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BACKGROUND
Arteriovenous malformations (AVMs) can usually only be treated by excision. When this is not feasible due either to extensive location or involvement of other critical organ systems then options become limited. In the case described, the young woman concerned wanted to become pregnant. This presented her cardiology and obstetric team with a number of potentially life-threatening scenarios that would need to be explained to the patient.
CASE PRESENTATION
A 21-year-old Caucasian female presented for an assessment of cardiac status. She reported shortness of breath on climbing stairs and general fatigue on exertion. Since early childhood it had been noted that her right lower limb was larger than the left. On examination she had a cutaneous naevus over the upper right thigh, buttock and right lower abdomen. The whole right leg was larger than the left ( fig 1 ) and felt hot to touch. Additionally, pupils of both eyes were oval in appearance. Ophthalmic examination confirmed bilateral uveo-retinal coloboma. Cardiovascular examination confirmed sinus rhythm at 80 bpm and normotensive at 110/70 mmHg. On auscultation, there was a grade 3/6 systolic murmur audible throughout the precordium. Bruits were audible throughout the right lower limb as far distal as the right foot.
Past history included recurrent episodes of cellulitis of the right leg and extensive arterial bleeding from ulceration. At age 10 years, surgery was performed on the right knee to slow growth of the leg. At the age of 16 years, intractable bleeding from vessels over the right shin required surgical intervention. There is no family history of AVMs, although her mother also has bilateral uveo-retinal coloboma.
INVESTIGATIONS
The plasma brain natriuretic peptide level in our patient was 7 [mu]mol/L (normal range for a woman of this age is 0-4 [mu]mol/L). MR-angiography has shown that the arterial system in the right leg...