Drainage Of Pulmonary Veins Research Articles

Persistent Left Superior Vena Cava: Incidence and Management in Patients Undergoing Repair of Partial Anomalous Pulmonary Venous Connection.

A association between partial anomalous pulmonary venous connection (PAPVC) and systemic venous anomalies has been described in the literature, but the true incidence is yet to be elucidated. At our institution, we sought to find the incidence of a persistent left superior vena cava (PLSVC) in patients undergoing an operation for PAPVC. A retrospective review of all pediatric and adult patients with PAPVC who underwent surgical repair from February 2006 to February 2016. All clinical, radiographic, and operative data were reviewed. Eighty-five patients underwent surgical repair. A PLSVC was identified in 15 (17.6%) patients. Every PLSVC drained/connected to the coronary sinus. A bridging vein was present in only 26.7% (4 of 15). Intraoperative management of the PLSVC consisted of direct cannulation in nine (60%) patients, temporary occlusion in one (6.7%) patient, and ligation in one (6.7%) patient. In the 15 PLSVC patients, 6 (40%) had a secundum atrial septal defect (ASD), 2 (13.3%) had a patent foramen ovale (PFO), 10 (66.6%) had a sinus venosus type defect, and 3 (20%) had both an ASD and sinus venosus-type defect. Of the 15 patients with PAPVC and PLSVC, 14 (93%) had anomalous drainage of pulmonary vein(s) on the right side, whereas 1 (7%) had veins on both sides with anomalous drainage. Persistent left superior vena cava is present in 17.6% of patients undergoing an operation for PAPVC. Awareness of this association as well as the intraoperative management of PLSVC is advised for those that perform operations for PAPVC. As improper myocardial protection and cardiopulmonary bypass strategies in the presence of a PLSVC can result in deleterious outcomes.

The limitation of transthoracic echocardiography in diagnosing partial anomalous pulmonary venous drainage

Objective: To improve the diagnostic accuracy of transthoracic echocardiography (TTE) by analyzing its limitations in diagnosing partial anomalous pulmonary venous drainage (PAPVD). Method: This was a retrospective analysis of PAPVD patients seen at the Children's Hospital of Fudan University from October 1 2006 to October 1 2016. The echocardiographic data were compared to findings on multi-slice spiral CT (MSCT), cardiac catheterization or surgery. The echocardiography machines used were Philip IE33, GE Vivid 7 and Vivid i with frequency ranging from 5.0 MHz to 7.5 MHz. The cardiac structure was analyzed according to Van Praagh segments. Result: A total of 43 cases of PAPVD were enrolled, male∶ female ratio 20∶23 with average age (27.9±21.4) months. Among them, 3 cases were simple PAPVD and 40 cases had other associated congenital heart diseases. TTE was successful in diagnosing 29 cases (67%) while 14 cases were missed. The diagnostic rate for right pulmonary vein drainage into superior vena cava, right atrium, inferior vena cava were 5/10, 17/20, and 3/5 respectively while left pulmonary vein drainage into left innominate vein was only 1/4. Added TTE images to re-exam the 9 of the 14 missed cases, 5 cases of abnormal drainage from right superior pulmonary vein were diagnosed, while 4 cases of drainage from right lower or left pulmonary vein were only picked up by indirect signs. Conclusion: The distance of the pulmonary veins from the routine ultrasound view and the possibility of branch number variation may limit the accuracy of TTE in diagnosing PAPVD, especially for drainage from right lower and left pulmonary vein. But TTE is still the preferred diagnostic method. The diagnostic rate could be increased by paying special attention to non-routine views including the suprasternal fossa, the right parasternal and subcostal area.

A сase of obstruction of SVC-right atrium appendage anastomosis after procedure Warden

The analysis of a 6-year patient’s medical history after correction of congenital heart disease (partial anomalous drainage of the right superior pulmonary vein into the superior vena cava) complicated by obstruction of the anastomosis between the SVC and the appendage right atrium is presented. The article discusses the stages of diagnosis and patient management features, as well as the choice of treatment.Received 12 December 2016. Accepted 14 February 2017.Financing: The study did not have sponsorship.Conflict of interest: The authors declare no conflict of interest.Svyazov E.A.: data analysis, article writing and editing. Podoksenov A.U.: article editing. Varvarenko V.I.: implantation of the stent-graft in superior vena cava.Martsinkevich G.I.: echocardiographic examination of the patient before and after endograft implantation, article editing. Krivoshchyokov E.V.: organizational work and referral of the patient to endovascular treatment, article editing.

Role of four-dimensional echocardiography with high-definition flow imaging and spatiotemporal image correlation in detecting fetal pulmonary veins.

Prenatal diagnosis of fetal total anomalous pulmonary vein connection (TAPVC) remains challenging for most screening sonographers. The purpose of this study was to evaluate the use of four-dimensional echocardiography with high-definition flow imaging and spatiotemporal image correlation (4D-HDFI) in identifying pulmonary veins in normal and TAPVC fetuses. We retrospectively reviewed and performed 4D-HDFI in 204 normal and 12 fetuses with confirmed diagnosis of TAPVC. Cardiac volumes were available for postanalysis to obtain 4D-rendered images of the pulmonary veins. For the normal fetuses, two other traditional modalities including color Doppler and HDFI were used to detect the number of pulmonary veins and comparisons were made between each of these traditional methods and 4D-HDFI. For conventional echocardiography, HDFI modality was superior to color Doppler in detecting more pulmonary veins in normal fetuses throughout the gestational period. 4D-HDFI was the best method during the second trimester of pregnancy in identifying normal fetal pulmonary veins. 4D-HDFI images vividly depicted the figure, course, and drainage of pulmonary veins in both normal and TAPVC fetuses. HDFI and the advanced 4D-HDFI technique could facilitate identification of the anatomical features of pulmonary veins in both normal and TAPVC fetuses; 4D-HDFI therefore provides additional and more precise information than conventional echocardiography techniques.

Interventional therapy for partial anomalous pulmonary venous connection with dual drainage.

A 6-year-old boy presented with dual drainage of left upper pulmonary vein, with connection to innominate vein inaddition to its normal connection to the left atrium. Despite relief of aortic stenosis at the age of 3 years, significant left to right shunt persisted. The dual drainage allowed successful percutaneous closure of the levoatriocardinal vein without obstruction to the pulmonary venous flow to the left atrium.

Mid- and long-term results of Warden’s procedure to repair partial anomalous drainage of right pulmonary veins into superior vena cava

Mid- and long-term results of Warden’s procedure to repair partial anomalous drainage of right pulmonary veins into superior vena cava

Persistent Left Superior Vena Cava Associated with Hemiazygos Vein Draining in It and Absence of Left Brachiocephalic Vein, in a Patient with Congenital Heart Defect

Abstract Persistent left superior vena cava is an anomalous vein that derives from a malfunction of obliteration of the left common cardinal vein during intrauterine life. The diagnosis can be suggested by a dilated coronary sinus as seen in echocardiography, or other imagistic methods. Due to the lack of hemodynamic impairment, and consequently with few or no symptoms, this vascular anomaly is frequently discovered incidentally. In this brief report we present the case of a 35-year-old male known with a complex congenital cardiovascular malformation that included atrial septum defect, persistent left superior vena cava and anomalous right pulmonary vein drainage in the PLSVC, diagnosed with sinoatrial block that required pacemaker implantation. Due to the patient’s medical history, investigations to decide the best approach needed for pacemaker implantation were performed, including a thoracic CT that incidentally found additional anomalies — the hemiazygos vein draining in PLSVC and the lack of the left brachiocephalic vein.

Total anomalous pulmonary venous return

TAPVR typically presents in early infancy due to excessive pulmonary blood flow and mild cyanosis. Presentation is earlier when there is obstruction to pulmonary venous flow where neonates present with severe cyanosis and cardiogenic shock, surgical repair must be planned immediately. Mixing of well oxygenated blood through the anomalous connection of pulmonary veins with the systemic blood in the right atrium results in similar oxygen saturation in all cardiac chambers and arteries, therefore pre and post ductal oxygen saturations are equal. Increased blood flow across the right heart causes delay in closure of the pulmonary valve, presenting as fixed splitting of second heart sound as well as systolic flow murmur over the pulmonic region. Supra-cardiac drainage of anomalous pulmonary veins to the superior vena cava result in figure-eight or snow man appearance of the cardiac silhouette on CXR.

Case 2/2016 - Scimitar Sign with Right Pulmonary Vein Drainage into the Right Atrium

Clinical data: the characteristic signs of the scimitar syndrome with right pulmonary hypoplasia were discovered in an asymptomatic patient through routine chest X-ray, in the presence of dengue. On physical examination, the patient was in good general health status, eupneic, normal skin color, with normal pulses. His weight was 54 kg, height 155 cm, blood pressure of 100/60 mmHg, heart rate of 88 bpm. The aorta was not palpable at the sternal notch. In the precordium, there were mild impulses at the left and right sternal borders and the apex beat was not palpable. Heart sounds were normal, with constant splitting of the second heart sound, with discreet and rough ejection systolic murmur in the pulmonary area. The liver was not palpable and in the lungs, breath sounds were less audible in the right lower pulmonary lobe.

Role of Perioperative Echocardiographic in Surgical Correction of the Pulmonary Venous Anomalies

ABSTRACT Anomalous pulmonary venous drainage is the anomalous drainage of one or more than one pulmonary vein draining into right atrium, superior vena cava, inferior vena cava, coronary sinus, or innominate vein. Right-sided pulmonary vein anomalous drainage is usually associated with sinus venosus type atrial septal defect. Pericardial patch repair and rerouting of the right pulmonary vein are commonly performed operations for such cases. These operations involve a risk of obstruction to the flow of superior vena cava or rerouted pulmonary vein in the postoperative period. The defects are well visualized on the transesophageal echocardiography (TEE). We are reporting three cases operated for anomalous drainage of right-sided pulmonary vein, highlighting the perioperative echocardiographic features. How to cite this article Kumar A, Dutta V, Negi S, Puri GD. Role of Perioperative Echocardiographic in Surgical Correction of the Pulmonary Venous Anomalies. J Perioper Echocardiogr 2016;4(1):19-26.

A case of asymptomatic patient with right ventricular dilatation

Sinus venous defect is an uncommon type of atrial septal defect (ASD), often associated with anomalous pulmonary vein drainage and left to right shunting. If undetected, it leads to right atrial and ventricular volume overload. The current case describes an asymptomatic athlete with missed large sinus venosus defect since birth, and the different types of cardiac imaging modalities that lead to the detection of the defect and guiding the appropriate surgical intervention.

Scimitar Syndrome a Rare Cause of Isolated Esophgeal Varices

We present a rare case of isolated esophageal varices arising from Scimitar syndrome, an anomalous connection of the right pulmonary vein to the Inferior vena cava (IVC). A 53 year old female with past medical history of Scimitar syndrome repaired at age 20, with dissection of the anomalous right pulmonary vein and connecting it to the right atrium, and then creating an intra-atrial pericardial baffle directing venous flow to the left atrium presented with epigastric pain. She denied history of gastrointestinal bleeding or chest pain. Vital signs and exam were normal. Labs were normal. CT Chest, abdomen and pelvis showed mild mediastinal enlargement, a small right pulmonary artery, loss of right lung volume and diffuse distal esophageal wall prominence. The liver, spleen & pancreas were unremarkable. For epigastric pain, she underwent an EGD showing H.pylori gastritis and grade III esophageal varices in the mid and distal esophagus. She was treated for H. pylori gastritis. Endoscopic US showed esophageal varices, a lymph node in the aortopulmonary region and normal flow in the portal and splenic veins. For further workup of her esophageal varices in the setting of Scimitar syndrome and in the absence of portal hypertension, she underwent a cardiac catheterization. The cardiac catheterization showed normal right atrial and left ventricular end diastolic pressures. Also right pulmonary venous hypertension was noted, with the pulmonary venous collaterals draining into the azygous venous system, intercostal veins and then into the para esophageal venous system producing esophageal varices. Blood flow in the baffle from the right lower pulmonary vein into left atrium was not visualized, suggestive of obstruction of the surgical baffle. The study concluded that the right pulmonary venous flow drained into the systemic circulation via the paraesophageal veins. Scimitar syndrome aka pulmonary venolobar syndrome is a rare congenital heart malformation, with a prevalence of 0.5-1%. It is characterized by partial or complete anomalous drainage of the right pulmonary vein into the IVC, right lung hypoplasia and mediastinal shift. The ‘Scimitar sign' is a curvilinear shadow resembling a curved Turkish sword ‘scimitar' created by an abnormal right pulmonary vein draining into the IVC. The common causes of esophageal varices in an adult are portal vein thrombosis, liver cirrhosis, SVC obstruction, mediastinal tumors, thymoma or chronic fibrosing mediastinitis. Esophageal varices arising from an anomalous pulmonary venous return is very rare. In this case, obstruction of the surgical baffle resulted in esophageal varices without portal hypertension. Patient was referred to surgery for evaluation of right lower lobe pneumonectomy or creation of another surgical baffle which may diminish the esophgeal varices.Figure 1Figure 2Figure 3

Re: Retrogradein situversus antegrade pulmonary preservation in clinical lung transplantation: a single-centre experience

Historically, grafts were preserved by means of autoperfusion with extracorporeal circulation, topical cooling or donor core cooling. In the 1980s, the standard lung preservation method was single pulmonary artery flush perfusion, using modified Euro-Collins solution at 4°C [1]. However, reperfusion injury remained a significant factor for morbidity and early mortality after lung transplant. Since the early years of lung preservation strategies, some advances have emerged in transplant teams, such as adding prostaglandin E2 to prevent counteract temperature and potassium-induced vasoconstriction. The use of low potassium dextran glucose solutions shows better overall lung function, superior early oxygenation, higher lung compliance, lower incidence of severe primary graft dysfunction and 30-day mortality [2]. As Gohrbandt et al. point out, the route to administer the preservation solution may also have an impact on early lung graft performance [3]. Anterograde flush initiating the infusion through the pulmonary artery with pulmonary vein drainage was the traditional approach for many years [4]. This technique, however, presents a number of limitations. Flushing the pulmonary artery leaves the bronchial tissue to be preserved by topical cooling alone, raising the complication from the bronchial suture. Also, clots and fat or brain tissue emboli, especially in donors with major trauma and multiple bone fractures, may hamper uniform flushing and cooling and, therefore, impair graft function upon reperfusion. Adding a retrograde flush, whereby the preservation solution is administered via the left atrial appendage or four pulmonary veins into the pulmonary venous system and drained through the pulmonary artery, has the potential to flush not only pulmonary vessels, but also the bronchial network, contributing to the adequate perfusion of the tracheobronchial submucosa tissue, and also limiting the effect of hypothermic pulmonary arterial vasoconstriction. On the other hand, less ventilated areas, such as the posterior segments of lower lobes, are better flushed. Also residual blood and harmful constituents and any possible microthrombi that may obstruct the pulmonary vessels are washed out. The evidence from clinical series of this technique is limited [5]; authors such as Gohrbandt et al. show us that there are no clinical benefits of retrograde lung preservation alone and that it may cause or prolong mechanical ventilation and primary graft dysfunction at 72 h and prolong ICU stay [3]. On the other hand, some evidence has been published, showing that biphasic lung preservation is not only clinically feasible [6], but also related with better early oxygenation ratio, lower mean airway pressure and chest radiography score [7, 8]. Many transplant teams have now adopted a biphasic technique with an antegrade flush through the pulmonary artery followed by a retrograde flush through each of the pulmonary veins after heart excision with the lung still ventilated inside the cadaver. However, there is lack of controlled randomized studies.

Sinus venosus atrial septal defect with severe mitral stenosis: A rare presentation

We present a case of 32-year-old female patient with rheumatic mitral stenosis with sinus venosus atrial septal defect (ASD) and anomalous drainage of the right superior pulmonary vein into right atrium. Sinus venosus ASD are uncommon and constitute 2-3% of interatrial communication. However, sinus venosus ASD with mitral stenosis is an extremely rare condition which was treated successfully by patch closure with left atrialization of superior pulmonary vein and mitral valve replacement.

Combined congenital heart disease — cor triatrium, atrial septal defects and total anomalous drainage of the right pulmonary veins into the right atrium

Combined congenital heart disease — cor triatrium, atrial septal defects and total anomalous drainage of the right pulmonary veins into the right atrium

Isolated Partial Anomalous Pulmonary Venous Connection

BACKGROUND: Isolated partial anomalous pulmonary venous connection (PAPVC) is a very rare variant of PAPVC, and involves the anomalous drainage of the right upper pulmonary vein into the superior vena cava (SVC) with no associated inter-atrial septal defect. CASE: We present a case of an isolated PAPVC occurring in 26-year old female patient who presented with palpitations, exertional dyspnea and easy fatigability. Physical examination revealed a dynamic precordium, a right ventricular heave, and a tricuspid regurgitation murmur. Transthoracic echocardiogram showed right atrial, ventricular, and main pulmonary artery dilatation with moderate pulmonary hypertension. Hemodynamic studies revealed an isolated PAPVC with severe pulmonary hypertension. She underwent Warden’s procedure (superior vena cava translocation with atriocaval anastomosis), which remarkably improved her symptoms. CONCLUSION: This case report shows a very rare case of isolated PAPVC presenting with heart failure symptoms and right-sided chamber enlargement. The clinical presentation is not specific and may mimic more common diseases, such as acyanotic atrial septal defect and primary pulmonary hypertension. A high index of suspicion is important in diagnosing the disease in patients with right-sided cardiac chamber dilatation.

Correlative imaging in congenital heart disease

For the evaluation of congenital heart diseases (CHDs), echocardiography is the initial diagnostic modality and Catheter angiography is the gold standard for delineation of the anomalies. Though echocardiography is the initial diagnostic modality in CHDs, it has its limitations and Catheter angiography is relatively invasive and should be reserved for therapeutic procedures. Multidetector computed tomography (MDCT) has emerged as an important tool for cardiac imaging owing to constant advancements in computed tomography scanners. With the judicious and innovative use of three-dimensional reconstructions, the depiction of congenital anomalies is more accurate and it is possible to perform a virtual surgery to aid the surgeon in planning the approach to the patient as a one or two step procedure, counseling the parents about the outcome and giving a fair estimate of the cost of treatment. MDCT acts as a one stop shop for a complete evaluation of the cardiac, extra-cardiac, visceral and skeletal anomalies; their combinations and subsequent effects on each other. Excellent delineation of the vascular compressions on the tracheo-bronchial tree, degree of main and branch pulmonary artery stenosis, anomalous drainage of pulmonary and systemic veins, interruptions and narrowing of the aorta, anomalies of origin and course of coronary arteries are unique to MDCT.

Single-Stage Surgical Correction of Combination Lesions Through Thoracotomy

Coarctation of the descending aorta is associated with significant cardiac lesions in up to 50% of the patients. Under such circumstances, surgical management requires consideration of the nature of the associated lesion which usually requires a median sternotomy for surgical correction. Coarctation of the aorta (COA) is, therefore, rarely associated with lesions that are amenable to surgical correction through a thoracotomy incision. In this case report, we report a case of COA with significant associated cardiac disease that was repaired through a left thoracotomy incision. A 13-year-old boy was found to have COA and also partial anomalous pulmonary venous drainage of the left upper pulmonary vein to the innominate vein. Both the lesions were surgically corrected simultaneously through a thoracotomy.

Leftward Displacement of Septum Primum in Children With Congenital Heart Disease

To the Editor, In a recent issue of your journal we read the interesting paper by Park et al. [4], entitled ‘‘Leftward displacement of septum primum in hypoplastic left heart syndrome’’. In this study, the investigators report on the frequency of leftward displacement of septum primum (LDSP) in children with hypoplastic left heart syndrome (HLHS), and using echocardiography they correlate this pattern of atrial septation with the other anatomic cardiac features [4]. In the Introduction and in Discussion sections of the article, the investigators affirm that ‘‘leftward displacement of septum primum is not known to occur in other forms of congenital heart disease’’. Actually, leftward malposition of the septum primum is a well know anatomic finding occurring not only in HLHS [1, 4, 6] but also in other rare cases of cardiac defect with situs solitus [1, 2, 5, 7], particularly in patients with heterotaxy and polysplenia (left isomerism) [3, 5]. In children with HLHS, the superior limbic band (septum secundum) is present; in some cases, the septum primum shows a leftward and posterior deviation of its superior attachment, which is far away from the septum secundum [1, 4, 6]. This anatomic pattern usually coexist with normal pulmonary venous drainage [1, 4, 6]. In polysplenia, the superior limbic band (septum secundum) (*95 %) is absent, and in some cases the superior margin of the septum primum is deviated to the left and is inserted on the left atrium wall [3]. This anatomic pattern usually results in partial anomalous pulmonary vein drainage with the right pulmonary veins draining in to the right atrium [3, 5]. LSDP has also been described in association with other rare cardiac defect with situs solitus, including total anomalous pulmonary venous drainage [1, 2, 5, 7]. These different anatomic phenotypes of atrial septum may be in pathogenetic relation with the presence of leftsided obstructions in children with HLHS, as suggested in the article by Park et al. [4] but also in those with polysplenia [3, 5], which frequently presents systemic obstructions [3, 5].

Transposition of great arteries and partial anomalous pulmonary venous drainage

We describe the technical aspects of performing an atrial switch operation in a 4-year-old boy with d-transposition of the great arteries and partial anomalous drainage of the left-sided pulmonary veins. The rarity of the condition is discussed.