A Massive Arteriovenous Malformation arising from the Aorta causing Severe Pulmonary Hypertension
Copyright (c) 2018 Kennelly et al.
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Aortic arteriovenous malformations (AVMs) are rare vascular anomalies where the aorta communicates with adjacent venous structures, bypassing the capillary system. We report a case of a neonate born at 34 weeks gestation who presented at 3 weeks of life in respiratory distress. Echocardiography demonstrated severe pulmonary hypertension with right ventricular dilatation but an otherwise structurally normal heart. Clinical examination revealed a pulsatile abdominal mass with an audible bruit.
Abdominal ultrasound was performed and it demonstrated a large cystic mass arising from the inferior vena cava (IVC) with both arterial and venous vascular flow within it. Further evaluation with magnetic resonance angiography (MRA) showed a massive arteriovenous malformation from both left and right iliac arteries, and lumbar arteries communicating with the IVC. A decision was made with interventional radiology to attempt coiling of the vascular lesion. Following this procedure his pulmonary hypertension worsened in severity, culminating in acute heart failure and multi-organ dysfunction. A subsequent interventional radiology procedure found that there was no blood flow to his bowel or abdominal viscera. Cardiac arrest followed with an unsuccessful attempt at resuscitation. This case describes a presentation of severe pulmonary hypertension initially considered to be secondary to either congenital heart disease or intrinsic pulmonary disease, but which was found to be as a result of a very rarely occurring massive aortic AVM.