Expansion of pulmonary arteriovenous malformations after grand mal seizures and other circumstances of PAVM growth

Abstract

A woman with asymptomatic pulmonary arteriovenous malformation (PAVM) discovered incidentally on admission developed recurrent generalised seizures. Immediately after, the PAVM demonstrated marked expansion, and was safely resected. Congenital PAVMs (associated with hereditary haemorrhagic telangiectasia or sporadic) are considered stable lesions that exhibit very slow growth if at all. A review of the literature reveals all circumstances of accelerated growth of PAVM (puberty, pregnancy, postpartum, pulmonary hypertension) and suggests a novel mechanism of seizure-associated expansion. This is important because the size and rapid growth of PAVMs correlate with the potential for rupture and other ominous complications such as right to left shunt and paradoxical emboli. The new seizures–PAVM progression association mandates recognition since the risk of seizures in patients with PAVM is substantial. Our observations strongly suggest the need to monitor PAVM in patients with or without haemorrhagic telangiectasis by repeated imaging after generalised seizures to evaluate potential expansion and risk.

Hereditary haemorrhagic telangiectasia with atrial septal defect and pulmonary hypertension during advanced pregnancy: a case report and literature review

“…However, the risk of imminent major haemorrhage is substantial as long as PAVMs are perfused at systemic pressure due to marked PH of any cause. 43 Unfortunately, we did not terminate the pregnancy in time to remove the factors of PAVM rupture, resulting in massive haemoptysis.…”