Abstract
Congenital left atrial aneurysm is a very rare congenital cardiac
anomaly, and very few cases have been reported with neonatal
presentation. Despite the congenital origin of this condition, it may
remain asymptomatic and patients may not present until their third
decade of life. Even in asymptomatic cases, prompt surgical intervention
is indicated for the prevention of fatal thromboembolic events such as
stroke. In neonates and infants presenting early in life, surgical
intervention is urgent to relieve respiratory distress symptoms. Herein,
we introduce the case of a 3-week-old neonate who presented with severe
respiratory distress symptoms requiring prompt intubation and mechanical
ventilation. Diagnosis of congenital left atrial aneurysm was
established and confirmed by transthoracic echocardiography and computed
tomography angiography. The patient underwent surgical resection of the
aneurysm using cardiopulmonary bypass and cardiac arrest. The patient
was discharged from the hospital after three weeks with normal
echocardiography.
Introduction :
Congenital left atrial aneurysm (CLAA) is an extremely rare congenital
cardiac anomaly with potential serious complications on long-term basis
such as arrhythmias, thromboembolic events and myocardial dysfunction
(1,2). The first description of CLAA was introduced by Semans and
Taussig in 1938 (3). Growth of CLAA may be attributed to dysplasia of
the pectinate muscles which leads to poor myocardial contractility of
the left atrium (LA). This results in progressive dilatation of LA
according to laplace law (2,4,5). It is usually an isolated lesion, and
the most common associated lesion is secondary mitral valve
regurgitation (1,2). It has been reported that diagnosis of CLAA depends
on its origin from a normal LA and its location within the pericardium
with well-defined communication with LA (6). The diagnostic modalities
of CLAA consist of non-invasive imaging such as transthoracic or
transesophageal echocardiography (TTE, TEE), computed tomography
angiography(CTA), and magnetic resonance imaging (MRI) (1,3). Despite
the congenital origin of this condition, it may remain asymptomatic and
patients may not present until their third decade of life, with very few
reported cases of neonatal presentation (2,4). Even in asymptomatic
cases, prompt surgical intervention is indicated, for the prevention of
fatal thromboembolic events such as stroke (1,2,7). In neonates and
infants presenting early in life, surgical intervention is urgent to
relieve respiratory distress symptoms (8). Herein we present a case of a
3-week-old neonate who presented with respiratory distress, and was
diagnosed to have a giant CLAA. Successful surgical resection of the
CLAA was performed as soon as the diagnosis was established .