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Truncus arteriosus

Phillip S Naimo, Tyson A Fricke, Yves d'Udekem, Johann Brink, Robert G Weintraub, Christian P Brizard, Igor E Konstantinov
OBJECTIVES: Preoperative moderate or greater truncal valve (TV) insufficiency is one of the most important factors influencing mortality in children with truncus arteriosus. We therefore sought to determine the impact of TV insufficiency and concomitant TV surgery on children who underwent truncus arteriosus repair at a single institution. METHODS: We reviewed 180 patients who underwent truncus arteriosus repair between 1979 and 2016. Preoperative echocardiography demonstrated TV insufficiency in 80 patients (mild: 33...
March 8, 2018: European Journal of Cardio-thoracic Surgery
Takashi Kido, Takaya Hoashi, Masataka Kitano, Masatoshi Shimada, Kenichi Kurosaki, Hatsue Ishibashi-Ueda, Hajime Ichikawa
The purpose of the study is to analyze the impact of hybrid stage 1 palliation on right ventricular myocardial pathology in hypoplastic left heart syndrome. Sufficient amount of right ventricular biopsies could be obtained from 16 of 32 patients who underwent Norwood operation between 2007 and 2013. Histopathological findings of right ventricle in patients who underwent primary Norwood operation (primary group, n = 5), patients with aortic atresia (HS1P AA group, n = 6) or aortic stenosis (HS1P AS group, n = 5) who underwent staged Norwood palliation following hybrid stage 1 palliation were compared...
March 9, 2018: Pediatric Cardiology
Erica Hasten, Donna M McDonald-McGinn, Terrence B Crowley, Elaine Zackai, Beverly S Emanuel, Bernice E Morrow, Silvia E Racedo
Non-allelic homologous recombination events on chromosome 22q11.2 during meiosis can result in either the deletion (22q11.2DS) or duplication (22q11.2DupS) syndrome. Although the spectrum and frequency of congenital heart disease (CHD) are known for 22q11.2DS, there is less known for 22q11.2DupS. We now evaluated cardiac phenotypes in 235 subjects with 22q11.2DupS including 102 subjects we collected and 133 subjects that were previously reported as a confirmation and found 25% have CHD, mostly affecting the cardiac outflow tract (OFT)...
March 2, 2018: Human Molecular Genetics
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
Ahmed Kheiwa, Punag Divanji, Vaikom S Mahadevan
Right ventricular outflow tract (RVOT) dysfunction is a common hemodynamic challenge for adults with congenital heart disease (ACHD), including patients with repaired tetralogy of Fallot (TOF), truncus arteriosus (TA), and those who have undergone the Ross procedure for congenital aortic stenosis and the Rastelli repair for transposition of great vessels. Pulmonary valve replacement (PVR) has become one of the most common procedures performed for ACHD patients. Areas covered: Given the advances in transcatheter technology, we conducted a detailed review of the available studies addressing the indications for PVR, historical background, evolving technology, procedural aspects, and the future direction, with an emphasis on ACHD patients...
March 2018: Expert Review of Cardiovascular Therapy
Clauden Louis, Michael F Swartz, Bartholomew V Simon, Jill M Cholette, Nader Atallah-Yunes, Hongyue Wang, Francisco Gensini, George M Alfieris
OBJECTIVE: Repair of truncus arteriosus often requires early right ventricular outflow tract (RVOT) re-operation. Using a modified repair the branch pulmonary arteries are left in-situ, which may avoid earlier RVOT reoperation. We hypothesized that our modified repair for Type I and II truncus arteriosus would extend the time to RVOT re-operation. METHODS: Infants with truncus arteriosus were divided into two groups: A) Traditional technique where the branch pulmonary arteries are excised from the truncal root, or B) Modified repair where the branch pulmonary arteries are left in-situ and septated from the truncal root...
February 8, 2018: Seminars in Thoracic and Cardiovascular Surgery
Zhe Yu, Paul Lf Tang, Jing Wang, Suying Bao, Joseph T Shieh, Alan Wl Leung, Zhao Zhang, Fei Gao, Sandra Yy Wong, Andy Lc Hui, Yuan Gao, Nelson Dung, Zhi-Gang Zhang, Yanhui Fan, Xueya Zhou, Yalun Zhang, Dana Sm Wong, Pak C Sham, Abid Azhar, Pui-Yan Kwok, Patrick Pl Tam, Qizhou Lian, Kathryn Se Cheah, Binbin Wang, You-Qiang Song
Incomplete penetrance of congenital heart defects (CHDs) was observed in a mouse model. We hypothesized that the contribution of a major genetic locus modulates the manifestation of the CHDs. After genome-wide linkage mapping, fine mapping, and high-throughput targeted sequencing, a recessive frameshift mutation of the heterogeneous nuclear ribonucleoprotein A1 (Hnrnpa1) gene was confirmed (Hnrnpa1ct). Hnrnpa1 was expressed in both the first heart field (FHF) and second heart field (SHF) at the cardiac crescent stage but was only maintained in SHF progenitors after heart tube formation...
January 25, 2018: JCI Insight
Dilek Dilli, Nazan Neslihan Doğan, Utku Arman Örün, Murat Koç, Ayşegül Zenciroğlu, Selmin Karademir, Hasan Akduman
OBJECTIVE: It is suggested that folic acid and/or multivitamins, taken periconceptionally, have a role in the prevention of many congenital anomalies. The aim of this study was to determine the serum micronutrient levels in mother-infant pairs with CHD compared with those with healthy newborns and their mothers. METHODS: Serum levels of folic acid, homocysteine, zinc, vitamin A, vitamin D, and vitamin B12 were measured from 108 newborns with CHD (study group) and 103 healthy newborns (control group)...
January 23, 2018: Cardiology in the Young
Jeffrey P Jacobs, John E Mayer, Sara K Pasquali, Kevin D Hill, David M Overman, James D St Louis, S Ram Kumar, Carl L Backer, Charles D Fraser, James S Tweddell, Marshall L Jacobs
The Society of Thoracic Surgeons Congenital Heart Surgery Database is a comprehensive clinical outcomes registry which captures almost all pediatric cardiac surgical operations in the United States. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This article summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency...
January 11, 2018: Annals of Thoracic Surgery
Barakat Adeola Animasahun, Aminat Titilayo Ogunlana, Henry Olusegun Gbelee
Background: The true incidence of truncus arteriosus in underdeveloped countries is difficult to determine. This is due largely to underreporting as a result of nonavailability of technologically advanced facilities to make definitive diagnosis prenatally. There is a lack of data on the profile and outcome of patients with persistent truncus arteriosus (PTA) in Nigeria. This study aims to document the demographic characteristics, mode of presentation, indications for echocardiography, associated anomalies, average age at diagnosis, and outcome of patients with truncus arteriosus in our center...
October 2017: Heart Views: the Official Journal of the Gulf Heart Association
Toshiyuki Iwaya, Koichi Sughimoto, Kagami Miyaji
We describe the case of a 21-year-old patient who underwent repairs for multiple lesions including aortic and pulmonary valve replacements, right ventricular outflow tract reconstruction, revision of the right pulmonary artery route, and a repair of partial anomalous pulmonary venous drainage, which was diagnosed during this fourth sternotomy. For these patients with adult CHD, it is most important to address all underlying factors as much as possible at the redo surgery.
January 8, 2018: Cardiology in the Young
Karl Reyes, Mohsen Ahmed, Alan Brock, Mark Bleiweis
Truncus arteriosus is an extremely rare and complex form of congenital heart disease. Surgical strategies vary depending on anatomic subtype.  In this tutorial, we present our surgical technique for single stage repair of a truncus arteriosus with interrupted aortic arch (Van Praagh type A4).
December 12, 2017: Multimedia Manual of Cardiothoracic Surgery: MMCTS
Koichiro Niwa
Marfan syndrome, bicuspid aortic valve, and/or coarctation of the aorta are associated with medial abnormalities of the ascending aortic or para-coarctation aorta. Medial abnormalities in the ascending aorta are prevalent in other type of patients with a variety of CHDs such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, and tetralogy of Fallot, encompassing a wide age range and may predispose to dilatation, aneurysm, and rapture necessitating aortic valve and root surgery...
December 2017: Cardiology in the Young
Selman Vefa Yıldırım, Ali Yıldırım
Yıldırım SV, Yıldırım A. Truncus arteriosus with double aortic arch: A rare association. Turk J Pediatr 2017; 59: 221-223. Truncus arteriosus (TA) is a congenital heart defect often diagnosed in neonatal period; it represents 0.7% of all congenital heart lesions. The pulmonary arteries originate generally above the coronary ostium. Aorto-pulmonary and interventricular defects are believed to represent an abnormality of conotruncal septation. TA is classified into four types, according to Van Praagh and Colette Edwards...
2017: Turkish Journal of Pediatrics
Travis J Wilder
No abstract text is available yet for this article.
October 26, 2017: Journal of Thoracic and Cardiovascular Surgery
Ling-Yi Wei, Yih-Sharng Chen, Ing-Sh Chiu, Shu-Chien Huang
OBJECTIVE: Quadricuspid truncal valves are susceptible to regurgitation, and tricuspid configuration is considered more durable after repairing the truncal valve. We report a new method by excising the excessive truncal cuspid to reconstruct a new competent aortic valve. METHODS: Bilateral pulmonary banding failed to stabilize a 4-month-old baby with type I truncus arteriosus and quadricuspid truncal valve with severe regurgitation, so surgery was performed. The aorta was transected, and the pulmonary arteries was separated from the aorta...
November 16, 2017: Journal of Thoracic and Cardiovascular Surgery
Lauge Østergaard, Nana Valeur, Nikolaj Ihlemann, Henning Bundgaard, Gunnar Gislason, Christian Torp-Pedersen, Niels Eske Bruun, Lars Søndergaard, Lars Køber, Emil Loldrup Fosbøl
Aims: Patients with prior infective endocarditis (IE), a prosthetic heart valve, or a cyanotic congenital heart disease (CHD) are considered to be at high risk of IE by guidelines. However, knowledge is sparse on the relative risk of IE between these three groups and compared controls. Methods and results: Using Danish nationwide registries (1996-2015), we identified all patients with prior IE, a prosthetic heart valve, or a complex CHD (defined as tetralogy of Fallot, truncus arteriosus, and transposition of great arteries) as well as matched control populations...
December 12, 2017: European Heart Journal
Katherine E Steckham, Catriona J Bhagra, Samuel C Siu, Candice K Silversides
Pregnancy in women with repaired truncus arteriosus (TA) is rare. We report the outcomes of 8 pregnancies in 4 women with surgically repaired TA. None of the women had adverse cardiac events during pregnancy. Two pregnancies, both in the same woman, were complicated by hypertensive disorders of pregnancy. The birth weight was less than the 10th percentile for gestational age in 3 pregnancies. Although women with repaired TA can do well during pregnancy, they require careful preconception assessment and surveillance...
December 2017: Canadian Journal of Cardiology
J M Monné Rodríguez, J Chantrey, S Unwin, R Verin
This case report describes congenital truncus arteriosus in a 1-month-old Eastern black rhinoceros (Diceros bicornis michaeli). From the first day of life the animal was underweight and from the 22nd day of life displayed respiratory signs that exacerbated with time leading eventually to collapse and death. Post-mortem examination revealed a single truncus arteriosus originating from the right ventricle leading to two separated pulmonary arteries and the aorta, with the ventricular septum showing a focal communicating defect...
November 2017: Journal of Comparative Pathology
Maryam Nabati
Persistent truncus arteriosus is a rare congenital cardiac malformation. It is characterized by a single great artery arising from the heart which supplies the aorta, the origin of coronary arteries and pulmonary arteries. Without surgery, prognosis is poor and 90% of these patients die before one year of age. We report a rare case of an asymptomatic 35-year-old woman with uncorrected persistent truncus arteriosus and hypoplastic right and left pulmonary arteries. Hypoplastic branch pulmonary arteries prevented the development of severe pulmonary arterial hypertension...
November 2017: Ultrasound: Journal of the British Medical Ultrasound Society
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