Decompensation of portopulmonary hypertension in a cirotic patient secondary to thrombosis of porto-caval communication

Introduction: The presence of pulmonary hypertensi-on in a patient with cirrhosis can have multiple etiolo-gies, Porto-pulmonary hypertension being responsible in 2-5% of cases, most being due to increased cardiac output of these patients. However the presence of severe pulmonary hypertension in a patient without a history and symptomatology should make us search for other causes of acute increase of pulmonary hypertension.

Methods: We present a case of a patient of 50 years, diagnosed 3 years ago with hepatic cirrhosis Child A of unspecified etiology, who was admitted to Gastro-enterology for investigation of a new episodes of loss of consciousness, suddenly appeared one week ago, assu-ming to be in the context of portal encephalopathy. The collected analyses showed: liver function and enzymes, within normal limits, hypoxaemia with orthodeoxia, dilation of portal vein, with splenomegaly, without oe-sophageal varices. The level of ammonia, EEG and ne-uropsych tests were normal, making the presence of an encephalopathy unlikely.

Results: Echocardiography reveals severe dilatation of right cavities with right ventricular hypertrophy, seve-re tricuspid regurgitation with a gradient of 90mmHg, PAPs estimated at 110mmHg, equaling systemic arte-rial pressure. The right ventricle function was mode-rately diminished. The left-sided cavities seem small by the systole-diastolic displacement of the interven-tricular septum to VS, with a preserved function, wi-thout significant valvulopathy, suggestive of preca-pillary pulmonary hypertension. Vascular abdominal echography reveals the presence of venous vascular packets with thrombotic material at this level. Suspi-cion of pulmonary thromboembolism was raised in a patient without a history of pulmonary hypertension and sudden onset of the symptoms. The tomographic evaluation with contrast substance reveals the presence of severe dilatation of the right cavities, without images suggestive of thrombosis up to the level of the subseg-mental pulmonary arteries, but with the presence of an important porto-caval circulation, some with diameter of 3cm, with thrombosis at this level. Under treatment with HGMM, diuretics, evolution was favorable, with a decrease in the gradient VD-AD at 50mmHg, as mea-sured by Doppler analysis and the size of right cavities. We believe that thrombosis at the level of the porto-systemic collaterals was responsible for the decompen-sation of a likely pre-existing hypertension, which was demonstrated by the improvement of symptomatology under anticoagulant treatment. The right cardiac ca-theterization was not performed. At discharge the pa-tient was directed to a centre for initiating treatment with pulmonary vasodilators and received anticoagu-lant treatment with acenocoumarol and diuretics.

Conclusions: We interpret the case as the decompen-sation of a most likely porto-pulmonary hypertension, in the context of presence of acute thrombosis of the porto-caval collaterals. There are several cases reported in literature that have shown that the thrombosis of the portocaval shunts can cause increased in portal pressu-re and a decrease in sanguine flow in the kidneys, with the subsequent increase in secretion of endothelin 1 and hydrosaline retention with further increase in pul-monary hypertension, leading to the decompensation of a pre-existing pulmonary hypertension.

ISSN
ISSN – online: 2734 – 6382
ISSN-L 1220-658X
ISSN – print: 1220-658X
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CNCSIS B+
CODE: 379
CME Credits: 10 (Romanian College of Physicians)
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