2PHPulmonary hypertension is an abnormal elevation of the pulmonary artery pressure (PAP) and the pulmonary vascular resistance (PVR) resulting in right ventricular (RV) failure and premature death.
3PH PH used to be recognized as a disease with a grim prognosis. Over the last decade, new medications have been developed to treat PH. These medications have improved both the quantity and quality of life for patients with PH.Since we can now treat PH, we need to be more aware of pursuing it as a diagnosis.
4PH What is PH? What are the most common symptoms? mPAP > 25 mmHg at restmPAP > 30 mmHg with activityWhat are the most common symptoms?Worsening SOBChest painFatiguePalpitationsLower extremity edemaSyncope
5PH WHO classification Group I- PAH Group II- PVH, PH secondary to LV failureGroup III- PH associated with lung disease or hypoxiaGroup IV- PH secondary to chronic thromboembolic diseaseGroup V- miscellaneous - HIV infection, drug exposure
6PH Group I - PAH Replaces primary pulmonary hypertension No known underlying risk factorsUsually seen in women of childbearing ageRare - 2 to 3 per million per yearGenetic predisposition
7PH Facts: Group II – PVH – most common, PCWP >15 mmHg Group III - lung diseaseCOPD – mild PH seen in up to 50% of ptsOSA – usually associated with mild PHOHS – more commonly seen with cor pulmonaleGroup IV - chronic PTED – up to 4%
8PHPathophysiologyPulmonary endothelial cell dysfunction or injury causing vascular changesIntimal proliferationHypertrophyProliferation of smooth muscle cellsVasoconstrictionIn situ thrombosis
9PH Making the diagnosis High index of suspicion PE – early, Nl; increased P2, TR, heptojugular refluxCXR, CT chest – enlarged PA’sEKG – V1, tall R wave and short S wave (RV hypertrophy); II, p-pulmonale (RAE)Transthoracic ECHO – evaluate LV function, estimate RVSP and PAP’SRight heart catheterization – measure PAP’s and PCWP
10PH Further Evaluation Lab – ANA, RF, HIV, CBC, LFT’s, TFT’s PFT’s – OLD or ILD; decrease in DLCOOvernight oximetry – desaturation is seen in 70% of ptsPSGV/Q scan, CT chest, pulmonary angiography
11PH Treatment – General measures O2 – keep sats > 90% Avoid vasoconstricting decongestants, B blockers, stimulants and anorexigensDo low level aerobic exerciseFollow a low sodium (<2400 mg) dietAvoid pregnancyAnticoagulationDiureticsDigoxin?
13PH Prostanoids – prostacyclin analogues Prostacyclin is a potent vasodilator and antiplatelet agentDeficient in pts with PHImprove symptomsImprove hemodynamicsOverdosage causes hypotension and hyperdynamic state with high-output cardiac failure
14PHProstanoids cont.Epoprostenol – only drug with proven survival benefit; 6 minute half-lifeMust be kept cold during storage and administrationContinuous IV infusion thru tunneled catheterTreprostinil – not shown to improve survival; 3 hour half lifeContinuous SQ infusionIloprost – inhaled route of administration; 6-9 times a day (Q2 hours while awake)
16PHPDE-5’s – improve sx and functional class; augments vasodilatory effects of nitric oxideSildenafil (Revatio) – oral, TIDHA’sFlu-like sxFlushingEpistaxis
17PH NYHA classification of functional status of pts with PH I – no limitations in nl physical activityII – mild limitation, no sx at rest, worsening sx with exertionIII – marked limitation, no sx at rest, worsening sx with light activityIV – sx at rest, unable to do any activity, signs of RV failure at rest
18PH Treatment by Classification I – monitor II – oral sildenafil (Revatio)III – oral sildenafil or bosentan (Tracleer) and inhaled or intravenous prostanoidsIV – intravenous prostanoids
19PH Goals of Treatment Improvement to class I or II Improvement in the 6 MWDT to 380 m or betterMax SBP with exercise of 120 mm Hg or greaterDecrease in BNP to < 180 pg/ml
20PH Other treatments Surgery Atrial septostomy – decrease right-sided pressures, may worsen hypoxiaLung transplant – curative, post op median survival 5 yearsPulmonary thromboendarterectomy – curative for PH from chronic PTED, tx of choice in appropriate candidates
21PHUpcoming therapiesTreprostinil – infusion and inhaled available now, working on oral formulationSitaxsentan – approved in Europe, application is pending with FDATadalafil (Cialis) – longer half-life and greater selectivity and potency than sildenafil; in trials now
22PHPrognosis1980s – grim, medial survival of 2.8 years from time of diagnosis in untreated ptsCurrent – newer medications have greatly improved the outlook for pts with PHPoor prognostic indicators – low 6 MWDT; pericardial effusion, RV dysfunction, and RAE on ECHO; increased mRAP (the most powerful hemodynamic predictor) and decreased cardiac index on RHC; and elevated BNP