Noonan Syndrome with Coronary Anomaly

CASE REPORT, September 2017, VOL V ISSUE III, ISSN 2042-4884
10.5083/ejcm.20424884.155 , Cite or Link Using DOI
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Ling D Twohig DO PGY-4, Ashley R Nickerson DO, John W Prather MD PhD

The prevalence of single coronary artery (SCA) is a rare congenital anomaly. In the largest angio- graphic review reported by Yamanaka and Hobbs, the incidence of anomalous right coronary artery in 126,595 Americans was 0.26%. Similar ndings have been con rmed on multiple other smaller studies. However, these secondary coronary anomalies supplementing primary Noonan Syndrome are rare; less than 10 cases have been reported.

This case report exhibits a 25 year old male with a very rare type R-I variant of single coronary artery, where the right coronary artery originates from the left anterior descending artery as a secondary coronary anomaly to the Noonan Syndrome. Further workups involving Cardiac CT Angiogram revealed a bicuspid aortic valve with aortic root ectasia and cardiac cath demonstrated normal coronary lumen without arterial disease. The patient was discharged on optimised medical therapy and arrangements were made for continued follow up.

This case highlights the importance of clari ed coronary anatomy, especially in anticipation of possible future surgical intervention. Knowledge of coronary anomaly improves patient care and avoids potential medical complications. This patient’s unique physiology and deranged cardiac condition would bene t from having close follow ups with expert consultation. Beyond clinical utilities, medical knowledge sharing and exchange continues to fuel our intellectual curiosities, as we strive to improve patient safety and reduce medical cost.