Only 1 patient (3%) was diagnosed with a secundum atrial septal defect. A partial anomalous pulmonary venous connection was seen in 0.2% of adults on CT. In contrast to previous series focusing on children, the anomalous vein in adults was most commonly from the left upper lobe, in women, and infrequently associated with atrial septal defects.

Dysphonia: a frequently encountered symptom in the evaluation of infants with unobstructed supracardiac total anomalous pulmonary venous connection.

Total anomalous pulmonary venous connection is a relatively uncommon congenital cardiac anomaly. When there is obstruction to pulmonary venous drainage or severe pulmonary hypertension due to increased pulmonary blood flow, the patient is usually critically ill and the diagnosis is unambiguous. However, in some patients the symptoms may be mild and often overlap with those of other noncardiac diseases. Tachypnea, failure to thrive, and mild cyanosis are the most frequently reported symptoms. In our patient population, we encountered a frequent observation by parents that the child had an alteration in voice which we elected to refer to as dysphonia for the purpose of this study. We retrospectively reviewed all patients with total anomalous pulmonary venous connection who were evaluated at our institution from January 1996 to January 1999 and found that 27% of patients had dysphonia as a presenting complaint to their primary care provider. We hypothesize that the etiology of this phenomenon lies in the possible compression of the left recurrent laryngeal nerve in the vicinity of the dilated pulmonary artery and the left vertical vein.

Echocardiographic Spectrum of Supracardiac Total Anomalous Pulmonary Venous Connection

Although infracardiac total anomalous pulmonary venous connection (TAPVC) is almost always associated with venous obstruction, the incidence and location of obstruction in supracardiac TAPVC has not been completely delineated. This report summarizes our experience with 20 cases of supracardiac TAPVC diagnosed by transthoracic echocardiography from Jan. 1989 to Mar. 1997. Fifty percent were obstructed, and five different sites of narrowing were found. The most common sites of obstruction were at the level of the left pulmonary artery (left vertical vein) and at the insertion into the superior vena cava (right vertical vein). Because nonobstructed Doppler flow patterns are present proximal to the actual site of obstruction in the anomalous pathway, a thorough interrogation of the entire venous channel with two-dimensional and Doppler echocardiography is essential to provide complete preoperative anatomic and hemodynamic details to determine the nature and timing of surgery in this condition. (J Am Soc Echocardiogr 1998;11:289-93).

Total anomalous pulmonary venous connection in adults

Between 1961 and 1989, 19 patients with total anomalous pulmonary venous connection underwent surgical correction. Ages ranged from 18 to 38 years (mean 26.2 +/- 6.5 years). The anatomic variants included 10 patients with total anomalous pulmonary venous connection to the vertical vein, 6 patients with total anomalous pulmonary venous connection to the coronary sinus, and 2 with total anomalous pulmonary venous connection directly to the right atrium. The last patient had mixed connection to the coronary sinus and left vertical vein. Two patients died, one because of left atrial rupture and another of low cardiac output. Long-term follow-up after surgical repair ranged from 2 to 24 years (mean 7 +/- 6.2 standard deviation). Preoperative disability, assessed according to New York Heart Association criteria, showed 12 patients in functional class II and seven in class III. After treatment, 16 patients were in class I and one in class II (p less than 0.001). Echocardiographic evaluation of six patients revealed in all a normal left ventricular ejection fraction; the diastolic function was also normal except in one patient. The postoperative evaluation of the pulmonary arterial systolic pressure performed by both Doppler echocardiography and right cardiac catheterization in 14 patients showed a significant reduction of the mean pulmonary arterial systolic pressure from 51.1 +/- 3.4 to 37.4 +/- 14.4 mm Hg (p less than 0.01). The remaining three patients evaluated in the follow-up period only by clinical examination are in New York Heart Association functional class I. The anatomic characteristics of our patients were responsible for the long-term outcome without correction. Surgical treatment of older patients can be performed with satisfactory results and excellent long-term survival.

Pulmonary Veins in Total Anomalous Pulmonary Venous Connection with Obstruction: Demonstration Using Silicone Rubber Casts

We studied the postmortem vascular anatomy of four patients with total anomalous pulmonary venous connection (TAPVC) with obstruction using a silicone rubber casting method. Two patients were studied clinically but died before therapeutic intervention, one died after corrective surgery, and another was not diagnosed before death. The morphologic types were supracardiac (2), infracardiac (1), and mixed (1). One of the supracardiac forms had a stenosis at the left vertical vein by a bronchoarterial vise, and a collateral channel to the right superior caval vein was also present. In the other supracardiac case, a left upper pulmonary vein was connected to the left vertical vein above the stenotic portion, resulting in postoperative pulmonary hemorrhage into the left upper lobe. The patient with infracardiac TAPVC had a tree-shaped descending vertical vein and small opening at the inferior caval vein. In the mixed type, a right upper pulmonary vein ran between the pulmonary artery and bronchus draining into the superior caval vein, and the rest of the drainage was to the portal vein.