PHS and PPHTN

Q: How transthoracic echocardiography (TTE) can differentiate between hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN)?



Answer: 

• HPS is a term used when pulmonary hypertension is present with liver disease but the absence of portal HTN.
• PPHTN is a term used exclusively when portal hypertension is present with or without the hepatic disease.

TTE with contrast (bubble study) can distinguish hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN). Patients with HPS usually have a shunt which can be demonstrated via bubble study. Shunt is usually absent in PPHTN.

Clinical exam can also provide this distinction at bedside as patients with HPS will have platypnea and orthodeoxia.

#pulmonary
#hepatology
#cardiology


References:

1. Rodríguez-Roisin R, Krowka MJ, Hervé P, et al. Pulmonary-Hepatic vascular Disorders (PHD). Eur Respir J 2004; 24:861.

 2. Hadengue A, Benhayoun MK, Lebrec D, Benhamou JP. Pulmonary hypertension complicating portal hypertension: prevalence and relation to splanchnic hemodynamics. Gastroenterology 1991; 100:520.

3. Henkin S, Negrotto S, Pollak PM, Cullen MW, O'Cochlain DF, Wright RS. Platypnea-Orthodeoxia Syndrome: Diagnostic Challenge and the Importance of Heightened Clinical Suspicion. Tex Heart Inst J. 2015;42(5):498–501. Published 2015 Oct 1.

4. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 2015; 46:903.