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Obstructed total anomalous pulmonary veins

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...
February 16, 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%...
March 9, 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...
November 9, 2016: 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
Milind S Phadke, Swapnil D Mate, Prafulla G Kerkar
A 17-year-old female presented with mild cyanosis. Imaging showed supracardiac total anomalous pulmonary venous connections with a vertical vein travelling between the left pulmonary artery and the left bronchus with significant obstruction. There was a huge post-stenotic aneurysm of the vertical vein.
June 2016: Cardiology in the Young
Anupama K Nair, Sitaraman Radhakrishnan, Krishna S Iyer
In this study, we present the case of a neonate with obstructed infracardiac total anomalous pulmonary venous connection with severe pulmonary hypertension and a patent ductus arteriosus with right-to-left shunting. The patient had an unusual finding of pandiastolic flow reversal in the upper descending thoracic aorta. He underwent emergency surgical re-routing of the pulmonary veins to the left atrium, and postoperative echocardiography showed disappearance of the descending aortic flow reversal. We hypothesise that in severely obstructed total anomalous pulmonary venous connection the left ventricular output may be extremely low, resulting in flow reversal in the descending aorta...
June 2016: Cardiology in the Young
Neelam Aggarwal, Shrinivas Gadhinglajkar, Rupa Sreedhar, Baiju S Dharan, Keerthi Chigurupati, Saravana Babu
OBJECTIVE: Review of intraoperative anesthetic challenges and the role of transesophageal echocardiography in children with sinus venosus atrial septal defect and partial anomalous pulmonary venous drainage undergoing Warden repair. DESIGN: A retrospective observational case series. METHODOLGY: Pediatric patients who underwent Warden repair between October 2011-September 2015 were recruited. Their preoperative clinical details, anesthetic techniques, intraoperative TEE findings and postoperative events were recorded from the medical records...
April 2016: Annals of Cardiac Anaesthesia
Hiroki Ito, Masaya Murata, Yujiro Ide, Mikio Sugano, Kazuyoshi Kanno, Kenta Imai, Motonori Ishido, Ryohei Fukuba, Kisaburo Sakamoto
OBJECTIVES: Fontan candidates with mixed totally anomalous pulmonary venous connection often have postoperative pulmonary venous obstruction after cavopulmonary anastomosis. Because some pulmonary venous obstructions have no intimal hypertrophy at reoperation, we considered such pulmonary venous obstructions to be caused by 3D deformities arising from dissection or mobilization of the vessels, and hypothesized that keeping the pulmonary venous branches in a natural position could avoid such obstruction...
May 2016: Interactive Cardiovascular and Thoracic Surgery
Hyun-Jin Jung, Ji Hyun Bang, Chun-Soo Park, Jeong-Jun Park, Yu-Mi Im, Tae-Jin Yun
Indications of sutureless repair (SR) for pulmonary vein anomalies have evolved from re-operational SR for pulmonary vein stenosis after the repair of total anomalous pulmonary venous drainage (TAPVD) to primary SR for TAPVD associated with right atrial isomerism or isolated TAPVD with small individual pulmonary veins (IPVs) and an unfavorable pulmonary vein anatomy. We sought to determine whether small IPVs outgrow somatic growth after primary SR. Between 2004 and 2013, 21 children underwent primary SR for TAPVD: 13 with a functionally single ventricle, 11 with right atrial isomerism, six with isolated TAPVD, and 13 with a pulmonary venous obstruction...
February 2016: Pediatric Cardiology
Tomoyuki Iwase, Junichi Koizumi, Hitoshi Okabayashi, Akio Ikai
A simple total anomalous pulmonary venous connection (TAPVC) coexisting with a persistent left superior vena cava (PLSVC) is extremely rare. Connection of the PLSVC with the coronary sinus behind the left atrium induces coronary sinus dilatation. This reduces the free posterior wall space to which the common pulmonary vein is anastomosed for repairing the anomalous connection. Postoperative recurrent pulmonary venous obstruction (PVO) is the most important complication. To prevent PVO, sufficient tension-free anastomosis is necessary...
December 2015: Interactive Cardiovascular and Thoracic Surgery
Mauro Lo Rito, Tamadhir Gazzaz, Travis Wilder, Arezou Saedi, Devin Chetan, Glen S Van Arsdell, Christopher A Caldarone, Shi-Joon Yoo, Osami Honjo
BACKGROUND: We hypothesized that primary sutureless (SL) repair of total anomalous pulmonary venous drainage (TAPVD) may have a lower incidence of postrepair pulmonary vein obstruction (PVO) and different modes of PVO compared with standard repair (SR). METHODS: One hundred ninety-five patients who underwent TAPVD repair (1990 to 2012) with the exception of congenital pulmonary vein stenosis, isomerism, and single-ventricle anomalies were included. Survival, reintervention, incidence, degree of PVO were compared between groups...
August 2015: Annals of Thoracic Surgery
Changping Gan, Peng Ji, Ke Lin, Yuan Feng
Pulmonary venous obstruction after the repair of total anomalous pulmonary venous connection is a serious complication. Transcatheter interventional treatment is a palliative choice to defer the timing of surgery; however, penetrating through the patched atrial septum may be difficult. We report a technique for pulmonary venous obstruction after mixed-type total anomalous pulmonary venous connection repair using balloon dilatation of the pulmonary veins through a novel hybrid approach.
July 2015: Journal of Cardiac Surgery
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