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(Chest. 1999;115:1748-1749.)
© 1999 American College of Chest Physicians

Improvement of Pulmonary Hypertension After Liver Transplantation*

Roland Schott , MD; Ari Chaouat , MD; Anne Launoy , MD; Thierry Pottecher , MD and Emmanuel Weitzenblum , MD, FCCP

* From the Departments of Respiratory Medicine (Drs. Schott, Chaouat, and Weitzenblum) and Anesthesiology and Surgical Intensive Care (Drs. Launoy and Pottecher), University Hospital, Strasbourg, France.


   Abstract
TOP
 Abstract
Introduction
 Case Report
 Discussion
 Conclusion
 References
 
Pulmonary hypertension at the end stage of chronic liver disease is not an uncommon situation. This association termed portopulmonary hypertension raises the question of the feasibility of performing orthotopic liver transplantation (OLT). In the case reported herein, there was a favorable outcome after OLT, even though the mean pulmonary artery pressure (MPAP) before transplantation was increased to 45 mm Hg. Before OLT, the cardiac index (CI) was considerably elevated (7.69 L/min/m2), giving evidence of a marked hyperdynamic circulatory state. The CI decreased significantly after OLT (3.38 L/min/m2), and this produced a significant decrease in the MPAP. Our observation suggests that portopulmonary hypertension due to a marked increase in the CI can be managed successfully by OLT.

Key Words: liver transplantation • portal hypertension • pulmonary hypertension


   Introduction
TOP
 Abstract
 Introduction
Case Report
 Discussion
 Conclusion
 References
 
The presence of pulmonary hypertension (PH) in patients with chronic liver disease was first described by Mantz and Craige1 in 1951. Later, Hadengue et al2 reported that the prevalence of PH in liver cirrhosis was approximately 2%. However, this figure probably underestimates the actual prevalence, since a more recent study3 has shown that PH is present in about 4% of patients undergoing orthotopic liver transplantation (OLT), whereas this incidence in the general population is < 0.1%. Portal hypertension (not cirrhosis) is in itself the factor that determines the development of PH.4 This association has been called portopulmonary hypertension, and it was classified in 1993 as a form of secondary PH.5 Severe PH is considered a contraindication to OLT because these patients are at risk of developing irreversible right heart failure during or after OLT. To our knowledge, this is the first reported case of a favorable outcome in pulmonary hemodynamics 1 year after OLT for portopulmonary hypertension, even though the mean pulmonary artery pressure (MPAP) before transplantation was increased to 45 mm Hg.


   Case Report
TOP
 Abstract
 Introduction
 Case Report
Discussion
 Conclusion
 References
 
A 38-year-old man with cirrhosis due to hepatitis C was admitted because of shortness of breath. On entry to our department, his exertional dyspnea was grade III of the New York Heart Association functional classification. The physical examination disclosed a systolic murmur of tricuspid regurgitation. A chest radiograph revealed a moderate enlargement of the heart and a marked increase in the caliber of the proximal pulmonary arteries. The ECG showed an increase of P wave in II, III, V1, and V2 and a right bundle branch block. A two-dimensional Doppler echocardiogram that included a contrast-enhanced test revealed right ventricular enlargement, tricuspid regurgitation, a systolic pulmonary artery pressure of 70 mm Hg, and excluded an intracardiac shunt. The arterial blood gas analyses in ambient air indicated mild hypoxemia and the presence of a chronic respiratory alkalosis (Table 1 ). The alveolar-arterial oxygen pressure difference (P[A-a]O2) was markedly increased. Lung function tests showed a mild restrictive pattern: total lung capacity was 76% of predicted. The FEV1 and FEV1/vital capacity ratio were in the normal range. Pulmonary angiography was performed, and the results ruled out chronic PH due to thromboembolic disease.


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  		Table 1. Pulmonary Hemodynamics and Arterial Blood Gases Before and 1 Year After OLT*

 
A right heart catheterization was performed on March 5, 1996. Pulmonary hemodynamic data (Table 1 ) indicated an increase in the MPAP with a normal pulmonary capillary wedge pressure, a very high cardiac index (CI), and a mild increase in the pulmonary vascular resistance index (PVRI). The inhalation of nitric oxide at 20 ppm had no effect on the MPAP and the PVRI. The CI was assessed by the thermal dilution method and was controlled according to Fick's equation applied to oxygen with the measurement of O2 uptake. The two measurements were similar.

OLT was performed on July 12, 1996. After anesthetic induction, a pulmonary artery catheter was placed, revealing an MPAP of 48 mm Hg. Unfortunately, the CI was not measured at that time. Following OLT, respiratory symptoms improved rapidly, dyspnea decreased from grade III to grade I of the New York Heart Association classification, and tricuspid regurgitation disappeared. Due to the poor prognosis of PH associated with liver disease, we decided to perform a pulmonary hemodynamic reevaluation in July 1997 (1 year after OLT). We observed a decrease in the MPAP due to the normalization of the CI (Table 1 ). The chronic respiratory alkalosis regressed, and the P(A-a)O2 decreased. In May 1998, almost 2 years after OLT, the patient was still in good condition and had no signs of PH. A Doppler echocardiogram showed normal-size right and left ventricles, as well as the complete disappearance of the tricuspid regurgitation that was observed initially.


   Discussion
TOP
 Abstract
 Introduction
 Case Report
 Discussion
Conclusion
 References
 
Our patient fulfilled all of the criteria for portopulmonary hypertension that were established in 1993.5 Furthermore, this patient verified the distinctive clinical features of this disease as recently reported by Kuo et al.6 Indeed, the MPAP and the PVRI were elevated with a simultaneous elevation in the CI. It must be emphasized that the hyperdynamic circulatory state was particularly severe in this patient.

Although it has been established that the common feature of PH patients with severe liver disease is the presence of portal hypertension, the exact mechanisms leading to the vascular abnormalities are still unknown. The main factor suspected is that vasoactive compounds from the splanchnic circulation are not metabolized by the liver and subsequently reach the pulmonary circulation.4 However, in the present case report, we believe that the very high CI (7.69 L/min/m2) has contributed to increasing the PVRI, because such a high pulmonary blood flow has been observed in children with a large ventricular septal defect7 leading to Eisenmenger's syndrome. In this situation, the elevated shear stress induces an irreversible remodeling of the muscular pulmonary artery walls.

When this patient reached the end stage of chronic liver disease, only three therapeutic possibilities remained: (1) OLT, (2) combined heart-lung-liver or lung-liver transplantation, (3) and medical treatment. Due to the scarcity of hearts and lungs that are available for transplantation, the high risk of combined transplantation, and the fact that the hyperdynamic circulatory state frequently resolves after OLT, we chose to perform OLT. After OLT, the CI normalized (as expected), allowing the MPAP to decrease significantly, although the PVRI increased slightly. The explanations for this additional increase in the PVRI could be the delay between the first right heart catheterization and OLT (4 months), or the persistence of portosystemic shunts after OLT that continue to expose the pulmonary circulation to vasoactive compounds. The fact that the MPAP increased only from 45 to 48 mm Hg between the two preoperative right heart catheterizations does not exclude a real increase in the PVRI between these two dates because (1) the patient was under anesthetic drugs during the second measurement, and (2) the CI was not assessed.

Concerning the arterial blood gas analyses, the important increase in the P(A-a)O2 before OLT was due to a ventilation/perfusion mismatch because an intracardiac or intrapulmonary shunt was excluded. Indeed, these two possible true shunts were excluded by a negative contrast-enhanced echocardiogram. Furthermore, an increase in the PVRI excluded an intrapulmonary shunt due to a hepatopulmonary syndrome.4 Therefore, the increase in the P(A-a)O2 was in part the consequence of a very high blood flow in the pulmonary circulation, because the P(A-a)O2 decreased when the CI returned to normal after OLT. The fact that the P(A-a)O2 remained moderately elevated could be the effect of irreversible pulmonary artery lesions and/or decreased ventilation in the pulmonary bases in a mildly obese patient (a body mass index of 30 kg/m2).


   Conclusion
TOP
 Abstract
 Introduction
 Case Report
 Discussion
 Conclusion
References
 
To our knowledge, there are no criteria that identify patients with portopulmonary hypertension who have an acceptable outcome after OLT. Our observation suggests that OLT can be performed successfully, even though PH is severe, when the main cause of PH is an important increase in the cardiac output. Therefore, severe PH (MPAP > 40 mm Hg) should not automatically be considered a contraindication to OLT.


   Footnotes
 
Correspondence to: Ari Chaouat, MD, Service de Pneumologie, Hôpital de Hautepierre, Avenue Molière, 67098 Strasbourg Cedex, France; e-mail: Ari.Chaouat@chru-strasbourg.fr

Abbreviations: CI = cardiac index; MPAP = mean pulmonary artery pressure; OLT = orthotopic liver transplantation; P(A-a)O2 = alveolar-arterial oxygen pressure difference; PH = pulmonary hypertension; PVRI = pulmonary vascular resistance index

Received for publication August 11, 1998. Accepted for publication December 24, 1998.


   References
TOP
 Abstract
 Introduction
 Case Report
 Discussion
 Conclusion
 References
 

  1. Mantz, FA, Craige, E (1951) Portal axis thrombosis with spontaneous portacaval shunt and resultant cor pulmonale. Arch Pathol 52,91-97
  2. Hadengue, A, Benhayoun, MK, Lebrec, D, et al (1991) Pulmonary hypertension complicating portal hypertension: prevalence and relation to splanchnic hemodynamics. Gastroenterology 100,520-528[ISI][Medline]
  3. Castro, M, Krowka, MJ, Schroeder, DR, et al (1996) Frequency and clinical implications of increased pulmonary artery pressures in liver transplantation. Mayo Clin Proc 71,543-551[ISI][Medline]
  4. Mandell, SM, Groves, BM (1996) Pulmonary hypertension in liver disease. Clin Chest Med 17,17-33[CrossRef][ISI][Medline]
  5. Rubin, LJ (1993) Primary pulmonary hypertension: ACCP consensus statement. Chest 104,236-250[Free Full Text]
  6. Kuo, PC, Plotkin, JS, Johnson, LB, et al (1997) Distinctive clinical features of portopulmonary hypertension. Chest 112,980-986[Abstract/Free Full Text]
  7. Dunbar, ID, Wolfe, RR, Abman, SH (1996) Congenital heart disease. Peacock, AJ eds. Pulmonary circulation ,449-466 Chapman & Hall Medical London, UK.



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