Síndroma hepatopulmonar: Relato de um caso clínico e revisão do tema

Resumo

A síndroma hepatopulmonar é uma complicação importante da doença hepática crónica, ocorrendo em 16–24% dos doentes. É caracterizada por aumento do gradiente alveolocapilar com ou sem hipoxemia resultante de vasodilatação intrapulmonar. O curso da doença é progressivo e associado a aumento da morbilidade e mortalidade. Não existe tratamento médico eficaz, sendo o transplante hepático a opção terapêutica prioritária. Como exemplo desta patologia, os autores apresentam o caso clínico de um doente, sexo masculino, 39 anos, enviado à consulta de Pneumologia com informatração de policitemia secundária e dispneia progressiva para esforços de média intensidade e cuja investigação permitiu identificar uma síndroma hepatopulmonar.

Palavras-chave: Síndroma hepatopulmonar. hipoxemia. hepatopatia. transplante hepático.

Bibliografia

1.Kennedy TC, Knudson RJ. Exercise-aggravated hypoxemia and orthodeoxia in cirrhosis. Chest. 1997; 72:305-9.
Medline
2.Krowka MJ, Cortese DA. Hepatopulmonary syndrome: an evolving perspective in the era of liver transplantation (editorial). Hepatology. 1990; 11:138-42.
Medline
3.Agusti AGN, Roca J, Bosch J, Rodriguez-Roisin R. The lung in patients with cirrhosis. J Hepatol. 1990; 10:251-7.
Medline
4.Binay K, Sen S, Biswas PK, Sanyal R, Jundar DM, Biswas J. Hepatopulmonary syndrome in inferior vena cava obstruction responding to cavoplasty. Gastroenterol. 2000; 118:192-6.
5.Gupta D, Vijaya DR, Grupta R, Dhiman RK, Bhargava M, Verma J. Prevalence of hepatopulmonary syndrome in cirrhosis and extrahepatic portal venous obstruction. Am J Gastroenterol. 2001; 96:3395-9.
Medline
6.Regev A, Yeshurun M, Rodriguez M, Sagie A, Neff G, Molina E. Transient hepatopulmonary syndrome in a patientes with acute hepatitis A. J Viral Hep. 2001; 8:83-6.
7.Rolla G, Brussino L, Colagrande P, et-al. Ehhaled nitric oxide and oxigenation abnormalities in hepatic cirrhosis. Hepatol. 1997; 26:842-7.
8.Rolla G, Brussino L, Colagrande P. Exhaled nitric oxide and impaired oxigenation in cirrhotic patients before and after liver transplantation. Ann Inter Med. 1998; 129:375-8.
9.Cremona G, Higenbottam TW, Mayoral V, et-al. Elevated exhaled nitric oxide in patients with hepatopulmonary syndrome. Eur Respir J. 1995; 8:1883-5.
Medline
10.Teuber G, Teupe C, Dietrich C, Caspary W, Buhl LR, Zeuzem S. Pulmonary disfunction in non-cirrhotic patients with chronic viral hepatitis. Eur J Intern Med. 2002; 13:311-8.
Medline
11.Fallon M, Abrams G. Pulmonary disfunction in chronic liver disease. Hapatol. 2000; 32:859-65.
12.Rodrigues-Roisin R, Roca J, Augusti AG, et-al. Gas exchange and pulmonary vacsular reactivity in patients with liver cirrhosis. Am Rev Respir Dis. 1987; 135:1085.
Medline
13.Krowka MJ, Dickson ER, Cortese DA. Hepatopulmonary syndrome: clinical observations and lack of therapeutic response to somatostatin analogue. Chest. 1993; 104:515-21.
Medline
14.Lima BL, Franca AV, Pazin-Filho A, et-al. Frequency, clinical characteristics and respiratpry parameters of hepatopulmonary syndrome. Mayo Clin Proc. 2004. 79-42
15.Rodriguez-Roisin R, Krowka MJ, Herve P, Fallon MB. On behalf of the ERS task force pulmonary-hepatic vascular disorders scientific comittee ERS task force PHD scientific comittee Pulmonary-hepatic vascular disorders (PHD). Eur Respir J. 2004; 24:861-80.
Medline
16.Abrams GA, Jaffe CC, Hoffer PB, Binder HJ, Fallon MB. Diagnostic utility of contrast echocardiography and lung perfusion scan in patients with hepatopulmonary syndrome. Gastroenterol. 1995; 109:1283-8.
17.Palma DT, Fallon MB. The hepatopulmonary syndrome. J Hepatol. 2006; 45:617-25.
Medline
18.McAdams HP, Erasmus J, Crockett R, Mitchell J, Godwin JD, McDermott VG. The hepatopulmonary syndrome: radiologic findings in 10 patients. AJR Am J Roentgenol. 1996; 166:1379-85.
Medline
19.Schenk P, Schoniger-Hekele M, Fuhrmann V, Madl C, Silberhumer G, Muller C. Prognostic significance of the hepatopulmonary syndrome in patients with cirrhosis. Gastroenterol. 2003; 125:1042-52.
20.Krowka M, Wiseman G, Burnett O, Spivey J, Therneau T, Porayko M, Wiesner R. Hepatopulmonary syndrome: a prospective study relationships between severity of liver disease, paO2 response to 100% oxygen and brain uptake after 99mTc MAA lung scanning. Chest. 2000; 118:615-24.
Medline
21.Arguedas M, Abrams GA, Krowka MJ, Fallon MB. Prospective evaluation of outcomes and predictors of mortality in patients with hepatopulmonary syndrome undergoing liver transplantation. Hepatol. 2003; 37:192-7.
22.Freire R, Mangualde J, Vieira AM, Lobato C, Alves LA, Cremers MI, Augusto F, Caetano F, Oliveira AP. Síndrome hepatopulmonar em doentes com cirrose hepática: importância da sua pesquisa sistémica e impacto no prognóstico. J Port Gastroenterol. 2007; 14:176-83.
23. http://www.unos.org/
24.Swanson KL, Wiesner RH, Krowka MJ. Natural history of hepatopulmonary syndrome: impact of liver transplantation. Hepatol. 2005; 41:1122-9.
25.Krowka MJ, Porayko MK, Plevak DJ, Pappas SC, Steers JL, Krom RAF, et-al. Hepatopulmonary syndrome with progressive hypoxemia as an indication for liver transplantation: case reports and literature review. Mayo Clin Proc. 1997; 72:44-53.
Medline
26.Lange PA, Stoller JK. The hepatopulmonary syndrome: effect of liver transplantation. Clin Chest Med. 1996; 17:115-23.
Medline
27.Philit F, Wiesendanger T, Gille D, et-al. Late resolution of hepatopulmonary syndrome after liver transplantation. Respiration. 1997; 6:173-5. (abstract)
Michele De Santis a, , Luis Vaz Rodrigues a, Fernando Matos b, Fernando Barata c,

a Interno de Pneumologia Centro Hospitalar de Coimbra, EPE
b Assistente Graduado em Pneumologia Centro Hospitalar de Coimbra, EPE
c Serviço de Pneumologia, Centro Hospitalar de Coimbra, Quinta dos Vales São Martinho do Bispo, 3046-853 Coimbra