Cardiology in the young
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Cardiology in the young · Dec 2018
Case ReportsVascular ring associated with d-transposition of the great arteries: when should we suspect aortic arch anomalies?
A male neonate with d-transposition of the great arteries was diagnosed with the concomitant anomaly of left circumflex aortic arch and right-sided ductus arteriosus, which formed a vascular ring. Initial postnatal echocardiography had demonstrated an obscured aortic isthmus mimicking coarctation of the aorta, which could be a diagnostic clue to circumflex aortic arch.
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Cardiology in the young · Nov 2018
Eighteen years of paediatric extracorporeal membrane oxygenation and ventricular assist devices: insight regarding late outcomes.
We reviewed all patients who were supported with extracorporeal membrane oxygenation and/or ventricular assist device at our institution in order to describe diagnostic characteristics and assess mortality. ⋯ This single-institutional 18-year review documents the differential probability of survival for various sub-groups of patients who require support with extracorporeal membrane oxygenation or ventricular assist device. The indication for mechanical circulatory support, underlying diagnosis, age, and setting in which cannulation occurs may affect survival after extracorporeal membrane oxygenation and ventricular assist device. The Kaplan-Meier analyses in this study demonstrate that patients who survive to hospital discharge have an excellent chance of longer-term survival.
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Cardiology in the young · Oct 2018
Bloodless priming of the cardiopulmonary bypass circuit: determinants of successful transfusion-free operation in neonates and infants with a maximum body weight of 7 kg.
We currently perform open-heart procedures using bloodless priming of cardiopulmonary bypass circuits regardless of a patient's body weight. This study presents results of this blood-saving approach in neonates and infants with a body weight of up to 7 kg. It tests with multivariate analysis factors that affect perioperative transfusion. ⋯ In conclusion, by adjusting the circuit, cardiopulmonary bypass could be conducted without donor blood in majority of patients, regardless of body weight. Transfusion-free open-heart surgery in neonates and infants requires team cooperation. It was more often achieved in procedures with lower mortality score.
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Cardiology in the young · Sep 2018
Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: collateral vessel disease burden and unifocalisation strategies.
IntroductionThe optimal approach to unifocalisation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (pulmonary artery/ventricular septal defect/major aortopulmonary collaterals) remains controversial. Moreover, the impact of collateral vessel disease burden on surgical decision-making and late outcomes remains poorly defined. ⋯ In all, 41 patients received single-stage unifocalisation (Group 1) and 43 patients underwent multi-stage repair (Group 2). Preoperative collateral vessel anatomy, branch pulmonary artery reinterventions, ventricular septal defect status, and late right ventricle/left ventricle pressure ratio were evaluated.
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Cardiology in the young · Aug 2018
Case ReportsIncidental finding of pulmonary arterial sling during patent ductus arteriosus surgery in a patient with Mowat-Wilson syndrome.
Mowat-Wilson syndrome is a genetic condition due to a mutation in the ZEB2 gene; it affects many systems including the cardiovascular system. The pulmonary arterial sling originates from a failure of development of the proximal portion of the left sixth aortic arch, resulting in an anomalous left pulmonary artery origin from the posterior wall of the right pulmonary artery and the left pulmonary artery crossing to the left lung between the trachea and the oesophagus. We present a 4-month-old infant with Mowat-Wilson syndrome and left pulmonary arterial sling, and discuss the association of these two rare conditions. Pulmonary arterial sling is significantly more frequent in patients with Mowat-Wilson syndrome than in the general population.