The clinical characteristics of the patients with the diagnosis of hepatopulmonary syndrome

Öz

Background/aim: Hepatopulmonary syndrome (HPS) is a clinical entity characterized by the triad of liver disease, increased alveolar arterial oxygen gradient (P(A-a) O2) and intrapulmonary vascular dilatation. In this study we aimed to determine the incidence and the specific clinical characteristics of HPS in our patients with chronic liver disease. Materials and methods: Seventy chronic liver disease patients without any concomitant cardiopulmonary disease were enrolled into the study. Arterial blood gas analysis was performed after a period of exercise. Patients with a high P(A-a) O2 underwent contrast echocardiography. Child score and physical examination findings were evaluated in all patients. Results: Of 70 patients, 12 were diagnosed as HPS. Clubbing and spider nevi were significantly higher in HPS patients than in non-HPS patients. While the diagnostic sensitivities of all the symptoms and physical findings were low, the specificities of platypnea, clubbing and spider nevi were high. There was no significant difference between the Child score of HPS (9.9±1.9) and non-HPS (8.5±2.3) patients. Although the incidence of HPS was observed to be increased from Child A to C (Child A: 9.1%, Child B: 12.5%, Child C: 25.9%), the difference was not statistically significant. Conclusion: HPS is a common clinical entity in patients with chronic liver disease. While the diagnostic sensitivities of all the symptoms and physical findings were low, the specificities of clubbing, spider nevi, and platypnea were found to be high.

Anahtar Kelimeler

• Hepatopulmonary syndrome, liver, clinic

Hepatopulmoner sendrom tanısı konulan olguların klinik özellikleri

Öz

Giriş ve amaç: Hepatopulmoner sendrom (HPS), karaciğer hastalığı, artmış alveolo-arteriyel oksijen gradiyenti (P(A-a) O2) ve intrapulmoner vasküler dilatasyon triadı ile karakterize bir klinik tablodur. Bu çalışmada kliniğimizde kronik karaciğer hastalığı tanısı ile takip edilen hastalarda HPS insidansının ve HPS'ye özgü klinik özelliklerinin ortaya konması amaçlanmıştır. Gereç ve yöntem: Çalışmaya ek kardiyopulmoner patolojisi olmayan 70 kronik karaciğer hastası alındı. Her hastada egzersiz sonrası dönemde arteriyel kan gazı incelemesi yapıldı. P(A-a) O2'si yüksek olanlara kontrast ekokardiyografi yapıldı. Tüm olguların Child skoru ve fizik muayene bulguları değerlendirildi. Bulgular: Çalışmaya alınan 70 hastanın 12'sine (%17) HPS tanısı kondu. HPS'li hastalarda parmaklarda çomaklaşma ve spider nevi HPS'si olmayanlara göre anlamlı düzeyde daha fazla görülmekteydi. Tüm semptom ve bulguların tanısal duyarlılıkları düşükken platipne, parmaklarda çomaklaşma ve spider nevi'nin tanısal özgüllüğü yüksekti. HPS'si olan (9,9±1,9) ve olmayan hastaları n (8,5±2,3) Child skorları arasında anlamlı fark görülmedi. Child grubu A'dan C'ye doğru ilerledikçe HPS insidansının arttığı gözlense de (Child A:%9,1, Child B:%12,5 ve Child C: %25,9) aradaki fark istatistiksel olarak anlamlı bulunmadı. Sonuç: HPS kronik karaciğer hastalarında sık görülen bir klinik tablodur. Tüm semptom ve bulguların tanıdaki duyarlılığı düşük olmakla beraber spider nevi, parmaklarda çomaklaşma ve platipnenin tanısal özgüllüğü yüksek bulunmuştur.

Anahtar Kelimeler

• Hepatopulmoner sendrom, karaciğer, klinik

Kaynakça

1. Krowka MJ. Hepatopulmonary syndrome and portopulmonary hypertension: distinctions and dilemnas. Hepatology 1997; 25: 1282-4.
2. Castro M, Krowka MJ. Hepatopulmonary syndrome: A pulmonary vascular complication of liver disease. Clin Chest Med 1996; 17: 35-48.
3. Hopkins WE, Waggoner AD, Brazilai B. Frequency and significan- ce of intrapulmonary right-to-left shunting in end-stage hepatic di- sease. Am J Cardiol 1992; 70: 516-19.
4. Hourani JM, Bellamy PE, Tashkin DP, et al. Pulmonary dysfuncti- on in advanced liver disease: Frequent occurence of an abnormal diffusing capacity. Am J Med 1991; 90: 693-700.
5. Chiesa A, Ciappi G, Baldi L, et al. Role of various causes of arteri- al desaturation in liver cirrhosis. Clin Sci 1969; 37: 803-14.
6. Cremona G, Higenbottam TW, Mayoral V, et al. Elevated exhaled nitric oxide in patients with hepatopulmonary syndrome. Eur Respir J 1995; 8: 1883-5.
7. Rolla G, Brussino L, Colagrande P, et al. Exhaled nitric oxide and impaired oxygenation abnormalities in hepatic cirrhosis. Hepato- logy 1997; 26: 842-7.
8. Luo B, Abrams G, Fallon M. Endothelin-1 in the bile duct ligation model of hepatopulmonary syndrome: correlation with pulmonary dysfunction. J Hepatol 1998; 28: 571-8.

9. Zhang M, Luo B, Chen G, et al. Endothelin-1 stimulation of endot- helial nitric oxide synthase in the pathogenesis of hepatopulmonary syndrome. Am J Physiol 1999; 277: 944-52.
10. King PD, Rumbaut R, Sanchez C. Pulmonary manifestations of chronic liver disease. Dig Dis1996; 14: 73-82.
11. Panos RJ, Baker SK. Mediators, cytokines, and growth factors in li- ver-lung interactions. Clin Chest Med 1996; 17: 151-69.
12. Ebeid AM, Escourrou J, Soeters PB, et al. Hepatic inactivation of vasoactive intestinal peptide in man and dog. Ann Surg 1978; 188: 28-33.
13. Hortnagl H, Singer EA, Lenz K, et al. Substance P is markedly inc- reased in plasma of patients with hepatic coma. Lancet 1984; 1: 480-83.
14. Caramelo C, Fernandez-Gallardo S, Santos JC, et al. Increased le- vels of platelet-activating factor in blood from patients with cirrho- sis of the liver. Eur J Clin Invest 1987; 17: 7-11.
15. Krowka MJ, Dickson ER, Cortese DA. Hepatopulmonary syndrome: clinical observations and lack of therapeutic response to somatosta- tin analogue. Chest 1993; 104: 515-21.
16. Sood G, Fallon MB, Niwas S, et al. Utility of a dyspnea-fatigue in- dex for screening liver transplant candidates for hepatopulmonary syndrome (Abstract). Hepatology 1998; 28 (Suppl): 742A.
17. Robin ED, Horn B, Goris ML, et al. Detection, quantitation, and pathophysiology of lung spiders. Trans Assoc Am Physicians 1975; 88: 202-16.
18. Andrivet P, Cadranel J, Housset B, et al. Mechanisms of impaired arterial oxygenation in patients with liver cirrhosis and severe res- piratory insufficiency. Effects of indomethacine. Chest 1993; 103: 500-7.
19. Heinemann HO, Emirgil C, Mijnssen JP. Hyperventilation and ar- terial hypoxemia in cirrhosis of the liver. AM J Med 1960; 28: 239- 46.
20. Krowka MJ, Cortese DA. Hepatopulmonary syndrome. Current concepts in diagnostic and therapeutic considerations. Chest 1994; 105: 1528-37.
21. The North Italian Endoscopic Club for the Study and Treatment of Esophageal Varices: Prediction of the first variceal hemorrhage in patients with cirrhosis of the liver and esophageal varices: A pros- pective multicenter study. N Engl J Med 1998; 319: 983.
22. Kaymakoğlu S, Kahraman T, Kudat H, et al. Hepatopulmonary syndrome in noncirrhotic portal hypertensive patients. Dig Dis Sci 2003; 48: 556-60.
23. Rodriguez-Roisin R, Roca J, Agusti AG, et al. Gas exchange and pul- monary vascular reactivity in patients with liver cirrhosis. Am Rev Respir Dis 1987; 135: 1085-92.