Author/Authors :
Khajali, Zahra Rajaei Heart Medical and Research Center - Iran University of Medical Sciences, Tehran, Iran , Jorfi, Fateme Rajaei Heart Medical and Research Center - Iran University of Medical Sciences, Tehran, Iran , Parsaee, Mozhghan Rajaei Heart Medical and Research Center - Iran University of Medical Sciences, Tehran, Iran , Kasaei, Mohammad Rajaei Heart Medical and Research Center - Iran University of Medical Sciences, Tehran, Iran , Jafarifesharaki, Mehrdad School of Medicine - Shahid Beheshti University of Medical Sciences, Tehran, Iran
Abstract :
Idiopathic right atrial dilation is known as a congenital anomaly in the absence of
obvious causes that produce right ventricular pressure or volume overload. Congenital
giant right atrium can cause functional tricuspid regurgitation, arrhythmia, and rightsided heart failure. A massive Idiopathic Dilation of Right Atrium (IDRA) was reported
in a 30-year-old man presented with intermittent palpitation and dyspnea on effort.
Transthoracic echocardiography revealed huge right atrium leading to severe tricuspid
regurgitation, moderate right ventricular enlargement with moderate dysfunction, and
no evidence of other valvular heart diseases increasing the right ventricular pressure or
volume, left to right shunt, and Ebstein anomaly. Cardiac Magnetic Resonance Imaging
(MRI) confirmed the right atrial enlargement. The estimated right atrium volume by
MRI was about 700 mL. With regard to the patient’s symptoms, he was scheduled for
surgical treatment. The patient underwent partial surgical resection of the dilated right
atrium wall and mechanical tricuspid valve replacement because the tricuspid valve
was unrepairable on the operation table. Idiopathic right atrial enlargement is a very
rare disease and may be easily confused with other anomalies that lead to right atrial
enlargement, such as Ebstein anomaly. Hence, the probability of this pathology should
be kept in mind when the usual etiologies of right atrial enlargement are excluded.
Partial surgical resection of the dilated right atrium is an effective therapeutic option in
symptomatic patients.
Keywords :
Ebstein Anomaly , Heart Atria , Cardiomyopathies , Congenital Heart Disease