Anomalous Pulmonary Vein Connection Research Articles

Corrected partial anomalous pulmonary vein connection associated with lung resection: a case report

IntroductionPartial anomalous pulmonary venous connection (PAPVC) is a potential cause of right-sided heart failure. Notably, a risk of sudden circulatory failure exists during lung surgery. Only a few reports of PAPVC complicated by lung cancer requiring lobectomy exist. Here, we report a case of left lower lung lobectomy complicated by an anomalous connection of the left upper pulmonary vein requiring revascularization.Case presentationA 66-year-old man was found with an abnormal mass shadow in the left lower lung field on chest radiography. Bronchoscopy revealed lung adenocarcinoma. Preoperative contrast-enhanced computed tomography showed that the left upper pulmonary vein did not perfuse the left atrium but was connected to the left brachiocephalic vein. Preoperative transthoracic echocardiography revealed right atrial and ventricular enlargement. The clinical diagnosis was stage IB (T2aN0M0). We decided to perform a left lower lobectomy and correct the PAPVC to maintain oxygenation after the lobectomy. The PAPVC was ligated, and the stump of the left upper pulmonary vein was anastomosed to the left atrial appendage without cardiopulmonary bypass. Postoperative contrast-enhanced computed tomography revealed intact reconstructed vessels. Postoperative transthoracic echocardiography showed no right ventricular overload. The patient’s postoperative clinical course following the surgical procedure was uneventful. Furthermore, the patient was followed up without any complications.ConclusionsThis rare case of successful surgical correction can inform clinicians of similar cases.

Surgical Outcomes of Patients With Heterotaxy Syndrome - The Japanese Congenital Cardiovascular Surgery Database.

Cardiac malformations are a major component of heterotaxy syndrome and result in significant mortality and morbidity. This multicenter nationwide Japanese study evaluated mortality and morbidity after initial surgical palliation for patients with heterotaxy syndrome and determined predictors for mortality and morbidity among patients enrolled in the study. The Japanese Congenital Cardiovascular Surgery Database (JCCVSD) collects clinical data from 119 domestic institutions specializing in congenital heart disease, covering almost all major congenital heart surgery programs in Japan. Clinical data on preoperative, operative, and postoperative characteristics and survival data within 30 and 90 days were available from the JCCVSD database. Of the 561 patients with heterotaxy syndrome who underwent any of 8 specific initial cardiovascular surgeries, 45 (8.2%) and 75 (13.4%) had died at 30 and 90 days, respectively. Preoperative emergency transport, type of heterotaxy syndrome, low hospital volume, the repair of total anomalous pulmonary vein connection, and the repair of a common atrioventricular valve were identified as significant predictive factors for operative mortality. Improvements in some medical circumstances, such as fetal diagnosis and the patient transport system, will be needed to improve outcomes for severely ill patients with heterotaxy syndrome. This study describes early outcomes for the largest number of main cardiovascular surgeries to date in infants with heterotaxy syndrome.

Video assisted thorascopic assisted correction of left partial anomalous pulmonary venous connection: one case report

IntroductionThe left partial anomalous pulmonary vein connection is a rare congenital heart disease, especially with intact atrial septum. Now we reported a case of the left superior pulmonary vein drainage to left innominate vein through a vertical vein, and corrected with video assisted thoracoscopy.Case presentationA-59-years old man diagnosed left anomalous partial pulmonary vein connection with presentation of short breathiness and palpation, and diagnosed with computer tomography pulmonary angiography. The operation was carried out under video assisted thoracoscopy with one manipulation incision and one observational incision, the vertical vein was dissected and anastomosis with left atrial appendage. The patients recovered smoothly and postoperative CTPA showed anastomosis ostium was unobstructed.ConclusionThe left lateral thoracotomy and video assisted thoracoscopic surgery is a feasible for correction of left PAPVC with intact interatrial septum without using CPB.

Case Report: Removal of septal occluder device and closure of atrial septal defect with pulmonary vein flow redirection

BACKGROUND: Atrial septal defect represents 10 to 15% of congenital heart disease and is more frequent in women with a 2:1 ratio. Sinus venosus atrial septal defect is usually associated with partial anomalous pulmonary venous drainage from the right superior pulmonary vein to the right atrium directly or indirectly through the superior vena cava. Rarer still, is the inferior sinus venosus, which may be associated with partial anomalous venous drainage from the right inferior pulmonary vein. CASE REPORT: 13-year-old patient with a history of intervention for interatrial defect closure with Amplatzer occluder at the age of six. He was brought to receive medical attention for fatigue and dyspnea that had been progressing for 2 years. Cardiac CT reported partial anomalous connection of pulmonary veins and echocardiogram reported a residual atrial septal defect of 10 mm and mild to moderate pulmonary hypertension. Based on the findings, surgical treatment was decided. EVOLUTION: In correction surgery, we observed Amplatzer occluder incorrectly positioned, the device was removed and flow was redirected from right pulmonary veins to left atrium with bovine pericardium patch. After the surgical intervention, the patient had a favorable evolution without no post-surgical complications. At follow-up, 15 days after medical discharge, the patient was asymptomatic, and a cardiac CT scan showed the corrected interatrial defect. CONCLUSION: The treatment of atrial septal defect should be individualized, since it will depend on the size of the defect, the age at the time of diagnosis, the magnitude of the shunt, the severity of the symptoms and the presence of other conditions. If the defect requires repair, it is important to weigh the benefits of choosing percutaneous closure vs. surgical intervention to avoid the need for reintervention, as in the present case. Closure and redirection of pulmonary vein flow with bovine patch is a safe and effective option.

Coronary artery and congenital heart diseases in adult patients: a case series

Background: The relevance of coronary artery disease treatment in congenital heart disease is related to the increasing number of older patients with congenital conditions, predisposing factors and continuous silent ischemia.Objective: To analyze clinical data and results of surgery in patients with a combination of congenital heart disease and hemodynamically significant coronary artery lesions.Methods: 17 patients (7 women (41.2%), 10 men (58.8%)) were included in the study. All of them underwent myocardial revascularization and congenital heart disease corrective surgery from 2003 to 2020. The mean age at the time of surgery was 57.2 years. The congenital diagnosis was an atrial septal defect (14 patients), partial anomalous pulmonary vein connection (1 patient), recanalization of atrial septal defect (1 patient), recanalization of ventricular septal defect (1 patient). In 7 cases congenital and coronary pathology correction were performed percutaneously — coronary stenting as the first stage, defect closure at an interval of 4 to 10 days as the second. One patient underwent stenting 7 months prior to an open-heart surgery. In 9 cases one-staged open-heart surgery was performed simultaneously. Results: At the hospital 1 patient died after surgery due to multiple organ dysfunction syndrome. In all other cases there were no symptoms of ischemia on discharge, the patients were in NYHA class I–II (New York Heart Association).Conclusion: The tactics of coronary revascularization is determined by the necessity of percutaneous or open-heart intervention. Myocardial revascularization may be preferable as the first stage, or a one-stage correction may be performed and proved by good results. Received 14 October 2022. Revised 25 November 2022. Accepted 27 November 2022. Informed consent: The patient’s informed consent to use the records for medical purposes is obtained. Funding: The study did not have sponsorship. Conflict of interest: The authors declare no conflict of interest. Contribution of the authorsLiterature review: A.V. MinaevDrafting the article: A.V. Minaev, A.V. Sobolev, I.V. ZemlyanskayaCritical revision of the article: V.P. PodzolkovSurgical treatment: M.R. Chiaureli, V.N. Cheban, E.P. Golubev, K.V. PetrosyanFinal approval of the version to be published: V.P. Podzolkov, A.V. Minaev, M.R. Chiaureli, V.N. Cheban, E.P. Golubev, K.V. Petrosyan, A.V. Sobolev, I.V. Zemlyanskaya

Is there a total anomalous connection of pulmonary veins with hypoplastic right ventricle?

Is there a total anomalous connection of pulmonary veins with hypoplastic right ventricle?

Pulmonary Hypertension Crisis in Patient with Tetralogy of Fallot and Mixed Total Anomalous Pulmonary Vein Connection after the Primary Correction: A Rare Case Report

Pulmonary Hypertension Crisis in Patient with Tetralogy of Fallot and Mixed Total Anomalous Pulmonary Vein Connection after the Primary Correction: A Rare Case Report

Is there a total anomalous connection of pulmonary veins with hypoplastic right ventricle?

Is there a total anomalous connection of pulmonary veins with hypoplastic right ventricle?

Descending thoracic aortic homograft for superior vena caval translocation in the Warden procedure: Report of six cases.

Warden procedure has been described for repair of partial anomalous pulmonary venous connections inserted high into the superior vena cava. In the current report, we describe a new modification. A total of six patients (five females, 83%) with a mean age of 18.8 ± 10.8 years, underwent a modified Warden procedure with a descending thoracic aortic (DTA) homograft connected to the free wall of the right atrium, rather than to the right atrial appendage in the period between June 2021 and July 2022. All patients had evidence of enlarged right-sided cardiac chambers. Two patients had intact interatrial septum (33.3%), while three patients had sinus venosus (50%) and one had secundum atrial septal defects (16.7%). The procedure was performed via minimally invasive right thoracotomy in half of the patients. Patients were extubated in the operating room or soon after. No early mortality or reoperation. One patient required concomitant aortic root replacement and one needed repair of anomalous left upper pulmonary vein connection to the left innominate vein. Follow-up was complete in all patients with a mean of 6.2 ± 4.6 months. No late mortality or reoperation. All patients were discharged on antiplatelet therapy. Follow-up imaging studies showed satisfactory repair with widely patent superior vena caval and pulmonary venous pathways. The use of DTA homograft to facilitate superior vena caval translocation in the modified Warden procedure appears to be beneficial as it decreases tension on the anastomosis and avoids the need for anticoagulation.

Long-term results of two different techniques in patients with supracardiac partial anomalous pulmonary venous connection via right thoracotomy approach. A prospective study

Aim: This study compared outcomes following the double-patch and Warden procedures for correcting partial anomalous connection of the right pulmonary veins to the superior vena cava via right thoracotomy approach. Material and Methods: Hundred patients, aged <18 years old, and with partial anomalous connection of the right pulmonary veins to the superior vena cava were randomly selected into double-patch method (n=50) and Warden procedure (n=50) groups. The median follow-up was 31.5 (range, 10-45) months. The primary endpoint was sinus node dysfunction at the mid-term follow-up period. Results: No early or late mortality occurred. In the early postoperative period, sinus node dysfunction was observed in 32.4% and 7% of cases after double-patch technique and the Warden procedure, respectively (risk ratio, 6.15; 92% confidence interval, 1.45-26.35; p=0.02). At follow-up, sinus node dysfunction remained in two (5%) patients after double-patch correction. All patients had normal sinus rhythm after the Warden procedure. No early or late pacemaker implantation occurred in either group. No patients had significant pulmonary veins or superior vena cava stenosis. Conclusion: The double-patch technique and Warden procedure both showed best mid - term consequences without mortality. The Warden procedure was linked with less sinus node dysfunction in the early postoperative period than the double-patch technique. There was no significant various differences in sinus node dysfunction at the mid-term follow-up.

Heart Failure Due to Anomalous Pulmonary Vein Connection Associated with Atrial Septal Defect

Introduction Anomalous pulmonary vein dreinage (APVD) is a rare disease, accounting for nearly 1 to 3% of congenital heart diseases. It may be total, when all PVs are involved, or partial, when only some PVs drain anomalously into one or more systemic veins or directly into the right atrium (RA)., This condition occurs alone or in association with an interatrial septal defect, more often sinus venosus interatrial communication (IAC) (which accounts for nearly 5% of all IAC cases). Patients with [...]

Non diagnosed PAPVC induce large reverse venovenous shunt after modified Fontan surgery: A case report of a rare anomaly and embolization therapy

Fontan operation is a reliable palliative surgery for patients with single ventricle physiology. Still, the development of complication is common; one of these complications that need to interventional approach is veno-venous collaterals between systemic and pulmonary veins. A 16-yearoldgirl with a history of modified Fontan operation at 9 years ago was referred with progressive cyanosis and dyspnea on exertion. In contrast trans-thoracic echocardiography (TTE), no fenestration was seen in Fontan circulation. Cardiac magnetic resonance revealed partial anomalous pulmonary vein connection (PAPVC) from left upper pulmonary vein to vertical vein and then into the in nominate vein and SVC with the reverse flow from superior vena cava (SVC) to left upper pulmonary vein(LUPV). This anomalous vein became severe engorged and tortuous. Possibly, LUPV and the verticalvein was dilated gradually as a result of increased pressure in the Fontan circuit. Finally, she underwent successful coil embolization in the midpart of the vertical vein. The oxygen saturation increased from80% to 93%.

胸腔镜下肺切除术伴部分型肺静脉异位连接的个案报道

胸腔镜下肺切除术伴部分型肺静脉异位连接的个案报道

Norwood procedure with right ventricle to pulmonary artery conduit: a single-centre 20-year experience.

The aim of this study was to evaluate the long-term outcomes of the Norwood procedure with right ventricle-pulmonary artery (RV-PA) conduit for hypoplastic left heart complex. A retrospective observational study was performed in 136 patients with hypoplastic left heart complex who underwent a Norwood procedure with RV-PA conduit between 1998 and 2017. The probabilities of survival, reintervention and Fontan completion were analysed. Stage 1 survival was 91.9% (125/136). Reintervention for PA stenosis was needed for 22% and 30% at stages 2 and 3, respectively, while 15% underwent reintervention for aortic arch recoarctation. Among 106 bidirectional Glenn survivors, 93 (68% of the total number of patients) had a Fontan completion, while 4 were not considered to be Fontan candidates. Risk factors for overall mortality included weighing <2.5 kg at the time of the Norwood procedure, intact atrium septum, total anomalous pulmonary vein connection and more than mild atrioventricular regurgitation at the time of the Norwood procedure. Overall survival was 80.9%, 72.3% and 62.8% at 1, 5 and 20 years, respectively. Probabilities of survival and Fontan completion were acceptable under the current surgical strategy incorporating RV-PA Norwood procedure as the first palliation. Incorporating a strategy to maintain PA growth and ventricular function through the staged repair is of prime importance. Further studies are necessary to observe changes in atrioventricular regurgitation as well as in right ventricular function, in patients who require atrioventricular valve interventions during the staged Fontan completion.

Progression of vascular remodeling in pulmonary vein obstruction

ObjectivesPulmonary vein obstruction (PVO) frequently occurs after repair of total anomalous pulmonary vein connection with progression of intimal hyperplasia from the anastomotic site toward upstream pulmonary veins (PVs). However, the understanding of mechanism in PVO progression is constrained by lack of data derived from a physiological model of the disease, and no prophylaxis has been established. We developed a new PVO animal model, investigated the mechanisms of PVO progression, and examined a new prophylactic strategy. MethodsWe developed a chronic PVO model using infant domestic pigs by cutting and resuturing the left lower PV followed by weekly hemodynamic parameter measurement and angiographic assessment of the anastomosed PV. Subsequently, we tested a novel therapeutic strategy with external application of rapamycin-eluting film to the anastomotic site. ResultsWe found the pig PVO model mimicked human PVO hemodynamically and histopathologically. This model exhibited increased expression levels of Ki-67 and phospho-mammalian target of rapamycin in smooth muscle-like cells at the anastomotic neointima. In addition, contractile to synthetic phenotypic transition; that is, dedifferentiation of smooth muscle cells and mammalian target of rapamycin pathway activation in the neointima of upstream PVs were observed. Rapamycin-eluting films externally applied around the anastomotic site inhibited the activation of mammalian target of rapamycin in the smooth muscle-like cells of neointima, and delayed PV anastomotic stenosis. ConclusionsWe demonstrate the evidence on dedifferentiation of smooth muscle-like cells and mammalian target of rapamycin pathway activation in the pathogenesis of PVO progression. Delivery of rapamycin to the anastomotic site from the external side delayed PV anastomotic stenosis, implicating a new therapeutic strategy to prevent PVO progression.

P718 Unexpected finding during follow up for a suspected arrythmogenic right ventricular cardiomyopathy (ARVC)

Abstract Interatrial communication is the most common congenital defect found in adulthood, being the most common ostium secundum variety (70-80%). Superior and inferior sinus venosus defects are less usual, found in the 5-10% of cases of interatrial communication and frequently associated with anomalous pulmonary venous return (APVR). These defects are located near the junction of the superior (5%) or inferior (&amp;lt;1%) vena cava with the right atrium, which makes them difficult to diagnose by transthoracic ecocardiography (TTE). Case description: A 44-year-old man who is being followed up in our electrophysiology consulting with suspicion of ARVC (suggestive CMR with no gene found) after an episode of ventricular tachycardia (VT) 11 years ago. Asymptomatic since then under treatment with atenolol, except for an episode of chest pain that required a coronary computed tomography which described an image compatible with a patent foramen ovale and normal coronary arteries. During the follow-up a cardio magnetic resonance (CMR) is performed which showed a severely dilated right ventricle with diskinetic areas, no volume changes since last CMR (5 years ago) and preserved ejection fraction. A small interatrial communication located infero-posteriorly in the septum drawed our attention. Estimated QP/QS was 1.4. After this finding, we reviewed the CT made 4 years ago, where a flow from the left atrium to the right atrium could be seen. We decided to ask for both a transthoracic echocardiography (TTE) and a transesophageal echocardiography (TEE). TTE showed normal left ventricle, a dilated right ventricle with preserved function, no valvulopathies and normal pulmonary pressure. Shunt test with agitated saline was slightly positive after Valsalva maneuver, and QP/QS was again 1.4. TTE showed a small interatrial communication measuring 1.9x0.8cm, next to inferior vena cava`s drainage. Since right ventricle dilation could be due both to the atrial septal defect (ASD) and to the dysplasia, the case was discussed in the heart team, and as the defect was small, QP/QS was 1.4 and pulmonary pressure was normal we adopted a conservative approach. Inferior sinus venosus defects are one of the least common atrial septal defects. They are located in the atrial septum immediately above the orifice of the inferior vena cava and are often associated with partial anomalous connection of the right pulmonary veins. This location makes it difficult to see by means of a common TTE or TEE, and usually as in our case multimodal approach can be very helpful. Usually patients with this kind of atrial septal defect (ASD), signs of significant shunt (right ventricular volume overload, QP/QS≥1.5) and systolic PA pressure less than 50% of systemic pressure (with pulmonary vascular resistance less than one third of the systemic vascular resistance) are suggested for surgery. In this case the possibility of two pathologies overlapping makes it challenging for diagnosis and treatment. Abstract P718 Figure. Multimodal imaging for diagnosis.

Late diagnosis of total anomalous connection of pulmonary veins during the puerperium

The total anomalous pulmonary venous connection (TAPVC) is a rare congenital anomaly. We present a case of a 36-year-old woman, in the postpartum period during the physical examination there was a heart murmur and beating in hands and feet. Cardiac magnetic resonance reported supracardiac TAPVC and large atrial septal defect. The survival of these patients is very uncommon in adults and even worse during pregnancy.

Fetal pulmonary atresia with ventricular septal defect: echocardiographic findings, associated anomalies and chromosomal characteristics

Objective To analyze the echocardiographic findings, associated anomalies and chromosomal characteristics in fetuses with pulmonary atresia with ventricular septal defect (PA/VSD). Methods The echocardiographic data and follow-up materials were retrospectively reviewed in 30 256 fetuses from December 2012 to March 2018 in the consultation center of fetal heart disease in maternal-fetal medicine in Anzhen hospital. Of all the fetuses, 59 cases (0.19%) had PA/VSD. The echocardiographic findings, associated anomalies and chromosomal characteristics were retrospectively analyzed in all the 59 fetuses with PA/VSD. Based on whether the presence of the native pulmonary arteries and the major aortopulmonary collateral arteries (MAPCAs) or not, the PA-VSD was classified into type A, type B, and type C. Results A large ventricular defect was demonstrated in five-chamber view with 61.7% of the mean ratio of the aortic overriding. Other fetal echocardiographic features of all the 59 fetuses with PA/VSD included: the right aortic arch (n=19), reversal flow in the ductus arteriosus (n=40), MAPCAs (n=24). The classification of the PA/VSD included: type A (n=35), type B (n=5) and type C (n=19). Associated anomalies: persistent left superior vena cava (n=13), anomalous pulmonary vein connection (n=5), complete atrioventricular septal defect (n=5); single umbilical artery (n=3), right atrial isomerism (n=3). Of all the 30 cases performed chromosomal test, 3 cases had aneuploidy and 7 cases had microdeletion of chromosome. Conclusions The fetal echocardiographic findings of the PA/VSD are characteristic. For prenatal diagnosis of PA/VSD, the type of PA/VSD should be defined and chromosomal test should be performed, which can be helpful for prenatal consulting. Key words: Echocardiography; Heart septal defect, ventricular; Pulmonary atresia; Prenatal diagnosis; Chromosomal aberrations

Outcomes of Double-patch and Warden Techniques in Patients With Supracardiac Partial Anomalous Pulmonary Venous Connection

This study compared outcomes following the double-patch and Warden procedures for correcting partial anomalous connection of the right pulmonary veins to the superior vena cava. Eighty (80) patients, aged <18years old, and with partial anomalous connection of the right pulmonary veins to the superior vena cava were randomly assigned into double-patch method (n=40) and Warden procedure (n=40) groups. The median follow-up was 22.5 (range, 12-39) months. The primary endpoint was sinus node dysfunction at the mid-term follow-up period. No early or late mortality occurred. In the early postoperative period, sinus node dysfunction was observed in 27.5% and 5% of cases after double-patch correction and the Warden procedure, respectively (risk ratio, 5.50; 95% confidence interval, 1.30-23.25; p=0.01). At follow-up, sinus node dysfunction persisted in two (5%) patients after double-patch correction. All patients had normal sinus rhythm after the Warden procedure. No early or late pacemaker implantation occurred in either group. No patients had significant pulmonary veins or superior vena cava stenosis. The double-patch technique and Warden procedure both showed excellent early and mid-term results with no mortality and minimal morbidity. The Warden procedure was associated with less sinus node dysfunction in the early postoperative period than the double-patch technique. There was no significant between-group difference in sinus node dysfunction at the mid-term follow-up.

‘Left Right Out’: A Rare Case of Left Superior and Inferior Anomalous Pulmonary Venous Return Diagnosed with Transoesophageal Echocardiography

We present the case of a 31-year-old woman who was referred to our institution for transoesophageal echocardiography to investigate the cause of right ventricular volume overload seen on transthoracic echocardiography, investigating exertional dyspnoea. On transoesophageal echocardiogram, close examination of the interatrial septum did not reveal any atrial septal defect. Similarly, the right superior and inferior pulmonary veins were unremarkable. However, the left superior and inferior pulmonary veins were noticeably absent. Careful observation of colour Doppler applied to the right atrium during the assessment of the inter-atrial septum revealed colour aliasing and increased flow velocity at the entry of the superior vena cava (SVC) into the right atrium. On further inspection, there was evidence of a communication to the SVC 3.6 cm above the connection with the right atrium, receiving flow from a confluence of the left-sided pulmonary veins. Cardiac magnetic resonance imaging confirmed the finding of anomalous left superior and inferior pulmonary veins draining into the SVC. The most common form of anomalous pulmonary venous connection is the connection of the left upper pulmonary vein to the left innominate vein. Less commonly, the right-sided pulmonary veins can drain into the SVC or the IVC (‘Scimitar syndrome’). Anomalous connection of both left-sided pulmonary veins is an extremely rare finding, and represents a very small proportion of anomalous drainage cases. This case demonstrates the value of simple and inexpensive transoesophageal echocardiography for the diagnosis of congenital heart disease in an era where there still remains high demand on cardiac MRI and computed tomography services.