20 November 2010
A term male infant presented at 3 weeks of age with signs of bronchiolitis and moderate respiratory distress. Cardiovascular examination was normal. No initial chest X-ray was performed due to typical clinical findings. He was admitted for oxygen therapy and nasogastric feeding. Respiratory syncytial virus was isolated from a nasopharyngeal aspirate.
A chest X-ray was performed after several days due to failure to improve as expected (Fig. 1). An echocardiogram demonstrated a large pericardial effusion. The heart was structurally normal with no evidence of tamponade. A small, discrete echogenic mass was noted in the right atrial wall. The significance of this was unclear, and follow-up echocardiography was recommended. A pericardial drain was inserted and 100 mL of straw coloured fluid drained. There were no organisms isolated or malignant cells seen on cytology. He improved symptomatically and serial echocardiograms showed no re-accumulation of the fluid or change in the right atrial mass. He was discharged with cardiology follow-up.
He was re-admitted 2 weeks later with tachypnoea. An echocardiogram showed re-accumulation of the pericardial effusion and increased size of the right atrial mass. A cardiac magnetic resonance imaging scan showed a nodular, encapsulated mass adherent to the posterior wall of the right atrium. He underwent repeat drainage of the effusion and received a course of oral corticosteroids; however, the effusion recurred. He therefore underwent complete excision of the right atrial mass, which was extensive and had extended from the right atrial free wall into the inter-atrial septum. Histology was consistent with a haemangioma. His post-operative recovery was uneventful.
On review at 6 months of age, he was clinically well with no evidence of recurrence of the mass or effusion evident on echocardiogram.
Primary cardiac tumours are very rare in the paediatric population. Haemangiomas are the fourth most common cardiac tumour in children (following rhabdomyoma, teratoma and fibroma) but account for less than 5% of all primary cardiac neoplasms.1,2 In a review of the literature, Mackie and colleagues found only 10 case reports of vascular cardiac tumours presenting during the first year of life, with a further 19 cases presenting later in childhood.3
The clinical presentation of a cardiac haemangioma depends on the size and location of the tumour and the age of the patient.3 The right atrium is the most common location in infancy.2,3 In this age group, pericardial effusion is the most common clinical manifestation and may result in cardiac tamponade.2,3 Other presentations include respiratory distress, arrhythmia, haemorrhage and cardiac failure.1,3,4 They may be asymptomatic and present with a murmur or incidental chest X-ray findings.3,4 Cutaneous lesions may be present in some infants.2
Treatment depends on the location and clinical presentation. Some tumours may regress without therapy, while steroid therapy has also been used successfully.1–3 Complete resection is most commonly the treatment of choice with a favourable prognosis and low recurrence rate.1,3,4