Idiopathic dilatation of the right atrium: a not so benign entity

Abstract

<jats:title>Abstract</jats:title><jats:p>Idiopathic dilatation of the right atrium is an isolated enlargement of the right atrium in the absence of other cardiac lesions. This rare anomaly has a clinical spectrum ranging from asymptomatic to heart failure or even sudden death. It can be associated with atrial arrhythmias and thrombus formation. Antiplatelet therapy is prescribed in most cases reported in the literature, and reduction plasty is indicated when there is rapid growth of the right atrium, compression of adjacent structures, or refractory arrhythmias. We report four cases of idiopathic dilatation of the right atrium diagnosed during prenatal screening. We describe the intrauterine course and management in postnatal life until early childhood.</jats:p>

Giant right atrium in a child with dilated cardiomyopathy: A case report

“…Most patients are asymptomatic, and the most common symptoms are palpitations, dyspnea, and syncope caused by atrial tachyarrhythmias ( 2 ). The diagnosis of IDRA is usually established with fetal echocardiography and transthoracic echocardiography, and CT or CMR may be beneficial for a definitive diagnosis ( 2 – 4 ). According to prenatal and postnatal imaging features, IDRA should be considered as the primary disorder in our case.…”

“…So far, many studies suggest that the long-term outcomes of patients post-surgery are also contradictory. Although many cases demonstrate that surgery may indeed relieve symptoms associated with IDRA, some children still suffer the recurrent attack of giant RA ( 4 , 6 ). Unfortunately, the situation of our case also suggests that the surgical indications for children with IDRA should be more strictly controlled.…”

“…Giant RA is considered to be of congenital origin in the absence of conditions such as congenital heart disease, pulmonary arterial hypertension, or tricuspid valve disease. IDRA is the most common disease mentioned in recent years due to a marked enlarged right atrium, a congenital anomaly with unknown etiology reported in fetuses, infants, children, and adults (2)(3)(4). Most patients are asymptomatic, and the most common symptoms are palpitations, dyspnea, and syncope caused by atrial tachyarrhythmias (2).…”

Idiopathic dilatation of the right atrium: a case report

“…4 Since it is a simple intervention with few post-operative complications, some authors defend surgical reduction as the treatment of choice in order to avoid the occurrence of thrombosis and arrhythmias. 4,5 Nonetheless other authors report significant post-operative atrial arrhythmias and recommend that this should be taken into consideration when considering surgery in asymptomatic patients. 6 Taking into account the wide range of clinical presentations, it is reasonable to individualise management based on each case.…”

“…3,4 So far, aetiology is unknown and the real pathogenesis has not been clarified. 4 Patients with this condition are usually asymptomatic, yet they can present with atrial arrhythmias, palpitations, fatigue, shortness of breath, chest pain, severe tricuspid regurgitation, congestive heart failure, thrombus formation leading to pulmonary embolism, paradoxical embolism or stroke, and even sudden death. 5,6 The progression varies from spontaneous regression, stabilisation of atrial size, and progressive enlargement.…”