Browse
Submit Data
Databases
API
Help

Dataset Information

21 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Total transcatheter correction of scimitar variant having dual pulmonary venous drainage.

ABSTRACT: Scimitar syndrome is a clinical triad of anomalous pulmonary venous drainage, lung hypoplasia, and anomalous aortic blood supply to the lung segment. When there is dual pulmonary venous drainage both to inferior vena cava and left atrium, it is called scimitar variant. A young child presenting with recurrent chest infections, dextroposition of the heart, and scimitar shadow on chest X-ray was identified to have scimitar variant after a detailed evaluation and managed successfully by catheter interventions. This report discusses the embryogenesis and angiographic findings of scimitar variants, indications for interventions, and issues in its management.

SUBMITTER: Singh AS

PROVIDER: S-EPMC7727905 | biostudies-literature | 2020 Oct-Dec

REPOSITORIES: biostudies-literature

ACCESS DATA

Json Xml

Publications

Total transcatheter correction of scimitar variant having dual pulmonary venous drainage.

Singh Arvind Sahadev AS Sivakumar Kothandam K

Annals of pediatric cardiology 20200901 4

Scimitar syndrome is a clinical triad of anomalous pulmonary venous drainage, lung hypoplasia, and anomalous aortic blood supply to the lung segment. When there is dual pulmonary venous drainage both to inferior vena cava and left atrium, it is called scimitar variant. A young child presenting with recurrent chest infections, dextroposition of the heart, and scimitar shadow on chest X-ray was identified to have scimitar variant after a detailed evaluation and managed successfully by catheter int ...[more]

PMID: 33311925

Similar Datasets

Complete transcatheter correction of variant scimitar syndrome-a case report.

Project description:BackgroundScimitar syndrome (SS) comprises of an anomalously draining right pulmonary vein (APV), to the inferior vena cava (IVC), maldevelopment of the right pulmonary artery (RPA), and the right lung, with variable number of aorto-pulmonary collaterals (APC) to the right lung. It can cause pulmonary hypertension if left untreated. Surgical correction is the method of choice. We report a case of variant SS with dual drainage of the APV to the IVC and left atrium (LA) that was addressed with a transcatheter approach.Case summaryA 13-year-old child was evaluated for dyspnoea. Chest x-ray and transthoracic echo (TTE) were suggestive of SS with an additional central atrial septal defect (ASD). Cardiac computed tomography (CT) revealed dual drainage of the APV to the IVC and via a meandering vein to the LA and three APCs. The ASD was closed, and the APCs were coiled. The connection of the APV to the IVC was closed with a device, rerouting the pulmonary vein blood to the LA. The child is doing well on follow-up after 2 years.DiscussionVariant forms of SS are rare. Our case had ASD, multiple APCs, well-developed RPA and right lung and a dual drainage of the APV. This allowed for transcatheter management. Otherwise, surgery is the default choice. Multimodality imaging with TTE, CT, magnetic resonance imaging, and cardiac catheterization will help in diagnosis and anatomical delineation.

| S-EPMC9671289 | biostudies-literature

Scimitar sign in the absence of anomalous pulmonary venous drainage: a case report.

Project description:Scimitar syndrome consists of anomalous pulmonary vein drainage to the inferior vena cava. Its name derives from the image this anomalous pulmonary vein creates on a chest radiograph. We describe a case of normal venous pulmonary vein drainage that also presented the scimitar sign due to an aorto-collateral vessel. A 15-month-old girl presented with mild dyspnoea and fever. Control chest X-ray showed an image of cardiac dextroposition, hypoplastic right lung, and the 'scimitar sign'. Although the transthoracic echocardiogram confirmed the initial suspicion of anomalous pulmonary venous drainage, the computed tomography (CT) scan showed normal right pulmonary veins connected to the left atrium and revealed that an aorto-collateral vessel caused the scimitar sign. Although the patient had several typical alterations of the scimitar syndrome, the pulmonary venous connection was normal, and the scimitar sign was due to an aorto-collateral vessel. It might be difficult to describe venous pulmonary connections on the basis of echocardiography, so an angio CT scan proved to be a valuable tool in this scenario.

| S-EPMC6601148 | biostudies-literature

Unusual drainage pattern of a supracardiac total anomalous pulmonary venous connection.

Project description: Not available

| S-EPMC4368941 | biostudies-literature

Partial anomalous venous connection with intrapulmonary dual drainage: Transcatheter treatment of a rare entity.

Project description:Partial anomalous pulmonary venous return (PAPVR) is a rare congenital condition, and dual-drainage connection PAPVR to the left atrium has been reported in a few cases in the literature; in which cases, percutaneous catheterization was successfully used in lieu of surgery. We, hereby, describe a 7-month-old boy with a functional single-ventricle physiology with dual drainage of the left upper pulmonary vein to the left atrium and the innominate vein. Appropriate recognition of this entity allowed safe occlusion of the anomalous draining vein.

| S-EPMC10593273 | biostudies-literature

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

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

| S-EPMC5241853 | biostudies-other

Bilateral pulmonary vein stenting for pulmonary vein obstruction after surgical correction of total abnormal pulmonary venous connection.

Project description: Not available

| S-EPMC5336951 | biostudies-literature

Total anomalous pulmonary venous return with mixed drainage and double connection: a rare case report not previously described.

Project description:BackgroundTotal anomalous pulmonary venous return is a rare cyanotic congenital heart disease in which pulmonary veins connect to the systemic veins, right atrium, or coronary sinus. Given its variability, it is essential to define the pathway and drainage site.Case summaryWe describe a 3-day-old patient with total anomalous pulmonary venous return, in which the four pulmonary veins drained into a common vertical vein that had a double connection at the cardiac (coronary sinus) and infracardiac (portal vein) levels. The echocardiographic diagnosis was confirmed by computed tomography scan. At 1.5 months, she underwent surgery by anastomosis of the venous collector with the left atrium, unroof of the coronary sinus, and clousure of its communication with right atrium. The vertical vein was ligated. There were no complications after 1 year of follow-up.DiscussionThis is an unusual anatomy that has not been described in the literature. In this article, we discuss the embryological origin. Furthermore, we highlight the importance of an accurate diagnosis of the venous pathway and its impact on surgical planning and patient prognosis.

| S-EPMC9631977 | biostudies-literature

Opitz GBBB syndrome with total anomalous pulmonary venous connection: A new MID1 gene variant.

Project description:BackgroundOpitz GBBB syndrome (GBBB) is an X-linked disease characterized by midline defects, including congenital heart defects. We present our diagnostic approach to the identification of GBBB in a consanguineous family in which two males siblings were concordant for a total anomalous connection of pulmonary veins and minor facial dysmorphias.MethodsTargeted exome sequencing analysis of a 380-gene panel associated with cardiovascular disease was performed on the propositus. Interpretative analysis of the exome results was conducted, and 3D models of the protein changes were generated.ResultsWe identified a NM_000381.4:c.608G>A;p.(Arg203Gln) change in MID1, affecting the conformation of the B-box 2 domain of the protein, with a zinc finger structure and associated protein interactions. This clinical phenotype is consistent with GBBB; however, the type of congenital heart disease observed in this case has not been previously reported.ConclusionA new likely pathogenic variant on MID1 c.608G>A was found to be associated with Opitz GBBB syndrome.

| S-EPMC10496055 | biostudies-literature

Total anomalous systemic venous drainage with heterotaxia syndrome: a rare case.

Project description:Total anomalous systemic venous return is a very rare anomaly, where vena cava inferior, vena cava superior, and coronary sinus drain into left atrium. Two-day-old male baby was admitted with cyanosis and tachypnea after the birth. Left atrial isomerism with anomalous systemic venous drainage was found on echocardiographic examination. We present an unusual case of total anomalous systemic venous drainage in to the left atrium.

| S-EPMC4145383 | biostudies-literature

Left superior vena cava or left vertical vein in supracardiac total anomalous pulmonary venous drainage - Does it make a difference?

Project description:What do we call a vascular structure that is in the left pneumopericardial space, drains systemic venous blood, and the total pulmonary venous return, into the right atrium, through a dilated coronary sinus (CS)? Can we preserve the CS drainage while correcting the total anomalous pulmonary venous connection?

| S-EPMC10015388 | biostudies-literature

OmicsDI is part of the ELIXIR infrastructure

OmicsDI is an Elixir interoperability service. Learn more ›

Tweets

OmicsDI Databases

PRIDE
PeptideAtlas
MassIVE
JPOST Repository
Physiome Model Repository

EGA
EVA
ENA
LINCS
PAXDB
Cell Collective

MetaboLights
Metabolomics Workbench
MetabolomeExpress
GNPS
BioModels
FAIRDOMHub

ArrayExpress
dbGaP
ExpressionAtlas
GEO
NODE

Information

Databases
Help
API
Contact us
Code on GitHub
Terms of use
Submit Data