Contemporary Outcomes of Surgical Repair of Total Anomalous Pulmonary Venous Connection in Patients With Heterotaxy Syndrome

2015 ◽  
Vol 99 (6) ◽  
pp. 2134-2140 ◽  
Author(s):  
Muhammad S. Khan ◽  
Roosevelt Bryant ◽  
Sung H. Kim ◽  
Kevin D. Hill ◽  
Jeffrey P. Jacobs ◽  
...  
Keyword(s):  
Surgical Repair ◽  
Heterotaxy Syndrome ◽  
Anomalous Pulmonary Venous Connection

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.

A situs solitus transposition of great arteries with obstructed sub-diaphragmatic totally anomalous pulmonary venous connection: a rare case treated with anatomical repair

2019 ◽  
Vol 29 (12) ◽  
pp. 1536-1538 ◽  
Author(s):  
Giovanni Meliota ◽  
Gabriele Scalzo ◽  
Ugo Vairo
Keyword(s):  
Venous Return ◽  
Arterial Switch Operation ◽  
Heterotaxy Syndrome ◽  
Transposition Of Great Arteries ◽  
Rare Association ◽  
Switch Operation ◽  
Atrial Switch ◽  
Situs Solitus ◽  
Atrial Switch Operation ◽  
Anomalous Pulmonary Venous Connection

AbstractTransposition of the great arteries combined with totally anomalous pulmonary venous connection is extremely rare outside of heterotaxy syndrome. Most reported cases have been treated by a modified atrial switch operation. We report the successful treatment of a neonate with this rare association, repaired by arterial switch operation and connection of the pulmonary venous return to the left atrium.

Persistent Left Superior Vena Cava: Incidence and Management in Patients Undergoing Repair of Partial Anomalous Pulmonary Venous Connection

2017 ◽  
Vol 8 (4) ◽  
pp. 440-444 ◽  
Author(s):  
Nicholas S. Clarke ◽  
Raghav A. Murthy ◽  
Kristine J. Guleserian
Keyword(s):  
Superior Vena Cava ◽  
Surgical Repair ◽  
Superior Vena ◽  
Vena Cava ◽  
Sinus Venosus ◽  
Left Superior Vena Cava ◽  
Persistent Left Superior Vena ◽  
Type Defect ◽  
Intraoperative Management ◽  
Anomalous Pulmonary Venous Connection

Introduction: A association between partial anomalous pulmonary venous connection (PAPVC) and systemic venous anomalies has been described in the literature, but the true incidence is yet to be elucidated. At our institution, we sought to find the incidence of a persistent left superior vena cava (PLSVC) in patients undergoing an operation for PAPVC. Methods: A retrospective review of all pediatric and adult patients with PAPVC who underwent surgical repair from February 2006 to February 2016. All clinical, radiographic, and operative data were reviewed. Results: Eighty-five patients underwent surgical repair. A PLSVC was identified in 15 (17.6%) patients. Every PLSVC drained/connected to the coronary sinus. A bridging vein was present in only 26.7% (4 of 15). Intraoperative management of the PLSVC consisted of direct cannulation in nine (60%) patients, temporary occlusion in one (6.7%) patient, and ligation in one (6.7%) patient. In the 15 PLSVC patients, 6 (40%) had a secundum atrial septal defect (ASD), 2 (13.3%) had a patent foramen ovale (PFO), 10 (66.6%) had a sinus venosus type defect, and 3 (20%) had both an ASD and sinus venosus–type defect. Of the 15 patients with PAPVC and PLSVC, 14 (93%) had anomalous drainage of pulmonary vein(s) on the right side, whereas 1 (7%) had veins on both sides with anomalous drainage. Conclusion: Persistent left superior vena cava is present in 17.6% of patients undergoing an operation for PAPVC. Awareness of this association as well as the intraoperative management of PLSVC is advised for those that perform operations for PAPVC. As improper myocardial protection and cardiopulmonary bypass strategies in the presence of a PLSVC can result in deleterious outcomes.

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 BIDIRECTIONAL GLENN SHUNT AND REPAIR OF TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION IN A 3 YEARS OLD CHILD WITH HETEROTAXY SYNDROME: A CASE REPORT

2021 ◽  
Vol 54 (3) ◽  
pp. 273-274
Author(s):  
Shahyad Salehi-ardebili ◽  
Mohammad Radvar ◽  
Behnam Askari
Keyword(s):  
Surgical Repair ◽  
Rare Condition ◽  
Right Atrial ◽  
Older Children ◽  
Glenn Shunt ◽  
Bidirectional Glenn Shunt ◽  
Atrial Isomerism ◽  
Suitable Group ◽  
Anomalous Pulmonary Venous Connection ◽  
Very High

Right Atrial Isomerism (RAI) and total anomalous pulmonary venous connection (TAPVC) is a rare condition with very high mortality in neonatal period, but outcomes may be better in older children. A cyanotic 3 years old boy with dyspnea and growth retardation diagnosed with RAI and supracardiac type of TAPVC undergone a successful repair of TAPVC and simultaneous Glenn shunt. Older patients with RAI may be a distinct, more suitable group for surgical repair.

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