Spontaneous hemothorax: first manifestation of pulmonary arteriovenous malformation in a patient with hereditary hemorrhagic telangiectasia.

Hereditary Hemorrhagio Telangiectasla (HHT) is a rare inherited autosomal dominant disorder characterized by systemic fibrovascular dysplasia making vessel walls more liable to spontaneous ruptures and injuries. Epistaxis is the first and the most common symptom; however patients may have a variety of serious complications due to vascular involvement of internal organs. In this report we present a case of a 39 year-old woman with recurrent epistaxis and family history of HHT who presented with right spontaneous hemothorax. Pulmonary angiography disclosed multiple pulmonary artery aneurysms with subsequent coil embolization. Considering that pulmonary arteriovenous malformations are associated with considerable morbidity and mortality, patients and family members with suspected HHT should be screened for these vascular malformations.

Total right pulmonary venous return to the inferior vena cava: a rare variant of Scimitar syndrome.

Scimitar syndrome is a rare congenital disorder characterized by a partial, or rarely total, unilateral anomalous pulmonary venous return to the inferior vena cava. This anomaly has a distinctive bimodal presentation with the infantile form having a higher incidence, severity and mortality than the adult form, which is usually asymptomatic on diagnosis. We present the case of a 36-year-old-male transferred to our institution due to incidental anomalous vascular findings on contrast enhanced chest tomography while being evaluated for dyspnea on exertion. Patient underwent right and left heart catheterization with evidence of left-to-right shunt secondary to complete anomalous right pulmonary venous return. Patient was referred to a cardiovascular surgeon with expertise in congenital heart disease for definitive surgical correction.