Case 4/2019 - Pulmonary Atresia, Ventricular Septal Defect, and Systemic-Pulmonary Collateral Circulation developing after prior Blalock-Taussig, in a Symptomatic 47-Year-Old Adult

Abstract The electrocardiogram (ECG) has been reinvigorated by the identification of electrical alterations that were not definitely clarified before. In this context, and mainly regarding the definition of arrhythmogenic substrates, the association of the ECG with the vectorcardiogram (VCG) has gathered much more information about the cardiac electrical phenomena, thus allowing us to differentiate potentially fatal cases from benign ones. Obtaining a VCG concomitantly with the performance of an ECG has led to a significant gain in the definition of extremely sophisticated pathologies, which function suffer some type of structural or dynamic alterations, involving either the reduction or enhancement of ionic channels and currents. The classic aspects of the ECG/VCG association in the differential diagnosis of myocardial infarctions, conduction disorders, atrial and ventricular hypertrophies, and the correlations between these electrical disorders are still valid and assertive. The association of these pathologies is further clarified when they are seen through the ECG/VCG dyad. The three-dimensional spatial orientation of both the atrial and the ventricular activity provides a far more complete observation tool than the ECG linear form. The modern analysis of the ECG and its respective VCG, simultaneously obtained by the recent technique called electro-vectorcardiogram (ECG/VCG), brought a significant gain for the differential diagnosis of some pathologies. Therefore, we illu...
Source: Arquivos Brasileiros de Cardiologia - Category: Cardiology Source Type: research

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A 2-year-old cyanotic child underwent echocardiography which revealed criss-cross heart and truncus arteriosus. CT Angiography done to confirm the diagnosis revealed aortic atresia with hypoplastic ascending aorta and proximal aortic arch (white arrow) with dilated patent ductus arteriosus (PDA) reforming the distal aortic arch (Figure 1a-b). The coronary arteries were arising from the hypoplastic aortic sinuses. There was also a supero-inferior relationship of ventricles with the presence of a horizontal interventricular septum and a ventricular septal defect.
Source: Journal of Cardiovascular Computed Tomography - Category: Radiology Authors: Tags: Case report 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
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
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
AbstractA massive but asymptomatic left subclavian artery pseudoaneurysm was diagnosed in a 30-year-old female patient with transposition of the great arteries, ventricular septal defect, and pulmonary atresia. After undergoing bilateral modified Blalock –Taussig shunts at the age of 4 months and 3 years, respectively, she underwent the Rastelli operation and division of both shunts at the age of 6 years of age. The pseudoaneurysm was not discovered at the follow-up investigation at age 14. During the time period from age 18 to 30 years, she w as lost to follow-up, she was confirmed to be free fro...
Source: General Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery 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
AbstractWe here report a neonate with prenatal echocardiographic diagnosis of tricuspid atresia, with normally related great vessels, and large ventricular septal defect. This diagnosis could be confirmed with echocardiography at birth. An additional double mitral orifice was also seen. This is a very rare association.
Source: Mammalian Genome - Category: Genetics & Stem Cells 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
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
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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