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home - Liver - Vascular Problems - Porto pulmonary Disease Written by Dr Sebastian Zeki
Knowledge


Understands the risk of variceal bleeding as a complication of with
portal hypertension


Knows risk of variceal haemorrhage in cirrhotics who have not bled

Knows risk of bleeding related to variceal size endoscopic findings
and severity of liver dysfunction


Knows range of therapeutic options (both endoscopic and
pharmacological).

Skills
Recognises and can treat portal hypertension.
Behaviours
Manages patients with oesophageal varices with skill and
compassion

Able to convey the serious risks to patients and their relatives.

Also...

Knowledge


Recognises and shows understanding of vascular liver disease
including Budd-Chiari syndrome veno-occlusive disease and
portomesenteric venous thrombosis; understands the underlying
anatomy and physiology of these often complex conditions

Aware of need for investigation for associated myeloproliferative and
procoagulant conditions

Understands the role of anticoagulation and indications for further
intervention including TIPS surgery or transplantation

Skills
Can make careful clinical of these conditions and has
heightened awareness of liver vascular disease in differential
diagnosis

Able to make a potentially difficult diagnosis of less common variants
of vascular conditions

Behaviours
Shows ability to keep patient and relatives informed and to refer
appropriately for specialist management

Porto pulmonary Disease

Features Young woman presenting with SOB and dizziness Loud P2 Chronic lung disease Written by Dr Sebastian Zeki Diagnosis confirmed when:PAH: (Mean pulmonary artery pressure (mPAP) >25 mmHg at rest or >30 mmHg with exercisePulmonary capillary wedge pressure (PCWP) <15 mmHg)+Elevated hepatic venous pressure during hepatic vein catheterization+No alternative cause of PAH Group 1- Pulmonary arterial hypertension (PAH)- This includes PPHTN , Idiopathic PAH (sporadic and familial) and PAH due to diseases that localize to small pulmonary muscular arterioles:Group 2-5 - Pulmonary hypertension (PH) a) Vasoconstrictionb) Remodeling of muscular pulmonary artery walls-Medial hypertrophy is early and reversible form of disease.c) In situ thrombosis. As the diseases advances, medial hypertrophy becomes a component of pulmonary arteriopathy.2 subtypes of pulmonary arteriopathy:-Plexogenic pulmonary arteriopathy is characterized by medial hypertrophy, intimal fibrosis, and lesions involving the entire vessel wall-Thrombotic pulmonary arteriopathy is characterized by medial hypertrophy, thrombosis, and eccentric, nonlaminar intimal fibrosis.It may arise from in situ thrombosis, rather than emboli. EpidemiologyPAH occurs in 2 % of portal hypertension patients.PAH increases with severity of PHTN.5 groups of pulmonary hypertension exist: Diagnostic Evaluation: PrognosisWith therapy 1 year survival is 88%, and 50% without therapy Transjugular intrahepatic portosystemic shunts (TIPS)May be harmful if have PPHTN so weigh up pros and cons Transplantation PAH resolves with transplantationSevere PAH (systolic PAP >60 mmHg) assoc with high perioperative risk and poor clinical outcomeMild to moderate PAH does not influence mortality after liver transplantation. One of: Iloprost —(Prostacyclin analogue)Anecdotal success-ful use of inhaled/iv iloprost in PPHTN.Not as good as bosentan Sildenafil —(Phosphodiesterase inhibi-tor)Successfully used to treat PPHTN- case reports Bosentan —(Oral endothelin receptor antagonist) Can be used success-fullyIs associated with deranged LFT's in 10% so careful moni-toring required Epoprostenol —(prostacyclin, Flolan; potent vasodilator with anti-platelet aggregating and anti-proliferative properties)Iv infusion improves hemodynamics and exercise performance; is bridge to liver transplantation.Side effects: jaw pain, diarrhea, erythema, arthralgias, and high cardiac output. + Anticoagulation Venous stasis, slowed pulmonary blood flow, and right heart enlargement combine to increase the risk of in situ pulmonary vascular thrombosis and thromboembolic disease in both IPAH and PPHTN.Goal INR of 1.5 due to the increased risk of hemorrhage in patients with chronic liver disease. Treatment Portal Hypertension Signs + Pulmonary Regurgitation Murmur Pathology Intimal fibrosis Medial hypertrophy PathologyThe pulmonary histopathologic findings in PPHTN and IPAH are indistinguishable: Pathogenesis theories:A humoral substance (which would normally be metabolized by the liver) is able to reach the pulmonary circulation through portosystemic collaterals, resulting in PPHTN.Candidates for a humoral substance include serotonin, interleukin-1, endothelin-1, glucagon, secretin, throm-boxane B2, and VIP.:Genetic Familial PAH has been localized to chromosome 2 (locus 2q33) and defective function of the bone morphogenetic protein receptor type II (BMPR2).Thromboembolism from the portal venous system via portosystemic shunts. Portopulmonary hypertension (PPHTN) = pulmonary arterial hypertension that is associated with portal hypertensionHepatopulmonary syndrome= hypoxemic respiratory insufficiency due to increased intrapulmonary shunting Portopulmonary hypertension= A type of group 1 pulmonary arterial hypertension

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