Pulmonary Vascular Disease |

Unusual Case of Chronic Thromboembolic Pulmonary Hypertension FREE TO VIEW

Syed Tousheed, MD; Tiyas Sen, MD; Binoy Chatturparambil, MBChB; Muralimohan Bv, MD; Hemanth Kumar, DTCD
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Intervention Pulmonology, Narayana Hrudayalaya, Bangalore, India

Chest. 2014;146(4_MeetingAbstracts):876A. doi:10.1378/chest.1958985
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SESSION TITLE: Pulmonary Vascular Disease Global Case Reports

SESSION TYPE: Global Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Right ventricular myxomas are one of the rare causes of pulmonary embolism which in turn can progress to chronic thrombo-embolic pulmonary artery hypertension( CTEPH). Cardiac Echinococcosis remains one of the major differentials for cardiac myxomas in an endemic area.We hereby report a case of right ventricular mass that presented to us with CTEPH and multiple lung nodules, which later turned out to be echinococcosis multilocularis.

CASE PRESENTATION: A 36 year old male presented with dyspnea, recurrent episodes of syncope, bilateral lower limb swelling and generalized anasarca in the last 1 year. The patient was operated for a liver hydatid in the past. He was hypoxic and was de-saturating on exercise. His chest radiograph revealed hilar prominence with multiple lung nodules. 2D-Echocardiography confirmed severe pulmonary artery hypertension and mass in the right ventricle. The computed tomography with pulmonary angiogram showed multiple nodules in the lung parenchyma, dilated main pulmonary artery,vessel wall thickening,webs formation in both right and left pulmonary arteries.There was a mass in the right ventricle with reduced tracer uptake on PET-CT. Patient was taken up for surgery for debulking of the right ventricular mass.Intra-operatively,he developed a cardiac arrest and he could not be revived. The histopathology of the right ventricular mass revealed Echinococcus multilocularis with myxomatous reaction.

DISCUSSION: Primary cardiac hyadtid cyst though rare,is a well known entity. The continous contraction of the heart makes the environment less suitable for the viability of hydatid cyst in the heart (1). Hence the frequency ranges only between 0-5% and 2% according to various authors (2). Echinococcus unilocularis is much more common when compared to multilocularis(3).Liver and lung are more frequently affected by E. multilocularis, but so far heart involvement is not reported. E.multilocularis causes destruction of surrounding tissue by aggressive invasion and hence may mimic malignancies(3). Echinococcosis commonly affects the left ventricular myocardium. Echinococcus on the right ventricular myocardium can rupture in to the cavity and may precipitate an acute pulmonary thromboembolism. Some times the daughter cysts migration through the right ventricle in to the pulmonary arteries and can lead to chronic pulmonary thromboembolism(3). Considering the possibilities of malignant myxoma versus hydatid and the potential grave consequences of both the conditions, the patient was subjected for surgery but the patient developed a cardiac arrest and expired on table. The autopsy revealed E multilocularis with a myxomatous reactions.

CONCLUSIONS: High index of suscipicion for cardiac echinococcosis must be raised in all patients who come from endemic areas,as a differential diagnosis for cardiac myxomas, specifically when the ventricular myocardium is involved.

Reference #1: . Ulgen SM, Yazici M, Kayrak M, et al. Three-year follow up of recurrent cardiac echinococcosis simulating myxoma: report of a rare case. Anadolu Kardiyol Derg 2007; 7: 436-48.

Reference #2: Calamai G, Perna A.M.,Venturini A. Hydatid disease of the heart Report of five cases and review of the literature. Thorax 1974; 29: 451-457

Reference #3: Abid L, Abdennadher M, Msaad S,et al. A cardiac hydatid cyst underlying pulmonary embolism: a case report ; Pan African Medical Journal - ISSN: 1937- 8688

DISCLOSURE: The following authors have nothing to disclose: Syed Tousheed, Tiyas Sen, Binoy Chatturparambil, Muralimohan Bv, Hemanth Kumar

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