SURGICAL MANAGEMENT OF PULMONARY ATRESIA WITH VENTRICULAR SEPTAL DEFECT AND MAJOR AORTOPULMONARY COLLATERAL ARTERIES: Part II —Midline unifocalization and Unifocalization revision

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a rare and heterogeneous form of congenital heart disease. Despite improvements in the surgical management of this disease, there is still an ongoing controversy regarding the optimal treatment. Part II of this paper will focus on the surgical results of unifocalization and the need and techniques used for unifocalization revision at Stanford University.
Source: Operative Techniques in Cardiac and Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: Congenital Source Type: research

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The aim of this article was to review computed tomography angiography and magnetic resonance angiography of pulmonary atresia with ventricular septal defect. This disorder is a rare complex congenital heart disease. Preoperative imaging of pulmonary atresia with ventricular septal defect with computed tomography angiography and magnetic resonance angiography is important for complete anatomical delineation and planning for treatment. Preoperative imaging used for assessment of the main pulmonary artery (its size, valve, and confluence), aortopulmonary collaterals (its origin, insertion, course, and size), presence of paten...
Source: Journal of Computer Assisted Tomography - Category: Radiology Tags: Cardiothoracic Imaging Source Type: research
This study is used to determine whether the VSD can be closed during surgery. A collateral flow study circuit is also set up for first-time operations to measure the residual collateral flow after all of the MAPCAs have been harvested. Patients who require midline unifocalization will invariably require very lengthy periods of support on cardiopulmonary bypass. We have adapted our perfusion circuitry to prepare for the demands on the bypass circuit to meet the requirements of this patient population. Our institution has developed a systematic approach for the conduct of perfusion to best serve our patients presenting with ...
Source: Journal of Extra-Corporeal Technology - Category: Cardiovascular & Thoracic Surgery Tags: J Extra Corpor Technol Source Type: research
Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a relatively rare and extremely heterogeneous form of congenital heart disease. Despite vast improvements in the surgical management of this disease, there is still an ongoing controversy regarding the optimal treatment. The purpose of this paper is to summarize the surgical algorithm and experience at Stanford University.
Source: Operative Techniques in Cardiac and Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: Congenital Source Type: research
If the left and right ventricles were siblings, the right would have a reasonable claim that the left receives preferential treatment during childhood treatment of congenital heart disease. This would be relevant to pathologies such as tetralogy of Fallot, truncus arteriosus, and pulmonary atresia with ventricular septal defect. By the time adolescence rolls around, the right ventricle has suffered years of volume and pressure load. The left ventricle remains perky, worried that its sick sibling might one day affect its longer-term prospects.
Source: The Journal of Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: Commentary 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
Rationale: Atrioventricular reentrant tachycardia (AVRT) is the most common supraventricular tachycardia occurring in children. However, in complex congenital heart disease patients with a different heart anatomy and conduction system morphology, accessory pathway modification may be particularly challenging because of distortion of typical anatomic landmarks. Patient concerns: A 10-year-old boy with tricuspid atresia and history of bidirectional Glenn operation had recurrent chest distress and palpitation for 3 months. He had multiple hospitalizations for narrow-QRS tachycardia with poor hemodynamic tolerance, despit...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
Pulmonary atresia witha ventricular septal defect and major aortopulmonary collateral arteries(PA/VSD/MAPCAs), is a cardiac malformation characterized by the lack of a pulmonary valve and a pulmonary vascular bed supplied by aortopulmonary collateral arteries.1 This rare cyanotic congenital heart disease is associated with a high mortality.2,3 Owing to deterioration of cardiac function during pregnancy, the anesthetic management for cesarean delivery is a particular challenge. As reported by Atanassoff,4 pre-labor decisions regarding preoperative, perioperative and postoperative management are vital for a successful delive...
Source: International Journal of Obstetric Anesthesia - Category: Anesthesiology Authors: Tags: Case report Source Type: research
The traditional indications for aortic valve preservation during root replacement (valve-sparing root replacement [VSRR]) include connective tissue disorders (Marfan and Loeys-Dietz syndromes), bicuspid aortic valve, and selected congenital heart defects. Patients with congenital heart disease (CHD) include those with (1) conotruncal abnormalities (tetralogy of Fallot, pulmonary atresia with ventricular septal defect, truncus arteriosus, and transposition of the great arteries), (2) palliated single-ventricle physiology, and (3) previous arterial switch or Ross procedures.
Source: The Journal of Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: Editorial commentary Source Type: research
ConclusionsPerforation followed by RVOT stenting may be a safe and effective alternative to surgical SPS in infants with PA/VSD.
Source: Congenital Heart Disease - Category: Cardiology Authors: Tags: ORIGINAL ARTICLE Source Type: research
Abstract BackgroundAn association between congenital heart disease (CHD) and infantile cholestasis, a key finding for the diagnosis of biliary atresia (BA), remains unknown. The aim of this study was to investigate the characteristics of direct hyperbilirubinemia (D‐HB) in infants with CHD. MethodsAll neonates who were admitted to our hospital and diagnosed with CHD in 2015 and 2016 were included. D‐HB (direct bilirubin≧ 2.0 mg/dL) within 60 days of age and other clinical parameters were retrospectively reviewed. Statistical analysis between patients with/without D‐HB was performed by chi square test or Wilcoxon ra...
Source: Pediatrics International - Category: Pediatrics Authors: Tags: Original Article Source Type: research
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