scholarly journals Pulmonary Vein: Embryology, Anatomy, Function and Disease

2021 ◽  
Author(s):  
Chan I-Ping ◽  
Hsueh Tung

Four pulmonary veins come from respective lung lobes drain oxygen-rich blood back to the left atrium. Failure of incorporation with the left atrium can lead to a condition, called Cor triatriatum sinister, that the left atrium is separated into two chambers by an abortive fibrous tissue. The venous system of lung and whole body communicate with each other in the earlier time and they will be disconnected in the following developmental process. Total or partial anomalous pulmonary venous connection refers to that there is/are some degree of the communication exists after birth, which can occur in different sites. In the veterinary field, retrospective studies and several case reports have been published to describe these rare congenital cardiovascular diseases in several species. More cases are need for better understanding their clinical manifestation, treatment options and outcomes.

2018 ◽  
Vol 6 (1-2) ◽  
pp. 73-77
Author(s):  
Nusrat Ghafoor ◽  
Md Rokonujjaman Selim ◽  
Nawshin Siraj

Total anomalous pulmonary venous connection is a rare congenital cardiac anomaly in which the pulmonary veins have no connection with the left atrium and connect directly to the right atrium or to one of the systemic veins. In the diagnosis of total anomalous pulmonary venous connection, the presence and severity of the venous obstruction is important. Computed Tomography (CT) imaging is known to be a useful method for the evaluation of total anomalous pulmonary venous connection. Here we report 3 cases of total anomalous pulmonary venous circulation (TAPVC) where 32 slice CT scan played an important role in pre-operative diagnosis and management. Age of the patients ranged from seven months to sixteen years. Symptoms of the patients’ varied from fatigue, shortness of breath, mild cyanosis and reduced growth for their age. Echocardiography done revealed dilated right cardiac chambers and atrial septal defect (ASD) with suspicion of TAPVC. CT scan was performed and it provided detailed information about intra-cardiac anatomy, pulmonary veins and their confluence, route, drainage and most importantly their distance and relation with the left atrium. It also provided information about coronary sinus and its drainage. This information is very important for surgical mapping and planning. Ibrahim Card Med J 2016; 6 (1&2): 73-77


2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Ignacio A. Zepeda ◽  
Peter Morcos ◽  
Luis R. Castellanos

A 73-year-old man with new onset atrial fibrillation with rapid ventricular response underwent transthoracic echocardiography that revealed an echogenic linear structure along the left atrium, suggestive of cor triatriatum sinister (CTS). CTS was confirmed with transesophageal echocardiography which demonstrated a proximal accessory atrium receiving pulmonary venous flow separated from a distal true atrium by a fibromuscular membrane with a large fenestration allowing flow between the chambers. In CTS, the left atrium is divided into proximal and distal chambers by a fenestrated fibromuscular septum. This cardiac anomaly accounts for 0.1% of cases of congenital heart disease and rarely presents in adults. CTS is primarily diagnosed with echocardiography and is associated with left atrial enlargement and development of atrial fibrillation. Treatment options depend on size of the communication between proximal and distal chambers, the gradient across the membrane, and the position of pulmonary veins. In some instances, surgical resection of the membrane that divides the left atrium is warranted.


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 ◽  
...  

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


2020 ◽  
Vol 18 (1) ◽  
pp. 28-30
Author(s):  
Zanda Grīnberga ◽  
Pauls Sīlis ◽  
Elīna Ligere ◽  
Ingūna Lubaua ◽  
Inta Bergmane ◽  
...  

SummaryCor triatriatum sinister is a rare congenital cardiac anomaly that has been identified in 0.1% of children with congenital heart disease. It is defined as a fibromuscular membrane that divides the left atrium into two chambers: a superior (proximal) that in most cases receives drainage from the pulmonary veins and an inferior (distal) chamber that communicates with the mitral valve and the left atrium. Cor triatriatum sinister can be an isolated lesion (approximately 25% of cases), but in many cases it is associated with other congenital cardiovascular anomalies, the most common one being – atrial septal defect(3). Symptoms in patients with cor triatriatum sinister are related to obstruction of pulmonary venous drainage, pressure loading of the right side of the heart and congestive cardiac failure. Depending on the severity of the obstruction and presence of associated cardiac anomalies it can be diagnosed at any age. Diagnosis is usually achieved by echocardiography in early infancy. Elective treatment method is surgical excision of the membrane. Here we present a pediatric patient (4 months old) presenting in cardiogenic shock with a successful correction of isolated cor triatriatum sinister. To confirm diagnosis and success of surgical repair, transthoracic and transesophageal echocardiography were used.


2021 ◽  

We describe the lateral approach to the surgical repair of a total anomalous pulmonary venous connection in this video tutorial. The goal of the operation is to create an unobstructed anastomosis between the pulmonary confluence and the left atrium, ligate the systemic venous connections, and close the atrial septal defect. After a median sternotomy and initial dissection of the structures surrounding the heart, cardiopulmonary bypass is initiated by aortobicaval cannulation. The patient is then cooled to attain mild hypothermia (30°C). The heart is arrested by a dose of antegrade cold cardioplegia. The right pleural cavity is opened widely. The heart is retracted and pushed into the right pleural cavity. The vertical vein is ligated near its connection with the innominate vein. An incision is made along the length of the confluence, stopping short of the individual pulmonary veins. The left atrial appendage is retracted, and the left atrium is opened in alignment with the opening in the confluence. The left atrium and the pulmonary confluence are anastomosed widely with 7-0 polypropylene suture material. The heart is put back into the mediastinum. Rewarming is started. The atrial septal defect is closed through the right atrium using a large untreated autologous pericardium patch. The patient is then weaned off cardiopulmonary bypass.


2021 ◽  
Author(s):  
Takahiro Ito ◽  
Ikuo Hagino ◽  
Mitsuru Aoki

Abstract Background: Total anomalous pulmonary venous connection with unilateral lung agenesis and Goldenhar syndrome is extremely rare and high mortality.Case presentation: We present a case of total anomalous pulmonary venous connection with unilateral lung agenesis and Goldenhar syndrome. It was diagnosed from Transthoracic echocardiography and enhanced Computed Tomography. Total absence of the lung, the bronchial tree, and vascular structures were detected on the right side, and the left pulmonary veins returned abnormally to the innominate vein. There was apparent indication of pulmonary venous obstruction, the operation was performed at 3 days after birth. The common pulmonary venous chamber with vertical vein and the left atrium was anastomosed using 7-0 PDS running sutures through a median sternotomy. Postoperative echocardiography and Computed Tomography 1 year after the surgery, between the common pulmonary venous chamber and the left atrium was no stenosis.Conclusion: A extremely rare case of total anomalous pulmonary venous connection with unilateral lung agenesis and Goldenhar syndrome successfully repaired at 3 day after birth was reported. Anastomosis between the common pulmonary venous chamber and the left atrium using the vertical vein is a reasonable choice in patient with small common pulmonary venous chamber.


2004 ◽  
Vol 14 (5) ◽  
pp. 553-556 ◽  
Author(s):  
Sara E. Monaco ◽  
Welton M. Gersony ◽  
Harshwardhan M. Thaker

We describe an infant with hypoplasia of the left heart diagnosed prenatally who, at birth, had signs of severe pulmonary venous obstruction. Echocardiography indicated normally connecting pulmonary veins, and showed a paradoxical right-to-left shunt across a patent oval foramen. Postmortem examination revealed that the obstruction was due to a divided left atrium, or cor triatriatum sinister, with an imperforate muscular diaphragm separating completely the two components of the divided atrium.


1970 ◽  
Vol 1 (1) ◽  
pp. 59-61
Author(s):  
Md Mazibur Rahman ◽  
Sheikh Muhammad Shaheedul Islam ◽  
Md Sirajul Islam ◽  
Nawshin Siraj ◽  
Sultana Khanum ◽  
...  

A one year and three months male child having Total Anomalous Pulmonary Venous Connection (TAPVC) presented at Ibrahim Cardiac Hospital and Research Institute with repeated cough, tachypnoea, fever, failure to thrive & diaphoresis particularly during feeding with a mild degree of cyanosis without any evidence of heart failure. He underwent successful rerouting of pulmonary veins to left atrium (LA). The procedure consisted of side to side wide anastomosis between confluence of pulmonary veins (CPV) and left atrium with the division and ligation of vertical vein at its opening to left innominate vein and closure of atrial septal defect (ASD). The patient is doing well without any symptoms at followup. Key words: TAPVC; CPV; Vertical vein Ibrahim Card Med J 2011; 1(1):56-58


2017 ◽  
Vol 21 (2) ◽  
pp. 132-137 ◽  
Author(s):  
Fawwaz R. Shaw ◽  
Jonathan M. Chen

Total anomalous pulmonary venous connection (TAPVC) is predominantly a neonatal congenital cardiac diagnosis in which the pulmonary veins fail to return to the left atrium. The presence or absence of obstruction defines the clinical presentation, and in the setting of obstructed pulmonary veins, this diagnosis represents a true surgical emergency. Improvements in perioperative care and refinement of surgical techniques continue to allow for improved survival and decreased morbidity. A description of the surgical anatomy and key concepts in the perioperative and surgical management of TAPVC are presented in this review.


2006 ◽  
Vol 2 (1) ◽  
pp. 1
Author(s):  
Josef Kautzner ◽  
Hanka Micochova ◽  
Petr Peichl ◽  
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