Recurrence of arterio-venous malformations with life-threatening complications in a pregnant woman with hereditary teleangiectasia.
Hereditary hemorrhagic teleangiectasia (HHT) is an autosomal dominant vascular disorder with incidence of 1 in 2300, characterized by teleangiectasia, arterio-venous malformations, and aneurysms. In this article we presented a case of a 43-year-old woman, diagnosed with HHT and treated with a lobectomy at the age of 5 and with transcatheter coil closure of pulmonary feeding artery at the age of 30, who developed a recurrence of arterio-venous fistulas in the lungs and the brain during pregnancy. The case was complicated by ischemic stroke with hemiparesis. The patient went into premature labor at 35 weeks and the child was delivered by cesarean section. The patient developed severe pulmonary insufficiency with hemothorax post partum, which required transcatheter embolotherapy. During a seven year follow-up, the patient developed progressing intrapulmonary shunt deterioration and hypertrophic pulmonary osteoarthropathy. The case described shows that significant morbidity and mortality may arise during pregnancy from this otherwise relatively benign condition.
Swietlik
E
E
Department of Internal and Pulmonary Medicine, Miedzyleski Hospital, Warsaw, Poland. em_swietlik@wp.pl
Doboszynska
A
A
D000328:Adult; D001164:Arteriovenous Fistula / Q000473:pathology; D001165:Arteriovenous Malformations / Q000150:complications / Q000209:etiology* / Q000473:pathology*; D002545:Brain Ischemia / Q000150:complications; D002585:Cesarean Section; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D010291:Paresis / Q000209:etiology; D049590:Postpartum Period; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000473:pathology*; D011652:Pulmonary Circulation / Q000502:physiology; D012008:Recurrence; D020521:Stroke / Q000150:complications; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000235:genetics / Q000473:pathology*