Combined Uphill and Downhill Varices as a Consequence of Rheumatic Heart Disease: A Unique Presentation

Abstract

Hemorrhage from downhill varices is a rare manifestation. The etiology of downhill varices is due to superior vena cava obstruction while uphill varices are secondary to portal hypertension. We report a rare case of 55-year-old female with bleeding downhill varices not associated with obstruction or compression of superior vena cava, but was due to severe pulmonary artery hypertension secondary to chronic rheumatic heart disease. ( J CLIN EXP HEPA-TOL 2014;4:63-65) E sophageal varices are classified as downhill or uphill varices. Uphill varices are common, found at the lower end of the esophagus, extend upwards and develop as a consequence of portal hypertension. Downhill varices are rare, found at upper esophagus, extend downwards and usually develop due to superior vena cava obstruction. Very rarely they can develop due to pulmonary artery hypertension. We report a case presenting with upper gastrointestinal bleed from the downhill varices, which was also found to have uphill varices due to portal hypertension. The downhill varices in this patient were due to moderate pulmonary artery hypertension as a consequence of chronic rheumatic heart disease with severe mitral regurgitation, mild mitral stenosis and severe tricuspid regurgitation. CASE REPORTA 55-year-old female was admitted with repeated episodes of hematemesis and melena of 15 days duration. Patient was a diagnosed case of liver cirrhosis. General physical examination revealed pallor, raised jugular venous pressure, pedal edema and icterus. Her pulse was 82/min, regular and a blood pressure of 110/70 mmHg. Her abdomen was distended and shifting dullness was present. Cardiovascular examination revealed apical impulse in 6th intercostal space 2 cm outside mid-clavicular line, grade II parasternal heave, palpable pulmonary artery pulsations and palpable 2nd heart sound at parasternal 2nd left intercostals space, loud P2, opening snap and grade III/VI pansystolic murmur in mitral and tricuspid area. Respiratory and central nervous system examination were normal. On investigation her hemoglobin was 8 g/dL, platelet counts 0.76 lakh/mm 3 , TLC 7200/mm 3 with normal differentials, aspartate aminotransferase (AST) 40 U/L, alanine transferase (ALT) 41 U/L, alkaline phosphatase 150 mg/dL, total bilirubin 2.4 mg/dL with unconjugated fraction of 1.6 mg/dL, total serum proteins 6.2 g/dL, albumin 2.1 g/dL, prothrombin time 20 s, urea 19 mg/dL, creatinine 1 mg/dL, Na 143 meq/l and K was 3.7 meq/L. Her X-ray chest showed cardiomegaly, straightening of left heart border, findings suggestive of right atrium (RA) and left atrium (LA) enlargement, dilated pulmonary artery and superior vena cava (SVC) [ Figure 1]. Her ECG showed normal sinus rhythm with evidence of RA and LA enlargement with evidence of right ventricular hypertrophy (RVH) and left ventricular (LV) volume overload. Her serology was negative for ANA, HBsAg, anti HCV antibodies and HIV. On upper gastrointestinal endoscopy she had large varices 2 columns extending from post cricoid region downwards decreasing...

Downhill esophageal varices: a systematic review of the case reports

“…However, a systematic review of individuals with atrial fibrillation receiving dialysis found similar efficacy in preventing venous thromboembolic events with direct oral anticoagulants (DOACs) versus warfarin [51]. Some of the other predisposing factors that are found less happened included retrosternal thyroids [16,21,23,40,41], Behçet's disease [6,22,32,42] and pulmonary hypertension [7,24,25,34]. Downhill varices in retrosternal goiter develop due to a similar obstruction in the thyroid veins resulting in the blood re-routing via the deep esophageal veins [16].…”

“…The definitive diagnosis is made via neck imaging and management mainly involves thyroidectomy to relieve the obstruction and resume normal blood flow. In addition, a mechanical obstructive mechanism increased blood drainage in the esophageal veins due to malignancy as well as pulmonary hypertension leading to venous backflow have also been proposed as etiologies for downhill varices [26,34,38].…”

A Rare but Reversible Cause of Hematemesis: “Downhill” Esophageal Varices

“…Most cases of SVC obstruction are associated with some forms of malignancy including lung, lymphoma, and mediastinal metastases [ 6 , 7 ]. Nonmalignancy etiologies include pacemaker implantation [ 8 ], goiter [ 9 , 10 ], central venous catheters (dialysis catheter in particular) [ 11 – 14 ], rheumatic heart disease [ 15 ], congenital heart disease [ 16 ], thymoma [ 5 ], and mediastinal fibrosis [ 17 ]. There are however no direct association or reported cases linking hypercoagulable state as a contributing cause.…”

Downhill esophageal varices: a therapeutic dilemma

“…Venous drainage of the esophagus predominantly occurs by the azygos and hemiazygos systems. When there is an obstruction, these vessels can dilate and form varices in the esophagus (1,2). An obstruction in the SVC forces retrograde flow of blood into the right atrium through collateral channels to the IVC (1).…”

“…If the obstruction is above the level of the azygous vein, blood flow back to the heart will be confined to the upper esophagus, leading to formation of varices in the upper third of the esophagus. In contrast, if the obstruction is below or involving the azygous vein, varices will form along the entire esophagus (2,3).…”