CC: 4 months of worsening dyspnea on exertion

ID: 39 yo female with Child’s A alcoholic cirrhosis (MELD 15) complicated by esophageal varices, duodenal varices, ascites, s/p TIPS procedure 3 years ago presents with 4 months of worsening shortness of breath, dyspnea on exertion, and palpitations. Exam was notable for oxygen saturation of 98% on room air, tachycardia, clear lungs bilaterally, digit clubbing, and absence of a fluid wave. Further studies, including TTE and R heart catheterization, revealed she had portopulmonary hypertension. We propose that she likely had hepatopulmonary syndrome as well, which may have improved with her TIPS, and subsequently developed portopulmonary hypertension.

table in PDF format

Pearls from morning report:

• Hepatopulmonary Syndrome (HPS) will cause digital clubbing; portopulmonary HTN (POPH) generally will not
• RVSP < 30 mmHg on TTE essentially rules out POPH

Random trivia:

The first case of hepatopulmonary syndrome was described by Dr. Fluckiger in 1884 after observing a woman with cirrhosis, cyanosis, and clubbed digits.

Want to read more?

Pulmonary Complications in Chronic Liver Disease (Hepatology)
Hepatopulmonary Syndrome – A Liver-Induced Lung Vascular Disorder (NEJM)

References: 

Porres-Aguilar M, Altamirano JT, Torre-Delgadillo A, et al. Portopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview. Eur Respir Rev. 2012;21:125,223-233.