SESSION TITLE: Cardiovascular Student/Resident Case Report Posters I
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Porto-pulmonary hypertension is most commonly seen in patients with liver cirrhosis and the occurrence of porto-pulmonary hypertension in non-cirrhotic portal hypertension patients is rare .
CASE PRESENTATION: The patient is a 57 years old Caucasian woman who presented with worsening shortness of breath for the past several months. A 2-D Echocardiogram with Doppler revealed an RVSP of 80 mmHg. Initial work up revealed normal PFT’s, negative ventilation-perfusion scan, enlarged pulmonary artery on a CT chest. Serology was positive for anti-centromere antibodies and anti-polymerase III antibodies (table 1). A percutaneous liver biopsy was performed which showed hepatoportal sclerosis of the liver without evidence of auto-immune hepatitis or cirrhosis. Upper endoscopy revealed small esophageal varices. A right heart catheterization revealed wide trans-pulmonary gradient (TPG), elevated pulmonary vascular resistance (PVR), and relatively normal left-sided filling pressures consistent with severe pulmonary hypertension (PA mean = 63 mmHg, PCWP=16 mmHg, TPG = 47, PVR= 6.6 Woods Units (529 Dynes/sec/cm), Cardiac index= 3.7 L/min/m2. A polysomnography showed sleep-related hypoxemia (SpO2 < 90% for 32 consecutive minutes) and an overall Apnea-Hypopnea index (AHI) of 13.9 per hour (supine AHI was 28.1 per hour). The diagnosis was pulmonary arterial hypertension (PAH) due to portal hypertension. OSA is likely to have contributed to pulmonary hypertension to lesser extent. The patient was started on vasodilators which included Ambrisentan 10 mg daily and sildenafil 20 mg three times daily. A follow up visit revealed significant improvement of symptoms after starting vasodilators. A repeat 2-D echocardiogram performed 5 months later revealed an RVSP of 59 mmHg. 6 minute walk test improved from 360 to 400 meters.
DISCUSSION: Our patient has both portal hypertension and systemic sclerosis, which are known etiologies for PAH. We have concluded that our patient’s PAH is secondary to portal hypertension in a non-cirrhotic patient and is not due to systemic sclerosis. This is supported by her FVC%/DLCO% ratio being 0.9 and DLCO being 81%  (table 2).
CONCLUSIONS: PAH in association with non-cirrhotic portal hypertension is a rare association reported in the literature. Portal hypertension with or without cirrhosis plays an essential role in this association but the exact mechanism is yet to be defined.
Reference #1: : Gayathri AP et al. Portopulmonary hypertension in a case of non-cirrhotic portal fibrosis. Indian J Pathol Microbiol. 2007. Oct;50(4):828-30.
Reference #2: : Fischer A, Bull TM, Steen VD. Practical approach to screening for scleroderma-associated pulmonary arterial hypertension. Arthritis Care Res (Hoboken). 2012 Mar;64(3):303-10.
DISCLOSURE: The following authors have nothing to disclose: Ghassan Kamel, Joseph Espiritu, Adrian Di Bisceglie, Guilan Chen, Reema Syed, Ravi Nayak
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