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Akash Kasatwar, Rajiv Borle, Nitin Bhola, Rajanikanth K, G S V Prasad, Anendd Jadhav
Background: Cleft lip and palate is one of the most common congenital craniofacial deformities seen in children. Various congenital anomalies are reported in the literature to be associated with cleft lip and palate. Cardiac anomalies are one of the most common congenital disorders associated in cleft lip and palate patientsIt includes Cyanotic and acyanotic cardiac diseases likel fallot's tetralogy, transposition of greater vessels, atresia of tricuspid, total anomalous pulmonary venous return (TAPVR), truncus arteriosus, ebstein's anomaly, hypoplastic left heart syndrome and pulmonary atresia, patent ductus arteriosus, ventricular septal defect, atrial septal defect, pulmonary stenosis, aortic stenosis and coarctation of aorta...
September 2018: Journal of Oral Biology and Craniofacial Research
Carlos M Mery, Rodrigo Zea-Vera, Martin A Chacon-Portillo, Huirong Zhu, William B Kyle, Iki Adachi, Jeffrey S Heinle, Charles D Fraser
BACKGROUND: Contemporary outcomes of complete atrioventricular septal defect (CAVSD) repair, particularly for defects with associated abnormalities, is unclear. The goal of this study is to report an all-inclusive experience of CAVSD repair using a consistent surgical approach. METHODS: All patients undergoing CAVSD repair between 1995 and 2016 at our institution were included. Patients were divided into 2 groups: isolated and complex (tetralogy of Fallot, aortic arch repair, double outlet right ventricle, and total anomalous pulmonary venous return)...
November 2018: Annals of Thoracic Surgery
Shelly Domadia, S Ram Kumar, Jodie K Votava-Smith, Jay D Pruetz
The objective of this study is to evaluate neonatal outcomes of total anomalous pulmonary venous return (TAPVR) and identify fetal echocardiography findings associated with preoperative pulmonary venous obstruction (PPVO). This retrospective study evaluated TAPVR cases from 2005 to 2014 for preoperative and postoperative outcomes based on prenatal diagnosis, PPVO, and heterotaxy syndrome. Fetal pulmonary and vertical vein Dopplers were analyzed as predictors of PPVO. Of 137 TAPVR cases, 12% were prenatally diagnosed; 60% had PPVO, and 21% had heterotaxy...
October 2018: Pediatric Cardiology
Norain Talib, Khairil Amir Sayuti, Mohd Shafie Abdullah, Mohd Rizal Mohd Zain
Total anomalous pulmonary venous return (TAPVR) is a rare congenital heart defect, and patients are usually symptomatic at a very young age. Survival to adulthood without surgical correction is extremely rare. We report a 33-year-old woman with a heart murmur and a history of a successful pregnancy. Echocardiogram revealed a large atrial septal defect with suspicious pulmonary vein anomaly. Chest radiograph demonstrated classical 'snowman' configuration. Cardiac catheterisation was consistent with anomalous pulmonary venous drainage...
March 5, 2018: BMJ Case Reports
Lakshmi R Gokanapudy, Kenneth E Remy, Sathappan Karuppiah, Eneida V Melgar Humala, Ibrahim Abdullah, Michael D Ruppe, William S Schechter, Robert Michler, Joseph D Tobias
Total anomalous pulmonary venous return (TAPVR) is a rare congenital cardiac defect, accounting for 1.5-3% of cases of congenital heart disease. With prenatal ultrasonography, the majority of these patients are diagnosed in utero with definitive surgery performed during the neonatal period. However, as prenatal screening may not be available in developing countries, patients may present in later infancy. We present successful surgical repair of a 6-month-old infant with TAPVR who presented for medical care at 5 months of age in Lima, Peru...
February 2018: Cardiology Research
Christine Levêque, Louise Paret, Tiffany Cochet, Félicia Joinau-Zoulovits, E Chantalat, F Vidal, Christophe Vayssière, Nadine David
INTRODUCTION: Total abnormal pulmonary venous return is a heart defect often missed prenatally, yet at birth, it is a surgical emergency. Antenatal detection could be improved by sonographic visualization of the anastomosis of 2 pulmonary veins in a sinus into the left atrium. The objective of this study is to evaluate the feasibility of this screening method. METHOD: Prospective observational study. Five operators selected one representative image taken during morphological ultrasound screening...
January 2018: Prenatal Diagnosis
Kazutoshi Okada, Midori Ishida, Hiroshi Okada, Hiroto Nakayama, Jun Aizawa
PURPOSE: To investigate the hematological changes during the perioperative period of open-heart surgery without homologous blood transfusion under simple deep hypothermia in infants and small children, and to define the limits of body weight for open-heart surgery without homologous blood transfusion under simple deep hypothermia. METHODS: We performed open-heart surgery without homologous blood transfusion under simple deep hypothermia on eight children, four infants, and a neonate with diagnoses of atrial septal defect, ventricular septal defect, on total anomalous pulmonary venous return (TATVR)...
September 1998: Journal of Anesthesia
Ranjit I Kylat
Mycophenolate is often used in the management of systemic lupus erythematosus. It has often been associated with significant fetal embryopathy, including fetal loss and multiple anomalies. The Food and Drug Administration has directed that women should be counseled regarding this prior to initiating treatment with this drug. Isolated total anomalous pulmonary venous return (TAPVR) is a rare association seen with its use in pregnancy.
June 2017: Journal of Pediatric Genetics
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
Jun Li, Shiwei Yang, Zhening Pu, Juncheng Dai, Tao Jiang, Fangzhi Du, Zhu Jiang, Yue Cheng, Genyin Dai, Jun Wang, Jirong Qi, Liming Cao, Xueying Cheng, Cong Ren, Xinli Li, Yuming Qin
As a rare type of Congenital Heart Defects (CHD), the genetic mechanism of Total Anomalous Pulmonary Venous Return (TAPVR) remains unknown, although previous studies have revealed potential disease-driving regions/genes. Blood samples collected from the 6 sporadic TAPVR cases and 81 non-TAPVR controls were subjected to whole exome sequencing. All detected variations were confirmed by direct Sanger sequencing. Here, we identified 2 non-synonymous missense mutations: c.C652T, p.R218W in activin A receptor type II-like 1 (ACVRL1), c...
April 25, 2017: Oncotarget
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
Marijo Aguilera, Kirsten Dummer
OBJECTIVE: We sought to examine the concordance of prenatally diagnosed congenital cardiac disease with postnatal echocardiography. STUDY DESIGN: Patients who underwent fetal echocardiograms performed by pediatric cardiologists at a single referral center from January to December 2014 were reviewed. Fetal echocardiography exams were performed in accordance with guidelines by the American Institute of Ultrasound in Medicine (AIUM) and the American Heart Association (AHA) guidelines (2013 and 2014, respectively)...
March 12, 2017: Journal of Maternal-fetal & Neonatal Medicine
Jacob S Parzen, Michael F Swartz, Jill M Cholette, Frank Smith, Francisco Gensini, George M Alfieris
Circulatory arrest (CA) is traditionally utilized during the repair of total anomalous pulmonary venous return (TAPVR). Since 2005, we have exclusively repaired all types of TAPVR using continuous cardiopulmonary bypass. We present our technique using continuous cardiopulmonary bypass throughout the duration of the repair, by temporarily occluding the vertical vein and placing a pump sucker within the pulmonary venous confluence. This technique has been used on 29 consecutive patients and resulted in limited morbidity and absence of pulmonary vein stenosis from most recent follow-up...
November 2016: World Journal for Pediatric & Congenital Heart Surgery
Youngok Lee, Joon Yong Cho, O Young Kwon, Woo Sung Jang
BACKGROUND: Recent developments in surgical techniques and hospital care have led to improved outcomes following total anomalous pulmonary venous return (TAPVR) repair. However, the surgical repair of TAPVR remains associated with a high risk of mortality and need for reoperation. We conducted this retrospective study to evaluate mid-term outcomes following in situ TAPVR repair without total circulatory arrest (TCA), and to identify the risk factors associated with surgical outcomes. METHODS: We retrospectively reviewed 29 cases of surgical intervention for TAPVR conducted between April 2000 and July 2015...
October 2016: Korean Journal of Thoracic and Cardiovascular Surgery
Rintaro Yokoyama, Satoshi Iihoshi, Kei Miyata, Kentaro Toyama, Katsuya Komatsu, Masahiko Wanibuchi, Nobuhiro Mikuni
OBJECT: We report a relatively rare case of pediatric iatrogenic extradural vertebral arteriovenous fistula. CASE: A five-year-old boy with a history of multiple surgeries for Total Anomalous Pulmonary Venous Return(TAPVR)exhibited vascular engorgement of his right neck from about 4 years old. We thought that this was caused by his past operations. He demonstrated swollen blood vessels in the right neck and pulmonary hypertension with increased right heart load...
October 2016: No Shinkei Geka. Neurological Surgery
Yoshiyuki Maekawa, Yoshinori Miyahara, Ko Yoshizumi, Masaaki Kawada, Takaomi Minami, Tomoyuki Sato, Akiko Yokomizo, Kensuke Oka, Sadahiro Furui, Koichi Kataoka
A combination of tetralogy of Fallot( TOF) and total anomalous pulmonary venous return(TAPVR) is rare and results in chronic volume and pressure load of the right side of the heart and underfilling of the left heart. We report a successful 2-staged surgical correction of TOF associated with TAPVR and atrial septal defect. The patient was unsuitable for total primary intracardiac correction because the volume of the left ventricle was considered to be small. First, repair of anomalous pulmonary venous return and palliative right ventricle outflow tract reconstruction were simultaneously performed in 2 months of birth...
September 2016: Kyobu Geka. the Japanese Journal of Thoracic Surgery
Tatsuya Oonishi, Yasunobu Hayabuchi, Miho Sakata, Kazuhiro Mori, Shoji Kagami
A newborn with right isomerism, infracardiac total anomalous pulmonary venous return (TAPVR), and obstruction of the ductus venosus underwent stent implantation in the ductus venosus at 4 h after birth. This procedure averted early pulmonary venous obstruction. Since gradual neointimal proliferation subsequently occurred within the stent, the patient underwent stent balloon dilatation at 35 days of age and underwent surgical repair of the TAPVR with a Blalock-Taussig shunt operation at 53 days of age. Finally, the patient was discharged without any complications...
March 2012: Journal of Echocardiography
Theera Tongsong, Suchaya Luewan, Phudit Jatavan, Fuanglada Tongprasert, Kornkanok Sukpan
The objective of this series was to describe a simple rule for prenatal diagnosis of total anomalous pulmonary venous return (TAPVR). Fourteen fetuses had a prenatal diagnosis of TAPVR by the simple rule, including the following components: (1) the major criterion, which was the absence of a connection between the pulmonary vein and the left atrium; and (2) at least 1 of the following minor criteria: (a) the presence of a vascular confluence behind the atria, (b) abnormal spectral Doppler waveforms in the pulmonary veins, (c) a smooth posterior wall of the left atrium, (d) increased retroatrial space, (e) a dilated coronary sinus (cardiac type), (f) a dilated superior vena cava or brachiocephalic vein, and (g) an additional vessel on the 3-vessel/3-vessel and trachea view or a vertical descending vein...
July 2016: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
Richelle Olsen, Zoe Doyle, Denis Levy, Tracy Anton, Delaram Molkara, Maryam Tarsa, Mark Sklansky, Dolores H Pretorius
OBJECTIVES: To review all cases of total anomalous pulmonary venous return (TAPVR) or partial anomalous pulmonary venous return (PAPVR) identified prenatally or postnatally at a single institution and to identify factors that may lead to a correct or missed diagnosis in both high- and low-risk fetuses on screening examinations. METHODS: Fetal images from 16 cases of prenatally or postnatally diagnosed T/PAPVR were retrospectively reviewed to analyze factors that influenced interpretations and diagnoses...
June 2016: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
Ufuk Çakır, Dilek Kahvecioğlu, Serdar Alan, Ömer Erdeve, Begüm Atasay, Tayfun Uçar, Saadet Arsan, Hasan Çakmaklı, Mehmet Ertem, Semra Atalay
Total anomalous pulmonary venous return (TAPVR) is a rare and frequently isolated defect identified in 1% to 3% of all congenital heart diseases. To the best of our knowledge, portal vein thrombosis (PVT) associated with TAPVR has not been reported in the literature. We report a successfully managed PVT in a newborn with infracardiac-type TAPVR and review the literature. Anticoagulation therapies were used during the neonatal period to prevent thrombus progression. PVT should be kept in mind in TAPVR patients who have open heart repair with total correction...
September 2015: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
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