Content area
Full text
A three-month-old male infant presented to the Royal Hospital, Muscat, Oman in April 2014 following two episodes of pneumonia. On presentation, the patient was not dysmorphic; however, he was tachycardic and tachypnoeic with severe respiratory distress. A pansystolic murmur was heard in his apex, radiating to the axilla. A chest examination showed bilateral wheezing and scattered crepitations. An abdominal examination revealed a soft abdomen with hepatomegaly (liver span of 12 cm), situated 4 cm below the right costal margin.
Echocardiography and an angiographic volume-rendered reconstructed computed tomograph showed a giant left atrium measuring 7.4 × 4.7 cm [Figure 1]. The atrium had severe mitral valve regurgitation from poor coaptation of the mitral valve leaflets due to annulus dilation of 22 mm. The mitral valve apparatus and left atrial appendage were normal, excluding the associated left atrial appendage aneurysm. There was mild tricuspid valve regurgitation with a peak gradient of 90 mmHg (systemic blood pressure: 80/55 mmHg), indicating severe pulmonary hypertension. The left ventricle was moderately dilated—the left ventricular posterior wall dimension in diastole was 4 mm; the interventricular septum dimension in diastole was 4 mm; the left ventricular end diastolic dimension was 40 mm; and the left ventricular end systolic dimension was 18 mm. Normal ventricular systolic function was noted (ejection fraction: 62%). The right ventricle was compressed by the dilated left ventricle, with severe compression of the right atrium secondary to the dilated left atrium. The inferior vena cava...