scholarly journals Neonatal Repair of Total Anomalous Pulmonary Venous Connection with Goldenhar Syndrome and Unilateral Lung Agenesis：A Case Report

2021 ◽  
Author(s):  
Takahiro Ito ◽  
Ikuo Hagino ◽  
Mitsuru Aoki
Keyword(s):  
Computed Tomography ◽  
Left Atrium ◽  
Pulmonary Veins ◽  
Median Sternotomy ◽  
Pulmonary Venous Obstruction ◽  
Goldenhar Syndrome ◽  
Vertical Vein ◽  
Lung Agenesis ◽  
The Common ◽  
Anomalous Pulmonary Venous Connection

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.

scholarly journals Neonatal repair of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome: a case report

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Takahiro Ito ◽  
Ikuo Hagino ◽  
Mitsuru Aoki ◽  
Kentaro Umezu ◽  
Tomohiro Saito ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Pulmonary Veins ◽  
Median Sternotomy ◽  
Bronchial Tree ◽  
Pulmonary Venous Obstruction ◽  
Goldenhar Syndrome ◽  
Venous Obstruction ◽  
Vertical Vein ◽  
Lung Agenesis ◽  
Anomalous Pulmonary Venous Connection

Abstract Background Total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome is extremely rare. Case presentation We present a case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome in a patient who was diagnosed based on transthoracic echocardiography and computed tomography. We observed complete absence of the lung, the bronchial tree, and vascular structures on the right side, with abnormal drainage of the left pulmonary veins into the innominate vein. The patient showed clear clinical evidence of pulmonary venous obstruction and underwent surgery 3 days after birth. The pulmonary venous chamber containing the vertical vein was anastomosed to the left atrium using 7–0 PDS running sutures via a median sternotomy. Echocardiography and computed tomography performed 1 year postoperatively revealed no pulmonary venous obstruction. Conclusion We report a rare case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome, which was successfully repaired 3 days after birth. A median sternotomy is a safe and effective approach for surgical repair of congenital heart disease with unilateral lung agenesis. Repair of the supra cardiac total anomalous pulmonary connection using the vertical vein is feasible in patients with a small pulmonary venous chamber.

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

scholarly journals Atypical Mixed Total Anomalous Pulmonary Venous Connection: A Case Report

2018 ◽  
Vol 33 (2) ◽  
pp. 138-140
Author(s):  
Md Abul Kalam Azad ◽  
Naharuma Aive Haider Chowdhury ◽  
Abul Kalam Shamsuddin
Keyword(s):  
Computed Tomography ◽  
Respiratory Tract Infections ◽  
Mixed Type ◽  
Pulmonary Veins ◽  
Vertical Vein ◽  
Ct Angiogram ◽  
History Of ◽  
Tract Infections ◽  
Anomalous Pulmonary Venous Connection ◽  
Bluish Discoloration

A 2 years old boy presented to us with a history of repeated respiratory tract infections and bluish discoloration of tongue, lips and figure tips for last 18 months. Echocardiography and Computed tomography (CT) angiogram revealed total anomalous pulmonary venous connection (TAPVC) mixed type (supracardiac and cardiac);all pulmonary veins drain into a common chamber behind left atrium (LA) and left lower pulmonary vein (LUPV) drains to vertical vein and common chamber both.The patient underwent rerouting of pulmonary veins and vertical vein ligation above the drainage of LUPV. Bangladesh Heart Journal 2018; 33(2) : 138-140

Repair of a mixed form of supracardiac total anomalous pulmonary venous connection

2020 ◽  
pp. 1-3
Author(s):  
Yuki Kawasaki ◽  
John N. Dentel ◽  
Henry L. Walters ◽  
James M. Galas ◽  
Daisuke Kobayashi
Keyword(s):  
Superior Vena ◽  
Pulmonary Veins ◽  
Vena Cava ◽  
Right Atrial ◽  
Pulmonary Venous Obstruction ◽  
Mixed Form ◽  
Vertical Vein ◽  
Vena Cava Superior ◽  
Anomalous Pulmonary Venous Connection

Abstract Total anomalous pulmonary venous connection is a rare congenital heart defect. We report an infant with a mixed form of supracardiac TAPVC, in whom all pulmonary veins, except the right upper, entered a pulmonary venous confluence that is connected to a vertical vein and drained into the superior vena caval–right atrial junction. Several segmental right upper pulmonary veins entered the superior vena cava, superior to the entry of the vertical vein. Surgical repair consisted of the Warden procedure combined with direct anastomosis of the vertical vein to the left atrium. Separate pulmonary venous drainage pathways decreased the risk of post-operative pulmonary venous obstruction. Our patient had an uneventful post-operative course and encouraging 2-month follow-up echocardiography. Careful follow-up is warranted to detect post-operative complications, including obstruction of the pulmonary venous and cavoatrial anastomoses.

ATRESIA OF THE COMMON PULMONARY VEIN

PEDIATRICS ◽  
1962 ◽  
Vol 29 (5) ◽  
pp. 729-739
Author(s):  
Russell V. Lucas ◽  
Bertram F. Woolfrey ◽  
Ray C. Anderson ◽  
Richard G. Lester ◽  
Jesse E. Edwards
Keyword(s):  
Left Atrium ◽  
Pulmonary Vein ◽  
Pulmonary Veins ◽  
Early Death ◽  
Pulmonary Venous Obstruction ◽  
Usual Sense ◽  
Venous Obstruction ◽  
Functional Connection ◽  
The Common ◽  
Common Pulmonary Vein

Among the causes of pulmonary venous obstruction is atresia of the common pulmonary vein. This entity is described in three patients. The anatomic abnormality in these patients was the absence of any functional connection between the pulmonary veins and the left atrium, and anomalous pulmonary venous connection in the usual sense was not present. The results of the severe pulmonary venous obstruction imposed by this abnormality are reflected clinically in severe cyanosis, congestive cardiac failure, and early death. Roentgenograms revealed the diffuse reticular pattern in the pulmonary fields associated with pulmonary venous obstruction. Angiocardiographic techniques permit anatomic diagnosis of this cardiac abnormality and allow its differentiation from the other cardiac abnormalities responsible for pulmonary venous obstruction. Because the cul-de-sac-like confluence of the pulmonary veins is of ample size and lies immediately behind the left atrium, surgical relief of this condition seems possible.

scholarly journals Multislice Computed Tomography Imaging of Total Anomalous Pulmonary Venous Circulation: Three Rare Case Reports

2018 ◽  
Vol 6 (1-2) ◽  
pp. 73-77
Author(s):  
Nusrat Ghafoor ◽  
Md Rokonujjaman Selim ◽  
Nawshin Siraj
Keyword(s):  
Computed Tomography ◽  
Ct Scan ◽  
Left Atrium ◽  
Case Reports ◽  
Pulmonary Veins ◽  
Cardiac Anomaly ◽  
Congenital Cardiac Anomaly ◽  
Venous Circulation ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

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

Dual drainage total anomalous pulmonary venous connection: a rare mixed variant

2018 ◽  
Vol 26 (4) ◽  
pp. 305-307 ◽  
Author(s):  
Ch Bharat Siddharth ◽  
Mayank Yadav ◽  
Amol Bhoje ◽  
Milind P Hote
Keyword(s):  
Computed Tomography ◽  
Adult Patient ◽  
Coronary Sinus ◽  
Mixed Type ◽  
Pulmonary Veins ◽  
Common Variant ◽  
Venous Anatomy ◽  
Vertical Vein ◽  
Anomalous Pulmonary Venous Connection ◽  
Mixed Variant

The mixed type of total anomalous pulmonary venous connection is the least common variant, occurring in approximately 5% of all patients. Dual drainage through a common venous confluence is much rarer. Computed tomography to delineate the exact pulmonary venous anatomy is a must in such cases. Correct preoperative recognition and intraoperative confirmation to check the drainage of all 4 pulmonary veins is essential in all cases of total anomalous pulmonary venous connection. We report the case of an adult patient with dual drainage to coronary sinus and left vertical vein to innominate vein.

scholarly journals Total Anomalous Pulmonary Venous Connection (TAPVC) - A Case Report

1970 ◽  
Vol 1 (1) ◽  
pp. 59-61
Author(s):  
Md Mazibur Rahman ◽  
Sheikh Muhammad Shaheedul Islam ◽  
Md Sirajul Islam ◽  
Nawshin Siraj ◽  
Sultana Khanum ◽  
...  
Keyword(s):  
Heart Failure ◽  
Left Atrium ◽  
Septal Defect ◽  
Pulmonary Veins ◽  
Failure To Thrive ◽  
Mild Degree ◽  
Vertical Vein ◽  
One Year ◽  
Left Innominate Vein ◽  
Anomalous Pulmonary Venous Connection

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

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.

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