2017 Resarch Forum

IM: C-11

Applicant & Principal Investigator: Samiollah Gholam MD

A rare case of downhill esophageal varices in the absence of superior vena cava obstruction Samiollah Gholam MD 1 ,Sehem Ghazala MD 2 , Bupesh Pokhra MD 3 , Archita Desai MD 3 1 Department of Medicine, Kern Medical 2 Department of Medicine, Banner University Medical Center – Tucson 3 Division of Gastroenterology, Department of Medicine, Banner University of Arizona – Tucson INTRODUCTION: Esophageal varices are dilated veins of the sub-epithelial connective tissue. They are characterized into two major classes: uphill - increased portal venous pressure leads to “upward” venous flow via dilated esophageal collaterals towards the superior vena cava (SVC). Downhill varices are primarily due to superior vena cava obstruction leading to “downward” venous flow via esophageal collaterals towards the portal vein and inferior vena cava. Downhill varices without superior vena cava (SVC) obstruction are rare. PURPOSE: To present the first published case of an 87 year old female patient with downhill esophageal varices secondary to severe aortic stenosis and pulmonary hypertension, with no intra-thoracic mass or evidence of SVC obstruction. DISCUSSION: Concurrent hypothyroidism, hypertension and cognitive impairment; Physical examination unremarkable (no pedal edema, CVP, nor ascites). Laboratory findings: hemoglobin of 5.6 g/dL, Platelet 386 (1000/uL), ALT 109 IU/L, AST of 60 IU/L, Sodium 140 mMol/L, INR 1.1, BNP >3000 and Hepatitis negative. Social history negative (EtOH, risk factors - liver disease). CXR: pulmonary edema/bilateral pleural effusion. 2-D echocardiogram showed severe valvular aortic stenosis and RVSP 60+ mmHg. MRI of the chest/abdomen/pelvis - discrete mid-esophageal varices without advanced chronic liver disease or portal hypertension, significant for cardiomegaly, bilateral pleural effusion. No prior EDG or colonoscopy. Upper endoscopy (two columns of grade II-III varices were found in the upper third of the esophagus without stigmata of bleeding) and findings (large hiatal hernia/multiple superficial erosions in the hernia sac suggestive of Cameron ulcers; diffuse severe inflammation/linear erosions, shallow ulcerations) found in the entire examined stomach. Biopsy sent for Helicobacter Pylori testing. Colonoscopy revealed diverticulosis in the sigmoid colon, internal hemorrhoids, no evidence of bleed. Anemia was attributed to hiatal hernia and gastric ulcers. She was placed on double dose PPI for 3 months, discharged in stable condition. A multidisciplinary meeting recommended outpatient follow-up & TAVR (cardiology). This case demonstrated the presence of “downhill” varices in the setting of pulmonary hypertension caused by aortic stenosis without of SVC obstruction and liver cirrhosis. The mild elevation (AST, ALT) are attributed to liver congestion secondary to pulmonary hypertension. CONCLUSION: Downhill varices are fairly common in the presence of SVC obstruction, reported in as many as 30% of patients. However, bleeding from downhill esophageal varices is rare and accounts for approximately 0.4 -10% of variceal bleeds. The treatment for downhill varices includes treating the underlying cause, e.g. aortic stenosis. Although there have been few case reports of pulmonary hypertension and downhill varices, to our knowledge, this is the first case of downhill esophageal varices in aortic stenosis induced pulmonary hypertension in the absence of liver disease. CT or MRI of the chest may be indicated in anemic patients with pulmonary hypertension for concern of downhill esophageal varices.