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PF.56 Cardiac Rhabdomyomas in Fetal Life and Beyond: A Single Centre 15-Year Experience
  1. M Walsh,
  2. J Te Water Naude,
  3. O Uzun
  1. University Hospital of UK UK, Cardiff, UK

Abstract

Aim This study describes the immediate complications and outcome of children with antenatally-diagnosed cardiac rhabdomyomas, arising as a consequence of the tuberous sclerosis complex (TSC). This group is compared with those diagnosed after birth.

Method The paediatric cardiology database was interrogated to identify children with cardiac rhabdomyomas: twenty-one cases were analysed, with nine diagnosed antenatally and twelve after birth.

Results Cardiac complications were identified in ¾ of the antenatal group (7 out of 9), compared with a third of the postnatal group (p = 0.08). The commonest antenatal abnormality identified was an outflow tract obstruction, which affected six fetuses. Two significant cases included an intrauterine death at 36 weeks gestation and an induction of labour at 38 weeks, due to a haemodynamically significant left ventricular outflow tract obstruction.

Cardiac arrhythmias affected five antenatally-diagnosed fetuses (56%), with one requiring emergency delivery at 28 weeks and ongoing neonatal management.

The majority of cardiac rhabdomyomas in both groups were located in the ventricles. Tumour growth continued up to 28 weeks of age amongst all surviving children, followed by spontaneous regression, with no need for resective surgery. There was a high prevalence of neurological morbidity in both groups.

Conclusion Antenatal cardiac rhabdomyomas, occurring as part of the TSC, can cause significant morbidity, which is rarely fatal, but warrants careful monitoring until the point of tumour regression. The burden of neurological disease is high in children, compared with the largely favourable cardiac outcome.

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