Hepatopulmonary Syndrome (HPS) — Simplified Notes for Clinical Use
What is HPS?
Hepatopulmonary Syndrome (HPS) is a complication of advanced liver disease (especially cirrhosis) where abnormal blood vessels in the lungs cause low oxygen levels (hypoxemia).
It is part of the spectrum of Cirrhotic Cardiopulmonary Complications.
Pathophysiology
Liver disease → Increased Nitric Oxide (NO) → Pulmonary Vasodilatation
Formation of Intrapulmonary Vascular Dilatations (IPVD)
Oxygen bypasses alveoli → Hypoxia
Diagnostic Criteria of HPS (Triad)
Chronic Liver Disease / Portal Hypertension
Intrapulmonary Vasodilatation
Arterial Hypoxemia (PaO₂ < 80 mmHg or A-a gradient > 15 mmHg)
Clinical Clues
| Symptom | Significance |
|---|---|
| Dyspnea (Breathlessness) | Especially worsening on sitting up (Platypnea) |
| Orthodeoxia | ↓ Oxygen saturation on sitting up (>5% drop) |
| Cyanosis | Late finding |
| Clubbing | Sometimes present |
Diagnosis
Screening:
Pulse Oximetry (SpO₂ < 96% suspicious)
Confirmation:
Contrast Echocardiography (Bubble Study) — Gold Standard
Perfusion Lung Scan
Arterial Blood Gas (ABG) — Low PaO₂
Severity Grading (Based on PaO₂)
| Severity | PaO₂ (mmHg) |
|---|---|
| Mild | > 80 |
| Moderate | 60-80 |
| Severe | 50-60 |
| Very Severe | < 50 |
Treatment
Definitive Treatment:
Liver Transplantation — Only curative option
Post-transplant oxygenation usually improves within 6-12 months.
Supportive Care:
Oxygen Therapy
Avoid aggravating factors (like anemia, infections)
Prognosis
Without transplant:
Poor survival (especially if PaO₂ < 60 mmHg)
Post-Transplant:
80-85% improve or normalize oxygenation
Severe cases may need prolonged oxygen post-transplant
Take-Home Points
"HPS should always be looked for in any cirrhosis patient complaining of breathlessness — especially if it worsens on sitting up."
Screen routinely with pulse oximetry.
Confirm with Bubble Echo.
Early referral for Liver Transplant Evaluation is critical.
Keywords
Hepatopulmonary Syndrome Treatment in India
Breathlessness in Liver Cirrhosis
Dr. Chetan Kalal HPS Specialist
When to Suspect HPS in Cirrhosis
Liver Transplant for HPS
 — Simplified Notes for Clinical Use){kind=link}