Presentation on theme: "PH: Who Ya Gonna Call Pulm Consults"— Presentation transcript:
1PH: Who Ya Gonna Call Pulm Consults Lana MelendresPulm/CC DivisionPulm Htn Program Director4/24/13
2ObjectivesDefine pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH)Identify the 5 different groups of PHWhen to call a lifeline (pulm consult)Review the workup for PHMaking the diagnosisTherapeutic options
3Case #135yo woman with PMHx significant for exercise induced asthma and anxiety presenting to the ED after “syncope”.Remembers trying to pick up her 2 year old daughter when the “curtains closed” and she “went down”SOB worsening despite use of inhalers
4Case #229yo man with HIV presenting to the ED with 2 month h/o of worsening SOB on exertion that has dramatically worsened over the past week to the point that he is now having SOB at rest and feeling dizzy when standing not on any medications.Recently moved from California and has no information in our system.
5Case #346yo morbidly obese woman with DM, htn, and presumed OSA/OHS presenting to the ED with fatigue and worsening lower ext edema.Recently established with a PCP and started on therapies for her DM, htn and was referred for a sleep study (will be seen in Oct 2013)
6Case #465yo woman with little PMHx presenting with SOB and exhaustion. Has no medical problems that she knows of, just retired from teaching for the past 40years.Previously playing 18 holes of golf, now only able to walk 15ft before needing to stop and rest.
7Case #547yo man with SOB that has been progressive for the past several months. Previously able to ride his bike miles a week, now to sob to ride more than 5-10 miles at a time.No medical problems that he is aware of. Works as a nuclear engineer. Takes no medication.CT scan showed nodules, lymphadenopathy, and septal thickening.PVOD:CT findings with
8Case #683yo woman has been healthy her whole life now presenting with worsening fatigue with exertion.Previously able to swim for 30 minutes a day and walk for 30min, now sob with much less. Unable to keep up with her friend.Experiencing palpitations and chest pressure intermittently.
9Case #752yo man with ESRD on HD, htn, DM, CAD, cirrhosis from hep C and prior ETOH abuse and mild COPD, no longer smoking, admitted after missing two HD appointments with profound fluid overload.Also notes that he has had worsening SOB over the past year and fluid retention.
11Diagnostic Definition: Pulmonary Hypertension Rest:- Mean PAP >25 mmHgPAH = above + PCWP or LVEDP <15 mmHgAssociated with adverse changes- In the pulmonary vasculature (arteriopathy)- At the level of the right ventricle (hypertrophy)No longer part of the definition:Exercise:- Mean PAP > 30 mmHgGenevea convention in 2003 the diagnostic classification was mPAP of >25, included exercise >30 and wedge of <15mmHg with some mention of PVR needing to be >2 or 3 Woods units. At the Dana point conference in 2008 this definition was altered for a variety of reasons. The first was that when reviewing 47 studies on normal patients PAP, at rest the mean was but with exercise there was a wide range especially respective to age (>50 could be as high as 47). Currently, it is felt that mPAP of 8-20 is normal; is a gray zone with insufficient evidence; 25 or greater was PH.Plexogenic arteriopathy (the parent muscular artery shows medial and intimal thickening) due to inflammatory process from cytokines and chemokines undergoes shear stress which results in damage causing transmural destruction that is repaired by grnaulation tissue= plexiform lesion.Pleciform lesion= granulation tissue resulting from the parent muscular artery sustaining medial and intimal thickening that combined with shear stress at branch pointsGaine et al. The Lancet, 1998.
12New Proposed Classification of Pulmonary Hypertension (Dana Point, 2008) 1. Pulmonary Arterial Hypertension 1.1 Idiopathic PAH 1.2 Heritable BMPR ALK1, endoglin (with or without hereditary hemorrhagic telangiectasia ) Unknown. 1.3 Drug- and toxin-induced 1.4 Associated with Connective tissue diseases HIV infection Portal hypertension Congenital heart diseases Schistosomiasis Chronic hemolytic anemia 1.5 Persistent pulmonary hypertension of the newborn 1’. Pulmonary veno-occlusive disease (PVO) and/or pulmonary capillary hemangiomatosis (PCH) 2. Pulmonary hypertension due to left heart disease 2.1 Systolic dysfunction 2.2 Diastolic dysfunction 2.3 Valvular disease3. Pulmonary hypertension due to lung diseases and/or hypoxiaChronic obstructive pulmonary diseaseInterstitial lung diseaseOther pulmonary diseases with mixed restrictive and obstructive patternSleep-disordered breathingAlveolar hypoventilation disordersChronic exposure to high altitudeDevelopmental abnormalities4. Chronic thromboembolic pulmonary hypertension (CTEPH)5. PH with unclear multifactorial mechanismsHematologic disorders: myeloproliferative disorders splenectomy.Systemic disorders, sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitisMetabolic disorders: glycogen storage disease, Gaucher disease, thyroid disordersOthers: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis.
13Back to the Cases What do all the patients presented have in common? Each patient was found to have pulmonary arterial hypertension after full w/u and diagnosis by right heart catheterization.
14Pulmonary Arterial Hypertension Case #1: IPAHCase #2: PAH associated with HIV
15PAHCase #3: PAH assoc with drugs/toxinsCase #4: PAH assoc with CTD
16PAH Case #5: PVOD Case #6: PAH assoc with CHD Case #7: PAH assoc with portal htn
17When to Call Pulmonary Definite Consult: Consider Consult: Patient with known PAH by RHC, on PAH specific therapiesAll patients followed by the PH clinic.Consider Consult:Patient with echo findings of elevated PASP:No known CTD, PASP >40mmHgKnown CTD, PASP >35mmHg
18Pulmonary Arterial Hypertension: Detection and Diagnosis Is there a reason to suspect PAHClinical history (symptoms, risk factors, family Hs.),Exam, CXR, ECGyesnoIs PAH likely?EchoRationaleTRV to measure RVSP; RVE; RAE; RV Dysfunction:No furtherevaluationfor PAHnoyesIs PAH due to LH disease?EchoyesDx LV systolic, diastolic dysfunction; valvular disease:Appropriate treatment and further evaluationif necessary, including R&LHCnoDx abnormal morphology; shunt:Surgery. Medical treatment of PAH or evaluation forfurther definition or other contributing causes,including R&LHC if necessaryIs PAH due to CHD?Echo with contrastyesnoThis detection and diagnosis algorithm was published in a recent Chest supplement focused on diagnosis and treatment of pulmonary hypertension. It provides a rigorous strategy to diagnose PH, and determine it’s cause which is key to determining the appropriate treatment approach. In some cases, medical history and/or the presence of risk factors will afford a re-order of this approach to initially hone in on and either confirm or eliminate the suspected cause.Dx Scleroderma, SLE, other CTD, HIV: Medicaltreatment of PAH and further evaluation forother contributing causes, including RHCIs PAH due to CTD, HIV?SerologiesyesnoIs chronic PE suspected?VQ scanMcGoon et al. Chest 2004;126:14S-34S
19Pulmonary Arterial Hypertension: Detection and Diagnosis Is chronic PE suspected?VQ scanyesIs chronic PE confirmed and operable?Pulmonary angiogramyesnoVQ normalAnatomic definition (CT, MRI may provide additional usefulbut not definitive information):Thromboendarterectomy if appropriate or medicaltreatment; clotting evaluation; a/cIs PAH due to lung diseaseor hypoxemia?PFTs, arterial saturationnoyesDx parenchymal lung disease, hypoxemia, or sleep disorder:Medical treatment, oxygen, positive pressure breathingas appropriate, and further evaluation for othercontributing causes, including RHC if necessarynoDocument exercise capacity regardless of cause of PH:Establish baseline, prognosis and document progression/response to treatment with serial reassessmentsWhat limitations are caused bythe PAH?Functional class; 6-minute walktest..... The final step in diagnosing PAH is right heart catheterizationDocument PA and RA pressures, PCWP (LV or LA pressureif PCWP unobtainable or uncertain), transpulmonary gradientCO, PVR, SvO2, response to vasodilators:Confirm PAH, or IPAH if no other cause identifiedDiscuss genetic testing and counseling of IPAHWhat are the precise pulmonaryhemodynamics?RHCMcGoon et al. Chest 2004;126:14S-34S
21Right Heart Catheterization is the Diagnostic Gold Standard SaturationsRule Out ShuntsIntra-cardiacIntra-PulmonaryHemodynamicsRAPmPAPPCWPRule out left sided heart diseaseCO/CIPVRAngiographyVessel propertiesCTEPHVasodilator ResponseRight heart catheterization is the diagnostic gold standard. This evaluation is necessary to sufficiently rule out secondary causes of disease and left sided heart disease. This evaluation is also necessary to obtain the critical measures of cardiac index and calculated PVR which have significant value prognostically.RHC can also Prognosticate!Rich et al. WHO Symposium on PPH. Evian, France,1998.
22Determinants of Disease Severity Determinants of riskLower riskHigher riskClinical evidence of RV failureNoYesProgressionGradualRapidWHO functional classII, IIIIV6MWDLonger (>400 m)Shorter (<300 m)BNPMinimally elevatedVery elevatedEchocardiographic findingsMinimal RV dysfunctionSignificant RV dysfunction, pericardial effusionHemodynamicsNormal/near normal RAP and CIHigh RAP, low CIBNP = brain natriuretic peptide; CI = cardiac index; RAP = right artery pressure; RV = right ventricular.McLaughlin and McGoon. Circulation. 2006;114:
24TherapiesThe only group that has been approved for the specialized medications for pulmonary hypertension are Group 1 (pulmonary arterial hypertension/PAH)The other groups require treatment of the underlying condition causing the elevated pressures.
25Therapeutic Options for PAH Traditional therapiesFDA approved for PAHSupplemental O2DiureticsOral vasodilators(CCB)AnticoagulantswarfarinInotropic agentsDigitalisProstanoidsEpoprostenol (flolan/veletri)Treprostinil (IV/SQ/Inhaled)Inhaled IloprostERA’sBosentanAmbrisentanPDE-5 InhibitorsSildenafilTadalafil
26Natural History of PAH: NIH Registry1,2 69%56%46%Percent survival38%Predicted survivalPredicted survival*YearsNIH = National Institutes of Health. Predicted survival according to the NIH equation. Predicted survival rates were 69%, 56%, 46%, and 38% at 1, 2, 3, and 4 years, respectively. The numbers of patients at risk were 231, 149, 82, and 10 at 1, 2, 3, and 4 years, respectively. *Patients with primary pulmonary hypertension, now referred to as idiopathic pulmonary hypertension.1. Rich et al. Ann Intern Med. 1987;107: D’Alonzo et al. Ann Intern Med. 1991;115:
27The Take Home Most crucial aspect: the patient’s history If concerned about PAH or have PAH, call the pulm consult service to help in managementTiming of RHC is essentialIf on PAH specific therapy, Do Not Abruptly Stop