Partial anomalous pulmonary venous connection and pulmonary venous varix coexist in Turner syndrome: a case report

2021 ◽  
pp. 1-3
Author(s):  
Qiao Li ◽  
Xiang Li ◽  
Xiao Li ◽  
Yuan Feng
Keyword(s):  
Turner Syndrome ◽  
Aortic Coarctation ◽  
Pulmonary Veins ◽  
Morphological Characteristics ◽  
Pulmonary Angiography ◽  
Clinical Syndrome ◽  
Cardiovascular Malformations ◽  
Anomalous Pulmonary Venous Connection ◽  
Pulmonary Vein Obstruction ◽  
Venous Varix

Abstract Turner syndrome is a clinical syndrome caused by autosomal abnormalities in women. It is often accompanied by congenital cardiovascular malformations commonly including a bicuspid aortic valve malformation and aortic coarctation, but the presence of multiple pulmonary venous abnormalities is extremely rare. We present a 27-year-old woman who was diagnosed with Turner syndrome. She was revealed an anomalous right upper pulmonary venous connection, left upper pulmonary vein obstruction, and varicose vein malformations of the left pulmonary veins by a series of examination. Cardiac catheterisation and selective pulmonary angiography can further confirm the diagnosis, morphological characteristics, haemodynamic significance and provide a reference for the next step of treatment.

scholarly journals Cardiovascular findings and management in Turner syndrome: insights from a French cohort

2012 ◽  
Vol 167 (4) ◽  
pp. 517-522 ◽  
Author(s):  
Bruno Donadille ◽  
Alexandra Rousseau ◽  
Delphine Zenaty ◽  
Sylvie Cabrol ◽  
Carine Courtillot ◽  
...  
Keyword(s):  
Turner Syndrome ◽  
Aortic Coarctation ◽  
Cardiovascular Events ◽  
Systematic Approach ◽  
Aortic Diameter ◽  
Aortic Dilatation ◽  
Cardiovascular Monitoring ◽  
Cardiovascular Malformations ◽  
Resonance Imaging ◽  
French Cohort

ObjectiveCongenital cardiovascular malformations and aortic dilatation are frequent in patients with Turner syndrome (TS). The objective of this study was to investigate the cardiovascular findings and management in a large cohort of patients, including children and adults.Design/methodsWe recruited 336 patients with TS from a network of tertiary centers. We reviewed their files, checking for cardiovascular events, cardiac valve abnormalities, and aortic diameters indexed to body surface area (BSA) from magnetic resonance imaging (n=110) or echocardiography (n=300).ResultsInformative cardiovascular data were available for only 233 patients. Vascular surgery was reported in 7.4% of the cohort. The first cause of surgery was aortic coarctation, detected in 6.9% at a median age of 9.5 (range: 0–60) years. Bicuspid aortic valve (BAV) was detected in 21% at a median age of 20 years (25th–75th percentiles: 15–30). At least one aortic diameter exceeded 32 mm in 12% of the cohort. This was detected at a median age of 19 (7–30) years. When indexed to BSA, at least one aortic diameter exceeded 20 mm/m2 in 39% of the cohort.ConclusionOur study shows that cardiovascular monitoring for TS patients is currently insufficient in France. BAV is present at birth, but often remains undiagnosed until later in life. Therefore, improved management in cardiovascular monitoring is required and a more systematic approach should be taken.

Supracardiac Total Anomalous Pulmonary Venous Connection

KYAMC Journal ◽  
2019 ◽  
Vol 10 (2) ◽  
pp. 118-121
Author(s):  
ASM Shariful Islam ◽  
Md Lutfar Rahman ◽  
Jayanta Kumar Saha ◽  
Mohammad Arifur Rahman ◽  
Mezanur Rahman ◽  
...  
Keyword(s):  
Left Atrium ◽  
Ductus Arteriosus ◽  
Pulmonary Veins ◽  
Hypertensive Crisis ◽  
Right Heart Failure ◽  
Residual Shunt ◽  
Low Cardiac Output Syndrome ◽  
Vertical Vein ◽  
Autologous Pericardial Patch ◽  
Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease in which there is developmental absence of connection of all four pulmonary veins with the left atrium. To report a rare case and share our experience in surgery and post-operative management for supracardiac TAPVC. Patient with supracardiac TAPVC with atrial septal defect (ASD) secundum variety with rudimentary patent ductus arteriosus (PDA) underwent rechanneling of pulmonary veins to left atrium (LA) with gluteryldehye treated autologous pericardial patch closure of ASD with ligation of ascending vertical vein and ligation of rudimentary PDA.Post operatively there were no events of pulmonary hypertensive crisis, low cardiac output syndrome, right heart failure or conduction defect were observed and echocardiogram showed adequate pulmonary venous drainage with no residual shunt across the interatrial septum. Marked development in surgical results of TAPVC has been observed in recent years with declining mortality rate from 65% in early sixties to 5% in current surgical scenerio. KYAMC Journal Vol. 10, No.-2, July 2019, Page 118-121

Cor Triatriatum in Association With a Unique Form of Partial Anomalous Pulmonary Venous Connection

2021 ◽  
pp. 215013512110469
Author(s):  
Lou Capecci ◽  
Richard D. Mainwaring ◽  
Inger Olson ◽  
Frank L. Hanley
Keyword(s):  
Superior Vena Cava ◽  
Superior Vena ◽  
Pulmonary Veins ◽  
Vena Cava ◽  
Venous Drainage ◽  
Cor Triatriatum ◽  
Unique Form ◽  
Elective Repair ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

Cor triatriatum may be associated with abnormalities of pulmonary venous anatomy. This case report describes a unique form of partial anomalous pulmonary venous connection. The patient presented at 5 weeks of age with symptoms of tachypnea and poor feeding. Echocardiography demonstrated cor triatriatum and partial anomalous pulmonary venous drainage of the right upper lung. The patient underwent urgent repair of cor triatriatum. It was elected to not address the partial anomalous pulmonary venous connection at that time. The patient returned at age 19 months for elective repair of the anomalous pulmonary venous connection. There was also a large vein connecting the right lower pulmonary veins to the superior vena cava. This was repaired by dividing the superior vena cava along a vertical axis to redirect the flow of the anomalous pulmonary veins through the connecting vein to the left atrium. This report describes the anatomy and surgical approach to a unique form of anomalous pulmonary venous connection.

scholarly journals Unligated vertical vein presenting as a large atrio-portal shunt in adulthood: a case report

2020 ◽  
Vol 2020 (10) ◽  
Author(s):  
Georgia R Layton ◽  
Marinos Koulouroudias ◽  
Eyad Issa ◽  
Steve Jepson ◽  
Antonio F Corno ◽  
...  
Keyword(s):  
Case Report ◽  
Portal Vein ◽  
Adult Patient ◽  
Pulmonary Veins ◽  
Systemic Circulation ◽  
Review Of The Literature ◽  
Right Heart ◽  
Vertical Vein ◽  
Initial Surgery ◽  
Anomalous Pulmonary Venous Connection

Abstract A 28-year-old male with infra-cardiac totally anomalous pulmonary venous connection (TAPVC) repaired as new-born presented in adulthood with right heart strain and very large left atrium to portal vein vessel. Residual connections from pulmonary veins to systemic circulation are believed to represent persistent ‘vertical veins’ (VV) not ligated at the time of the initial surgery. In our patient, since endovascular occlusion was not judged suitable, the anomalous vessel was surgically ligated and resected. A review of the literature failed to find such a procedure reported in an adult patient and analyzed the intra-operative ligation of VV during repair of TAPVC.

Descending aortic flow reversal in obstructed total anomalous pulmonary venous connection

2016 ◽  
Vol 26 (5) ◽  
pp. 1002-1004
Author(s):  
Anupama K. Nair ◽  
Sitaraman Radhakrishnan ◽  
Krishna S. Iyer
Keyword(s):  
Ductus Arteriosus ◽  
Pulmonary Veins ◽  
Left Ventricular ◽  
Flow Reversal ◽  
Aortic Flow ◽  
Severe Pulmonary Hypertension ◽  
Descending Thoracic Aorta ◽  
Descending Aorta ◽  
Left Ventricular Output ◽  
Anomalous Pulmonary Venous Connection

AbstractIn this study, we present the case of a neonate with obstructed infracardiac total anomalous pulmonary venous connection with severe pulmonary hypertension and a patent ductus arteriosus with right-to-left shunting. The patient had an unusual finding of pandiastolic flow reversal in the upper descending thoracic aorta. He underwent emergency surgical re-routing of the pulmonary veins to the left atrium, and postoperative echocardiography showed disappearance of the descending aortic flow reversal. We hypothesise that in severely obstructed total anomalous pulmonary venous connection the left ventricular output may be extremely low, resulting in flow reversal in the descending aorta.

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