Clinical analysis of portopulmonary hypertension

doi:10.3877/cma.j.issn.1674-6902.2020.01.006

Abstract

Abstract:

Objective

To explore the clinical features of portopulmonary hypertension (PoPH) and the relationship between the right atrial liner index and the heart function.

Methods

Eleven patients were diagnosed as PoPH in Beijing Anzhen Hospital, Capital Medical University in China from January 2012 to December 2018. The general information, clinical manifestation, medical imaging results, laboratory tests, right heart catheter data, cardiopulmonary exercise test, treatment and prognosis of the patients were analyzed retrospectively. The laboratory tests included the complete blood count, serum chemistry profile, coagulation function, B type brain natriuretic peptide, and arterial blood gas analysis, which were used to calculate the scales of Child-Pugh and Model for the end-stage liver disease (MELD).

Results

The patients (5 males and 6 females) aged from 29 to 85 years. Hepatocirrhosis was found in 9 cases and non-hepatocirrhosis was found in 2 cases. According to the medical history, the onset time of pulmonary hypertension was 0.5-19 years after portal vein hypertension, with the median time of 5 years. The scale of New York Heart Association (NYHA) was positively correlated with the modified Medical Research Council (mMRC) scale [r=0.9608, 95%CI (0.8460, 0.9896), P<0.0001], however, it had no correlation with Child-Pugh scale. The correlation coefficient was 0.7930 between the right atrial linear index and the BNP with the 95%CI of 0.3684-0.9439 (P=0.0036). However, no significant difference was found between the right atrial linear index and the MELD. All the patients suffered from hypoxemia. The alveolar-arterial oxygen differential pressure (PA-aO₂) was higher significantly than the predicted value (t=3.669, P=0.0018). The patients with severe heart failure had poor prognosis.

Conclusion

The patients with PoPH are more likely to experience exertional dyspnea, which is associated with the degree of the heart dysfunction. And it is a fact that all the patients have hypoxemia. Therefore, the clinical decision for the patients with PoPH should not be made only in the consideration of the liver function. The right atrial linear index is an available option to follow such patients.

Key words: Liver cirrhosis, Hypertension, portal, Pulmonary hypertension, Clinical analysis

Yan Wang, Xiangfeng Zhang, Qian Sun, Wenmei Zhang, Guangfa Zhu, Yang Gao, Ruchong Chen. Clinical analysis of portopulmonary hypertension[J]. Chinese Journal of Lung Diseases(Electronic Edition), 2020, 13(01): 28-33.

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Figures/Tables 7

References 30

1	Galie N, Humber M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension[J]. Eur Heart J, 2016, 37(1): 67-119.
2	Hoeper MM, Bogaard HJ, Condliffe R, et al. De finitions and diagnosis of pulmonary hypertension[J]. J Am Coll Cardiol, 2013, 62(Suppl 25): D42-D50.
3	Simón-Talero M, Roccarina D, Martínez J, et al. Association between portosystemic shunts and increased complications and mortality in patients with cirrhosis[J]. Gastroenterology, 2018, 154(6): 1694-1705.
4	Le Pavec J, Souza R, Herve P, et al. Portopulmonary hypertension: survival and prognostic factors[J]. Am J Respir Crit Care Med, 2008, 178(6): 637-643.
5	Sasiharan S, Arun N, Loqan T, et al. Survival in portopulmonary hypertension: outcomes of the United Kingdom National pulmonary arterial hypertension registry[J]. J heart and lung transplant, 2017, 36(7): 770-779.
6	ColleI O, Moreau R, Godinho E, et al. Diagnosis of portopulmonary hypertension in candidates for liver transplantation: a prospective study[J]. Hepatology, 2003, 37(2): 401-409.
7	Chen HS, Xing SR, Xu WG, et al. Portopulmonary hypertension in cirrhotic patients: Prevalence, clinical features and risk factors[J]. Exp Ther Med, 2013, 5(3): 819-824.
8	Hervé P, Lebrec D, Brenot F, et al. Pulmonary vascular disorders in portal hypertension[J]. Eur Respir J, 1998, 11(5): 1153-1166.
9	Herve P, Le Pavec J, Sztrymf B, et al. Pulmonary vascular abnormalities in cirrhosis[J]. Best Pract Res Clin Gastroenterol, 2007, 21(1): 141-159.
10	Tilg H, Wilmer A, Vogel W, et al. Serum levels of cytokines in chronic liver diseases[J]. Gastroenterology, 1992, 103(1): 264-274.
11	Miot-Noirault E, Faure L, Guichard Y, et al. Scintigraphic in vivo assessment of the development of pulmonary intravascular macrophages in liver disease: experimental study in rats with biliary cirrhosis[J]. Chest, 2001, 120(3): 941-947.
12	Hervé P, Lebrec D, Brenot F, et al. Pulmonary vascular disorders in portal hypertension[J]. Eur Respir J, 1998, 11(5): 1153-1166.
13	Roberts KE, Fallon MB, Krowka MJ, et al. Pulmonary Vascular Complications of Liver DiseaseStudy Group. Genetic risk factors for portopulmonary hypertension in patients with advanced liver disease[J]. Am J Respir Crit Care Med, 2009, 179(9): 835-842.
14	Nunes H, Lebrec D, Mazmanian M, et al. Role of nitric oxide in hepatopulmonary syndrome in cirrhotic rats[J]. Am J Respir Crit Care Med, 2001, 164(5): 879-885.
15	Schraufnagel DE, Kay JM. Structural and pathologic changes in the lung vasculature in chronic liver disease[J]. Clin Chest Med, 1996, 17: 1-15.
16	Guido M, Sarcognato S, Sacchi D, et al. Pathology of idiopathic non-cirrhotic portal hypertension[J]. Virchows Arch, 2018, 473(1): 23-31.
17	Nikolic I, Yung LM, Yang PR, et al. Bone morphogenetic protein 9 Is a mechanistic biomarker of portopulmonary hypertension[J]. Am J Respir Crit Care Med, 2019, 199(7): 891-902.
18	Rodríguez-Roisin R, Krowka MJ, Agustí A. Hepatopulmonary disorders：gas exchange and vascular manifestations in chronic liver disease[J]. Comp Phys, 2018, 8(2): 711-729.
19	Finkelhor RS, Lewis SA, Pillai D, et al. Limitations and strengths of doppler/echo pulmonary artery systolic pressure-right heart catheterization correlations: a systematic literature review[J]. Echocardiography, 2014, 32(1): 10-18.
20	Hua R, Sun YW, Wu ZY, et al. Role of 2-dimensional doppler echo-cardiography in screening portopulmonary hypertension in portal hypertension[J]. Hepatobiliary Pancreat Dis Int, 2009, 8(2): 157-161.
21	Maceira AM, Cosín-Sales J, Roughton M, et al. Reference right atrial dimensions and volume estimation by steady state free precession cardiovascular magnetic resonance[J]. J Cardiovasc Magn Reson, 2013, 15(1): 29-38.
22	Raymond RJ, Hinderliter AL, Willis PW, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension[J]. J Am Coll Cardiol, 2002, 39(7): 1214-1219.
23	Bustamante LM, Perrone S, De La Fuente RL, et al. Right atrial size and tricúspide regurgitation severity predict mortality or transplantation in primary pulmonary hypertension[J]. J Am Soc Echocardiogr, 2002, 15: 1160-1164.
24	AbuHalimeh B, Krowka MJ, Tonelli AR. Treatment barriers in portopulmonary hypertension[J]. Hepatology, 2019, 69(1): 431-443.
25	Farber HW, Miller DP, Poms AD, et al. Five-year outcomes of patients enrolled in the REVEAL registry[J]. Chest, 2015, 148(4): 1043-1054.
26	Ghofrani HA, Galiè N, Grimminger F, et al. Riociguat for the treatment of pulmonary arterial hypertension[J]. N Engl J Med, 2013, 369(4): 330-340.
27	Cosarderelioglu C, Cosar AM, Gurakar M, et al. Portopulmonary hypertension and liver transplant: recent review of the literature[J]. Experimental and Clinical Transplantation, 2016, 14(2): 113-120.
28	Hollatz TJ, Musat A, Westphal S, et al.Treatment with sildenafil and treprostinil allows successful liver transplantation of patients with moderate to severe portopulmonary hypertension[J]. Liver Transpl, 2012, 18(6): 686-695.
29	Raevens S, De Pauw M, Reyntjens K, et al. Oral vasodilator therapy in patients with moderate to severe portopulmonary hypertension as a bridge to liver transplantation[J]. Eur J Gastroenterol Hepatol, 2013, 25(4): 495-502.
30	Krowka MJ, Swanson KL, Frantz RF, et al. Portopulmonary hypertension：results from a 10-year screening algorithm[J]. Hepatology, 2006, 44(6): 1502-1510.

项　目		单位	数值
年龄		岁	55(47～74)
男性		[n(%)]	4(36.4)
女性		[n(%)]	7(73.6)
体重		kg	62.36±8.27
身高		m	1.66±0.10
BMI		kg/m²	22.63±2.41
原发性肝脏疾病
	病毒性肝炎肝硬化	[n(%)]	7(63.63)
	酒精性肝炎肝硬化	[n(%)]	1(9.09)
	自身免疫性肝炎肝硬化	[n(%)]	1(9.09)
	非原发肝脏疾病
	布加综合征	[n(%)]	1(9.09)
	门静脉闭锁	[n(%)]	1(9.09)
NYHA分级
	Ⅱ级	[n(%)]	6(54.55)
	Ⅲ级	[n(%)]	2(18.18)
	Ⅳ级	[n(%)]	3(27.27)
mMRC评分
	1分	[n(%)]	1(9.09)
	2分	[n(%)]	5(45.45)
	3分	[n(%)]	2(18.18)
	4分	[n(%)]	3(27.27)
Child-pugh分级
	A级	[n(%)]	2(18.18)
	B级	[n(%)]	7(63.63)
	C级	[n(%)]	2(18.18)

项　目		单位	数值
年龄		岁	55(47～74)
男性		[n(%)]	4(36.4)
女性		[n(%)]	7(73.6)
体重		kg	62.36±8.27
身高		m	1.66±0.10
BMI		kg/m²	22.63±2.41
原发性肝脏疾病
	病毒性肝炎肝硬化	[n(%)]	7(63.63)
	酒精性肝炎肝硬化	[n(%)]	1(9.09)
	自身免疫性肝炎肝硬化	[n(%)]	1(9.09)
	非原发肝脏疾病
	布加综合征	[n(%)]	1(9.09)
	门静脉闭锁	[n(%)]	1(9.09)
NYHA分级
	Ⅱ级	[n(%)]	6(54.55)
	Ⅲ级	[n(%)]	2(18.18)
	Ⅳ级	[n(%)]	3(27.27)
mMRC评分
	1分	[n(%)]	1(9.09)
	2分	[n(%)]	5(45.45)
	3分	[n(%)]	2(18.18)
	4分	[n(%)]	3(27.27)
Child-pugh分级
	A级	[n(%)]	2(18.18)
	B级	[n(%)]	7(63.63)
	C级	[n(%)]	2(18.18)

序	右心房短径mm	右心房长径mm	右心房面积cm²	TRVa cm/s	估测sPAP mmHg	BSA m²	右心房线性指数cm/m²	MELD评分	BNP pg/ml
1	39	56	21.8	480	100	1.62	3.46	11	150
2	52	59	30.7	347	55	1.72	3.44	14	162
3	57	64	36.5	417	86	1.98	3.23	11^a	374
4	41	52	21.3	366	79	1.47	3.54	9	128
5	43	53	22.8	379	63	1.60	3.31	13	969
6	51	54	27.5	436	85	1.77	3.05	13	72
7	50	64	32.0	342	85	1.53	4.20	11	1 570
8	57	66	37.6	426	63	1.56	4.22	10	1 788
9	50	60	30.0	367	64	1.80	3.33	11	466
10	54	67	36.2	370	67	1.72	3.90	9	1 219
11	44	57	25.1	290	42	1.85	3.08	12	686
	49	59	29.2	383	72	1.69	3.52	11	689
SD	6	5	60.0	54	13	0.15	0.41	2	613

序	右心房短径mm	右心房长径mm	右心房面积cm²	TRVa cm/s	估测sPAP mmHg	BSA m²	右心房线性指数cm/m²	MELD评分	BNP pg/ml
1	39	56	21.8	480	100	1.62	3.46	11	150
2	52	59	30.7	347	55	1.72	3.44	14	162
3	57	64	36.5	417	86	1.98	3.23	11^a	374
4	41	52	21.3	366	79	1.47	3.54	9	128
5	43	53	22.8	379	63	1.60	3.31	13	969
6	51	54	27.5	436	85	1.77	3.05	13	72
7	50	64	32.0	342	85	1.53	4.20	11	1 570
8	57	66	37.6	426	63	1.56	4.22	10	1 788
9	50	60	30.0	367	64	1.80	3.33	11	466
10	54	67	36.2	370	67	1.72	3.90	9	1 219
11	44	57	25.1	290	42	1.85	3.08	12	686
	49	59	29.2	383	72	1.69	3.52	11	689
SD	6	5	60.0	54	13	0.15	0.41	2	613

序号	RAP mmHg	mPAP mmHg	POAP mmHg	PVR Dny·s/cm⁵	CO L/min	CI L/min/m²
1	6/1	30	2	789.47	3.04	1.86
2	8/0	36	6	591.99	4.86	2.89
3	10/1	36	15	449.49	6.40	3.22

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