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Surgical resection of multiple right atrial masses in a child with β thalassaemia and receiving desferioxamine
  1. M West,
  2. G A Malik,
  3. J C S Pollock
  1. sdmwest{at}

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A 14 year old boy was referred for surgical treatment of multiple right atrial masses. At the time of referral he was asymptomatic. His right atrial masses (T) had been discovered incidentally on a routine chest x ray (middle panel) some five months before his referral. He had been diagnosed as having β thalassaemia at the age of 2 years and had a splenectomy at 13 years of age. His medications included penicillin, vitamin C, and desferioxamine. His desferioxamine was administered via a Hickman line which was inserted seven years previously. His clinical examination was essentially normal except for his well healed splenectomy scar and the Hickman line which was palpable in the right infraclavicular fossa. Haematological investigations revealed a haemoglobin of 10.3 g/dl, a white cell count of 12.3 × 109 g/l, and platelet count of 166 × 109 g/l. Transthoracic echocardiography showed a larger right atrial mass which prolapsed through the tricuspid valve (V) (right, upper panel) and also several smaller right atrial masses.

At surgery a large 37 × 30 × 16 mm pedunculated calcified mass was found in the right atrium. This was found to be occluding the orifice of the tricuspid valve. The stalk lay on the atrial septum immediately posterior to the coronary sinus; this stalk was calcified into the tissue wall and was removed as much as possible, as was the mass (right, lower panel). There was an 11 × 5 × 2 mm second mass on the right atrial wall inferiorly above the inferior vena cava orifice and numerous small calcified masses on the atrial septum. These were all removed. The anterior leaflet of the tricuspid valve was found to have central perforation. This appeared to be where the large atrial mass impacted on the tricuspid valve. This perforation was repaired. Histology of the masses showed them to be calcified thrombus with no signs of iron deposition. The patient’s recovery was complicated by a pericardial effusion, which required draining. The patient was discharged home after 14 days and was well two months after final discharge. We conclude that this is an unusual case of dystrophic calcification of multiple atrial thrombi caused by long term insertion of a Hickman line.

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