Pulmonary hypertension in scimitar syndrome in infancy.

Pulmonary hypertension in scimitar syndrome in infancy.

Four infants with the scimitar syndrome presented with failure to thrive, cardiac failure, and pulmonary hypertension. In all cases systemic arteries arose from the upper abdominal aorta to perfuse the lower part of the right lung, and a large unobstructed anomalous pulmonary vein drained venous blood from almost the entire right lung to the inferior vena cava. An additional small pulmonary vei...

Pulmonary hypertension after lung irradiation in infancy.

[Scimitar syndrome].

80 Background --Scimitar syndrome, which presents with an anomalous right pulmonary vein that drains part or the entire right lung into the inferior vena cava (IVC) as its hallmark, is a rare association of congenital cardiopulmonary anomalies with an incidence of approximately 1 to 3 per 100,000 live births worldwide. It commonly presents with symptoms of respiratory distress and has an over-a...

Scimitar syndrome.

Scimitar syndrome is a relatively uncommon constellation of cardio-pulmonary anomalies, its typical feature being partial anomalous pulmonary venous connection. It can present in the neonatal period as well as later in life. We present the case of a girl diagnosed in the newborn period, along with a brief review of literature.

Evaluation of Pulmonary Hypertension In First Degree Relatives Of Patients With Primary Pulmonary Hypertension

Background: Pulmonary hypertension (PH) was defined for the first time in 1951 as primary pulmonary hypertension (PPH). Some studies emphasized on the role of genetics in the development of pulmonary hypertension in family members of affected patients. So, in this study we evaluated the prevalence of pulmonary hypertension in first degree family of patients with documented PPH. Methods: In thi...