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.

scholarly journals Right Upper Lobe Partial Anomalous Pulmonary Venous Connection

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Christos Tourmousoglou ◽  
Christina Kalogeropoulou ◽  
Efstratios Koletsis ◽  
Nikolaos Charoulis ◽  
Christos Prokakis ◽  
...  
Keyword(s):  
Lung Cancer ◽  
Right Atrium ◽  
Venous Return ◽  
Superior Vena ◽  
Pulmonary Veins ◽  
Vena Cava ◽  
Systemic Vein ◽  
Anomalous Pulmonary Venous Return ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

Partial anomalous pulmonary venous return (PAPVR) is a left-to-right shunt where one or more, but not all, pulmonary veins drain into a systemic vein or the right atrium. We report a case of a 45-year-old male with PAPVR to superior vena cava which was incidentally discovered during a right lower bilobectomy for lung cancer.

scholarly journals Novel technique of repairing right partial anomalous pulmonary venous connection with intact atrial septum using in situ interatrial septum as a flap in a 68-year-old-woman: a case report

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Atsushi Morishita ◽  
Ikuo Hagino ◽  
Hideyuki Tomioka ◽  
Seiichiro Katahira ◽  
Takeshi Hoshino ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Right Atrium ◽  
Septal Defect ◽  
Surgical Repair ◽  
Interatrial Septum ◽  
Pulmonary Artery Hypertension ◽  
Right Heart ◽  
Intact Atrial Septum ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

Abstract Background Partial anomalous pulmonary venous connection draining into the right atrium with an intact atrial septum is a very rare clinical entity in the adult population. Partial anomalous pulmonary venous connection must be suspected as a differential diagnosis when the cause of right heart enlargement and pulmonary artery hypertension is unknown. Case presentation This study describes the surgical case of an isolated right partial anomalous pulmonary venous connection to the right atrium in a 68-year-old woman, who underwent tricuspid ring annuloplasty and right-sided maze procedure simultaneously. She had complaints of gradually progressing dyspnea on exertion. However, a diagnosis could not be established despite consultations at multiple hospitals for over a year. Right heart catheterization revealed severe pulmonary artery hypertension with a mean pulmonary artery pressure of 46 mmHg, step-up phenomenon of oxygen saturation at the mid-level of the right atrium with a pulmonary-to-systemic blood flow ratio of 2.4, and a pulmonary vascular resistance of 3.1 Wood Units. As medical treatment with pulmonary artery vasodilator therapy did not improve her symptoms, she underwent surgical repair. An atrial septal defect was created surgically with a curvilinear tongue-shaped cut. The right anomalous pulmonary veins were rerouted through the surgically created atrial septal defect into the left atrium with a baffle comprised of the interatrial septum flap, kept in continuity with the anterior margin and sutured while mobilizing the enlarged right atrium. The patient had an uneventful postoperative course and remains asymptomatic. Conclusions The described surgical technique could be considered an effective alternative for patients undergoing surgical repair for a partial anomalous pulmonary venous connection isolated to the right atrium. The indication for surgery must be judged on a case-by-case basis in these patients with prevalent systemic-to-pulmonary shunting.

scholarly journals Repair of an Isolated Right Partial Anomalous Pulmonary Venous Connection by Rerouting Blood Flow Through an Interatrial Septum Flap in a 68-Year-Old-Woman: A Case Report

2020 ◽  
Author(s):  
Atsushi Morishtia ◽  
Ikuo Hagino ◽  
Hideyuki Tomioka ◽  
Seiichiro Katahira ◽  
Takeshi Hoshino ◽  
...  
Keyword(s):  
Blood Flow ◽  
Pulmonary Artery ◽  
Right Atrium ◽  
Septal Defect ◽  
Surgical Repair ◽  
Interatrial Septum ◽  
Pulmonary Artery Hypertension ◽  
Right Heart ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

Abstract Background: Partial anomalous pulmonary venous connection draining into the right atrium with an intact atrial septum is a very rare clinical entity in the adult population. Partial anomalous pulmonary venous connection must be suspected as a differential diagnosis when the cause of right heart enlargement and pulmonary artery hypertension is unknown.Case presentation: This study describes the surgical case of an isolated right partial anomalous pulmonary venous connection to the right atrium in a 68-year-old woman, who underwent tricuspid ring annuloplasty and right-sided maze procedure simultaneously. She had complaints of gradually progressing dyspnea on exertion. However, a diagnosis could not be established despite consultations at multiple hospitals for over a year. Right heart catheterization revealed severe pulmonary artery hypertension with a mean pulmonary artery pressure of 46 mmHg, step-up phenomenon of oxygen saturation at the mid-level of the right atrium with a pulmonary-to-systemic blood flow ratio of 2.4, and a pulmonary vascular resistance of 3.1 Wood Units. In addition, it was mandatory to comprehensively utilize valuable imaging modalities, such as transthoracic echocardiography, transesophageal echocardiography, and multidetector computed tomography angiography for the accurate diagnosis and efficient surgical planning of this partial anomalous pulmonary venous connection. As medical treatment with pulmonary artery vasodilator therapy did not improve her symptoms, she underwent surgical repair. An atrial septal defect was created surgically with a curvilinear tongue-shaped cut. The right anomalous pulmonary veins were rerouted through the surgically created atrial septal defect into the left atrium with a baffle comprised of the interatrial septum flap, kept in continuity with the anterior margin and sutured while mobilizing the enlarged right atrium. The patient had an uneventful postoperative course and remains asymptomatic. Conclusions: The described surgical technique could be considered an effective alternative for patients undergoing surgical repair for a partial anomalous pulmonary venous connection isolated to the right atrium. The indication for surgery must be judged on a case-by-case basis in these patients with prevalent systemic-to-pulmonary shunting.

scholarly journals Novel technique of repairing right partial anomalous pulmonary venous connection with intact atrial septum using in situ interatrial septum as a flap in a 68-year-old-woman: a case report

2020 ◽  
Author(s):  
Atsushi Morishita ◽  
Ikuo Hagino ◽  
Hideyuki Tomioka ◽  
Seiichiro Katahira ◽  
Takeshi Hoshino ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Right Atrium ◽  
Septal Defect ◽  
Surgical Repair ◽  
Interatrial Septum ◽  
Pulmonary Artery Hypertension ◽  
Right Heart ◽  
Intact Atrial Septum ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

Abstract Background: Partial anomalous pulmonary venous connection draining into the right atrium with an intact atrial septum is a very rare clinical entity in the adult population. Partial anomalous pulmonary venous connection must be suspected as a differential diagnosis when the cause of right heart enlargement and pulmonary artery hypertension is unknown.Case presentation: This study describes the surgical case of an isolated right partial anomalous pulmonary venous connection to the right atrium in a 68-year-old woman, who underwent tricuspid ring annuloplasty and right-sided maze procedure simultaneously. She had complaints of gradually progressing dyspnea on exertion. However, a diagnosis could not be established despite consultations at multiple hospitals for over a year. Right heart catheterization revealed severe pulmonary artery hypertension with a mean pulmonary artery pressure of 46 mmHg, step-up phenomenon of oxygen saturation at the mid-level of the right atrium with a pulmonary-to-systemic blood flow ratio of 2.4, and a pulmonary vascular resistance of 3.1 Wood Units. As medical treatment with pulmonary artery vasodilator therapy did not improve her symptoms, she underwent surgical repair. An atrial septal defect was created surgically with a curvilinear tongue-shaped cut. The right anomalous pulmonary veins were rerouted through the surgically created atrial septal defect into the left atrium with a baffle comprised of the interatrial septum flap, kept in continuity with the anterior margin and sutured while mobilizing the enlarged right atrium. The patient had an uneventful postoperative course and remains asymptomatic.Conclusions: The described surgical technique could be considered an effective alternative for patients undergoing surgical repair for a partial anomalous pulmonary venous connection isolated to the right atrium. The indication for surgery must be judged on a case-by-case basis in these patients with prevalent systemic-to-pulmonary shunting.

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.

Modified Warden Operation With the Use of Femoral Vein Homograft for Repair of a Variant of Right-Sided Partial Anomalous Pulmonary Venous Connection

2020 ◽  
Vol 11 (2) ◽  
pp. 217-219
Author(s):  
Josue Chery ◽  
Karthik Ramakrishnan ◽  
Russel Cross ◽  
Richard A. Jonas
Keyword(s):  
Superior Vena Cava ◽  
Superior Vena ◽  
Femoral Vein ◽  
Pulmonary Veins ◽  
Vena Cava ◽  
Pulmonary Venous Obstruction ◽  
Anatomical Variant ◽  
Anomalous Pulmonary Venous Return ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

Surgical repair of right-sided partial anomalous pulmonary venous return (PAPVR) involves baffling the pulmonary vein across a naturally occurring or surgically created atrial septal defect without causing pulmonary venous or superior vena cava obstruction. A nine-year-old male presented to us with an unusual anatomical variant of right-sided partial anomalous pulmonary venous connection. The pulmonary veins draining the right upper and middle lobes connected to the azygous vein that drained in the usual fashion into the superior vena cava. The Warden operation was modified, with the use of femoral vein homograft, to avoid pulmonary venous obstruction.

Surgical repair of total anomalous pulmonary venous connection: Lateral approach

2021 ◽  
Keyword(s):  
Cardiopulmonary Bypass ◽  
Atrial Septal Defect ◽  
Left Atrium ◽  
Septal Defect ◽  
Surgical Repair ◽  
Pleural Cavity ◽  
Pulmonary Veins ◽  
Lateral Approach ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

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.

scholarly journals Perioperative Echocardiographic Features of Total Anomalous Pulmonary Venous Connection

2016 ◽  
Vol 4 (2) ◽  
pp. 51-58
Author(s):  
Parag Barwad ◽  
Goverdhan Dutt Puri ◽  
Vikas Dutta ◽  
Alok Kumar
Keyword(s):  
Right Atrium ◽  
Pulmonary Veins ◽  
Flow Imaging ◽  
Color Flow ◽  
Interatrial Communication ◽  
Color Flow Imaging ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection ◽  
Anomalous Pulmonary Venous Connections

ABSTRACT Total anomalous pulmonary venous connection (TAPVC) refers to when all four pulmonary veins connect anomalously to the right atrium, directly or indirectly. Perioperative echocardiography, both transthoracic and transesophageal (TEE) with color flow imaging and Doppler interrogation, can identify the venous confluence, its connections, obstructions if any in the pathway, and any interatrial communication. They supplement each other in delineating the anatomy of such anomalous pulmonary venous connections. Perioperative TEE evaluation of patients with repaired TAPVC confirms the adequacy of repair, leaks or stenosis of the venous baffle. We summarize the role of perioperative echocardiography in understanding cases of TAPVC. How to cite this article Kumar A, Dutta V, Puri GD, Barwad P. Perioperative Echocardiographic Features of Total Anomalous Pulmonary Venous Connection. J Perioper Echocardiogr 2016;4(2):51-58.

scholarly journals An Unusual Case of Total Anomalous Pulmonary Venous Connection in Pregnancy

2017 ◽  
Vol 9 (2) ◽  
pp. 190-191
Author(s):  
Priyanka Mehta ◽  
Usha Vishwanath ◽  
M Anitha ◽  
Archana Kandasamy
Keyword(s):  
Unusual Case ◽  
Septal Defect ◽  
Pulmonary Veins ◽  
Pulmonary Atresia ◽  
First Trimester ◽  
Pulmonary Venous Obstruction ◽  
Transposition Of Great Arteries ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection ◽  
In Pregnancy

ABSTRACT Total anomalous pulmonary venous connection (TAPVC) is a rare congenital anomaly of the heart in which there is an absence of direct communication between the pulmonary veins and the left atrium, resulting in a mixture of oxygenated and deoxygenated blood in the right atrium. It is often associated with atrial septal defect or with transposition of great arteries, pulmonary atresia, truncus arteriosus, or single ventricle. The factors determining the severity are the severity of pulmonary venous obstruction and the restriction of interatrial communication. Uncorrected TAPVC rarely survives up to adulthood. Pregnancy in a patient with TAPVC is very rare unless surgically corrected in childhood. It often requires termination of pregnancy in first trimester as the heart cannot withstand the hemodynamic changes associated with pregnancy. We report a very rare case of pregnancy in a patient with uncorrected TAPVC, delivered successfully in our hospital without any complications. How to cite this article Kandasamy A, Vishwanath U, Anitha M, Mehta P. An Unusual Case of Total Anomalous Pulmonary Venous Connection in Pregnancy. J South Asian Feder Obst Gynae 2017;9(2):190-191.

Sign in / Sign up

Export Citation Format

Share Document