Medical management of primary pulmonary hypertension

Kneußl, Meinhard; Lang, Irene M.; Brénot, F

doi:10.1183/09031936.96.09112401

Cited by 20 publications

(9 citation statements)

References 97 publications

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“…The proportions of patients having a reduction of PVR of at least 20% were similar in the two groups (43 versus 57%), and 37% of patients in the portopulmonary hypertension group versus 36% in the PPH group were subsequently treated with various oral vasodilators. Following KNEUSSL et al [57], pati-ents were classified as responders (reduction of PVR Š20% and reduction of mean pulmonary artery pressure Š20%) or nonresponders to prostacyclin. Using this criterion, 16% of patients were classified as responders in the PPH group and none in the portopulmonary hypertension group.…”

Section: Clinical Presentation Haemodynamics Response To Vasodilatomentioning

confidence: 99%

Pulmonary vascular disorders in portal hypertension

Hervé

Lebrec²,

Brénot³

et al. 1998

Eur Respir J

330

239

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aaThis review focuses on the pulmonary vascular complications of chronic liver failure and portal hypertension. The wide spectrum of pulmonary vascular disorders in liver disease and portal hypertension ranges from the hepatopulmonary syndrome (HPS), which is characterized by intrapulmonary vascular dilatations, to pulmonary hypertension (portopulmonary hypertension), in which pulmonary vascular resistance (PVR) is elevated. The exact pathophysiological mechanisms of these pulmonary vascular disorders are unknown. However, since HPS and portopulmonary hypertension have been reported in patients with nonhepatic portal hypertension, the factor that determines their development must be portal hypertension.A brief review of the haemodynamic changes observed in cirrhosis with portal hypertension is summarized, and the clinical and experimental evidence suggesting that the liver exerts a critical influence on the regulation of pulmonary vascular tone and angiogenesis is described. Portopulmonary hypertension is then discussed, focusing in turn on its clinical presentations, management and potential mechanisms, and referring to our findings in 39 patients with portopulmonary hypertension and 140 patients with primary pulmonary hypertension (PPH) admitted between 1986 and 1996 at Antoine Béclère Hospital. Finally, HPS will be examined, with a discussion of the mechanisms of hypoxaemia and intrapulmonary vascular dilatation, as well as its clinical presentation, diagnosis and treatment, with special attention to liver transplantation. Haemodynamics in cirrhosis with portal hypertensionAn understanding of pulmonary haemodynamics in liver disease with portal hypertension is important to a study of the pulmonary vascular disorders seen in this setting. A hyperdynamic circulatory state with high cardiac output, low systemic vascular resistance and low PVR is present in 30-50% of patients with cirrhosis and also in animal models of portal hypertension [1][2][3][4]. Compared with healthy subjects ( fig. 1) The wide spectrum of pulmonary vascular disorders in liver disease and portal hypertension ranges from the hepatopulmonary syndrome characterized by intrapulmonary vascular dilatations, to pulmonary hypertension (portopulmonary hypertension), in which pulmonary vascular resistance is elevated. Since hepatopulmonary syndrome and portopulmonary hypertension have been reported in patients with nonhepatic portal hypertension, the common factor that determines their development must be portal hypertension. The clinical presentations are very different, with gas exchange impairment in the hepatopulmonary syndrome and haemodynamic failure in portopulmonary hypertension. The severity of hepatopulmonary syndrome seems to parallel the severity of liver failure, whereas no simple relationship has been identified between hepatic impairment and the severity of portopulmonary hypertension. Resolution of hepatopulmonary syndrome is common after liver transplantation, which has an uncertain effect in portopulmonary hypertension. The pathoph...

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Section: Clinical Presentation Haemodynamics Response To Vasodilatomentioning

confidence: 99%

Pulmonary vascular disorders in portal hypertension

Hervé

Lebrec²,

Brénot³

et al. 1998

Eur Respir J

330

239

View full text Add to dashboard Cite

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“…By definition the aetiology of PPH is unknown and its diagnosis thus relies on exclusion criteria [2]. Treatment of PPH is based on vasodilator therapy, oral anticoagulation and supplemental oxygen (O 2 ) [3]. Further therapeutic options are atrial septostomy and lung transplantation [4,5].…”

mentioning

confidence: 99%

Nocturnal periodic breathing in primary pulmonary hypertension

Schulz

Baseler²,

Ghofrani³

et al. 2002

Eur Respir J

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Cheyne-Stokes respiration is frequently observed in congestive heart failure. Among other factors, prolongation of circulation time, hypocapnia and hypoxia are thought to underlie this sleep-related breathing disorder. Primary pulmonary hypertension (PPH) is also characterized by reduced cardiac output and blood gas alterations. Therefore, the aim of the present study was to determine whether a nocturnal periodic breathing (PB) occurs in PPH.A total of 20 consecutive patients with PPH who had been admitted for pharmacological investigation of pulmonary vasoreactivity were investigated by lung function testing, right heart catheterization and full-night attended polysomnography.PB was detected in six patients (30%) (mean¡SEM: apnoea/hypopnoea index 37¡5 h -1 ; arterial oxygen saturation was v90% during 56¡6.5% of total sleep time). The patients with PB had more severe haemodynamic impairment than those without. They also had a more marked reduction in the pulmonary diffusion capacity and greater arterial hypoxia. PB was markedly improved or even eradicated by nasal oxygen during the night.Periodic breathing occurs in patients with advanced primary pulmonary hypertension and can be reversed by nocturnal nasal oxygen. The clinical and prognostic significance of periodic breathing in primary pulmonary hypertension needs to be determined by further studies.

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“…In patients not receiving digoxin, spironolactone is an attractive agent because of its aldosterone-inhibiting properties in the setting of sympathetic nervous system and reninangiotensin-aldosterone system activation due to increased right-sided heart filling pressure and upstream venous congestion in patients with IPAH. 20 Spironolactone's ability to increase digoxin concentrations, however, may complicate therapy if these drugs are coadministered. Patients requiring additional diuresis can receive low-dose loop diuretics, with dosage titrated to maximum effect, with metolazone added in refractory cases.…”

Section: Conventional Therapymentioning

confidence: 99%