Abstract:
OBJECTIVE:Published case reports suggest that congenital portosystemic venous connections (PSVC) and other abdominal venous anomalies may be relatively frequent and potentially important in patients with polysplenia syndrome. Our objective was to investigate the frequency and range of portal and other abdominal systemic venous anomalies in patients with polysplenia and inferior vena cava (IVC) interruption who underwent a cavopulmonary anastomosis procedure at our center, and to review the published literature on this topic and the potential clinical importance of such anomalies. DESIGN:Retrospective cohort study and literature review were used. RESULTS:Among 77 patients with heterotaxy, univentricular heart disease, and IVC interruption who underwent a bidirectional Glenn and/or modified Fontan procedure, pulmonary arteriovenous malformations were diagnosed in 33 (43%). Bilateral superior vena cavas were present in 42 patients (55%). Despite inadequate imaging in many patients, a partial PSVC, dual IVCs, and/or renal vein anomalies were detected in 15 patients (19%). A PSVC formed by a tortuous vessel running from the systemic venous system to the extrahepatic portal vein was found in six patients (8%). Abdominal venous anomalies other than PSVC were documented in 13 patients (16%), including nine (12%) with some form of duplicated IVC system, with a large azygous vein continuing to the superior vena cava and a parallel, contralateral IVC of similar or smaller size, and seven with renal vein anomalies. In patients with a partial PSVC or a duplicate IVC that connected to the atrium, the abnormal connection allowed right-to-left shunting. CONCLUSIONS:PSVC and other abdominal venous anomalies may be clinically important but under-recognized in patients with IVC interruption and univentricular heart disease. In such patients, preoperative evaluation of the abdominal systemic venous system may be valuable. More data are necessary to determine whether there is a pathophysiologic connection between the polysplenia variant of heterotaxy, PSVC, and cavopulmonary anastomosis-associated pulmonary arteriovenous malformations.
journal_name
Congenit Heart Disjournal_title
Congenital heart diseaseauthors
McElhinney DB,Marx GR,Newburger JWdoi
10.1111/j.1747-0803.2010.00478.xsubject
Has Abstractpub_date
2011-01-01 00:00:00pages
28-40issue
1eissn
1747-079Xissn
1747-0803journal_volume
6pub_type
杂志文章,评审abstract:OBJECTIVE:The current study aims to identify the rates of lapses in care and loss to follow-up before age one through age five for white and nonwhite congenital heart disease (CHD) survivors. Nonwhite CHD survivors were hypothesized to experience an earlier lapse in care and be lost to follow-up than whites. DESIGN:Pa...
journal_title:Congenital heart disease
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journal_title:Congenital heart disease
pub_type: 杂志文章
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abstract:OBJECTIVE:We hypothesized that a right aortic arch in situs solitus, with or without an associated cardiovascular malformation, is often associated with a vascular ring. METHODS:From those born in Southern Nevada between March 2012 and March 2017, we identified 50 (3.6 per 10,000 live births) with a right aortic arch ...
journal_title:Congenital heart disease
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journal_title:Congenital heart disease
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abstract::Crisscross heart is a rare congenital cardiac anomaly in which systemic and pulmonary venous streams cross without mixing at atrioventricular level. We report a case of crisscross heart with tricuspid atresia, double outlet right ventricle, and pulmonary stenosis, which was diagnosed prenatally. ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2012.00716.x
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abstract:BACKGROUND:It has been reported that gender differences in cardiovascular outcomes found in adults also are present in children who undergo surgical repair for congenital heart disease. METHODS:California statewide hospital discharge data 1989-99 were used to study outcomes in children <18 years undergoing cardiac sur...
journal_title:Congenital heart disease
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doi:10.1111/j.1747-0803.2006.00013.x
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journal_title:Congenital heart disease
pub_type: 杂志文章
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journal_title:Congenital heart disease
pub_type: 杂志文章
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journal_title:Congenital heart disease
pub_type: 杂志文章
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journal_title:Congenital heart disease
pub_type: 杂志文章
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更新日期:2016-01-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2008.00174.x
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journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12568
更新日期:2018-03-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12192
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journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12001
更新日期:2013-07-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2011.00513.x
更新日期:2012-01-01 00:00:00
abstract:OBJECTIVE:Congenital cardiac defects represent the most common group of birth defects, affecting an estimated six per 1000 births. Genetic characterization of patients and families with cardiac defects has identified a number of genes required for heart development. Yet, despite the rapid pace of these advances, mutati...
journal_title:Congenital heart disease
pub_type: 杂志文章,meta分析
doi:10.1111/chd.12179
更新日期:2015-05-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章,评审
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更新日期:2014-05-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2011.00514.x
更新日期:2012-01-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章
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abstract::Carditis is a common manifestation of adult patients with Lyme disease affecting 4-10% of Lyme patients in the United States. However, children with Lyme disease rarely present with acute carditis. The management of pediatric patients with complete heart block (CHB) secondary to Lyme carditis has not been well describ...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
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更新日期:2007-09-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章
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更新日期:2007-01-01 00:00:00
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更新日期:2017-05-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章,meta分析,评审
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更新日期:2015-01-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章
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更新日期:2011-07-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章
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journal_title:Congenital heart disease
pub_type: 杂志文章,多中心研究
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更新日期:2013-09-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章
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更新日期:2015-05-01 00:00:00
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journal_title:Congenital heart disease
pub_type: 杂志文章
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journal_title:Congenital heart disease
pub_type: 杂志文章,评审
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journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2009.00373.x
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