Anomalous Pulmonary Venous Connection Research Articles

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.

Congenital Pulmonary Lymphangiectasia Masked by Postoperative Pulmonary Venous Obstruction in an Infant with Total Anomalous Pulmonary Venous Connection.

Congenital pulmonary lymphangiectasia (CPL) is associated with fetal pulmonary venous obstructive physiology. The precise morbidity of CPL is unknown as CPL is generally fatal in neonates. Here, we report an infant with secondary CPL in total anomalous pulmonary venous connection (TAPVC). He developed severe pulmonary hypertension (PH) after corrective surgery for TAPVC. However, cardiac catheterization showed mild left pulmonary venous obstruction (PVO), which was deemed unnecessary for re-intervention. He died at 11 months-old due to an exacerbation of PH. Autopsy revealed medial hypertrophy of the pulmonary arteries, mild left PVO, and marked dilatation and proliferation of the pulmonary lymphatics which might have been involved in the PH, although CPL was not conclusively identified based on the previous biopsy findings. We should be aware of the possibility of CPL in addition to postoperative PVO when encountering patients with fetal pulmonary venous obstructive physiology. Furthermore, a cautious approach to the interpretation of lung biopsy results is warranted.

A Very Rare Case of Bilateral Partial Anomalous Pulmonary Venous Connection in an Adult

A Very Rare Case of Bilateral Partial Anomalous Pulmonary Venous Connection in an Adult

Four-dimensional imaging of fetal infracardiac total anomalous pulmonary venous connection with double drainage.

Four-dimensional imaging of fetal infracardiac total anomalous pulmonary venous connection with double drainage.

Successful surgical management of total anomalous pulmonary ve-nous return in a pediatric patient

Successful surgical management of total anomalous pulmonary ve-nous return in a pediatric patient

Unusual Doppler in Pulmonary Veins Leads to the Diagnosis of Unusual Partial Anomalous Pulmonary Venous Return with Dual Connections

Unusual Doppler in Pulmonary Veins Leads to the Diagnosis of Unusual Partial Anomalous Pulmonary Venous Return with Dual Connections

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.

From Other Journals: A Review of Recent Articles by Our Editorial Team.

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) A new index for prenatal diagnosis of total anomalous pulmonary venous return, (2) Outcomes of patients with Tetralogy of Fallot after pulmonary valve replacement (PVR), and (3) Short-term outcomes of the self expanding Harmony valve for transcatheter PVR.

Unilateral Anomalous Pulmonary Venous Return into Coronary Sinus.

Unilateral Anomalous Pulmonary Venous Return into Coronary Sinus.

Risk factors and outcomes for surgical repair of obstructed total anomalous pulmonary venous connection.

Surgical repair of obstructed total anomalous pulmonary venous connection (TAPVC) is a high-risk surgical cohort. This study aimed to assess surgical risk factors and outcomes in infants with TAPVC treated at a single centre. This was a prospective single-centre, observational study that included consecutive patients presenting with obstructed TAPVC. Patients with obstructed supra-cardiac, cardiac, and infra-cardiac TAPVC were included in the study. In-hospital mortality was the primary outcome. Multivariable logistic regression was carried out to identify risk factors for in-hospital mortality. Of the 41 patients, 31 (75.6%) were males. Ages were distributed as follows: 20 (48.8%) between 1 and 3 months, 11 (26.88%) between >3 months, and 10 (24.4%) neonates. The in-hospital mortality was 19.5% (n=8). Of the remaining 33 patients, there were no deaths during the follow-up. Short-term follow-up was achieved for 100% of cases (up to 6 months post-operatively). The mean duration of follow-up was 43.6± 3.6 months. One (3%) early and 2 (6%) late pulmonary vein obstructions were observed during follow-up. Age less than 30 days and pre-operative ventilation were associated with significantly increased risk of in-hospital mortality. Despite logistical challenges, reasonable surgical outcomes are attainable in obstructed TAPVC cases. Age less than 30 days and need for pre-operative ventilation were independent predictors of in-hospital mortality, while pulmonary venous obstruction in the post-operative period contributed to re-interventions.

A 15-Year Experience With Total Anomalous Pulmonary Venous Connection in Vietnam.

This article aims to demonstrate the morphology of 261 total anomalous pulmonary venous connection (TAPVC) cases operated at Children's Hospital 1 with in-hospital mortality of 19.5% (51/261). All the surgical protocols of TAPVC cases repaired between 2008 and June 2023 were reviewed. The descriptions of TAPVC were based on operative findings by surgeons. A total of 261 TAPVC patients were operated, including 124 (47.5%) supra, 83 (31.8%) intra, 41 (15.7%) infra, and 13 (5%) mixed cases. The in-hospital mortality was 19.5% (51/261). Fifteen cases are associated with other anomalies of the heart. Four subtypes of 124 supra TAPVC were found, with 42 (33.9%) obstructed cases. The standard was all pulmonary veins (PVs) forming a common vein (CV) and draining into the innominate veins, then going to the superior vena cava (SVC) (100/124, 80.6%). Eleven supra TAPVC cases were vascular vise type. Ten cases had the vertical vein running from the right of the CV and draining directly into the SVC. Of 83 intracardiac TAPVCs with 9 (10.8%) obstructed cases, the most common was all PVs draining directly into the coronary sinus (60/83, 72.3%). The second was all PVs draining directly into the right atrium (RA) via separated ostia or forming a CV before entering the RA (17/83, 20.5%). Also, there were three cases with rare variants and 100% obstruction when the diagnosis was explored. The in-hospital mortality of intracardiac type was 13.3% (11/83) 41 infra TAPVC with obstructed rate of 61% (25/41) and in-hospital mortality of 29.3% (12/41). Thirteen mixed TAPVCs were repaired, with most cases having three PVs forming a CV. This article provides valuable information about the morphology of TAPVC types in Asian patients.

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.

Partial anomalous pulmonary venous return: a casual finding in many cases

Partial anomalous pulmonary venous return: a casual finding in many cases

A case of partial anomalous pulmonary venous connection (PAPVC) detected during left upper lobectomy for simple pulmonary aspergilloma

A case of partial anomalous pulmonary venous connection (PAPVC) detected during left upper lobectomy for simple pulmonary aspergilloma

Partial anomalous pulmonary venous return: a casual finding in many cases.

Partial anomalous pulmonary venous return: a casual finding in many cases.

Total Anomalous Pulmonary Venous Connections, Human Genetics.

Partial anomalous pulmonary venous connections (PAVC) have been found after abnormal gene expressions involving several syndromes. Total anomalous pulmonary venous connection (TAPVC) is found in conjunction with heterotaxia syndrome as well as several other syndromes. It has been reported with an autosomal dominance with variable expression and incomplete penetrance. The occurrence is also related to environmental factors which may superimpose on a familial susceptibility for TAPVC. Many pathways are involved in the normal development of the pulmonary venous connections and as a consequence disturbance of many genetic and epigenetic pathways lead to partial or total pulmonary venous misconnections. In this chapter, an overview of current knowledge regarding human genetics of anomalous venous connections is provided.

Kiosk 2R-TC-10 - Detection of Previously Undiagnosed Partial Anomalous Pulmonary Venous Return via 4D Flow Cardiac Magnetic Resonance Imaging in Patients with Turner Syndrome

Kiosk 2R-TC-10 - Detection of Previously Undiagnosed Partial Anomalous Pulmonary Venous Return via 4D Flow Cardiac Magnetic Resonance Imaging in Patients with Turner Syndrome

Kiosk 2Q-TA-02 - A Rare Case of Ebstein's Anomaly with Partial Anomalous Pulmonary Venous Connection

Kiosk 2Q-TA-02 - A Rare Case of Ebstein's Anomaly with Partial Anomalous Pulmonary Venous Connection

TAPVR: Molecular Pathways and Animal Models.

The venous pole of the heart where the pulmonary veins will develop encompasses the sinus venosus and the atrium. In the fourth week of development, the sinus venosus consists of a left and a right part receiving blood from the common cardinal vein, the omphalomesenteric and umbilical veins. Asymmetrical expansion of the common atrium corresponds with a rightward shift of the connection of the sinus to the atrium. The right-sided part of the sinus venosus including its tributing cardinal veins enlarges to form the right superior and inferior vena cava that will incorporate into the right atrium. The left-sided part in human development largely obliterates and remodels to form the coronary sinus in adults. In approximately the same time window (4th-fifth weeks), a splanchnic vascular plexus surrounds the developing lung buds (putative lungs) with a twofold connection. Of note, during early developmental stages, the primary route of drainage from the pulmonary plexus is toward the systemic veins and not to the heart. After lumenization of the so-called mid-pharyngeal endothelial strand (MPES), the first anlage of the pulmonary vein, the common pulmonary vein can be observed in the dorsal mesocardium, and the primary route of drainage will gradually change toward a cardiac drainage. The splanchnic pulmonary venous connections with the systemic cardinal veins will gradually disappear during normal development. In case of absence or atresia of the MPES, the pulmonary-to-systemic connections will persist, clinically resulting in total anomalous pulmonary venous return (TAPVR). This chapter describes the developmental processes and molecular pathways underlying anomalous pulmonary venous connections.