Pulmonary Vascular Disease |

Idiopathic Pulmonary Arterial Hypertension (IPAH) Mimicking Mitral Stenosis by Causing Pulmonary Vein Compression FREE TO VIEW

Sara Hegab, MD; Michael Mendez, MD; Hector Cajigas, MD; Rana Awdish, MD
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Henry Ford Health System, Detroit, MI

Chest. 2015;148(4_MeetingAbstracts):968A. doi:10.1378/chest.2270861
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SESSION TITLE: Pulmonary Vascular Disease Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Dilatation of right heart chambers can occur in severe pulmonary hypertension (PH), but it is unusual to cause compression of surrounding vasculature, particularly of the pulmonary veins (PV). Our patient had right superior pulmonary vein (RSPV) compression due to massive right atrial (RA) enlargement resulting from IPAH.

CASE PRESENTATION: A 40 year old female with a history of hypertension and sarcoidosis presented with syncope superimposed on subacute exertional dyspnea, dizziness, lightheadedness and presyncope. CTPA ruled out acute pulmonary embolism but showed severe dilatation of main pulmonary artery (PA), left and right PA with extrinsic compression of RSPV by the enlarged RA. There was mediastinal lymphadenopathy with no parenchymal lung disease. 2D echocardiogram showed normal left ventricular (LV) ejection fraction with small LV cavity size. Right ventricle (RV) was moderately enlarged with reduced function and severely dilated RA. Estimated pulmonary arterial systolic pressure was 129 mmHg. VQ lung scan ruled out chronic thromboembolism. ANA, RF, liver profile and serum chemistries were normal. Subsequent right heart catheterization showed (in mmHg) a mean RA pressure 8, RV 148/8, mean pulmonary artery pressure 86, mean pulmonary capillary wedge pressure 24, LVEDP 2, Fick cardiac output and cardiac index of 3.09 L/min and 1.8 L/min/m2 respectively and PVR of 20.06 WU. Cardiac MRI did not reveal mitral stenosis (MS) or MS mimetic lesions, such as left atrial anomalies, to explain the PCWP-LVEDP gradient. CT of PV showed external compression of the RSPV by the enlarged RA, explaining the elevated PCWP-LVEDP gradient. Diagnosis of IPAH was made and sildenafil was started, with plans to sequentially initiate IV prostacyclin therapy.

DISCUSSION: As severity of PH increases, RV hypertrophy and progressive contractile dysfunction occurs, followed by chamber dilatation leading to malcoaptation of tricuspid valve leaflets resulting in RA dilatation. The degree of atrial dilatation does not usually cause mass effect on the PVs.

CONCLUSIONS: PV compression from RA enlargement is a mimicker of MS and should be considered in the setting of PCWP-LVEDP gradient. To our knowledge, this is the first reported case of RA enlargement resulting from IPAH leading to compression of PVs.

Reference #1: J. Kreutzer, J.F. Keane, J.E. Lock, E.P. Walsh, R.A. Jonas, A.R. Castaneda, J.E. Mayer Jr Conversion of modified Fontan procedure to lateral atrial tunnel cavopulmonary anastomosis JThorac Cardiovasc Surg,111(1996),pp. 1169-1176

DISCLOSURE: The following authors have nothing to disclose: Sara Hegab, Michael Mendez, Hector Cajigas, Rana Awdish

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