WORLD JOURNAL OF ADVANCE
HEALTHCARE RESEARCH

( An ISO 9001:2015 Certified International Journal )

An International Peer Review Journal for Medical Science and Pharma Professionals
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Indexing

Abstract

HEPATOPULMONARY SYNDROME ASSOCIATED WITH PORTOPULMONARY HYPERTENSION: CASE REPORT

Gabriel S. Thiago Cavalleiro*, Pedro Santos Moraes, Daniel Augusto Goulart Filho, Marina Bispo Santiago Lima, Liliana Semionato da Silva Lima, Jessica de Andrade Freitas, Rayssa Kethlyn Alves de Campos, Livia Cafundó Almeida, Ada Al

ABSTRACT

Case Presentation: Man, 48 years old, with cirrhosis due to non-alcoholic steatohepatitis (NASH) with portalhypertension, already decompensated with ascites, hepatic encephalopathy, esophageal varices, CHILD-PUGHC11 and MELD -Na 25, referred for pre - evaluation liver transplant. During the evaluation, an increase in thealveolar-arterial gradient (GA-aO2) was evidenced by blood gas analysis, continuing investigation withtransthoracic echocardiogram (ECOTT), which showed passage of microbubbles after the sixth cardiac cycle,compatible with intra-pulmonary shunt, configuring hepatopulmonary syndrome (HPS), in this moderate caseseen PaO2 of 69 mmHg. Furthermore, ECOTT revealed signs suggestive of pulmonary hypertension, withpulmonary artery systolic pressure (PASP) being 64mmHg. A hemodynamic study was indicated, through rightheart catheterization, which confirmed moderate to severe pulmonary arterial hypertension with a mixedcomponent (pre and post capillary), presenting the following parameters in the evaluation of the pulmonary arterytrunk: Systolic pressure 59mmHg, diastolic pressure 33mmHg and mean pressure of 44mmHg. Therapy forpulmonary hypertension was initiated with sildenafil in association with ambrisentan, in addition to furosemideand spironolactone due to the hypervolemic component. The patient continues to be monitored by specialties toassess the response to therapy. Discussion: HPS is a complication resulting from portal hypertension and is themain cause of respiratory failure in cirrhotic patients. It is a priority on the liver transplant list and is the treatmentof choice. Prevalence reaches 32% in cirrhotic patients, being the result of microvascular changes, hinderingpulmonary gas exchange due to diffuse dilations at the pre -capillary level, or capillaries, or arteriovenouscommunications presenting changes in the ventilation-perfusion relationship and a decrease in gas exchange time.The diagnosis is: change in GA-aO2 or hypoxemia with evidence of intrapulmonary shunt, in a patient withchronic liver disease, and its severity is graded based on PaO2. Pulmonary hypertension can be a prohibitivecondition for liver transplantation, depending on its severity (patients with mPAP >50mmHg), and even afterresponding to therapy, it presents a high mortality rate. This patient presented a mixed component of pulmonaryhypertension, which was explained by portopulmonary hypertension (type I) and hypervolemic status (type II).Therapy was initiated for better clinical control of the disease and subsequent reevaluation for livertransplantation. Final considerations: HPS is a condition that should be investigated in all cirrhotic patients dueto its prevalence and severity. The association with pulmonary hypertension is rare, directly interfering in theplanning of liver transplantation and the survival of these patients.

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