Decompensated porto-pulmonary hypertension in a cirrhotic patient with thrombosis of portocaval shunt.
We report a case of decompensated porto-pulmonary hypertension closely associated with the development of intra-portocaval shunt thrombosis. A woman with Laennec's cirrhosis was hospitalized because of severe dyspnea and edema. She underwent surgical portocaval anastomosis ten years ago. Imaging studies showed massive intra-shunt thrombosis, portal hypertension, ascites, pleuro-pericardial effusions and enlargement of right cardiac cavities. Cardiac catheterization allowed to rule out coronary and left-sided heart abnormalities and led to the diagnosis of pre-capillary pulmonary hypertension. Antithrombotic treatment with low molecular weight heparin was instituted. The management also included ACE inhibitors, spironolactone, low-salt diet and lactulose. The patient was discharged and three months later we observed the disappearance of edema, ascites and pleuro-pericardial effusions, a marked body weight reduction and improved dyspnea and liver function tests. A possible link between the development of intra-shunt thrombosis and clinical decompensation in our patient was hypothesized. In fact, it has been demonstrated that the increased portal pressure, caused by occlusion of portosystemic shunt, reduces renal plasma flow and increases systemic endothelin-1 concentration. In our patient the disappearance of edematous state and improved dyspnea observed after recanalization of the shunt strongly support this hypothesis.
Giannarelli
Chiara
C
Department of Internal Medicine, University of Pisa, via Roma 67, Pisa 56126, Italy. c.giannarelli@virgilio.it
De Giorgi
Antonio
A
De Negri
Ferdinando
F
Carmassi
Franco
F
D000368:Aged; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use; D004039:Diet, Sodium-Restricted; D004232:Diuretics / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004417:Dyspnea / Q000209:etiology; D004487:Edema / Q000209:etiology; D005260:Female; D005343:Fibrinolytic Agents / Q000627:therapeutic use; D005765:Gastrointestinal Agents / Q000627:therapeutic use; D006495:Heparin, Low-Molecular-Weight / Q000627:therapeutic use; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D007792:Lactulose / Q000627:therapeutic use; D008103:Liver Cirrhosis / Q000601:surgery*; D017082:Portal Pressure; D011170:Portasystemic Shunt, Surgical / Q000009:adverse effects*; D013148:Spironolactone / Q000627:therapeutic use; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000627:therapeutic use; D020246:Venous Thrombosis / Q000150:complications* / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology
D000806:Angiotensin-Converting Enzyme Inhibitors; D000959:Antihypertensive Agents; D004232:Diuretics; D005343:Fibrinolytic Agents; D005765:Gastrointestinal Agents; D006495:Heparin, Low-Molecular-Weight; D014665:Vasodilator Agents; D013148:Spironolactone; D007792:Lactulose