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PH: Who Ya Gonna Call Pulm Consults

PH: Who Ya Gonna Call Pulm Consults. Lana Melendres Pulm /CC Division Pulm Htn Program Director 4/24/13. Objectives. Define pulmonary hypertension (PH ) and pulmonary arterial hypertension (PAH) Identify the 5 different groups of PH When to call a lifeline ( pulm consult)

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PH: Who Ya Gonna Call Pulm Consults

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  1. PH: Who YaGonna CallPulm Consults Lana Melendres Pulm/CC Division PulmHtn Program Director 4/24/13

  2. Objectives • Define pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH) • Identify the 5 different groups of PH • When to call a lifeline (pulm consult) • Review the workup for PH • Making the diagnosis • Therapeutic options

  3. Case #1 • 35yo 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

  4. Case #2 • 29yo 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.

  5. Case #3 • 46yo 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)

  6. Case #4 • 65yo 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.

  7. Case #5 • 47yo man with SOB that has been progressive for the past several months. Previously able to ride his bike 100-150 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.

  8. Case #6 • 83yo 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.

  9. Case #7 • 52yo 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.

  10. Normal Cardiac Hemodynamics

  11. Diagnostic Definition: Pulmonary Hypertension Rest: - Mean PAP >25 mmHg PAH = above + PCWP or LVEDP <15 mmHg Associated 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 mmHg Gaine et al. The Lancet, 1998.

  12. New Proposed Classification of Pulmonary Hypertension (Dana Point, 2008) • 3. Pulmonary hypertension due to lung diseases and/or hypoxia • 3.1 Chronic obstructive pulmonary disease • 3.2 Interstitial lung disease • 3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern • 3.4 Sleep-disordered breathing • 3.5 Alveolar hypoventilation disorders • 3.6 Chronic exposure to high altitude • 3.7 Developmental abnormalities • 4. Chronic thromboembolic pulmonary hypertension (CTEPH) • 5. PH with unclear multifactorial mechanisms • 5.1 Hematologic disorders: myeloproliferative disorders splenectomy. • 5.2 Systemic disorders, sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitis • 5.3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders • 5.4 Others: tumoral obstruction, fibrosingmediastinitis, chronic renal failure on dialysis. 1. Pulmonary Arterial Hypertension 1.1 Idiopathic PAH 1.2 Heritable 1.2.1. BMPR2 1.2.2. ALK1, endoglin (with or without hereditary hemorrhagic telangiectasia ) 1.2.3 Unknown. 1.3 Drug- and toxin-induced 1.4 Associated with 1.4.1. Connective tissue diseases 1.4.2 HIV infection 1.4.3 Portal hypertension 1.4.4 Congenital heart diseases 1.4.5 Schistosomiasis 1.4.6 Chronic hemolyticanemia 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 disease

  13. Back 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.

  14. Pulmonary Arterial Hypertension • Case #1: IPAH • Case #2: PAH associated with HIV

  15. PAH • Case #3: PAH assoc with drugs/toxins • Case #4: PAH assoc with CTD

  16. PAH • Case #5: PVOD • Case #6: PAH assoc with CHD • Case #7: PAH assoc with portal htn

  17. When to Call Pulmonary • Definite Consult: • Patient with known PAH by RHC, on PAH specific therapies • All patients followed by the PH clinic. • Consider Consult: • Patient with echo findings of elevated PASP: • No known CTD, PASP >40mmHg • Known CTD, PASP >35mmHg

  18. Pulmonary Arterial Hypertension: Detection and Diagnosis Is there a reason to suspect PAH Clinical history (symptoms, risk factors, family Hs.), Exam, CXR, ECG yes no Rationale Is PAH likely? Echo TRV to measure RVSP; RVE; RAE; RV Dysfunction: No further evaluation for PAH no yes yes Is PAH due to LH disease? Echo Dx LV systolic, diastolic dysfunction; valvular disease: Appropriate treatment and further evaluation if necessary, including R&LHC no Dx abnormal morphology; shunt: Surgery. Medical treatment of PAH or evaluation for further definition or other contributing causes, including R&LHC if necessary yes Is PAH due to CHD? Echo with contrast no Dx Scleroderma, SLE, other CTD, HIV: Medical treatment of PAH and further evaluation for other contributing causes, including RHC yes Is PAH due to CTD, HIV? Serologies no Is chronic PE suspected? VQ scan McGoon et al. Chest 2004;126:14S-34S

  19. Pulmonary Arterial Hypertension:Detection and Diagnosis Is chronic PE suspected? VQ scan yes Is chronic PE confirmed and operable? Pulmonary angiogram yes no VQ normal Anatomic definition (CT, MRI may provide additional useful but not definitive information): Thromboendarterectomy if appropriate or medical treatment; clotting evaluation; a/c Is PAH due to lung disease or hypoxemia? PFTs, arterial saturation no yes Dx parenchymal lung disease, hypoxemia, or sleep disorder: Medical treatment, oxygen, positive pressure breathing as appropriate, and further evaluation for other contributing causes, including RHC if necessary no Document exercise capacity regardless of cause of PH: Establish baseline, prognosis and document progression/ response to treatment with serial reassessments What limitations are caused by the PAH? Functional class; 6-minute walk test Document PA and RA pressures, PCWP (LV or LA pressure if PCWP unobtainable or uncertain), transpulmonary gradient CO, PVR, SvO2, response to vasodilators: Confirm PAH, or IPAH if no other cause identified Discuss genetic testing and counseling of IPAH What are the precise pulmonary hemodynamics? RHC McGoon et al. Chest 2004;126:14S-34S

  20. NYHA Classification

  21. Right Heart Catheterization is the Diagnostic Gold Standard • Saturations • Rule Out Shunts • Intra-cardiac • Intra-Pulmonary • Hemodynamics • RAP • mPAP • PCWP • Rule out left sided heart disease • CO/CI • PVR • Angiography • Vessel properties • CTEPH • Vasodilator Response RHC can also Prognosticate! Rich et al. WHO Symposium on PPH. Evian, France,1998.

  22. Determinants of Disease Severity BNP = brain natriuretic peptide; CI = cardiac index; RAP = right artery pressure; RV = right ventricular. McLaughlin and McGoon. Circulation. 2006;114:1417-1431.

  23. Therapeutic Pathways

  24. Therapies • The 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.

  25. FDA approved for PAH Therapeutic Options for PAH Traditional therapies Supplemental O2 Diuretics Oral vasodilators (CCB) Anticoagulants warfarin Inotropic agents Digitalis Prostanoids Epoprostenol(flolan/veletri) Treprostinil(IV/SQ/Inhaled) Inhaled Iloprost ERA’s Bosentan Ambrisentan PDE-5 Inhibitors Sildenafil Tadalafil

  26. 69% 56% 46% 38% Natural History of PAH: NIH Registry1,2 Percent survival Predicted survival Predicted survival* Years NIH = 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;107:216-223. 2. D’Alonzo et al. Ann Intern Med. 1991;115:343-349.

  27. The Take Home • Most crucial aspect: the patient’s history • If concerned about PAH or have PAH, call the pulmconsult service to help in management • Timing of RHC is essential • If on PAH specific therapy, Do Not Abruptly Stop

  28. Questions?

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