A 9 month old boy with a double chambered left ventricle presented with congestive cardiac failure refractory to medical treaunent. He was successfully treated by surgical exclusion of the accessory chamber.The double chambered left ventricle is a rare condition and to our knowledge only six cases have been previously reported1-4 We present the first case of this condition successfully treated by exclusion of the accessory chamber.
Case reportThis child was born at 38 weeks gestation after a normal pregnancy. His birthweight was 3.2 kg.He remained well until the age of 3 months when he started to experience feeding difficulties, and at the age of 4 months he was admitted to the Royal Belfast Hospital for Sick Children in congestive cardiac failure. This was treated with digoxin 0-03 mg bd, frusemide 10 mg bd, and aldactone 6.25 mg bd. Cardiac catheterisation at this time showed a double chambered left ventricle (Fig 1). There was free communication between the two chambers, both of which contracted poorly. The pressures in the two chambers were identical (90/10 mmHg). In view of persistent failure to thrive he was referred to the Brompton Hospital, London, for surgical management.Examination of the chest showed a prominent cardiac impulse at the left sternal border. The heart sounds were normal, with a grade 2/4 ejection systolic murmur in the fourth left intercostal space. The rest of the physical examination was normal.Chest x-ray film disclosed gross cardiomegaly (cardiothoracic ratio 16/20), with prominent pulmonary vascular markings. Electrocardiogram showed sinus rhythm with a mean QRS frontal axis of +30, left ventricular hypertrophy, and T wave inversion in leads V5 and V6. Cross-sectional echocardiography showed situs solitus, atrioventricular concordance, and ventriculoarterial concordance. A large accessory chamber appeared to be arising from the lateral border of the left ventricle. The ejection fraction was calculated at 26%.
OPERATIVE DETAILSSurgery was performed under deep hypothermia and circulatory arrest. The child was surface cooled to 23°C when the chest was opened via a median sternotomy incision. The left ventricle was observed to be contracting poorly. There was no external evidence of a discrete diverticulum or aneurysm. Cardiopulmonary bypass was established between a single right atrial cannula and the ascending aorta. The patient was then further cooled to a nasopharyngeal temperature of 15°C and the circulation stopped. A vertical incision was made at the apex of the left ventricle between the left anterior descending coronary artery and its third diagonal branch (Fig. 2a). The "accessory chamber" was entered and its wall appeared to contain viable myocardium. The chamber communicated freely with the main left ventricular chamber (Fig. 2b) through a large fibrous orifice (2 5 cmx2 cm). Using a series of vertical buttressed 4/0 Prolene mattress sutures the fibrous orifice was approximated to the wall of the accessory chamber excluding the accessory chamber while preserving its functiona...
Complete 'anatomic' repair of transposition of the great arteries has now been achieved. The ideal timing of this surgery is during neonatal cardiovascular transition, when the fetal elevation of pulmonary vascular resistance is still present.
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