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Bereiding van de dosis Flolan voor patiënten met pulmonale hypertensie. Step by step uitleg bij oplossen probleem met pomp

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Gynécologie --- Obstétrique --- Appareil génital féminin, maladies. --- Gynaecologie - verloskunde --- Gynécologie - obstétrique

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Background: Portopulmonary hypertension (PoPH) has poor prognosis without orthotopic liver transplantation (OLT), but its severe form is considered to be a contraindication for OLT. In patients with PoPH, pulmonary arterial hypertension (PAH) specific therapy is used to improve haemodynamics to a level at which OLT can be performed. There is currently few data on the use of PAH therapy in PoPH since this group has been excluded from the large clinical trials in PAH, and furthermore the effect of this therapy on liver function has not been reported. Objective: Our aim was to assess the evolution of liver function under PAH therapy, to evaluate efficacy of PAH therapy on haemodynamics, functional status and clinical outcome and to determinate survival in PoPH. Methods: 35 patients with PoPH referred to UZ Leuven Centre for Pulmonary Vascular Diseases (1997 to 2016) were retrospectively evaluated. The model for end-stage liver disease score (MELD score), presence of complications of portal hypertension, New York Heart Association (NYHA) functional class, six-minute walk distance (6MWD) and haemodynamic parameters measured by right heart catheterisation (RHC) were recorded for baseline and follow-up. Results: In OLT candidates compared with patients who were referred for PAH without OLT being considered, the MELD score at baseline was significantly higher (14 vs. 8, p < 0.001) and ascites was more frequently observed (65% vs. 7%, p < 0.001). Hemodynamic profile at diagnosis was worse in patients referred for PAH, with PVR of 784 vs. 536 dyne.s.cm-5 (p = 0.005), CO of 4.31 vs. 6.01 l.min-1 (p < 0.001), and CI of 2.15 vs. 3.12 l.min-1.m2 (p < 0.001). Comparing MELD-score at baseline with last measured MELD score, PAH therapy resulted in a significant 12% decrease in MELD score (-1.2 points, p = 0.025). At 6 months, a significant 8% decrease (-0.8 points, p = 0.022) in MELD score, improvement of NYHA functional class (-0.9 points, p < 0.001) and increase in 6MWD (+65 m, p < 0.001) was found. An average period of 6-month treatment (range 1 to 12 months) resulted in a 15% decrease in mPAP (p = 0.008), 42% decrease in PVR (p = 0.012) and increase in CO and CI of 36% (p = 0.010) and 40% (p = 0.001) respectively. Overall 1-year, 3-year and 5-year survival in this cohort was 82%, 62% and 46% respectively. Conclusion: These data confirms the benefit of PAH therapy in patients with PoPH. Besides efficacy on haemodynamics and functional status, we were able to demonstrate the positive impact of PAH therapy on the liver function.

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Pulmonary arteriovenous malformations, also referred to as pulmonary arteriovenous fistulae are abnormal direct connections between pulmonary veins and arteries without an intervening pulmonary capillary bed. This creates a right-left shunt (RLS). They occur more commonly in patients with hemorrhagic hereditary telangiectasia (HHT), also known as Osler-Weber-Rendu disease. Other associations are hepatic cirrhosis, schistosomiasis, mitral stenosis, trauma, actinomycosis, Fanconi’s syndrome, metastatic thyroid carcinoma and iatrogenic cases. The current estimated prevalence of these malformations is 1/2600. They can present as simple or complex lesions. Some have a single lesion, others have a diffuse or even disseminated form. Patients with PAVMs are mostly asymptomatic. One important consequence of the right-to-left shunting is the possibility of venous thromboemboli to bypass the pulmonary capillary bed. These paradoxical emboli cause ischemic cerebral events in up to 16% of patients with PAVMs. Another important comorbidity are cerebral abscesses, with an estimated prevalence between 6-26%. Pregnancy is considered a high risk situation, with a possible pulmonary hemorrhage as a complication. Assessment of risk and possible embolisation is preferred prior to the pregnancy. A right-left shunt is diagnosed with the use of transthoracic contrast echocardiography (TTCE). If a shunt is present, a high resolution chest tomography will be performed to visualize the lesion(s). It is now recommended to treat even small (<3 mm) malformations, compared to older publications. Treatment with transcutaneous embolisation is preferred. This can either be done with coils or vascular plugs. Another, much less preferred, option is lobectomy or lung transplantation.

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