Niloufar Samiei - Heart Valve Research Center, Rajaie Cardiovascular, Medical, and Research Center, Iran University of Medical Sciences, Tehran, Iran
Sepehr Gohari - Student Research Center, School of Medicine, Zanjan University of Medical Sciences, Zanjan, Iran
Hassan Ahangar - Department of Cardiology, Mousavi Hospital, Zanjan University of Medical Sciences, Zanjan, Iran.

Introduction: Shone syndrome is a rare congenital cardiac abnormality; however, many of the cases remain undiagnosed until early and mid-adulthood. Different Imaging modalities are used to assess the related structural abnormalities. Case presentation: In this case study, we report a ۳۲ yo woman who was planning for her first pregnancy. In consideration to her childhood heart problems, besides a history of extended penicillin prescriptions for several years, she was referred for complementary assessments. At the time of presentation she was asymptomatic. Imaging results showed several structural obstructive left sided lesions and pulmonary artery hypertension.  Conclusion: Ultimately, patient diagnosed with congenital shone syndrome which was misdiagnosed in the early ages. Shone complex in our case was presented in its complete form that could be potentially fatal in case of pregnancy.

Congenital heart disease, Echocardiography, Shone syndrome, Pregnancy