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Pulmonary atresia

Christina L Greene, Richard D Mainwaring, Douglas Sidell, Vamsi V Yarlagadda, William L Patrick, Frank L Hanley
OBJECTIVES: Injury to the phrenic nerves may occur during surgery for Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries (PA/VSD/MAPCAs). These patients may develop respiratory failure and require diaphragm plication. The purpose of this study was to evaluate the impact of phrenic nerve palsy on recovery following surgery for PA/VSD/MAPCAs. METHODS: Between 2007 to 2016, approximately 500 patients have undergone surgery for PA/VSD/MAPCAs at our institution...
March 12, 2018: Seminars in Thoracic and Cardiovascular Surgery
Zoel A Quinonez, Laura Downey, Rania K Abbasi, Calvin Kuan, Ritu Asija, Doff B McElhinney, Frank L Hanley, Richard D Mainwaring, Lisa Wise-Faberowski
Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals (TOF/PA/MAPCAs) is a heterogeneous disease with varying degrees of severity, requiring complex anesthetic management. Our institution has adopted the approach of early complete repair with incorporation of all lung segments, extensive lobar and branch segmental pulmonary artery reconstruction, and ventricular septal defect closure. While the surgical management of TOF/PA/MAPCAs has been extensively described and varies depending on the institution, there is a paucity of literature on the anesthetic management for such procedures...
March 2018: World Journal for Pediatric & Congenital Heart Surgery
Laura Mercer-Rosa, Okan U Elci, Nelangi M Pinto, Ronn E Tanel, Elizabeth Goldmuntz
Deletion of 22q11.2 (del22q11) is associated with adverse outcomes in patients with tetralogy of Fallot (TOF). We sought to investigate its contribution to perioperative outcome in patients with a severe form of TOF characterized by pulmonary atresia (PA) or severe pulmonary stenosis (PS) and major aortopulmonary collateral arteries (MAPCAS). We conducted a retrospective review of patients with TOF/MAPCAS who underwent staged surgical reconstruction between 1995 and 2006. Groups were compared according to 22q11...
March 8, 2018: Pediatric Cardiology
Renuka E Peterson, Grace Freire, Cynthia J Marino, Saadeh B Jureidini
Right ventricular-dependent coronary circulation (RVDCC) is associated with pulmonary atresia with intact ventricular septum and is defined by two or more epicardial coronary arteries with atresia or severe stenosis resulting in the dependency of coronary supply by retrograde flow. The hypothesis of this study is that coronary Doppler flow patterns on echocardiography can be used to distinguish patients with RVDCC. Between 2007 and 2016, we reviewed 16 patients with pulmonary atresia or critical pulmonary stenosis...
March 8, 2018: Pediatric Cardiology
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
Yohei Yamada, Ken Hoshino, Takayuki Oyanagi, Ryohei Gatayama, Jun Maeda, Nobuyuki Katori, Yasushi Fuchimoto, Taizo Hibi, Masahiro Shinoda, Kentaro Matsubara, Hideaki Obara, Ryo Aeba, Yuko Kitagawa, Hiroyuki Yamagishi, Tatsuo Kuroda
Children with single ventricle physiology have complete mixing of the pulmonary and systemic circulations, requiring staged procedures to achieve a separation of these circulations, or Fontan circulation. The single ventricle physiology significantly increases the risk of mortality in children undergoing non-cardiac surgery. As liver transplantation for patients with single ventricle physiology is particularly challenging, only a few reports have been published. We herein report a case of successful LDLTx for an 8-month-old pediatric patient with biliary atresia, heterotaxy, and complex heart disease of single ventricle physiology...
February 19, 2018: Pediatric Transplantation
Christopher W Mercer, Shawn C West, Mahesh S Sharma, Masahiro Yoshida, Victor O Morell
OBJECTIVE: Our institution uses a valved polytetrafluoroethylene conduit as an alternative to homografts. The objective of this study was to investigate the performance of bicuspid valved polytetrafluoroethylene conduits used for right ventricular outflow tract reconstruction in children aged less than 2 years and to evaluate risk factors for earlier conduit explant. METHODS: We performed an Institutional Review Board-approved retrospective chart review of all patients aged less than 2 years who underwent surgical right ventricular outflow tract reconstruction with a bicuspid valved polytetrafluoroethylene conduit or homograft conduit from July 2004 to December 2014...
January 31, 2018: Journal of Thoracic and Cardiovascular Surgery
Deepa Sasikumar, Sabarinath Menon, Sudip Dutta Baruah, Baiju S Dharan, Debabrata Gohain, Bijulal Sasidharan, Sivasubramanian Sivasankaran
A baby with pulmonary atresia with intact ventricular septum and hypoplastic right ventricle, with suspected right ventricle to coronary communications, was operated on for placing an aortopulmonary shunt. Postoperatively, the baby deteriorated with features of myocardial ischemia. Postmortem examination revealed anomalous origin of left coronary artery from pulmonary artery that caused significant coronary ischemia on ligation of the ductus arteriosus. Although coronary anomalies, including right ventricle dependent coronary circulation, has been well described, this is the first report of anomalous origin of coronary artery from pulmonary artery in a baby with pulmonary atresia and intact ventricular septum...
March 2018: Annals of Thoracic Surgery
Nathalie Jeanne Bravo-Valenzuela, Alberto Borges Peixoto, Edward Araujo JĂșnior
This article reviews important features to improve the diagnosis of congenital heart disease (CHD) by applying ultrasound in prenatal cardiac screening. As low and high-risk pregnancies for CHD are subject to routine obstetric ultrasound, the diagnosis of structural heart defects represents a challenge that involves a team of specialists and subspecialists on fetal ultrasonography. In this review, the images highlight normal anatomy of the heart as well as pathologic cases consistent with cardiac malposition and isomerism, septal defects, pulmonary stenosis/atresia, aortic malformations, hypoplastic left ventricle, conotruncal anomalies, tricuspid dysplasia, and Ebstein's anomaly, and univentricular heart, among other congenital cardiovascular defects...
January 2018: Indian Heart Journal
Xuelei Li, Zhongping Mu, Xu Li, Zongjie Weng
BACKGROUND: Anomalous origin of the pulmonary arteries is a rare congenital pulmonary vascular malformation, that includes unilateral absence of the pulmonary artery (UAPA), anomalous origin of unilateral pulmonary artery (AOPA) and left pulmonary artery sling (LPAS). METHODS: We analyze 15 cases of fetal pulmonary artery abnormalities from 2011 to 2017, detected via prenatal ultrasound at our center. RESULTS: The 15 cases include UAPA (five), AOPA (six), and LPAS (four)...
February 16, 2018: Prenatal Diagnosis
Richard D Mainwaring, William L Patrick, Michael Ma, Frank L Hanley
OBJECTIVES: Midline unifocalization has been developed for the surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. All patients will eventually require reoperation because of the presence of a conduit, and some patients may also require revision of the distal unifocalized bed. The purpose of this study was to analyse the need for unifocalization revision following midline unifocalization. METHODS: This was a retrospective review of 241 patients who underwent midline unifocalization for the treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries...
February 13, 2018: European Journal of Cardio-thoracic Surgery
Alessia Faccini, Gianfranco Butera
Patients with congenital heart disease and duct-dependent pulmonary circulation can undergo stenting of the patent ductus arteriosus (PDA). This case shows that, due to the physiological changes occurring after stent implantation, sometimes it is necessary to close the stented PDA rather than to redilate it.
February 2018: Clinical Case Reports
Patrick Elias, Chin Leng Poh, Karin du Plessis, Diana Zannino, Kathryn Rice, Dorothy J Radford, Andrew Bullock, Gavin R Wheaton, David S Celermajer, Yves d'Udekem
OBJECTIVES: The specific outcomes of patients with pulmonary atresia with intact ventricular septum late after Fontan palliation are unknown. Patients with smaller right ventricles and myocardial sinusoids are known to have worse survival in the first years of life. Whether the potential for coronary ischaemia affects the long-term outcomes of these patients after Fontan palliation is still unknown. METHODS: All patients with pulmonary atresia with intact ventricular septum who underwent the Fontan procedure from 1984 to 2016 in Australia and New Zealand were identified, and preoperative, perioperative and follow-up data were collected...
February 12, 2018: European Journal of Cardio-thoracic Surgery
Yoshie Ochiai, Yusuke Ando, Shigehiko Tokunaga, Jun Muneuchi
We present a very rare case of isolated right pulmonary veins atresia in a 4-year-old girl. As the presence of a pulsatile blind pulmonary venous confluence adjacent to the left atrium was demonstrated by preoperative right pulmonary arterial wedge angiography, we applied sutureless pericardial marsupialization for the repair of right pulmonary veins atresia. She is currently 8 years old without pulmonary veins stenosis.
February 8, 2018: Interactive Cardiovascular and Thoracic Surgery
Xiu-Qi Chen, Wen-Hai Tan, Fang-Fang Jiang, Xun Chen
OBJECTIVE: To investigate the clinical features of invasive pulmonary fungal infections (IPFIs) after biliary atresia (BA) surgery and related risk factors. METHODS: A retrospective analysis was performed for the clinical data of 49 children with IPFIs after BA surgery, including clinical features, lung imaging findings, and pathogenic features. The risk factors for IPFIs after BA surgery were also analyzed. RESULTS: The most common pathogens of IPFIs after BA surgery was Candida albicans (17 strains, 45%), followed by Candida tropicalis (7 strains, 18%), Aspergillus (6 strains, 16%), Candida krusei (3 strains, 8%), Candida glabrata (3 strains, 8%), and Candida parapsilosis (2 strains, 5%)...
February 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
David J Barron, Phil Botha
Pulmonary atresia with major aortopulmonary collateral arteries (MAPCAs) is one of the most challenging surgical conditions to manage-not only because of the technical complexity of the surgery but also in terms of defining the anatomy of the pulmonary vasculature, the timing of surgery, and decision making on staged vs complete repair. The importance of early definition of pulmonary blood supply is paramount, establishing which areas of the lung are supplied by MAPCAs alone and which have dual supply with the native system (noting that 20% of patients have absent intrapericardial native vessels)...
March 2018: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
Wang Qing, Wu Yu Rong, Jiao Xian Ting, Wu Peng Fei, Zhao Li Qing, Chen Sun, Sun Kun
OBJECTIVES: To compare the fetal echocardiographic measurements and neonatal outcome of fetuses with diagnosis of critical pulmonary stenosis (CPS/IVS) and pulmonary atresia with intact ventricular septum (PA/IVS) in order to identify the predictors of neonatal ductus dependence and the need for neonatal intervention. METHODS: 44 fetuses with a diagnosis of membranous PA/IVS or CPS/IVS referred to Shanghai Xinhua Hospital Affiliated to Shanghai Jiaotong University between June 2009 and November 2014 were respectively analyzed...
February 7, 2018: Prenatal Diagnosis
Sathish M Chikkabyrappa, Rohit S Loomba, Justin T Tretter
Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare complex cyanotic congenital heart disease with heterogeneous morphological variation. Prenatal diagnosis allows for developing a safe plan for delivery and postnatal management. While transthoracic echocardiography allows for detailed delineation of the cardiac anatomy, additional imaging modalities such as computed tomography, magnetic resonance imaging, and catheterization may be necessary to further outline features of the cardiac anatomy, specifically coronary artery anatomy...
February 1, 2018: Seminars in Cardiothoracic and Vascular Anesthesia
Shafkat Anwar, Toby Rockefeller, Demetrios A Raptis, Pamela K Woodard, Pirooz Eghtesady
Patients with tetralogy of Fallot, pulmonary atresia, and multiple aortopulmonary collateral arteries (Tet PA MAPCAs) have a wide spectrum of anatomy and disease severity. Management of these patients can be challenging and often require multiple high-risk surgical and interventional catheterization procedures. These interventions are made challenging by complex anatomy that require the proceduralist to mentally reconstruct three-dimensional anatomic relationships from two-dimensional images. Three-dimensional (3D) printing is an emerging medical technology that provides added benefits in the management of patients with Tet PA MAPCAs...
February 3, 2018: Current Treatment Options in Cardiovascular Medicine
Onkar B Auti, Varun Shetty, Vinay Belaval, Vimal Raj
Levoatrial cardinal vein (LACV) is anomalous connection between left atrium or pulmonary veins and systemic veins such as innominate vein or superior vena cava. This persistence of splanchnic circulation occurs when there is left-sided obstructive cardiac lesions such as hypoplastic left heart or mitral atresia. In this report we present three cases of LACV with well-developed left heart, without any obstructive lesions. All our cases presented with pulmonary arterial hypertension (PAH) and had associated intracardiac shunt such as ventricular/atrial septal defect and supracardiac partial anomalous pulmonary venous connection...
October 2017: Indian Journal of Radiology & Imaging
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