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Medicine and Health Sciences Commons

Open Access. Powered by Scholars. Published by Universities.®

Diseases

The Texas Medical Center Library

2019

Abnormalities

Articles 1 - 2 of 2

Full-Text Articles in Medicine and Health Sciences

Anomalous Origin Of The Right Coronary Artery From The Pulmonary Artery In A Neonate With Turner Syndrome And Aortic Arch Hypoplasia, Bryan P Stefek, Jason R Imundo, Joseph B Clark Jun 2019

Anomalous Origin Of The Right Coronary Artery From The Pulmonary Artery In A Neonate With Turner Syndrome And Aortic Arch Hypoplasia, Bryan P Stefek, Jason R Imundo, Joseph B Clark

The Texas Heart Institute Journal

Anomalous origin of the right coronary artery from the pulmonary artery, a rare congenital cardiac defect, is typically not diagnosed during infancy. On the other hand, Turner syndrome is usually diagnosed early, and it is classically associated with bicuspid aortic valve and aortic coarctation. Individuals with Turner syndrome are also at increased risk for coronary artery anomalies. We present a case of anomalous right coronary artery from the pulmonary artery in a week-old neonate who also had Turner syndrome, patent ductus arteriosus, transverse aortic arch hypoplasia, and impaired ventricular function. Prostaglandin therapy through the ductus increased the patient's myocardial perfusion. …


Unconventional 2:1 Ventricular Pacing In A Neonate With Congenital Heart Block And Biventricular Noncompaction, Andrew E Schneider, Philip L Wackel Apr 2019

Unconventional 2:1 Ventricular Pacing In A Neonate With Congenital Heart Block And Biventricular Noncompaction, Andrew E Schneider, Philip L Wackel

The Texas Heart Institute Journal

Congenital complete heart block with concomitant biventricular noncompaction cardiomyopathy has been reported once previously. Although not universal, when restrictive physiology is present, impaired diastolic filling may pose a distinct challenge to pacing during the neonatal period. We present the case of a neonate with congenital complete heart block and biventricular noncompaction that resulted in severe diastolic dysfunction and atrioventricular dyssynchrony. We intentionally used 2:1 ventricular pacing to provide atrioventricular synchrony with every paced beat, and this resulted in hemodynamic and clinical improvement. This unconventional pacing technique may be beneficial in other neonates who have complete heart block and diastolic dysfunction.