Bicuspid pulmonic valve with stenosis and marked pulmonary artery dilation: A case report

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Conference Proceeding

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Purpose: Congenital stenosis of t he pulmonic valve (PV) has been associated with pulmonary artery (PA) dilation, possibly leading to compression or distortion of surrounding structures like the left main coronary artery (LMCA) and left mainstem bronchus. There is no established criterion regarding the size of t he PA as it relates to the angle of origin of the LMCA and at what intervals these should be imaged or intervened upon. The goal of this case report is to explore the role of multi-modality imaging in diagnosing a symptomatic patient with PA dilation and a narrow angle of incidence of his LMCA and to review the literature identifying incidence and outlines for intervention. Case Report: A 31 year old man with congenital PV stenosis and spontaneously closed ventricular septal defect presented with syncope. His echocardiogram demonstrated a left ventricular (LV) ejection fraction of 59%, mild PV stenosis, and a PA measured at 4.3 cm. Cardiac magnetic resonance imaging (MRI) was performed and revealed a bicuspid PV with stenosis and a dilated PA measuring 6.2 cm. The patient did well and had no issues until five years later. At the age of 36, the patient presented with unprovoked palpitations and light headed sensation. He denied syncope. Ambulatory heart monitoring did not reveal arrhythmia. Repeat echocardiogram demonstrated mild PV stenosis with a dilated PA measuring 6.02 cm. CT was performed to evaluate the PA and LMCA. This revealed a main PA measuring 5.8 cm, a LMCA with normal anatomic course without evidence of compression or stenosis, and no other coronary artery disease. The angle of take-off of the LMCA was < 60 degrees from the aorta. The patient underwent exercise stress test with technetium-99m sestamibi myocardial perfusion imaging (MPI) which demonstrated no perfusion defect. He has since done well with no recurrence of symptoms. Discussion: This patient has unusual findings. Bicuspid PV is rare and has been associated with maternal rubella infection. This patient had no history of such. This case is significant in its demonstration of PV stenosis complicated by PA dilation and narrow angle or origin of the LMCA, yet without compression or stenosis of the LMCA. It is also important in its demonstration of multi-modality imaging to help assess patients' symptoms and pathologies comprehensively. Further studies are needed to develop imaging guidelines on PA size as it relates to LMCA angle of take-off and when to monitor/intervene.





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