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.

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 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.

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 with Lethal Pulmonary Venous Obstruction Managed by Multidisciplinary Cooperation

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Kana Ito ◽  
Ayako Chida-Nagai ◽  
Osamu Sasaki ◽  
Nobuyasu Kato ◽  
Takeshi Umazume ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Caesarean Section ◽  
Atrial Septal Defect ◽  
Congenital Heart ◽  
Septal Defect ◽  
Superior Vena ◽  
Vena Cava ◽  
Pulmonary Venous Obstruction ◽  
Venous Obstruction ◽  
Anomalous Pulmonary Venous Connection

Background. Total anomalous pulmonary venous connection (TAPVC) is a critical congenital heart disease for which emergency surgery is required after birth. In cases of no intervention, TAPVC is associated with a high mortality rate in the first year of life. Although foetal echocardiographic techniques for diagnosing TAPVC have improved, TAPVC remains one of the most difficult congenital heart diseases to diagnose via foetal echocardiography. Here, we report a case of TAPVC with pulmonary venous obstruction (PVO), which was diagnosed via foetal echocardiography. Case Presentation. On foetal echocardiography at 32 weeks’ gestation, a large atrial septal defect, enlarged superior vena cava, and continuous flow pattern in the vertical vein from the common chamber were observed in the foetus. Paediatric cardiologists and cardiac surgeons, neonatologists, and obstetricians planned to perform a caesarean section and emergency heart surgery. The male infant was born at 37 weeks’ gestation via caesarean section, and postnatal echocardiography revealed PVO at the confluence of the superior vena cava and common chamber. Similarly, chest computed tomography confirmed the foetal diagnosis. The postnatal diagnoses were TAPVC type Ib, PVO, atrial septal defect, and patent ductus arteriosus. Surgical repair of the TAPVC was initiated within the first 3 hours of life. Screening brain echocardiography and head computed tomography revealed intracranial haemorrhage and hydrocephalus. Therefore, the patient underwent emergency bilateral external drainage on day 13. On day 48, a ventriculoperitoneal shunt was inserted owing to progressive brain ventricular dilatation. The patient was discharged home on postoperative day 68. Conclusions. Although the prognosis of TAPVC with PVO remains poor, continuous observation through foetal echocardiography and early interdepartmental collaboration can result in good outcomes.

Total Anomalous Pulmonary Venous Connection: Preoperative Anatomy, Physiology, Imaging, and Interventional Management of Postoperative Pulmonary Venous Obstruction

2016 ◽  
Vol 21 (2) ◽  
pp. 123-131 ◽  
Author(s):  
Matthew D. Files ◽  
Brian Morray
Keyword(s):  
Postoperative Complications ◽  
Right Atrium ◽  
Surgical Repair ◽  
Venous Blood ◽  
Pulmonary Veins ◽  
Pulmonary Venous Obstruction ◽  
Venous Obstruction ◽  
Systemic Vein ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection refers to a spectrum of cardiac anomalies where the pulmonary veins fail to return to the left atrium and the pulmonary venous blood returns through a systemic vein or directly to the right atrium. There is a wide anatomical variety of venous connections and degrees of pulmonary venous obstruction that affect the presentation, surgical repair, and outcomes. In this review, we explore the preoperative physiology, echocardiographic diagnosis, and approach to postoperative complications.

Repair of Total Anomalous Pulmonary Venous Connection in Early Infancy

2003 ◽  
Vol 11 (1) ◽  
pp. 18-22 ◽  
Author(s):  
Anil Kumar Dharmapuram ◽  
Raghavan Nair Suresh Kumar ◽  
Pantula Narasinga Rao ◽  
Hassan Mohamed Mahmoud ◽  
Sushil Chandran ◽  
...  
Keyword(s):  
Anastomotic Stricture ◽  
Pulmonary Arteries ◽  
Early Infancy ◽  
Surgical Correction ◽  
Pulmonary Vein Stenosis ◽  
Pulmonary Venous Obstruction ◽  
Venous Obstruction ◽  
Mortality And Morbidity ◽  
Vertical Vein ◽  
Anomalous Pulmonary Venous Connection

From May 1995 through October 2001, 19 infants less than 90 days old underwent surgical correction of total anomalous pulmonary venous connection. In 15 babies with isolated total anomalous pulmonary venous connection, there was one operative death. In 4 with complex anomalies, there were 2 operative deaths. The vertical vein was not ligated in 6 cases for various reasons. Two patients died during reoperation for early pulmonary venous obstruction. In the late follow-up, 2 babies required reoperation for late anastomotic stricture; one needed additional balloon dilatation. Of the 14 surviving patients, one had a small residual gradient and infrequent supraventricular tachycardia, the others were asymptomatic and without gradients. Surgical correction of total anomalous pulmonary venous connection can be carried out in early infancy with low mortality and morbidity. However, associated complex cardiac anomalies and small caliber pulmonary arteries and veins carry higher risks. Recurrent pulmonary venous obstruction and diffuse pulmonary vein stenosis are causes of early reoperation and poor surgical outcome.

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 Total Anomalous Pulmonary Venous Connection with Severe Pulmonary Venous Obstruction

Circulation ◽  
1962 ◽  
Vol 25 (6) ◽  
pp. 916-928 ◽  
Author(s):  
ALOIS R. HASTREITER ◽  
MILTON H. PAUL ◽  
MARIAN E. MOLTHAN ◽  
ROBERT A. MILLER
Keyword(s):  
Pulmonary Venous Obstruction ◽  
Venous Obstruction ◽  
Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection

ESC CardioMed ◽  
2018 ◽  
pp. 831-834
Author(s):  
Marietta Charakida ◽  
John Deanfield
Keyword(s):  
Long Term Results ◽  
Pulmonary Venous Obstruction ◽  
Venous Obstruction ◽  
Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection is repaired in childhood and the long-term results are usually excellent. Recent surgical advances have reduced further the rate of recurrent pulmonary venous obstruction.

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