Right Ventricle Dependent Coronary Circulation: Location of Obstruction is Associated with Survival

Publication date: Available online 30 September 2019Source: The Annals of Thoracic SurgeryAuthor(s): Zachary Spigel, Athar M. Qureshi, Shaine A. Morris, Carlos M. Mery, S. Kristen Sexson-Tejtel, Rodrigo Zea-Vera, Ziyad Binsalamah, Michiaki Imamura, Jeffrey Heinle, Iki AdachiAbstractBackgroundPulmonary atresia with intact ventricular septum (PAIVS) with right ventricle dependent coronary circulation (RVDCC) carries suboptimal outcomes primarily due to cardiac ischemia. We hypothesize clinical outcomes are affected by the level of coronary obstruction, a surrogate for vulnerable myocardium.MethodsWe conducted a single-institution retrospective analysis of all neonates with PAIVS with RVDCC from 1995-2017. RVDCC was defined as the presence of any coronary-cameral fistula with coronary obstruction proximal to the fistula and angiographic evidence of RV perfusion of the myocardium through the fistulous communication. Location of coronary obstruction was categorized as either proximal or distal segments using the SYNTAX score criteria. Transplant-free survival was compared between those with proximal and distal obstruction, then these groups were compared to those without RVDCC.ResultsOf 103 neonates with PAIVS, 28 (27%) had RVDCC: 18 proximal (64%), 10 distal (36%). Median age at last follow-up for RVDCC patients was 1.8 years (interquartile range 0.3-8.1). All deaths (10/28, 36%) occurred at six months old or earlier. Proximal coronary artery obstruction was associated with decre...
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research

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Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Invited Commentaries Source Type: research
Conclusion: The significantly higher production of IL-33 in patients with BA compared to non-BA suggests a potential role of IL-33 for initiation and progression of the disease process, also, IL-33 may have a diagnostic role in infants with BA.
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Original Articles: Hepatology Source Type: research
Objectives: Gamma-glutamyl transferase levels (GGT) are typically elevated in biliary atresia (BA), but normal GGT levels have been observed. This cohort of “normal GGT” BA has neither been described nor has the prognostic value of GGT level on outcomes in BA. We aimed to describe outcomes of a single-centre Australian cohort of infants with BA and assess the impact of GGT level at presentation on outcomes in BA. Methods: Infants diagnosed with BA between 1991 and 2017 were retrospectively analysed. Outcomes were defined as survival with native liver, liver transplantation (LT), and death. Patients were ca...
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Original Articles: Hepatology Source Type: research
Biliary atresia (BA), which is the most important cause of liver cirrhosis in children, is characterized by progressive fibro-obliterative inflammation involving the extra- and intrahepatic bile ducts in infancy.1 A successful Kasai portoenterostomy procedure establishes bile drainage and may  prevent hepatic fibrogenesis and the need for liver transplantation. However, the procedure is unsuccessful in a significant portion of patients, who have persistent jaundice and liver dysfunction. Intestinal bile acids modulate the gut microbiota and may have a role in disease progression in BA.
Source: Pediatrics and Neonatology - Category: Perinatology & Neonatology Authors: Tags: Short Communication Source Type: research
To assess the phenotypic variations found amongst hearts diagnosed at autopsy with hypoplastic left heart syndrome, with attention to implications related to this syndrome as an acquired disease of fetal life, rather than being the consequence of abnormal embryogenesis. We assessed 119 specimens, from two archives, diagnosed initially as representing hypoplastic left heart syndrome. Among the 119 specimens, the majority of which had been entered into the archives prior to the availability of surgical treatment for the syndrome, 36 (30%) had the combination of mitral and aortic atresia, 26 (22%) had mitral and aortic stenos...
Source: Seminars in Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: CONGENITAL – Original Submission Source Type: research
We describe a rare case of newborn with aortic atresia and transposition of the great arteries who underwent successful surgical repair. To the best of our knowledge, no such case has been previously reported. We demonstrated that, even with a complex diagnosis, the patient could survive after rapid two-stage Norwood procedure.
Source: General Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
Conclusion: The current observations revealed that the SM is located more posterior to the FN in CAA patients, and this is mainly attributed to the laterally and anteriorly displaced FN. PMID: 32068476 [PubMed - as supplied by publisher]
Source: Acta Oto-Laryngologica - Category: ENT & OMF Authors: Tags: Acta Otolaryngol Source Type: research
Condition:   Biliary Atresia Interventions:   Other: blood sampling;   Other: skin biopsy sampling;   Other: explanted liver of BA patients sampling Sponsor:   Institut National de la Santé Et de la Recherche Médicale, France Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Publication date: Available online 12 February 2020Source: Computational and Structural Biotechnology JournalAuthor(s): Xin Shi, Li Zhang, Kai Bai, Huilin Xie, Tieliu Shi, Ruilin Zhang, Qihua Fu, Sun Chen, Yanan Lu, Yu Yu, Kun SunAbstractPulmonary atresia (PA) is a rare congenital heart defect (CHD) with complex manifestations and a high mortality rate. Since the genetic determinants in the pathogenesis of PA remain elusive, a thorough identification of the genetic factors through whole exome sequencing (WES) will provide novel insights into underlying mechanisms of PA. We performed WES data from PA/VSD (n=60), PA/IVS(n=20...
Source: Computational and Structural Biotechnology Journal - Category: Biotechnology Source Type: research
Conclusions: All patients enjoyed improvement in AC PTA after surgery (preoperative minus the best postoperative PTA, mean = 34 dB, range = 3.3–52 dB). Hearing declined by an average 8.2 dB over the long-term leaving a final average AC PTA of 37.4 dB HL. Sixty-four percent of patients exhibited stable (
Source: Otology and Neurotology - Category: ENT & OMF Tags: PEDIATRIC OTOLOGY Source Type: research
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