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

Right Ventricle Dependent Coronary Circulation: Location of Obstruction is Associated with Survival. Ann Thorac Surg. 2019 Sep 30;: Authors: Spigel Z, Qureshi AM, Morris SA, Mery CM, Sexson-Tejtel SK, Zea-Vera R, Binsalamah Z, Imamura M, Heinle J, Adachi I Abstract BACKGROUND: Pulmonary 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. METHODS: We 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. RESULTS: Of 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 wa...
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: Ann Thorac Surg Source Type: research

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Publication date: Available online 25 February 2020Source: Journal of Pediatric Surgery Case ReportsAuthor(s): Elroy P. Weledji, Naiza Monono, Theophil Nana
Source: Journal of Pediatric Surgery Case Reports - Category: Surgery Source Type: research
22q11.2 Deletion syndrome (22q11.2DS) is common in patients with tetralogy of Fallot (TOF) or pulmonary atresia with ventricular septal defect (PA/VSD) and is associated with worse outcomes in children. Whether this impaired prognosis is also translated into adulthood is unknown, as data in adult patients are limited. We aimed to compare long-term outcomes in adults with TOF or PA/VSD both with and without 22q11.2DS.
Source: International Journal of Cardiology - Category: Cardiology Authors: Source Type: research
We report a 3-year-old boy with recurrent hemoptysis for 2 years. The child had tachycardia, tachypnea, tender hepatomegaly, and left-sided decreased air entry with crepitations. Coagulation and Koch's workup was negative. X-ray of the chest showed a small left hemithorax. Computed tomography with angiography showed left unilateral PVA with pulmonary artery hypoplasia and dysplastic left lung. The child has been posted for left pneumonectomy. Although uncommon, PVA should be diagnosed early to prevent life-threatening complications such as hemoptysis and pulmonary hypertension. The patient can be managed conservatively...
Source: Lung India - Category: Respiratory Medicine Authors: Source Type: research
We present 6 cases of confirmed biliary atresia in infants who had biliary patency reported on HIDA scan. We demonstrate that misinterpreted HIDA scans led to delayed diagnosis and surgical intervention for biliary atresia.
Source: The Journal of Pediatrics - Category: Pediatrics Authors: Tags: Brief Reports Source Type: research
Minimally invasive repair of esophageal atresia with tracheoesophageal fistula (EA/TEF) and congenital diaphragmatic hernia (CDH) is feasible and confers benefits compared to thoracotomy or laparotomy. However, carbon dioxide (CO2) insufflation can lead to hypercapnia and acidosis. We sought to determine the effect of lower insufflation pressures on patients' surrogate markers for CO2 absorption — arterial partial pressure of CO2 (PaCO2), end tidal CO2 (EtCO2) and pH.
Source: Journal of Pediatric Surgery - Category: Surgery Authors: Source Type: research
No abstract available
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
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