Thank you Dr. Rosenstock for an excellent case presentation of a middle-aged man with HCV Cirrhosis who presented with acute onset cough and shortness of breath for three days, which was attributed to development of hepatopulmonary syndrome.
Key learning points:
- The pathophysiology is not entirely understood, but is thought to be due to increased production or decreased clearance of nitric oxide (NO) by the liver, leading to dilation of vessels and overperfusion relative to ventilation (V/Q mismatch), contributing to hypoxemia
- Platypnea (increased dyspnea when moving from recumbent to upright) and Orthodexia (decrease in arterial O2 sat when moving from recumbent to upright) are commonly seen
- Diagnosis includes shunt assessment via TTE with bubble study, transesophageal echo, and macroaggregated albumin scanning
- Definitive treatment is liver transplant. Medications including somatostatin analogs, NO synthase inhibitors and garlic (thought to decrease NO synthesis)