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Obstructed total anomalous pulmonary veins

Cornelia Tremblay, Shi-Joon Yoo, Luc Mertens, Mike Seed, Frederic Jacques, Cameron Slorach, Rachel Vanderlaan, Steven Greenway, Christopher Caldarone, John Coles, Lars Grosse-Wortmann
BACKGROUND: Two different surgical techniques are used to repair anomalous pulmonary venous connection or pulmonary vein (PV) stenosis: the classic repair (CR) and the sutureless repair (SR). The purpose of this study was to compare the prevalence of PV stenosis between the two surgical approaches. METHODS: Patients were prospectively recruited irrespective of symptoms or previous imaging findings. Cardiac magnetic resonance imaging and echocardiography were performed in a blinded fashion on the same day...
February 23, 2018: Annals of Thoracic Surgery
Hirofumi Obinata, Shinichi Nishibe, Yoko Ishihara
Background: Heterotaxy syndrome (HS) is characterized by a wide variety of cardiac and extra-cardiac malformations, including pulmonary valve stenosis, interruption of the inferior vena cava, total anomalous pulmonary venous connection (TAPVC), asplenia, polysplenia, intestinal malrotation, and preduodenal portal vein (PDPV). We report the case of a heterotaxic infant with an infracardiac TAPVC and preduodenal portal vein who experienced repetitive hemodynamic instability during urgent laparotomy for duodenal obstruction...
2018: JA Clin Rep
Rong Liufu, Guocheng Shi, Fang Zhu, Yongmei Guan, Zhaohui Lu, Weimin Chen, Zhongqun Zhu, Huiwen Chen
BACKGROUND: Studies on the outcomes of surgical repair for supracardiac total anomalous pulmonary venous connection through the superior approach are uncommon. METHODS: From January 2004 to January 2016, 198 patients with supracardiac total anomalous pulmonary venous connection underwent side-to-side anastomosis between the common pulmonary vein and left atrium through the superior approach. Kaplan-Meier curve was used to demonstrate the survival estimates. Cox proportional hazard model and competing risk regression model were used to identify risk factors for death and postoperative pulmonary venous obstruction...
February 15, 2018: Annals of Thoracic Surgery
Rachel D Vanderlaan, Christopher A Caldarone
Total anomalous pulmonary venous connection (TAPVC) constitutes a spectrum of congenital lesions whereby the pulmonary veins remain connected to systemic venous vessels or aberrantly connect to the right atrium. Definitive management requires surgical intervention and, in patients with obstruction to pulmonary venous flow, urgent operation is required. Use of temporizing catheter-based interventions allow for optimization in hemodynamically unstable neonates. Overall, survival has significantly improved over the past decades through better perioperative management and evolution of surgical approaches to minimize post-repair pulmonary vein stenosis, which persists as a major determinant of long-term outcomes...
March 2018: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
Matthew S Yong, Nima Yaftian, Robert G Weintraub, Christian P Brizard, Yves d'Udekem, Igor E Konstantinov
Total anomalous pulmonary venous drainage (TAPVD) is an uncommon cardiac defect in children. The mixed subset accounts for 5%-10% of the TAPVD and is variable in its anatomy. The outcomes associated with this subset of patients are rarely reported. A retrospective review of all patients with mixed TAPVD undergoing repair at a single institution (1984-2014) was conducted. A descriptive analysis was performed. Twenty-four patients underwent repair for mixed TAPVD (6 univentricular physiology, 18 biventricular physiology)...
2017: Seminars in Thoracic and Cardiovascular Surgery
Dario Paladini, Angela Pistorio, L H Wu, Gabriella Meccariello, Ting Lei, Giulia Tuo, Gloria Donarini, Maurizio Marasini, Hong-Ning Xie
OBJECTIVES: Aim of this study is to describe the sonographic features and associations of Total and Partial Anomalous Pulmonary Venous Connection (TAPVC, PAPVC) and scimitar syndrome (SS) and to review the relevant literature systematically in order to underline the associations and the sonographic features more frequently associated with these conditions. METHODOLOGY: Retrospective cohort study. Assessed variables include: TAPVC subtype, gestational age at diagnosis, area behind LA, ventricular disproportion, vertical vein, pulmonary venous obstruction, mode of diagnosis, association with cardiac and extra-cardiac conditions, pregnancy and feto-neonatal outcome...
September 19, 2017: Ultrasound in Obstetrics & Gynecology
Hunbo Shim, Ji-Hyuk Yang, Tae-Gook Jun
BACKGROUND: The small size of the pulmonary veins in infants increases the risk of pulmonary vein obstruction (PVO) after surgical repair of type IV total anomalous pulmonary venous connection (TAPVC). Here, we described the outcomes of our strategy, which delayed total correction after initial partial correction. METHODS: We reviewed the data of patients who underwent total correction for type IV TAPVC. In total, 11 out of 103 patients with TAPVC had type IV TAPVC with biventricular physiology...
July 6, 2017: Journal of Cardiothoracic Surgery
Felipe Aluja Jaramillo, Cristian Hernandez, Juan Pablo Garzón, Angela Paola Sánchez Herrera, Martha Lucia Velasco Morales
Total anomalous pulmonary venous return (TAPVR), also known as total anomalous pulmonary venous connection, is a congenital cardiovascular malformation that presents itself in the neonatal period, with cyanosis and tachypnea. There are 4 types of TAPVR with the mixed type being the least common. Any type of TAPVR may be associated with obstruction as result of flow redirection through the liver parenchyma before it may return to the heart, but infracardiac is the most common one. We report a case of a 10-hour-old female, with a mixed (cardiac and infracardiac) TAVPR with obstruction, that showed drainage to the coronary sinus and the portal vein, as the other classic findings in TAVPR, made with computed tomography angiography and echocardiogram...
June 2017: Radiology Case Reports
James D St Louis, Elizabeth M Turk, Jeffrey P Jacobs, James E O'Brien
BACKGROUND: Mortality associated with correction of type IV total anomalous pulmonary venous connection (TAPVC) is generally reported in combination with other anatomic types. The objective of this study is to review surgical outcomes associated with the repair of type IV TAPVC by analyzing a multi-institutional cohort specific for this group. We also analyze patient-specific variables that may contribute to poor operative outcomes. METHODS: A retrospective review of the Pediatric Cardiac Care Consortium (PCCC) registry identified patients who underwent repair of type IV TAPVC between 1982 and 2007...
March 2017: World Journal for Pediatric & Congenital Heart Surgery
Fabio Cuttone, Khaled Hadeed, François Lacour-Gayet, Hugues Lucron, Sebastien Hascoet, Philippe Acar, Bertrand Leobon, Richard Van Praagh
OBJECTIVES: Leftward displacement of the septum primum is usually described as associated with hypoplastic left heart syndrome or visceral heterotaxy. This rare malformation results in partially or totally anomalous pulmonary venous drainage with a normal connection of the pulmonary veins to the left atrium, depending on the degree of septal shift. We report the 3D echocardiographic and anatomic findings as well as the surgical repair in a series of isolated severe leftward displacement of the septum primum, responsible for totally anomalous pulmonary venous drainage...
May 1, 2017: Interactive Cardiovascular and Thoracic Surgery
Elizabeth A Siacunco, Garrett S Pacheco, Dale P Woolridge
BACKGROUND: Total anomalous pulmonary venous return (TAPVR) is an uncommon congenital heart defect. Obstructed forms are more severe, and typically present earlier in life, usually in the immediate newborn period, with symptoms of severe cyanosis and respiratory failure. CASE REPORT: A 13-day-old boy presented to the emergency department (ED) with respiratory extremis. He appeared cyanotic and limp, and was found to have significant hypoxia with oxygen saturation of 40%...
June 2017: Journal of Emergency Medicine
Iolanda Muntean, Claudiu Mărginean, Răzvan Stanca, Rodica Togănel, Marian Pop, Liliana Gozar
INTRODUCTION: Total anomalous pulmonary venous connection is an uncommon congenital heart disease. Four types are described based on the site of pulmonary venous drainage: supracardiac, cardiac, infradiaphragmathic, and mixed connection. In most cases of supracardiac type, the common venous confluence drains through an ascending left vertical vein into the brachiocephalic vein, right superior vena cava, and then into the right atrium. Anomalous connection of the pulmonary venous confluence directly to the right SVC, especially the obstructed form is an unusual and severe supracardiac variant...
February 2017: Medicine (Baltimore)
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...
January 1, 2017: Seminars in Cardiothoracic and Vascular Anesthesia
Matthew D Files, Brian Morray
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...
June 2017: Seminars in Cardiothoracic and Vascular Anesthesia
Deepa Prasad, James P Strainic, Khyati Pandya, Peter C Kouretas, Ravi C Ashwath
We report a rare causal association between obstructed supracardiac totally anomalous pulmonary venous drainage and coronary sinus ostial atresia. Our 12-week-old patient developed venous myocardial infarction secondary to coronary venous hypertension because her sole route of coronary venous drainage was obstructed. She recovered after the obstruction was relieved by balloon dilation. Surgical repair then included anastomosis of the pulmonary venous confluence to the left atrium, ligation of the vertical vein, and unroofing of the coronary sinus...
October 2016: Texas Heart Institute Journal
Tariq Alam, Hidayatullah Hamidi, Mer Mahmood Shah Hoshang
Total anomalous pulmonary venous connection (TAPVC) is a rare congenital anomaly of the pulmonary veins drainage. In this entity, the pulmonary veins, instead of draining to left atrium, connect abnormally to the systemic venous circulation. A right-to-left shunt is obligatory for survival. Based on its type and degree of pulmonary venous obstruction, TAPVC may result in pulmonary hypertension and congestive heart failure. In severe cases, urgent diagnosis and surgical correction is essential to reduce morbidity and mortality...
September 2016: Radiology Case Reports
Marc Figueras-Coll, Anna Sabaté-Rotés, Noemí Cañete-Abajo, Raúl Felipe Abella
No abstract text is available yet for this article.
November 15, 2016: International Journal of Cardiology
Taiyu Hayashi, Katsusuke Ozawa, Rika Sugibayashi, Seiji Wada, Hiroshi Ono
We report a fetal case of double outlet right ventricle, mitral atresia, and intact atrial septum. Although the pulmonary veins were connected to the left atrium, pulmonary venous blood drained into the right superior vena cava via the stenotic levoatriocardinal vein (LACV), which resulted in a circulation resembling total anomalous pulmonary venous connection (TAPVC) with pulmonary venous obstruction. Since the pulmonary veins were connected to both the stenotic LACV and the "dead-end" left atrium, the pulmonary venous flow had a to-and-fro pattern along with atrial relaxation and contraction...
July 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
W K Lim, M C Leong, H Samion
A 1.7 kg infant with obstructed supracardiac total anomalous pulmonary venous drainage (TAPVD) presented with severe pulmonary hypertension secondary to vertical vein obstruction. The child, in addition, had a large omphalocele that was being managed conservatively. The combination of low weight, unoperated omphalocele, and severe pulmonary hypertension made corrective cardiac surgery very high-risk. Therefore, transcatheter stenting of the stenotic vertical vein, as a bridge to corrective surgery was carried out...
May 2016: Annals of Pediatric Cardiology
Efrén Martinez-Quintana, Fayna Rodríguez-González
The goal of total anomalous pulmonary venous connection repair is to obtain an unobstructed communication between the pulmonary veins and the left atrium and removing intracardiac shunting. However, pulmonary venous obstruction orstenosis may be seen in 5-10% of patients, is usually evident in the first 6 months following surgery and may lead to pulmonary congestion, pulmonary arterial hypertension, and late mortality. In such cases, early intervention may be indicated before irreversible secondary changes occur...
January 2016: Pneumologia: Revista Societății Române de Pneumologie
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