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Faith J Ross, Denise Joffe, Gregory J Latham
Total anomalous pulmonary venous connection (TAPVC) is a potentially devastating form of congenital heart disease in which all pulmonary blood flow returns to the systemic venous circulation rather than the left atrium. Anomalous pulmonary venous flow may be obstructed at birth, and affected infants present with severe cyanosis and poor cardiac output unresponsive to standard resuscitation with prostaglandin. Obstructed TAPVC remains one of the few indications for emergent neonatal cardiac surgery. This review will discuss the physiology and perioperative management of isolated TAPVC without associated cardiac lesions...
September 29, 2016: Seminars in Cardiothoracic and Vascular Anesthesia
Yujiro Ide, Masaya Murata, Hiroki Ito, Kisaburo Sakamoto
Right atrial isomerism and pulmonary atresia combined with major aortopulmonary collateral arteries are very rare. Surgical treatment becomes more challenging when an extracardiac total anomalous pulmonary venous connection (TAPVC) requiring surgical repair neonatally is also present. We describe a successful staged Fontan operation for a neonate with a single ventricle, right atrial isomerism, pulmonary atresia with major aortopulmonary collateral arteries, a small central pulmonary artery and a supracardiac TAPVC...
September 13, 2016: Interactive Cardiovascular and Thoracic Surgery
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
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
Venkatraman S Bhat
Functional information regarding fluid movement within the body can be derived by magnetic resonance methods. Some of the observations were overlooked in early days of magnetic resonance imaging (MRI) application. This presentation illustrates an unique, intra-hepatic haemodynamic phenomenon, demonstrated by magnetic resonance imaging, in a case of infra-cardiac Total Anomalous Pulmonary Venous Connection (TAPVC) Demonstration of such phenomena is unreported even after widespread use of cardiac MRI, although patho-physiology of such a phenomenon is expected in TAPVC...
March 2016: Journal of Clinical and Diagnostic Research: JCDR
Hujun Cui, Xinxin Chen, Li Ma, Yuansheng Xia, Shengchun Yang, Minghui Zou, Weidan Chen
OBJECTIVE: To discuss the experience of surgical treatment of total anomalous pulmonary venous connection (TAPVC) in infants. METHODS: The clinic data of 84 cases with TAPVC under 6 months of age underwent surgical treatment at Department of Cardiac Surgery, Guangzhou Women and Children's Medical Center from January 2012 to October 2015 were analyzed retrospectively. There were 58 male and 26 female patients. The patients were aged 1 days to 6 months with a mean of (2...
April 1, 2016: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
Minghui Zou, Yanfei Wang, Hujun Cui, Li Ma, Shengchun Yang, Yuansheng Xia, Weidan Chen, Xinxin Chen
BACKGROUND: The aim of this study was to review the early and mid-term outcomes of the total cavopulmonary connection (TCPC) procedure and evaluate risk factors for prolonged pleural effusions. METHODS: The clinical records of 82 consecutive patients, who underwent a TCPC operation between January 2008 and December 2013, were reviewed for incidence of prolonged pleural effusions, duration of ventilation time and pleural drainage, length of intensive care unit (ICU) stay, and early and mid-term morbidity and mortality...
January 2016: Journal of Thoracic Disease
Sachin Talwar, Palkesh Agarwal, Shiv Kumar Choudhary, Balram Airan
Patients with right atrial isomerism and total anomalous pulmonary venous connection (TAPVC) in a functionally univentricular heart are a challenging subset with a high mortality rate. We present the case of a patient with univentricular heart who had right atrial isomerism and associated mixed TAPVC (supracardiac, cardiac, and infracardiac). The anatomy was delineated precisely, and the patient underwent first-stage univentricular palliation consisting of TAPVC repair and bilateral bidirectional superior cavopulmonary anastomosis...
May 2016: World Journal for Pediatric & Congenital Heart Surgery
Hamid Amoozgar, Maryam Ahmadipoor, Ahmad Ali Amirghofran
Total anomalous pulmonary venous connection (TAPVC) is an anomaly in which the pulmonary veins are directly connected to one of the systemic veins or drain into the right atrium. Management of pulmonary hypertension after the total correction of this congenital cardiac anomaly is very important. Unligation of the vertical vein in the supracardiac type of this anomaly can be a draining pathway for the prevention of postoperative pulmonary hypertension crisis. Late onset transcatheter closure of the unligated vertical vein after a decrease in pulmonary pressure with the Amplatzer vascular plug type 1can prevent residual left-to-right shunting...
July 3, 2015: Journal of Tehran Heart Center
Tomomi Hasegawa, Yoshihiro Oshima, Yumi Sato, Akiko Tanaka
Trisomy 8 mosaicism is a relatively rare chromosomal abnormality and has extremely variable phenotype with a wide range of clinical manifestations. Although no well-defined criteria for cardiac surgical indications are available for patients with mosaic trisomy 8, we present a case of hypoplastic left heart syndrome with total anomalous pulmonary venous connection (TAPVC) in a neonate with mosaic trisomy 8. Although primary sutureless repair of TAPVC with concomitant bilateral pulmonary artery banding was performed successfully in this case, the indications for cardiac surgery in patients with mosaic trisomy 8 should be carefully individualized...
March 2016: World Journal for Pediatric & Congenital Heart Surgery
Keyan Zhao, Huishan Wang, Zengwei Wang, Hongyu Zhu, Minhua Fang, Xianyang Zhu, Nanbin Zhang, Hengchang Song
BACKGROUND: We retrospectively reported our 26-year experience with operative repair of total anomalous pulmonary venous connection (TAPVC) with biventricular physiology. METHODS: Between December 1982 and December 2008, 122 TAPVC patients with biventricular heart underwent surgical repair in our department. Moderate or deep hypothermia was induced at the time of cardiopulmonary bypass (CPB). Follow-up was conducted for 5 postoperative years. Surgical outcomes of early and intermediate deaths after TAPVC repair were retrospectively analyzed...
2015: Journal of Cardiothoracic Surgery
Sudeep Verma, Anand Subramanian, Rajan Saileela, Nageswara Rao Koneti
BACKGROUND: Vertical vein is left patent in some cases of supra-cardiac total anomalous pulmonary venous connection (TAPVC) when there is hemodynamic instability due to noncompliant left atrium and ventricle. After the peri-operative period, this results in features of pre-tricuspid shunt. MATERIALS AND METHODS: Three cases with patent vertical vein following repair of supra-cardiac TAPVC presented with features of pre-tricuspid shunt on follow-up. Trans-catheter closure of patent vertical vein was performed using vascular plug in all three subjects...
September 2015: Annals of Pediatric Cardiology
Mohammed Al-Ghafri, Said Al-Hanshi, Suad Al-Ismaily
Pulmonary pneumatocele is a thin-walled, gas-filled space within the lung that usually occurs in association with bacterial pneumonia and is usually transient. The majority of pneumatoceles resolve spontaneously without active intervention, but in some cases they might lead to pneumothorax with subsequent hemodynamic instability. We report two cases presented to the pediatric intensive care unit at the Royal Hospital, Oman with pneumatoceles. The first was a 14-day-old baby who underwent surgical repair of total anomalous pulmonary venous connection (TAPVC) requiring extracorporeal membrane oxygenation (ECMO) support following surgery...
July 2015: Oman Medical Journal
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
Mehmet Ozgur Akkurt, And Yavuz, Iltac Akkurt, Gokhan Karakoc, Mekin Sezik
OBJECTIVE: We aimed to evaluate the correlation between clinical parameters and the post-left atrium (LA) space index, which is a candidate screening marker for total anomalous pulmonary venous connection (TAPVC). We also calculated the inter- and intra-observer variabilities of measurements for this parameter. METHODS: The LA-descending aorta distance/descending aorta diameter ratios (post-LA space index) were obtained from 165 fetuses between 20 and 24 weeks of gestation...
August 1, 2016: Journal of Perinatal Medicine
Cheng Cheng, Lian-qin Kuang, Min-rui Jiang, Yi-jie Hu, Yi Wang
All cases with total anomalous pulmonary venous connection (TAPVC) were reported in neonates and children in the previous literature. This report describes the only case in which a mixed supra- and intracardiac TAPVC was discovered in a 25-year-old adult female and multidetector-row computed tomography (MDCT) angiography was used in its pre- and postoperative evaluation. Multidetector-row computed tomography angiography is superior to echocardiography in showing the draining sites and courses of the anomalous connected pulmonary veins, as well as postoperative evaluation in a patient with mixed TAPVC...
November 2015: Heart, Lung & Circulation
Yukihiro Kaneko
Total anomalous pulmonary venous connection (TAPVC) is one of the most typical congenital heart defects requiring emergent surgery soon after birth. Obstruction of the pulmonary venous drainage that can lead to profound cyanosis, respiratory failure and shock indicates emergent intervention. Prenatal diagnosis of TAPVC contributes to a smooth transition from delivery to surgery. However, prenatal ultrasound screening frequently fails to reveal isolated TAPVC. In neonates with isolated obstructive TAPVC, common pulmonary venous chamber-to-left atrial anastomosis is the intervention of choice except in patients with severe intraventricular hemorrhage, extremely low birth weight, or gestational age of 30 weeks or less...
July 2015: Kyobu Geka. the Japanese Journal of Thoracic Surgery
T Hazelzet, I Durand, N David
Total anomalous pulmonary venous connection (TAPVC) is a serious congenital anomaly. TAPVC with obstruction of pulmonary venous return is an emergency requiring urgent intervention. Before 2010, very few isolated TAPVC were diagnosed prenatally. It has been suggested in the past literature that the use of Color flow Doppler is particularly useful for the screening of TAPVC prenatally. In fact, although color-Doppler is often useful to confirm a TAPVC diagnosis, it can be quite misleading in the screening process of the condition...
July 2015: Gynécologie, Obstétrique & Fertilité
Muhammad S Khan, Roosevelt Bryant, Sung H Kim, Kevin D Hill, Jeffrey P Jacobs, Marshall L Jacobs, Sara K Pasquali, David L S Morales
BACKGROUND: Total anomalous pulmonary venous connection (TAPVC) is prevalent in patients with atriovisceral heterotaxy. Although functionally univentricular heart defects are common in heterotaxy syndromes, the extent to which this association influences overall risk for TAPVC repair is undefined. This study examines multiinstitutional experience with TAPVC repair in infants with heterotaxy using a national clinical registry. METHODS: The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) (2002-2012) was queried for patients with heterotaxy syndrome who underwent TAPVC repair, with or without concomitant procedures at age of 90 days or younger...
June 2015: Annals of Thoracic Surgery
Yutaka Imoto, Yoshiya Shigehisa, Tomoyuki Matsuba, Yoshie Ochiai
Darling's classification for total anomalous pulmonary venous connection (TAPVC) is based only on the level of the site of drainage of the anomalous pulmonary veins (PVs) to the systemic venous circulation. Although it is a clinically useful classification, atypical course of the PVs is occasionally encountered as well. We report a case of infracardiac-type TAPVC in which the left upper PV traversed the posterior mediastinum and merged into the right PVs at the right hilum. The combined vein coursed medially and caudally, meeting the left lower PVs, and finally penetrated the diaphragm; the pulmonary venous drainage formed a shape of "C...
April 2015: World Journal for Pediatric & Congenital Heart Surgery
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