INTRODUCTION: Pulmonary arterial hypertension(PAH) is a progressive, symptomatic and if untreated an ultimately fatal disorder. While idiopathic PAH is most common, often there is an underlying condition which results in pulmonary hypertension. It is essential to identify any potentially treatable or reversible causes for PAH even though some of the treatable causes may be rare. We present one such case of severe PAH associated with pulmonary venous stenosis (PVS) which is a potentially reversible and treatable etiology of pulmonary hypertension which if diagnosed in time can lead to preservation of cardiac function.
CASE PRESENTATION: This is a case of 30 year old gentleman with medical history of premature birth (26 weeks) resulting in broncho-pulmonary dysplasia and cognitive deficit. The patient had been on oxygen and scheduled furosemide until 12 yrs of age. He had been symptom free until recently when he was admitted to the hospital with a complaint of worsening dyspnea (NYHA grade IV) for 1 week. He was diagnosed to have severe pulmonary hypertension (right ventricle systolic pressure estimated - 106 mm hg) and right side heart failure. This responded to furosemide and sildenafil. Lung perfusion scan suggested no V/Q mismatch. Outpatient pulmonary function tests showed very severe obstructive pattern and severely reduced diffusion capacity. Right heart catheterization done subsequently showed significantly elevated mean pulmonary artery pressures and right ventricular systolic pressures of 72mmhg and 110mmhg, respectively. Unexpectedly, the patient had a difference in the pulmonary capillary wedge pressures on the right (12mmhg) and left (33mmhg) side. This combined with angiographic images lead to the diagnosis of severe left lower pulmonary vein stenosis. This vein was stented and post stent mean pulmonary artery pressure and right systolic pressure had improved to 37mmhg and 46mmhg. Right heart catheterization done 7 months post stent showed normalization of pulmonary capillary wedge pressure on both sides with patency of the stented vein.
DISCUSSION: Pulmonary vein stenosis, both congenital and acquired can lead to severe and progressive pulmonary hypertension. Congenital pulmonary vein stenosis is a rare disease that occurs in only 0.4%of all congenital heart disease. Focal pulmonary vein stenosis has better prognosis than diffuse disease(1). Isolated case reports in adults have been varied from involvement of all the veins to involvement of isolated veins(1, 2). These cases stress the importance of right heart catheterization by experts well versed with congenital disorders. A high wedge pressure in such cases may simply be attributed to left ventricular dysfunction or an inability to obtain appropriate pulmonary capillary wedge pressure. Isolated increase in left pulmonary capillary wedge pressure in our patient suggested a localized cause compared to dysfunction of left atrium or ventricle. Without this finding the PAH could easily have been attributed to severe airway obstruction from his broncho-pulmonary dysplasia and hypoxemia.
CONCLUSIONS: Pulmonary venous stenosis is a potentially treatable cause for PAH and if treated early can prevent cardiac remodeling, improve hemodynamics as well as clinical outcome. High index of suspicion is the key to early diagnosis and treatment of PAH related to pulmonary vein stenosis.
Reference #1 A case of congenital pulmonary vein stenosis in an adult. Omasa M, Hasegawa S, Bando T, Okano Y, Otani H, Nakashima Y, Wada H. Respiration. 2004 Jan-Feb; 71(1):92-4.
Reference #2 Congenital pulmonary vein stenosis beyond childhood. Kelly DJ, Brodison A, Millner RW, Goode GK. Int J Cardiol. 2008 Feb 29; 124(2):e31-3. Epub 2007 Mar 2.
DISCLOSURE: The following authors have nothing to disclose: Rajat Kapoor, Asra Khan, Jairo Barrantes, Robert Ross, Srinath Gowda, Ghulam Saydain
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