Anomalous Pulmonary Venous Drainage Research Articles

Appraisal of partial anomalous pulmonary venous drainage through a lumped-parameter mathematical model: a new pathophysiological proof of concept.

Haemodynamic determinants of the ratio between pulmonary and systemic flow (Qp/Qs) in partial anomalous pulmonary venous return (PAPVR) are still not fully understood. Indeed, among patients with the same number of lung segments draining anomalously, a great variability is observed in terms of right ventricular overload. The aim of this study was to test the hypothesis that the anatomic site of drainage, affecting the total circuit impedance, independently influences the magnitude of shunt estimated by Qp/Qs. A zero-dimensional (0D) lumped parameter mathematical model was developed and validated on a sample of patients. We developed a 0D lumped parameter model, using time varying elastances for heart chambers, RLC Windkessel circuits for the systemic and pulmonary circulations. Patients, were categorized into vena cava (VC) type (including left drainage to anomimous vein) and right atrium type (RA). The mathematical model is a system of ordinary differential equations that are numerically solved by means of the ode15s solver in the Matlab environment. The model showed an increase of Qp/Qs with the increase of the number of anomalous veins. With the same number of anomalous veins, Qp/Qs was lower in patients with anomalous drainage to the vena cava (VC) as compared with right atrium (RA) The validation sample consisted of 49 patients (27, 55% females). As predicted by the model, patients with PAPVR with VC type displayed a lower invasive and CMR Qp/Qs as compared with drainage to RA: 1.4 (1.2-1.7) and 1.45 (1.25-1.6) versus 2 (1.75-2.1) and 1.9 (1.6-2) p < 0.05. After stratifying for number of lung territories a lower Qp/Qs was measured in patients with VC PAPVR as compared with RA. In patients with PAPVR, the site of anomalous drainage modulates the Qp/Qs. According to the model, this effect is mediated by the post-capillary impedance of the circuit and significantly decreases with the increase of pulmonary vascular resistances.

Outcomes of the Modified Warden Procedure for Partial Anomalous Pulmonary Venous Connection Drainage to a High Location in the Superior Vena Cava

To report the safety and effectiveness of a modified Warden procedure. Twenty-six (26) patients underwent modified Warden surgery at our centre from September 2017 to September 2021. In all patients, the superior vena cava (SVC) was reconstructed by turning the atrial wall in the main body of the right atrium upwards and applying fresh autologous pericardial patches. There were 13 male patients and 13 female patients, and the median age of the patients was 7.0 (range, 0.3-47.0) years. The median follow-up time after surgery was 47.0 (range, 32.0-80.0) months. The aortic cross-clamp time was 108.50±34.72 minutes, the cardiopulmonary bypass time was 154.81±41.65 minutes, the median postoperative mechanical ventilation time was 8.5 (range, 0.0-91.0) hours, the median intensive care unit stay was 43.5 (range, 15.0-352.0) hours, and the median length of postoperative hospital stay was 7.0 (range, 6.0-19.0) days. All patients were safely and successfully discharged from the hospital. No deaths or sinus node dysfunction occurred during the follow-up period. The mean gradient of the reconstructed SVC and/or the right pulmonary vein (PV) increased in eight patients and decreased to less than 1 mmHg in six patients. One (1) adult patient had already undergone two vascular interventions, and one child had persistent stenosis but did not undergo reoperation at the time of the study. In addition, two patients were subjected to pericardial windowing. A modified Warden technique using a right atrial flap and autologous pericardium effectively corrected the high-plane connection between the PVs and SVC. This technique effectively avoids sinus node dysfunction; however, a short-term increase in the differential pressure of the re-established SVC or PV after the procedure is acceptable, and reintervention is required only in rare cases.

Echocardiogram Diagnosis of Complex Congenital Cardiac Malformations

Background: Complex congenital cardiac malformations rarely reported in literature Objective: Study was undertaken to define complex congenital cardiac malformations by Echocardiogram Materials and Methods: Prospective study included 101 children aged 2 to14 years, both male and female during March 2004 to February 2005 and July 2006 who attended in-patients &amp; outpatient departments at Vyedhi Institute of Medical Sciences and Research Centre (VIMS &amp; RC) and Sri Jayadeva Institute of Cardio-vascular Sciences and Research of Cardiology (SJICR), Bangalore, India. Clinical diagnosis was confirmed by relevant investigations and Echocardiography. Results: Complex congenital cardiac malformations presented in 16 (15.99%) of 101 children raising the total number of cardiac defects to 121. Majority 77 (76.2%) acyanotic cardiac malformations comprised of 29 (28.72%) Atrial Septal Defects (ASD) had 11 complex cardiac malformations. Three cases ASD + Partial Anomalous Pulmonary Venous Drainage (PAPVC), 2 cases ASD + Patent Ductus Arteriosus (PDA) + Mitral Valve prolapse (MVP), 2 cases ASD + Pulmonary Stenosis (PS,) 1 case ASD + PDA + PS, 1 case ASD + Ventricular Septal Defect (VSD), 1 case ASD + Coarctation of Aorta (CoA) and 1 case ASD + MVP. Next 23 (22.77%) VSD had 4 complex cardiac malformation cases, 3 cases of VSD + Aortic Stenosis (AS) of which one case had dextrocardia and 1 case VSD + PS. Followed by AS 8 (7.9%) with 1 case of AS + CoA, another case had VSD + AS. Among 24 (23.76%) cyanotic defects, TOF18 (17.8%) was the commonest, other rare variants included Double Outlet Right Ventricle (DORV) 4(3.96%) one case with dextrocardia. Pentalogy of Fallot 1 (0.99%) and Triology of Fallot 1 (0.99%). The age distribution revealed most 30(29.8%) were young children aged 2-4 years with overall male predominance Male:Female::1.3:1, observed contrasted to female predominance noted in ASD and TOF, M:F::1:2 and M:F::1:1.3 respectively Conclusion: Complex congenital cardiac malformations presented in 16(15.99%) of 101 children raising the total number of cardiac defects to 121

Double Outlet Right Ventricle with Non-Committed Ventricular Septal Defect and Other Complex Associated Anomalies in an Infant: Overview of the Surgical Management Approach

In the first place, the right ventricle is the primary or only point of origin for both main arteries in a patient with double outlet right ventricle (DORV). In order to guarantee proper repair, a wide variety of surgical approaches, considerations, and procedures are required for the wide spectrum of disorders that make up DORV. A functional classification based on physiological characteristics is one technique to classifying it, which might help determine the appropriate surgical strategy. Presentation of the case: Our case study focused on a female newborn infant who was 2 months old when she arrived at the hospital complaining of worsening shortness of breath and anorexia. The patient was diagnosed with pulmonary over-circulation, an abnormal accumulation of blood flowing into the lungs, through the following cardiac abnormalities: double outlet right ventricle (DORV) with a non-committal ventricular septal defect (VSD), no pulmonary stenosis (PS), mitral atresia, atrial septal defect (ASD), single ventricle dominance (SVD), anomalous pulmonary venous drainage (APVD), and patent ductus arteriosus (PDA). After a palliative procedure including the closure of the PDA and the implantation of a PA band, the patient was to have a surgical procedure known as univentricular repair. During the surgical meeting, this course of action was agreed upon. Conclusion: Different atrial configurations, atrioventricular connections, ventricular morphologies, and spatial interactions between the main arteries may all contribute to the wide anatomical spectrum of DORV. In order to decide between biventricular repair and univentricular palliation, several anatomical features must be present in the patient. In addition, palliative treatments may be necessary for certain patients before they may have a permanent surgical repair.

Virtual reality modelling for planning of percutaneous first step palliation in a newborn with heterotaxy syndrome.

We report the first-stage percutaneous palliation in a newborn with a rare heterotaxy syndrome variant including interrupted inferior vena cava, partial anomalous pulmonary venous drainage, and restrictive interatrial communication. Virtual reality imaging aided visualisation, decision-making, and planning. Successful atrial septoplasty performed via the internal jugular vein and anomalous pulmonary vein was followed by stenting of ductus arteriosus.

Multimodality Cardiovascular Imaging for Totally Video-Guided Thorascopic Cardiac Surgery.

Totally video-guided thorascopic cardiac surgery (TVTCS) represents one of the most minimally invasive access routes to the heart. Its feasibility and safety can be guaranteed by an experienced surgeon with skilled operative techniques under the guidance of a video signal via thoracoscopy and the imaging from transesophageal echocardiography. At present, this surgical approach has been applied for atrioventricular valve disease, atrial septum defects plus and partial anomalous pulmonary venous drainage, cardiac tumors, hypertrophic obstructive cardiomyopathy, aortic valve disease, and atrial fibrillation. Multimodality cardiovascular imaging, including echocardiography, X-ray, computed tomography (CT), magnetic resonance imaging (MRI) and cardiac catheterization, provides morphologic characteristics and function status of the cardiovascular system and a comprehensive view of the target anatomy. In this review, the benefits of multimodality cardiovascular imaging are summarized for the clinical practice of TVTCS, including the preoperative preparation, intraoperative guidance and postoperative supervision. The disease categories are also individually reviewed on the basis of multimodality cardiovascular imaging, to ensure the feasibility and safety for TVTCS. Cardiovascular imaging technologies not only confirm who is a candidate for this surgical technique, but also provide technical support during the procedure and for postop follow to assess the clinical outcomes. Multimodality cardiovascular imaging is instrumental to provide the requirements to solve the problems for conduction of TVTCS; and to provide individualized protocols with high-resolution and real-time dynamic imaging fusion.

Scimitar Syndrome in a Pediatric Cohort.

Scimitar syndrome is a rare form of congenital heart disease (CHD) characterized by anomalous pulmonary venous drainage of the right lung to the inferior vena cava. We describe the presentation, diagnosis, therapeutic management and long-term follow-up of 10 pediatric patients with Scimitar Syndrome. We performed a retrospective observational study of all pediatric patients from our institution with scimitar syndrome (March 1996-July 2023). Patients underwent systematic evaluation including medical and family history, chest x-ray, 12-lead electrocardiogram, echocardiogram, angiography and/or computed tomography; or magnetic resonance angiography. Ten patients with scimitar syndrome were included. The median age at diagnosis was 10.4 [0.1-150.2] months and the median follow-up time was 7.7 [1.3-15.3] years. Eight patients presented with aortopulmonary collateral arteries which were embolized. Two patients had dual connections to the inferior vena cava and left atrium; embolization of the inferior vena cava connection was only feasible in one of them. No patients underwent surgery of the scimitar vein. Three patients had surgical correction of CHDs. There were no deaths related to scimitar syndrome during follow-up. All patients with scimitar syndrome need prompt cardiovascular evaluation and follow-up. Our study demonstrates that a conservative approach with aortopulmonary collateral artery embolization, scimitar vein embolization when dual drainage to the left atrium is identified, along with correction of concomitant CHDs might have good results in patients with scimitar syndrome in order to postpone surgical correction of the anomalous pulmonary venous return to an older age when clinically or hemodynamically indicated. Further studies with longer-term follow-up and a larger sample size are needed to more effectively determine treatment strategy.

Total Anomalous Pulmonary Venous Drainage with Malaligned Atrial Septum: A Case Series and Descriptive Study

Abstract Total anomalous pulmonary venous drainage (TAPVD) is a complex congenital heart anomaly characterized by abnormal venous return from the pulmonary veins. The classification of TAPVD has been a matter of debate, particularly when pulmonary veins open into the anatomically left atrium but functionally into the right atrium due to a malaligned interatrial septum. This case series aimed to assess clinical significance of malalignment of interatrial septum and to differentiate between TAPVD and total anomalous pulmonary venous connection (TAPVC) by echocardiography before sending the patient for surgery. This retrospective case series involved seven patients diagnosed as TAPVD between 2011 and 2023. The age of the patients ranged from 1 month to 3 years, mostly presenting within the first few months of life. One case was diagnosed during fetal echocardiography. The commonest complaints were failure to thrive, fast breathing and recurrent chest infection. All cases were diagnosed by echocardiography and confirmed during surgery. 5 patients had situs solitus, 1 had situs inversus and one had left isomerism. In all cases, surgical repair was done which included excision of malaligned interatrial septum and closing the interatrial defect with dacron patch, thus directing the flow of the pulmonary vein to the left atrium. In summary, TAPVD with malaligned interatrial septum and TAPVC are two completely different entities and must be differentiated before sending the patient for surgical repair. The normal anatomical connection of pulmonary veins to left atrium is preserved in TAPVD.

Recognizing Total Anomalous Pulmonary Venous Drainage (TAPVD) from Natural History to the Supportive Imaging Modalities

Congenital Heart Disease (CHD) prevalence was 8 until 9 for each thousand live births, and among all of the CHD, there is in one of condition that involved the malformation of the venous system that called Total Anomalous Pulmonary Venous Drainage (TAPVD). TAPVD can be found and to be diagnosed in all ages, not only in the childhood era. Adult individuals with TAPVD are susceptible to being misdiagnosed as having a significant secundum atrial septal defect if the condition is not known or suspected. A systematic history taking, physical examination, and another supportive imaging modalities is needed to ensure this structural anomaly. Keyword: Congenital Heart Disease, TAPVD, Venous System Anomalies.

Clinical implications of dextrocardia based on four visceroatrial situs studies

BackgroundPrevious studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of dextrocardia in the general population. Additionally, these studies had complex classification and presentation. Nor did these studies elaborate on the distribution of the associated CHD's complexity, the various segmental connections, and associated CHD among the four visceroatrial situs. MethodsWe retrospectively reviewed the medical records of 211 children with primary dextrocardia. We used a segmental approach to diagnose CHD. We then analyzed and compared the distribution of the above-mentioned issues among the four visceroatrial situs. ResultsDextrocardia occurred most commonly with situs inversus (52.6%), followed by situs solitus (28.4%), asplenia (17.1%), and polysplenia (1.9%). Although some patients had a structurally normal heart (22.7%) or they were associated with simple CHD (17.5%), most patients had complex CHD (59.7%) consisting of a single ventricle (34.6%) or conotruncal anomaly (25.1%) (double-outlet right ventricle [7.6%], corrected transposition of the great arteries [6.2%], complete transposition of the great arteries [5.7%], tetralogy of Fallot [4.7%], etc.). Situs inversus or polysplenia had a higher prevalence of a structurally normal heart or associated with simple CHD, two patent atrioventricular (AV) valves connections, and biventricular AV connections. Situs solitus or asplenia had a higher prevalence of associated complex CHD, common AV valve connection, univentricular AV connection, pulmonary outflow tract obstruction, and anomalous pulmonary venous drainage. ConclusionOur study finds that situs inversus is the most common visceroatrial situs in dextrocardia. Although some patients had a structurally normal heart or were associated with simple CHD, most patients have associated complex CHD consisting of a single ventricle or conotruncal anomaly. Dextrocardia is associated with a higher incidence of complex CHD in situs solitus and asplenia groups than in situs inversus and polysplenia groups.

Scimitar Syndrome with Acquired Cardiac Disease: A Once-in-a-blue-moon Occurrence

Abstract Scimitar syndrome (SS) is a rare congenital anomaly characterized by various abnormalities observed in both children and adults, primarily involving partial anomalous pulmonary venous drainage from the right lung to the inferior vena cava and right lung hypoplasia. It is also known by alternative names such as congenital venolobar syndrome, Halasz syndrome, mirror-image lung syndrome, hypogenetic lung syndrome, and vena cava bronchovascular syndrome. The clinical presentation of this syndrome varies widely, ranging from asymptomatic cases to severe forms. Surgical intervention is typically required for severe cases, and the diagnosis relies predominantly on imaging studies. We present two instances of SS: one involving a patient with underlying rheumatic heart disease scheduled for valve replacement surgery and the other as an incidental finding in an asymptomatic individual, managed through medical means.

A rare variant of scimitar syndrome characterised by right pulmonary vein drainage to portal vein with eventeration of diaphragm.

While infradiaphragmatic total anomalous pulmonary venous drainage to portal vein is well described, hemianomalous drainage of right pulmonary veins to portal vein in Scimitar syndrome has not yet been reported.

Diagnosis and treatment of neonatal machete syndrome

Aim: To provide suggestions for clinical diagnosis, treatment and eugenics through the retrospective analysis of a case of neonatal machete syndrome.Methods: The clinical data of a newborn with machete syndrome admitted to the pediatrics department of Baogang Hospital of Inner Mongolia were analyzed, and suggestions of eugenics were provided based on the literatures.Results: The child, male, natural conception, G2P2, gestational age 38+4 weeks, was born by cesarean section (at am 09:24 on November 13th, 2021). He was hospitalized in our department for 9 days and given NCPAP positive pressure ventilation to give oxygen and anti-infection symptomatic treatment. The dyspnea of the child was not significantly alleviated, and the heart murmurs became increasingly obvious. The child was transferred to the neonatology Department of the Seventh Medical Center of the PLA General Hospital for further treatment on 9 days after birth. After admission, piperacillin and tazobactam were treated with anti-infection, high-frequency oscillation mode of invasive ventilator, limited fluid volume and other symptomatic treatments. Fiberbronchoscopy showed atelectasis of right lung and tracheomalacia of left lung. Echocardiography showed partial anomalous pulmonary venous drainage, atrial septal defect (secondary foramina), inferior vena cava dilatation, pulmonary hypertension, initial coronary artery dilatation, and patent ductus arteriosus. CT 3D imaging of pulmonary veins showed: Congenital heart disease: partial anomalous pulmonary venous drainage (subcardial), consideration of "machete syndrome," atrial septal defect, consideration of pulmonary hypertension, right pulmonary artery stenosis, right lower lung receiving abdominal aorta blood supply, right lung tissue dysplasia, and widening of inferior vena cava pulmonary veins. Therefore, the comprehensive diagnosis was "machete syndrome." Considering the high surgical risk of the child, conservative symptomatic treatment was recommended, and the family requested discharge after 22 days of treatment. The patient died at home at 3 months follow-up after discharge.Conclusions: Machete syndrome is a rare congenital property of abnormal partial or complete pulmonary venous drainage from the right or left lung to the inferior vena cava. With an incidence of approximately 2 in 100,000 live births, the syndrome is commonly associated with right lung dysplasia, pulmonary sequestration, persistent left superior vena cava, and right tilt of the heart. In this case, the child had these syndromes after birth, since the primary hospital did not recognize the disease, the child was clearly diagnosed in the higher hospital. Children with machete syndrome can be treated with surgery in the early neonatal period, but the indication of surgical treatment is still controversial, and the need for surgery is evaluated according to the severity of the patient's symptoms and pulmonary circulation blood flow.

Kiosk 1Q-FA-07 - Partial Anomalous Pulmonary Venous Drainage in an Operated Adolescent with Tetralogy of Fallot, Is This Significant? Role of 4-dimensional (4D) Flow

Kiosk 1Q-FA-07 - Partial Anomalous Pulmonary Venous Drainage in an Operated Adolescent with Tetralogy of Fallot, Is This Significant? Role of 4-dimensional (4D) Flow

Kiosk 2Q-TA-05 - Transcatheter Treatment of Superior Sinus Venosus Atrial Septal Defect (SVASD) with Partial Anomalous Pulmonary Venous Drainage (PAPVD): The Critical Role of 3D Modelling

Kiosk 2Q-TA-05 - Transcatheter Treatment of Superior Sinus Venosus Atrial Septal Defect (SVASD) with Partial Anomalous Pulmonary Venous Drainage (PAPVD): The Critical Role of 3D Modelling

Determination of Stenosis and Arrhythmia After Surgical Operation of Partial Anomalous Pulmonary Venous Drainage in Mid &amp; Longterm (Comparison of Double/Single Patch and Warden Operation in Partial Anomalous Pulmonary Venous Drainage): A Comparative Study of Two Different Time Intervals

We retrospectively investigated 20 patients who underwent partial anomalous pulmonary venous drainage (PAPVD) surgery with single-double patch, and Warden operation after surgical treatment in Hacettepe Medical School, department of cardiovascular surgery (group A) and 62 patients who underwent surgery with double patch in Eskişehir Osmangazi Medical School and 10 patients in Muğla Sıtkı Koçman Medical School (group B including 72 patients) at different time intervals. We compared postoperative v. cava superior stenosis, pulmonary vein stenosis and arrhythmia in regards of different techniques. We constituted 2 groups: 20 patients of Hacettepe Medical School between 2005-2011 (group A) and 72 patients of Muğla Sıtkı Koçman and Eskişehir Osmangazi Medical School between 2015-2022 (group B). Group A included 12 females, 8 males, the mean age was 5.7±3 years, ranging between 0.42 and 11 years, the mean weight was 18.67±9.01 kg, ranging between 5.50 and 41 kg. Group B included 39 females, 33 males, the mean age was 6.68±3.70 years, ranging between 1 and 17 years, mean weight was 23.58±14.75 kg, ranging between 8 and 80 kg. Clinical findings, electrocardiographic, echocardiographic evaluations were obtained. We found neither early nor late mortality in both groups. The mean follow-up duration were 39.73 months and 49.82 months respectively. No pulmonary venous or v. cava superior stenosis occurred in both groups. Reoperation for residual atrial septal defects required in 1 patient who underwent Warden procedure,1 patient who underwent double patch technique in group A. It didn’t require reoperation in group B. In group A, 6 patients presented rhythm disturbance in early postoperative period, as well as 4 patients in group B. In follow-up all patient recovered to sinus rhythm in both groups. Pacemaker wasn’t required. PAPVD can be safely operated using different procedures. Meticulous dissection nearby sinus node (SN) should be emphasized to avoid injury of SN for all techniques.

Device Closure of Superior Sinus Venosus Atrial Septal Defects: A single centre experience.

Sinus venosus atrial septal defects present a wide variety of anatomical features and are frequently associated with partial anomalous pulmonary venous drainage of one or more right pulmonary veins. Surgical correction used to be the standard treatment. In recent times, transcatheter correction of superior sinus venosus atrial septal defects has come into vogue. The transcatheter closure of these defects with covered stents at a tertiary care centre in Oman between 2018 and 2023 is reported.

Right subaxillary muscle-sparing thoracotomy for less invasive open-heart surgery: flap-double patch repair of sinus venosus atrial septal defect and partially anomalous pulmonary venous drainage.

Right subaxillary muscle-sparing thoracotomy for less invasive open-heart surgery: flap-double patch repair of sinus venosus atrial septal defect and partially anomalous pulmonary venous drainage.

Totally anomalous pulmonary venous drainage presenting with cardiovocal (Ortner's) syndrome.

Totally anomalous pulmonary venous drainage presenting with cardiovocal (Ortner's) syndrome.

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.