jefferson heart institute 925 chestnut street. referring physician name of physician office address...
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Jefferson Heart Institute925 Chestnut Street
Referring Physician
Name of physician
Office Address
Pride
Communication
More referrals
“Carbon Copy”
Interested physicians
Office Address
Pride
Communication
More referrals
Reason for the visit (a.k.a., chief complaint)
Reason for the visit
Pulmonary hypertension evaluation
Elevated pulmonary artery pressure by echocardiogram
Elevated pulmonary artery pressure by right heart catheterization
History of Present Illness
W.H.O. Functional Class
W.H.O. Group
Drugs trialed and response to therapy
Relevant family history
Relevant testing
Symptoms of PH
• Dyspnea Fatigue
• Syncope Edema
• Dizziness Chest Pain
Non-specific nature of complaint can lead to:• Confusion with other conditions
• Delayed diagnosis
Gaine et al. Gaine et al. The Lancet,The Lancet, 1998. 1998. 352; 719352; 719
W.H.O. Functional ClassificationClass I: Patients with PH but without resulting limitation of physical activity.
Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain or near syncope.
Class II: Patients with PH resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnea or fatigue, chest pain or near syncope.
Class III: Patients with PH resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary physical activity causes undue dyspnea or fatigue, chest pain or near syncope.
Class IV: Patients with PH with inability to carry out any physical activity without symptoms. These patients manifest signs of right heart failure. They are comfortable at rest. Dyspnea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity. (Syncope)
W.H.O. Classification - 2008
1. Pulmonary arterial hypertension (PAH) 1.1 Idiopathic1.2 Heritable1.2.1 BMPR21.2.2 ALK1, endoglin (with or without hereditary hemorrhagic telangiectasia)1.2.3. Unknown1.3 Drug- and toxin-induced1.4 Associated with1.4.1 Connective tissue diseases1.4.2 HIV1.4.3 Portal hypertension1.4.4 Congenital Heart Diseases1.4.5 Schistosomiasis1.4.6 Chronic hemolytic anemia1.4 Associated with significant venous or capillary involvement1.5 Persistent pulmonary hypertension of the newborn1’. Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary
hemangiomatosis (PCH)
2. Pulmonary hypertension owing to left heart disease 2.1 Systolic dysfunction2.2 Diastolic dysfunction2.3 Valvular disease
3. Pulmonary hypertension owing to lung diseases and/or hypoxemia 3.1 Chronic obstructive pulmonary disease3.2 Interstitial lung disease3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern3.4 Sleep disordered breathing3.5 Alveolar hypoventilation disorders3.6 Chronic exposure to high altitude3.7 Developmental abnormalities
4. Chronic thromboembolic pulmonary hypertension (CTEPH)
5. Pulmonary hypertension with unclear multifactorial mechanisms5.1 Hematologic disorders: myeloproliferative disorders, splenectomy5.2 Systemic disorders: sarcoidosis, Langerhans cell histiocytosis: lymphangioleiomymatosis , neurofibromatosis, vasculitis5.3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders5.4 Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis
W.H.O. Classification - 2008
Past Medical History
W.H.O. Group 1
Heritable
Collagen vascular disease
HIV
Portal hypertension
Anorexigenic agents
Hemoglobinopathies
Past Medical History
W.H.O. Group IILeft heart disease
W.H.O. Group IIILung diseases and/or hypoxemia
W.H.O. Group IVChronic thromboembolic PH
W.H.O. Group V Unclear multifactorial mechanisms
Heritable pulmonary arterial hypertension
Dresdale, 1953 reported family
NIH Registry, 1987: 6% with one or more affected family members
Autosomal dominance
Fetal wasting
Genetic anticipation
Incomplete penetrance
Idiopathic PAH - Epidemiology
• Female• Young to middle age• NIH Registry, 1991
Distribution of patients with pulmonary arterial Distribution of patients with pulmonary arterial hypertension according to age based on sexhypertension according to age based on sex
Pulmonary Arterial Hypertension in France Pulmonary Arterial Hypertension in France
Results from a National Registry Results from a National Registry
Am. J. Am. J. RespirRespir. Crit. Care Med. Crit. Care Med 2006,2006, 173:1023173:1023
Idiopathic PAH - Epidemiology
Shear stress from increased pulmonary blood flow
Increased pulmonary artery pressures
Majority of unrepaired truncus arteriosus develop PH
Large VSD, 50% develop PH
ASD, 10% develop PH
Eisenmenger’s syndrome
Medical or surgical therapy effective
Congenital Heart DiseaseEpidemiology
Connective Tissue Diseases Epidemiology
-Limited systemic sclerosiso SLE, MCTD, RA, Sjogren’s
Survival Curves of Scleroderma Patients with Pulmonary Survival Curves of Scleroderma Patients with Pulmonary Hypertension, Lung Involvement, or No Major Organ InvolvementHypertension, Lung Involvement, or No Major Organ Involvement
Su
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PHPH
NoneNone
Lung Involvement Lung Involvement (without PH)(without PH)
Years from Diagnosis of PHYears from Diagnosis of PH
0
10
20
30
40
50
60
70
80
90
100
1 2 3 4 5 6 7 8 9 10 11 12 130
KohKoh, et al. , et al. Br J Br J RheumatolRheumatol. 1996;35:989. 1996;35:989--993993
HIVEpidemiology
0.5% prevalence– no decline since HAART
Occurrence depends upon length of infection, not CD4 count or prior opportunistic infections
Portopulmonary Hypertension Epidemiology
-2-6% prevalence in cirrhotics, higher in liver transplant candidates (8%)-Risk increases with duration of portal hypertension-High cardiac flow states and LV diastolic dysfunction complicate PH
Le Pavec J et al. Am J Respir Crit Care Med 2008
Portopulmonary Hypertension: Survival -21stCentury
Pulmonary Hemodynamic Scenarios in the Setting of Portal Hypertension
Type MPAP PAOP CO PVR TPG
I. Hyperdynamic, high flow state ↑ n ↑ ↓ n
II. Increased pulmonary venous vol. ↑ ↑ ↑ ↓ n
III. Portopulmonary hypertension
Pulmonary vascular obstruction; normal volume ↑ ↓ ↑ ↑ ↑
Pulmonary vascular obstruction; ↑ ↑ ↑ ↑ ↑ excess volume
M Krowka. Medscape Cardiology 2006
Associated Drugs and ToxinsEpidemiology
Definite:
Appetite suppressant drugs (anorexigens)Fenfluramine and dexfenfluramineAminorex Toxic Rapeseed Oil
Likely:
L-tryptophanMethamphetamineCocaine
HemoglobinopathiesEpidemiology
Sickle cell disease
• PH 10-30%• Yearly echocardiogram• 50% - 2 year mortality with PH• Thromboembolic disease• Restrictive pulmonary disease• Left heart disease
Homozygous beta-thalassemia
Hereditary spherocytosis
Chronic ThromboEmbolic Pulmonary Hypertension (CTEPH)
Cumulative Incidence
Historically considered rare:
0.1-0.5% of acute, non-fatal pulmonary embolism:Fedullo PF et al. N Engl J Med 2001
6 months 1.0%
12 months 3.1%
24 months 3.8%
Pengo V, et al NEJM 2004
Past Surgical History
Lung resection
Thyroidectomy
Splenectomy
CABG
Cardiac valve repair/replacement
Repair of congenital heart defect
Family History
Pulmonary hypertension
CHF/sudden cardiac death at a young age
Sarcoid
Connective tissue disorders
Clotting disorders – DVT, PE, CVA
Social History
TobaccoETOHRecreational Drugs methamphetamines
CocaineIVDU
Prescription diet pillsBush teaPets (birds)
Social Network – “Friends and Family”, not “Verizon”
Allergies /Adverse Reactions
Beware of hypotensive response to vasodilators
CCBs
Nitrates
Sildenafil
Medications
Nitrates
Calcium channel blockers
Warfarin
Beta blockers
Oxygen
Diuretics
Digoxin
Medications
ERAs (Bosentan)Hepatotoxins – monitor LFTs, Hgb
Glyburide may increase risk of hepatoxicity
Cyclosporine
Ketoconazole
Statins
Warfarin
(Ambrisentan*)
Medications
PDE V Inhibitors
• Sildenafil (Revatio)
• Tadalafil (Adcirca)
Medications
Prostacyclins
Parenteral Route of administration Inhaled
ng/kg/minute mcg/dose
ml/24 hrs frequency
Inhaled Iloprost (Ventavis)
25
TYVASO Inhalation System
1. Inhalation device assembled
2. Measuring cup 3. One inhaled treprostinil ampule
4. Carrying case provided
Device base
Inhalation piece
Mouthpiece
Filter shell
Review of Systems
Neurologic: Headache, prior TIA/CVA, lightheadedness or syncope
HEENT: Epistaxis, dry eyes, dry mouth, oral ulcers.
CV: Anginal quality chest pain, orthopnea, PND, palpitations, peripheral edema
Rheumatologic: Joint pain or swelling, Raynaud’s phenomenon
GU/Gyn: Hematuria, Gravida : Para (spontaneous abortions)
GI: Liver disease, dysphagia, heartburn, hematemesis, varices or hemorrhoids, ascites
Review of Systems
Hematologic/Lymphatic: DVT, PE, CVA, TIA (hypercoaguable state) swollen lymph glands (Sjogren’s, sarcoid)
Dermatologic: Rashes, skin lesions, painful ulcers on fingertips.
Psychiatric: Confusion, memory loss, depression, anxiety
HIV risk factors: Blood transfusions, known contacts, HIV serum test (date)
Sleep: Snoring, witnessed apneas, restless sleeper, awakens unrefreshed, daytime hypersomnolence
Physical Examination
Jugular venous distention at 45o
Widened split S2
Loud pulmonic valve closure (P2)
TR murmur
Right ventricular heave, PA pulsation
Enlarged, pulsatile liver – hepatojugular reflux
Peripheral edema, ascites
Skin/Mouth:Telangiectasias, spider hemangiomas
*Infusion catheters and site problems
Barst, R. J. et al. J Am Coll Cardiol
2004
Guidelines for evaluating pulmonary hypertension
Laboratory evaluation
LV
RV
LARA
IVS
INCREMENTAL SHUTTLE WALK TEST (ISWT)
CPT code (pulmonary stress testing): 94620 ISWT: mls/JPA (7/09) page 1/1 Date ___/____/_____ Ordering physician M. Scharf Patient name _____________________________ Date of birth _____________ Patient indices Pre-test Post-test BP ______ mmHg ______ mmHg HR ______ /min ______ /min Dyspnea ______ ______ (Borg scale, 0-10) Fatigue ______ ______ (Borg scale, 0-10) SpO2 ______% ______% Supplemental O2:
____ No ____ Yes, ____ L/min ISWT results Number of laps ______ x 10 m + _____ m (partial lap) = _____ m, total distance shuttled Lap counter: place a vertical line below corresponding for each 10 m lap shuttled Symptoms experienced during testing ____ none ____ yes ____ angina ____ lightheadness/dizziness ____ hip/leg/foot pain ____ other, explain ____________________________________________ Interpretation No Mild Moderate Severe Exercise limitation Exercise-induced hypoxemia: ______Yes ______ No Reviewing physician M. Scharf Reviewing physician signature (on file)
Patient name ___________________________ Physican(s) requested: Fischman, Savage, or Wolinsky Date of birth __________________________ Date of order _______________________________
Cardiac Catheterization Evaluation for
Pulmonary Artery Hypertension
o Right heart catheterization only Diagnostic, To detect PAH at rest
o Right heart catheterization with exercise Diagnostic, (PA mean <= 25 mmHg and Pcw<=18 mmHg) To detect PAH with exercise
o Exercise challenge
o Right heart catheterization Assess vasodilator (PA mean > 25 mmHg and Pcw <= 18mmHg): responsiveness
o inhaled NO protocol
o Right heart catheterization with To assess for occult diastolic (PA mean >25 mmHg and 12< Pcw <= 18 mmHg) diastolic dysfunction in PH
o Exercise or NS fluid challenge
o Right heart catheterization with “O2 sat run” Assess for shunt
o Left heart catheterization
o Measure LVEDP To confirm Pcw accuracy (At discretion of cath physician)
o Coronary angiogram CAD
o LV gram Pump, valves
Ordering physician ___________________________
Signature of physician ________________________
Cardiac Cath Evaluation for PAH. MLS/Jefferson Pulmonary Associates 5/10
Impression
1. (Suspected) Pulmonary (arterial) hypertension (with/without) right ventricular dysfunction due to __________: W.H.O. Functional Class 1 - 4
2. Other conditions contributing to cardio-respiratory complaints
Recommendations and Plan
Defend you reason for your assessmentDefend your reason for testing orderedDefend your reason for medical therapy
One paragraph per “Impression #”
Write so that the referring doctor will understandWrite so you may use this note as a reminder for what
you wish to do at next visit.Jefferson Heart Institute – follow-up
Tools for Patients
Cardiology nurses for RHC teaching
PH literature tearouts
Teaching aides – RHC booklets
Parenteral infusion CDs
Inhalation device models
Tools for Fellows/Faculty
• Pulmonary order sheets– Sleep lab
– Pulmonary function lab
PH Reading List
Projects
• ISWT - PAH– retrospective: ongoing
– prospective: needs design
– other groups, e.g. pre-op assessment – needs collaboration with Surgery
• CT evaluation of PAH and RVD in subjects with PH W.H.O. Group 1 – collaboration with Radiology: ongoing
• Echocardiographic evaluation of RV function in PH subsets – collaboration with cardiology, ongoing.
• PROSPECT Registry
