Anomalous Pulmonary Venous Connection Research Articles

A Case Report of an Uncommon Association of Total Intracardiac Anomalous Pulmonary Venous Return and Tetralogy of Fallot

A Case Report of an Uncommon Association of Total Intracardiac Anomalous Pulmonary Venous Return and Tetralogy of Fallot

Ultrasound and computed tomography angiography diagnosis of fetal right atrial isomerism with cardiac total anomalous pulmonary venous connection and intestinal malrotation: a case report and literature review.

Right atrial isomerism is a rare and severe isomerism. It is frequently associated with complex congenital heart disease and various extracardiac anomalies. Imaging diagnosis of right atrial isomerism is a challenge. Multisystem and complex anomalies in a 24-week-old fetus were diagnosed with prenatal ultrasound, postnatal computed tomography angiography (CTA), and autopsy. The ultrasound detected most major cardiovascular anomalies including right atrial isomerism and total anomalous pulmonary venous connection. The CTA further detected thoracic and abdominal malformations such as bilateral morphologically right bronchus, diaphragmatic hernia, asplenia, midline liver, and intestinal malrotation. The autopsy confirmed both ultrasound and CTA findings with additional findings, namely, bilateral trilobed lungs and bilateral morphological right auricles. Prenatal ultrasound and postnatal CTA can be complementary to each other in detecting multi-system complex anomalies. Their combined use can be useful for prenatal counseling and postpartum management.

Advanced Imaging Guidance for Ductus Venosus Stenting in Obstructed Total Anomalous Pulmonary Venous Return.

Obstructed infracardiac total anomalous pulmonary venous return is nearly always a surgical emergency in which infants present in severe cardiopulmonary distress. Ductal venosus stenting can provide a temporizing option for premature, low birth weight infants with high risk for surgical complications. In challenging anatomic cases, virtual reality, 3D-printed models, and fusion image guidance can aid in procedural planning and provide support for successful intervention.

Images of Extremely Rare Cantrell Phenomenon

We present a case of a neonate born with prenatal diagnosis of Cantrell syndrome and ectopia cordis. This extremely rare congenital disorder underscores the significant need for multimodality imaging to plan further management. The aim of the study was to present the thoracoabdominal syndrome using a three-dimensional computed tomography angiography. The CT scans confirmed complex intracardiac defects consisting of tetralogy of Fallot, total anomalous pulmonary venous return and persistent left superior vena cava. In conclusion, Cantrell syndrome necessitates a multidisciplinary approach, from the onset of the prenatal diagnosis followed by prompt medical imaging and surgical interventions after birth. The thoracoabdominal wall defect including complete ectopia cordis is an extremely rare disorder with a fatal outcome.

Insight into the research history and trends of total anomalous pulmonary venous connection: a bibliometric analysis

BackgroundTotal anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease characterized by the inability of all pulmonary veins to connect to the left atrium. Our previous bibliometric article summarized the characteristics of only the 100 most cited papers in TAPVC research. The purpose of this study was to use comprehensive bibliometric analysis to examine the development history, current status, and future trends in the field of TAPVC.MethodsAll publications on TAPVC published between 2000 and 2023 were collected from the Web of Science Core Collection. The publication and citation data were quantitatively analyzed by publication year, country, institution, author, and journal. Co-authorship and co-occurrence analyses were performed using VOSviewer, and keyword and reference bursts were identified using CiteSpace. Pearson’s test was used to examine the correlations between two continuous variables.ResultsAs of July 20, 2023, we identified 368 publications with 3320 citations. These publications were published in 132 journals and authored by 1835 researchers from 457 institutions in 47 countries. For the number of publications, the top country, top institution, top author, and top journals were the United States (n = 82), Shanghai Jiao Tong University (n = 13), Huiwen Chen (n = 9), and Annals of Thoracic Surgery and Pediatric Cardiology (n = 29 each), respectively. For the number of citations, the top country, top affiliation, top author, and top journal were the United States (n = 1348), University of Toronto (n = 250), Christopher A. Caldarone (n = 315), and Annals of Thoracic Surgery (n = 746), respectively. The number of national publications significantly correlated with GDP (R = 0.887, P < 0.001), research & development (R&D) expenditure (R = 0.375, P = 0.013), population (R = 0.694, P < 0.001), and journals (R = 0.751, P < 0.001). The number of national citations significantly correlated with GDP (R = 0.881, P < 0.001), R&D expenditure (R = 0.446, P = 0.003), population (R = 0.305, P = 0.037), and journals (R = 0.917, P < 0.001). International collaboration in the field of TAPVC was not well developed. The most commonly cited publication discussed era changes in mortality and reoperation rate in TAPVC patients. The most common keywords were “total anomalous pulmonary venous connection” and “congenital heart disease”. The keyword “case report” appeared most recently, with an average occurrence year of 2021.8. The co-occurrence analysis grouped 26 keywords into six themes: surgical repair of TAPVC, postoperative pulmonary vein stenosis, surgical repair of TAPVC patients with heterotaxy, application of echocardiography in diagnosing TAPVC, application of echocardiography in the prenatal diagnosis of TAPVC, and application of the sutureless technique in the surgical repair of TAPVC patients with right atrial isomerism or a single ventricle. Citation burst detection identified 32 references with citation bursts, seven of which had ongoing citation bursts until 2023.ConclusionsThis study conducted a bibliometric analysis to provide a comprehensive overview of TAPVC research. We hope to offer new ideas for promoting development in the field of TAPVC.

Percutaneous closure of a sinus venosus defect with partial anomalous pulmonary venous return.

A 73-year-old man with a history of hypertension, hyperlipidemia, and obesity presented for cardiovascular evaluation. He was experiencing mild fatigue and dyspnea on exertion. Transthoracic echocardiogram (TTE) showed right ventricular dilation, which was otherwise unremarkable.

Surgical anastomosis of vertical vein to left atrial appendage: A case report of technical aspects

Management of partial anomalous pulmonary venous connection (PAPVC) differs significantly between the defect on the left side, which is adjacent to left atrial appendage, and that on the right one adjacent to the interatrial septum. Here, we report two patients operated at our center. The first case is an adult diagnosed with the left-sided PAPVC draining to an innominate vein through a left-sided vertical vein, and with a large ostium secundum atrial septal defect. The second case is a neonate with mixed-type total anomalous pulmonary venous connection with all right pulmonary veins opening into the coronary sinus, and all the left pulmonary veins connecting to the innominate vein through a left-sided vertical vein. Both patients experienced severe pulmonary arterial hypertension, necessitating surgical treatment involving disconnection of the vertical vein from its junction, or, ligation of the vertical vein-innominate vein junction, coupled with its redirection to the left atrial appendage. We employed different surgical procedures on these patients, given the distinct age difference, intending to anastomose their respective vertical veins to the adjacent left atrial appendage. In this report, the technical aspects of anastomosing vertical vein to left atrial appendage, to avoid pulmonary venous obstruction in these two patients, are discussed.

Case Report: A comprehensive rehabilitation protocol in a post-operative case of ASD closure along with PAPVC

An array of congenital cardiac disorders known as partial anomalous pulmonary venous connections (PAPVC) occur when pulmonary veins, abnormally drain into the right atrium. This can happen directly into the right atrium or via draining into systemic veins. A 10 year old female patient had chief complaints of difficulty in breathing and frequent fever hence she visited rural hospital where 2D echo was done which reviled PAPVC associated with 16 mm ASD (Atrial Septal Defect). She underwent ASD closure along with pulmonary valvotomy as a part of surgical management after which a well-planned cardiac rehabilitation has been ingrained through several breathing methods such as breathing exercises, respiratory muscle strength and home exercise program, helping the patient to carry out her activities of daily living. The Visual Analog Scale (VAS), Respiratory Muscle Strength using MicroRPM device, 6 Minute Walk Distance (MWD), Borg Scale and Fatigue Severity Scale (FSS). In this present instance, carefully thought-out physical therapy rehabilitation has been shown to be successful in enhancing functional capacity, respiratory muscle strength, optimizing functional capacity, and lowering fatigue.

Managing bilateral discontinuous pulmonary arteries of ductal origin in single ventricle anatomy

We describe outcomes and management strategies for single ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus. We reviewed 22 patients with aforementioned anatomy and PA centralization from 1995-2023, excluding those with biventricular repair. Median age at centralization was 9 days (range 0 days-2 years). Centralization was performed with systemic-to-pulmonary shunt (n=20, 91%; 2 following bilateral ductal stents) or bidirectional cavopulmonary connection (n=2, 9%) via pericardial roll (n=14, 64%), patch-augmented direct anastomosis (n=7, 32%), and interposition graft (n=1, 5%) techniques. Concurrent total anomalous pulmonary venous connection (TAPVC, n=11, 50%) was associated with significantly inferior survival (P=.01). Five patients (23%) died at a median of 59 (6-257) days post-centralization, all with non-cardiac TAPVC. At latest follow-up for 17 survivors (median 13.5 [0.5-25.1] years post-centralization), 12 completed Fontan, 4 completed second stage palliation, and 1 was transplanted prior to second stage palliation. Fourteen patients (64%) required PA reintervention, including 3 with reoperations independent of staged palliation. Baseline to pre-second stage echocardiography demonstrated branch PA growth with significantly increased diameters (left P=.0006, right P=.0002); z-scores significantly increased for right (P=.004) but not left (P=.11). Successful single ventricle palliation is possible, though high-risk, for patients with bilateral discontinuous ductal PAs. Early post-centralization mortality remains substantial, particularly with associated non-cardiac TAPVC. Many require reintervention to maintain PA growth, typically concurrently with staged palliation.

Prenatal diagnosis of isolated total anomalous pulmonary venous connection (TAPVC) to coronary sinus.

Prenatal diagnosis of isolated total anomalous pulmonary venous connection (TAPVC) to coronary sinus.

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.

Complete Abnormal Venous Return Associated with Complex Heart Disease: Case Report S. Faiz, M. Boussif, B. Zouita, D. Basraoui, H. Jalal Published: April 27, 2024 | 4 2 DOI: 10.36347/sjmcr.2024.v12i04.035 Pages: 531-533 Downloads Abstract Total anomalous pulmonary venous return (TAPVR) is a cardiac malformation defined by a complete absence of communication between the pulmonary veins and the left atrium. All

Total anomalous pulmonary venous return (TAPVR) is a cardiac malformation defined by a complete absence of communication between the pulmonary veins and the left atrium. All oxygenated pulmonary venous return flows directly or indirectly into the right atrium. Survival is only possible via a right-to-left atrial shunt. Non-blocked forms present as high-flow shunts with moderate cyanosis. Symptoms in blocked forms are dominated by obstruction of venous return, and life-threatening distress with intense cyanosis and severe pulmonary hypertension sets in from the very first days of life. Treatment consists in connecting the pulmonary venous receptacle to the left atrium, tying off the collector and closing the inter-atrial septum. The risks of surgery remain high in severe forms, and failures usually occur in the first year of life. In the case of good surgical correction, the long-term prognosis is excellent.

Prenatal Ultrasound Markers of Isolated Total Anomalous Pulmonary Venous Return and a Sequential Approach to Reach Diagnosis

Abstract This article comprehensively reviews the literature concerning prenatal ultrasound findings of isolated total anomalous pulmonary venous return (TAPVR) and the application of specific sonographic markers to differentiate among the TAPVR types. These markers can be categorized as direct and indirect, based on either morphological features or hemodynamic changes in TAPVR. Indirect markers include the ventricular disproportion, an increased distance between the left atrium (LA) and the descending aorta, as well as the dilatation of superior vena cava or coronary sinus for supracardiac or cardiac TAPVR, along with abnormal pulmonary venous spectral Doppler patterns. Direct markers predominantly focus on the absence of a connection between the pulmonary veins and the LA. Some direct markers are specific to certain TAPVR types, such as the vertical vein. Diagnosing isolated TAPVR can pose challenges, but following a sequential approach can improve detection rates and outcomes. In cases with equivocal findings, additional follow-ups are recommended. The sequential approach described in the current article provides a step-wise methodology and sonographic markers for prenatal diagnosis of TAPVR, which can be utilized by fetal–maternal medicine specialists, obstetricians, and radiological technicians to ensure timely interventions.

Delayed Diagnosis of Right Heart Failure Due to Sinus Venosus Atrial Septal Defect, Persistent Left Superior Vena Cava and Partial Anomalous Pulmonary Venous Return

Sinus venosus atrial septal defects (SVASD) are uncommon and often underdiagnosed as they are visualised poorly on transthoracic echocardiography (TTE). A 59-year-old man was referred following worsening dyspnoea. A TTE demonstrated dilated right chambers, with torrential tricuspid regurgitation and a dilated coronary sinus. Agitated saline test raised the suspicion of both persistent left superior vena cava (PLSVC) and an intracardiac shunt, despite interrogation of the interatrial septum not demonstrating any defects. CT imaging of the thorax was performed revealing a superior SVASD alongside a PLSVC, and partial anomalous pulmonary venous return. SVASD are considerably uncommon, compared to other types of atrial septal defects (ASD). TTE has poor sensitivity in diagnosing SVASD, highlighting the importance of multimodality imaging in such cases. The purpose of this case report is to highlight unique features demonstrated on TTE, especially during the agitated saline study, which showcased the presence of a PLSVC alongside an intracardiac shunt that was undetected through conventional TTE alone.

Total Anomalous Pulmonary Venous Connection in Adults: Should We Offer Surgery?

Background: We aim to determine the surgical outcomes of adult patients with total anomalous pulmonary venous connection (TAPVC) and examine the regression of pulmonary artery (PA) pressures after the procedure. Methods: We reviewed the hospital records from 2003 to 2022 and identified 49 adult patients with TAPVC. We assessed their surgical outcomes and the trend of PA pressures after the procedure. Continuous data are presented as mean ± SD or median (interquartile range) and categorical variables are presented as percentages. Results: The median age of the patients was 23 years (range 18-42) and 31 (63.3%) were male. Thirty-six patients (73.5%) had supracardiac TAPVC. The mean systolic PA pressure was 65.8 ± 16.4 mm Hg and it decreased by 47.9% (34%, 61.8%) after surgery. Moderate or more tricuspid regurgitation was seen in 27 (55.1%) patients before surgery; however, it was present in only 3 (6.1%) patients during early follow-up. There was no intraoperative or 30-day mortality, and the median hospital length of stay was six days. Long-term follow-up data were available for 29 patients with the average duration of follow-up being 5.6 years (range 6 months to 15 years) and the mean systolic PA pressures of this cohort was 29.8±7.9 mm Hg. Forty-six (93.1%) patients were asymptomatic; four women had uneventful pregnancies and delivered healthy children. Conclusion: Surgical repair of the naturally selected group of adult TAPVC patients can be performed safely with good results. Regression in flow-related pulmonary hypertension and an improvement in functional quality of life are seen in nearly all patients.

Supracardiac totally anomalous pulmonary venous connection with an unusual drainage pathway

Supracardiac totally anomalous pulmonary venous connection with an unusual drainage pathway

Clinical characteristics, imaging findings, management, and outcomes of patients with scimitar syndrome at a tertiary referral healthcare center in Colombia.

Scimitar Syndrome is part of a complex spectrum of congenital cardiovascular anomalies related to anomalous pulmonary venous return. Depending on the extent of involvement, treatment can be either expectant or surgical. Prognosis and survival have been controversial, with some results supporting early surgical management. This research aims to disclose the outcomes and describe the management, clinical and imaging characteristics of patients diagnosed with Scimitar Syndrome treated in a tertiary referral healthcare center. Longitudinal descriptive observational study. The study included all patients diagnosed with scimitar syndrome in our institution between January/2011 and December/2022. A description of the sociodemographic and clinical characteristics, diagnostic tools used, treatment features, and patient outcomes is provided. Eleven patients were included, with a mean age at diagnosis of five years (CI 0-17), six of which were female (54.55%). Nine (81.82%) patients had evidence of a scimitar vein on the chest radiograph, six (54.55%) cardiac dextroposition, six (54.55%) pulmonary hypoplasia, five (45.45%) right pulmonary artery hypoplasia, and three (27.27%) had aortopulmonary collaterals. Four (36.36%) patients had horseshoe lungs, and four (36.36%) had bronchopulmonary sequestration. In the associations, two (18.18%) patients were found to have an atrial septal defect, three (27.27%) ventricular septal defect, and one (9%) had Tetralogy of Fallot. Pulmonary hypertension was demonstrated in two (18.18%) patients. Seven (63.64%) required surgical management to correct the scimitar vein, and two patients died due to unrelated complications. Scimitar syndrome presents diagnostic and treatment challenges, necessitating a multidisciplinary approach for timely care. Chest radiography and CT scans are primary diagnostic tools, with surgical intervention often warranted alongside other heart defects or significant hemodynamic repercussions. Medical management is effective for mild to moderate cases. Long-term patient outcomes remain uncertain due to study limitations, but improved life expectancy is anticipated with ongoing care.

Complex pulmonary venous drainage pattern with obstructive partial anomalous pulmonary venous connection and associated meandering pulmonary vein

Complex pulmonary venous drainage pattern with obstructive partial anomalous pulmonary venous connection and associated meandering pulmonary vein

Partial Anomalous Pulmonary Venous Connection with Ventricular Septal Defect and Intact Atrial Septum

Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiac abnormality in which the pulmonary veins drain into the right atrium directly or the superior vena cava 90% of PAPVC are accompanied by atrial septal defect. However, PAPVC with ventricular septal defect is a rare entity.

Exposure to Endocrine-Disrupting Chemicals and Congenital Heart Diseases: The Pooled Results Based on the Current Evidence.

The relationships between maternal exposure to endocrine-disrupting chemicals (EDCs) and congenital heart diseases (CHD) are not elucidated yet. The exposure levels of EDCs are generally estimated based on self-reported questionnaires or occupational exposure evaluations in the literature. Therefore, a study based on epidemiological data from human biospecimens is required to provide stronger evidence between maternal exposure to EDC and CHD. Embase, Pubmed, Scopus, and the Cochrane Library databases were searched for related research which provided risk estimates regarding the relationships between maternal EDC exposure and CHD in human offspring. Baseline characteristics and outcomes of CHD were extracted from each included study. Odds ratios (ORs) with 95% confidence intervals (CIs) were pooled to calculate the overall estimates of CHD. Subgroup and meta-regression analyses were performed to identify the sources of heterogeneity. Bootstrapping techniques were used in analyses where several studies originated from a similar population. A total of seventeen studies were involved in the meta-analyses. Maternal EDC exposure was significantly related to CHD in offspring (OR 2.15; 95%CI 1.64 to 2.83). EDC exposure was significantly associated with septal defects (OR 2.34; 95%CI 1.77 to 3.10), conotruncal defects (OR 2.54; 95%CI 1.89 to 3.43), right ventricular outflow tract obstruction (OR 2.65; 95%CI 1.73 to 4.07), left ventricular outflow tract obstruction (OR 3.58; 95%CI 2.67 to 4.79), anomalous pulmonary venous return (OR 2.31; 95%CI 1.34 to 4.00), and other heart defects (OR 2.49; 95%CI 1.75 to 3.54). In addition, maternal exposure to heavy metals, which included lead (OR 2.19; 95%CI 1.29 to 3.71), cadmium (OR 1.81; 95%CI 1.28 to 2.56), mercury (OR 2.23; 95%CI 1.13 to 4.44), and manganese (OR 2.65; 95%CI 1.48 to 4.74), increased risks for CHD significantly. In conclusion, based on the latest evidence, maternal EDC exposure may increase CHD risks in human offspring, especially in heavy metal exposure conditions.