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Having recently moved to the UK, a 27-year-old woman with known uncorrected congenital heart disease was referred for cardiovascular MRI (CMRI) due to increased shortness of breath. CMRI revealed a large (40 mm) aortopulmonary window (figure 1) with complete mixing of the pulmonary and systemic circulations (SaO2 73%), moderate mixed aortic valve disease and mild-moderate pulmonary valve regurgitation. Left and right ventricular volumes and systolic function were normal.
An aortopulmonary window is a communication between the ascending aorta and the pulmonary trunk. It is a distinct entity from truncus arteriosus due to the presence of two separate arterial valves rather than a single arterial valve, and is one of the rarest congenital cardiac lesions, accounting for under 0.1% of all congenital heart defects. Associated vascular lesions are common with anomalies of the aortic arch being the most prevalent, including interruption, coarctation, isthmal hypoplasia, double arch and right aortic arch. Cardiac lesions are also commonplace, including ventricular septal defect, atrial septal defect and left and right ventricular outflow valve stenoses.
In view of the fact that irreversible pulmonary hypertension and premature death are the natural history of uncorrected left-to-right shunt physiology in the setting of aortopulmonary window surgical correction which is the gold standard of treatment, is usually offered to patients at the time of diagnosis before the development of lung injury and irreversible pulmonary hypertension.
Contributors OJR scanned and analysed the data; MB drafted the manuscript; SGM reviewed the images and aided in manuscript revision.
Competing interests None.
Patient consent Obtained.
Ethics approval Oxford REC.
Provenance and peer review Not commissioned; internally peer reviewed.
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