Author + information
- Received May 29, 2013
- Revision received July 2, 2013
- Accepted July 30, 2013
- Published online January 7, 2014.
A 30-year-old female presented to us with exertional breathlessness for 2 years, orthopnea, and paroxysmal nocturnal dyspnea for 2 months. Electrocardiography showed sinus tachycardia and right bundle branch block with clockwise rotation (A). The chest roentgenogram (B) revealed normal heart size, prominent pulmonary trunk, reticular appearance of the lungs or ground glass opacification and evidence of Kerley B lines, and right-sided pleural effusion. Transthoracic echocardiogram revealed dilated right atrium (RA), right ventricle (RV), and main pulmonary artery, and small atrial septal aneurysm in superior position without any shunt (C). Abnormal nonphasic continuous high-velocity color signal seen in the left atrium (LA) originating at the ostia of pulmonary veins (both right and left pulmonary veins) in multiple views is suggestive of pulmonary vein stenosis (gradient ∼25 mm Hg) and severe pulmonary arterial hypertension (pulmonary artery systolic pressure ∼72 mm Hg, moderate tricuspid regurgitation [TR]) (D and E; Online Videos 1, 2, and 3). Multidetector computed tomography showed stenosis of all pulmonary veins (F). Congenital pulmonary vein stenosis presenting in adulthood is very rare. The patient continues to be severely symptomatic despite medical therapy and the prognosis is poor. AO = aorta; IAS = intra-atrial septum.
- Received May 29, 2013.
- Revision received July 2, 2013.
- Accepted July 30, 2013.
- American College of Cardiology Foundation