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Cardiovascular Disease |

A Rare Case of Carcinoid-Associated Pulmonary Hypertension in Absence of Valvular Involvement

Shikha Gupta, MD; Karen Wolf, MD; Tim Lahm, MD
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Indiana University School of Medicine, Indianapolis, IN


Chest. 2013;144(4_MeetingAbstracts):119A. doi:10.1378/chest.1689160
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Abstract

SESSION TITLE: Cardiovascular Case Report Posters I

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary hypertension (PH) in the setting of carcinoid syndrome (Ca-PH) is usually due to fibrous lesions on the endocardium of valves. PH in the absence of valvular heart disease (VHD) has not been reported. We report a rare case of Ca-PH without valvular involvement.

CASE PRESENTATION: A 60 year-old Caucasian female with metastatic carcinoid tumor, on octreotide, diagnosed 4 years ago, presented with dyspnea. She first noticed dyspnea when visiting Colorado, but continued to experience it with less than daily activities (WHO functional class III) upon return. She denied chest pain, syncope or palpitations. Her review of systems was negative, except for chronic diarrhea. Additional history was significant for hypertension and chronic kidney disease. On examination, she required 3 liters oxygen/minute via nasal canula, had loud P2 and pedal edema. Transthoracic echocardiogram revealed left ventricular ejection fraction of 65%, flattened septum and reduced right venticular systolic function. No valvular lesions were reported (Figure 1). Diastolic dysfunction was excluded. Hemodynamics from right heart catheterization are shown in the table 1. Laboratory work-up for other causes of PH, including connective tissue disease and HIV serology was unremarkable. High-resolution chest tomography, ventilation-perfusion scan, polysomnogram, and spirometry were non-diagnostic. She was diagnosed with Ca-PH, and started on amlodipine 10mg and sildenafil 20mg three times a day. Over the next few months, she had improvement in symptoms, WHO functional class and six-minute-walk distance.

DISCUSSION: Heart involvement is seen in ~50% patients with carcinoid syndrome (1). The pathognomonic feature is the presence of plaque-like fibrous tissue on the endocardium of the heart valves, cardiac chambers and occasionally pulmonary artery and aorta. Excessive serotonin levels are believed to be the main culprit behind these lesions. Recent studies have emphasized the role of serotonin in the pathogenesis of PH by causing vasoconstriction, smooth muscle hyperplasia and pulmonary vascular remodeling (2). It is therefore plausible that high serotonin levels from carcinoid syndrome caused PH in our patient by directly affecting the pulmonary vasculature. To our knowledge, this is the first reported case of Ca-PH without VHD. The positive vasodilator response in our patient suggests a strong vasoconstrictive component.

CONCLUSIONS: VHD and WHO group 2 PH are not uncommon in patients with carcinoid syndrome. However, PH can also develop in the absence of VHD, likely mediated by a direct effect of circulating serotonin on the pulmonary vasculature. PH should be suspected in dyspneic carcinoid patients even in the absence of VHD.

Reference #1: Bhattacharyya, et al. Circulation 2007;116(24):2860-5

Reference #2: Marcos, et al. Circulation 2004;94:1263-70

DISCLOSURE: The following authors have nothing to disclose: Shikha Gupta, Karen Wolf, Tim Lahm

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