Understanding Dyspnea in a Patient with Pulmonary Vein Stenosis

Abstract

<h3>Introduction</h3> 53-year-old female with a medical history of familial dilated cardiomyopathy, status post atrial fibrillation ablation in 2014 complicated by left pulmonary vein stenosis (PVS) requiring stenting, presents with dyspnea post surgical valve repair for tricuspid regurgitation. <h3>Case Report</h3> The patient presented with dyspnea and orthopnea for 1 month post cardiac surgery which did not improve with diuresis. Right heart catheterization confirmed euvolemia and preserved cardiac output. Given the history of PVS, lung perfusion scan was obtained which revealed left lung perfusion of 6% and right lung perfusion of 94% (1A). CTA confirmed occluded left sided pulmonary vein (1B). Further review of records confirmed that lung perfusion scan demonstrated similar results 4 years ago, and thus PVS did not adequately explain her acute dyspnea. Pulmonary function testing was obtained revealing FEV1 39% predicted, FVC 37% of predicted, FEV1/FVC 106% predicted, suggestive of new restrictive physiology. There was no significant parenchymal lung disease on chest CT to explain this finding, so chest fluoroscopy was performed. This confirmed right hemidiaphragm paralysis (1C), most likely secondary to phrenic nerve injury during cardiac surgery. Home noninvasive positive pressure ventilation at night was instituted, and over time her symptoms improved. <h3>Summary</h3> Patients with chronic unilateral PVS may be asymptomatic if there is adequate contralateral pulmonary reserve. Additional etiologies of dyspnea should be excluded in such patients. Unilateral hemidiaphragm paralysis is frequently asymptomatic. However, in rare circumstances, these two syndromes can occur on contralateral sides resulting in debilitating symptoms.