Signs and Symptoms of Chest Diseases: Student/Resident Case Report Poster - Signs and Symptoms of Chest Disease |

An Unexpected Turn: Hepatopulmonary Syndrome From Breast Cancer-Induced Liver Metastases FREE TO VIEW

Denise Sese, MD; Joseph Khabbaza, MD
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Cleveland Clinic Akron General, Akron, OH

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1257A. doi:10.1016/j.chest.2016.08.1370
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SESSION TITLE: Student/Resident Case Report Poster - Signs and Symptoms of Chest Disease

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Hepatopulmonary Syndrome (HPS) portends worse survival in patients with liver disease presenting with dyspnea. Early confirmation improves 5-year survival rates to 76%, comparable to that of liver transplant patients without HPS1. Diagnosis requires a documented defect in arterial oxygenation (elevated alveolar-arterial gradient) induced by intrapulmonary vascular dilatation 2,3. It is known to occur in different types of liver injury from acute ischemic hepatitis to cirrhosis with or without portal hypertension, and does not correlate with the severity of liver disease.

CASE PRESENTATION: A 56-year-old female diagnosed with HER2 positive breast cancer presents to the outpatient pulmonary office complaining of intermittent progressive exertional dyspnea since starting Mitomycin two months prior. Her course is complicated by brain and liver metastases. Physical exam is unremarkable. Ambulation of less than twenty yards revealed dyspnea and decreased oxygen saturations of 80%. A remote history of deep vein thrombosis prompted a chest CT angiography that was negative. Pulmonary function tests showed a mildly reduced maximal voluntary ventilation and a diffusing capacity corrected for lung volume of 85% predicted. Finally, a transthoracic contrast enhanced echocardiogram with saline (TTCE) showed a small right atrial mass, ejection fraction of 60-65%, and the delayed appearance of saline bubbles in the left atrium with a normal right ventricular systolic pressure confirming an intrapulmonary shunt.

DISCUSSION: While uncertain, the inciting event in HPS is thought to be intrapulmonary vascular dilatation from increased pulmonary nitric oxide (NO) production, increased expression of inducible NO synthase by bacterial endotoxins, or induced release of vasoactive mediators. Exertional dyspnea is the most frequent complaint while orthodeoxia is characteristic of the disorder due to an inability to adapt to gravitational blood flow changes and worsening ventilation perfusion mismatch.Intrapulmonary shunting is most practically demonstrated by TTCE. Other tests include a radionuclide lung perfusion scan and pulmonary angiography1, while other centers use direct injection of saline bubbles into the pulmonary artery with intracardiac echocardiography3.

CONCLUSIONS: The patient described presented with progressive exertional dyspnea and hypoxemia with intrapulmonary shunting confirmed by TTCE. To our knowledge, this is the first reported case of liver metastases causing HPS.

Reference #1: Swanson KL, Wiesner RH, Krowka MJ. Natural history of hepatopulmonary syndrome: impact of liver transplantation. Hepatology, 2005; 41(5):1122-1129

Reference #2: Rodriguez-Roisin, R & Krowka MJ, Hepatopulmonary Syndrome- A Liver-Induced Lung Vascular Disorder. N Engl J Med, 2008; 358 (22): 2378-2387

Reference #3: Khabbaza JE, Krasuski RA & Tonelli AR, Intrapulmonary shunt confirmed by intracardiac echocardiography in the diagnosis of hepatopulmonary syndrome. Hepatology, 2013; 58(4): 1514-1515. doi:10.1002/hep.26482

DISCLOSURE: The following authors have nothing to disclose: Denise Sese, Joseph Khabbaza

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