A Review of the Management of Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries

ConclusionsVariable surgical strategies are used in the management of PA/VSD/MAPCAs. Most teams report a repair rate of 70% to 80% and a mortality rate lower than 10%. Standardization in data reporting is necessary to better compare the existing strategies.Visual Abstract
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research

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AbstractObjectiveThe systemic ‐to‐pulmonary shunt (SPS) and right ventricle to pulmonary artery (RV‐PA) connection were evaluated to pursue the goal of rehabilitating dysplastic native PAs via establishment of antegrade blood flow. However, the application of these two palliative operations was still confusing. We compared the two operations to determine their different effects on patients who have pulmonary atresia, ventricular septal defects, and major aortopulmonary collateral arteries (MAPCAs).MethodsFrom January 2011 to January 2016, 44 patients received the SPS procedure, and 54 patients received the RV ‐PA c...
Source: Journal of Cardiac Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: ORIGINAL ARTICLE Source Type: research
Abstract OBJECTIVE: To examine the performance of the routine 11-13 weeks scan in detecting fetal non-chromosomal abnormalities. METHODS: This was a retrospective study of prospectively collected data from 100,997 singleton pregnancies attending for a routine ultrasound examination for fetal anatomy, according to a standardized protocol, at 11-13 weeks' gestation. All continuing pregnancies had an additional scan at 18-24 weeks and 71,754 had a scan at either 30-34 or 35-37 weeks. The final diagnosis of fetal abnormalities was based on the results of postnatal examination in the case of livebirths and on the ...
Source: The Ultrasound Review of Obstetrics and Gynecology - Category: Radiology Authors: Tags: Ultrasound Obstet Gynecol Source Type: research
In the current issue of the Seminars, Patel and colleagues from India report outcomes in 25 patients with ventricular septal defect and pulmonary atresia (VSD/PA) who underwent valveless right ventricle to pulmonary artery (RV-PA) shunt as initial palliative surgery.1 There was one operative and one late death. Among the remaining 23 patients, 17 achieved full repair while 6 were awaiting full repair. The distinctive aspect of this series is that the mean age of patients was 12.3 ±3.2 years, much older than VSD/PA patients in the North America or Europe who typically receive surgery during infancy.
Source: Seminars in Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: CONGENITAL – Editorial Commentary Source Type: research
This report presents a case of anatomic repair in a young child with double discordance, ventricular septal defect, and pulmonary atresia. A novel technique of hemi-Senning is described, combined with a bidirectional cavopulmonary anastomosis and Rastelli repair. The possible advantages and applications of this technical modification are briefly discussed.
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
AbstractAn extremely low birth weight infant (810  g) was born with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries accompanied by pulmonary overcirculation, which eventually resulted in intestinal hypoperfusion and focal intestinal perforation from the very early stage of life. Based on the echographic f indings, we performed banding operations twice to regulate the pulmonary blood flow on day 2 and day 9. At 6 months of age, a definitive repair simultaneous with unifocalization of major aortopulmonary collateral arteries was performed. At 1 year of age, the right ...
Source: General Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
Publication date: Available online 27 March 2019Source: The Annals of Thoracic SurgeryAuthor(s): Richard D. Mainwaring, Tristan D. Margetson, Patrick McCarthy, Justin Sleasman, Ozzie Jahadi, Paul Shuttleworth, Don Sheff, Sami Kollmann, William L. Patrick, Frank L. HanleyAbstractBackgroundPulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a rare form of congenital heart disease characterized by the entirety of pulmonary blood flow originating from systemic vessels. The purpose of this study was to measure the residual collateral flow following harvesting of the M...
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
We report a successful case of complete repair with unilateral lung in PAVSD and MAPCAs.
Source: General Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
ConclusionsVariable surgical strategies are used in management of PA/VSD/MAPCAs. Most teams report a repair rate of 70-80% and a mortality rate lower than 10%. Standardization in data reporting is necessary to better compare the existing strategies.Graphical abstract
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
CONCLUSION:  The prognosis in cases without chromosomal or syndromal anomalies is good. MAPCAs did not influence prognosis or postoperative health. The incidence of repeat interventions due to recurrent stenoses is significantly higher after staged compared with single-stage repair. PMID: 30616264 [PubMed - as supplied by publisher]
Source: Ultraschall in der Medizin - Category: Radiology Authors: Tags: Ultraschall Med Source Type: research
ConclusionsAC is a common cause of postoperative respiratory distress and tends to be associated with higher post-repair PA pressure, more frequent RV dysfunction and worse medium-term survival. The study underscores the importance of incorporating all available lung segments to achieve a low PA pressure, potentially preventing pathologic dilatation of the reconstructed PA. Management of patients with poor MAPCAs anatomy physiology remains a challenge.
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
More News: Anatomy | Atresia | Cardiovascular & Thoracic Surgery | Gastroschisis Repair | Hole in the Heart | Rehabilitation | Study | Ventricular Septal Defect