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benfluorex
Likely RF: amphetamine, methamphetamine, L-tryptophan, dasatinib
Possible RF: Cocaine, Amphetamine-like drugs (i.e., phenylpropanolamine), St.
Johns wort, several ChemoThx agents, IFN- and IFN-
o Disease-associated
CHD Prevalence 5-10%
CT Dz
MC: Systemic sclerosis Prevalence 9%
Other: Mixed CT Dz, SLE, RA, Sjogren
Portal hypertension Prevalence 2-6%
HIV Prevalence 0.5%
Schistosomiasis mansoni
Autoimmune thyroid dz
Pathogenesis: Multifactorial
o Increased pulmonary vascular resistence occurs 2/2 vasocontriction, inflammation,
remodeling of the pulmonary artery wall via obstruction and proliferation, and
thrombosis, resulting in:
Abnormal function or expression of K+ channels in smooth mm. cells
Endothelial dysfunction
Impaired nitrous oxide and prostacycle production causes decreased
History
o Sx: dyspnea, fatigue, weakness, angina, near syncope or syncope, abdominal
distention, chest pain, edema, palpitations
o HPI
Progressive SOB
Exertional chest pain may occur due to hypoperfusion during exertion
Exertional dizziness and/or syncope may develop 2/2 RV dysfxn
Late findings: peripheral edema and ascites
o Medication Hx: anorectics (aminorex, fenfluramine, dexfenlafurine, benfluorex),
rapeseed oil
o Social Hx: Amphetamine, methamphetamine and cocaine abuse
o Sexual Hx: HIV RF
Physical Exam
o Early dz: possibly unremarkable
o Lungs
Mostly normal
May find inspiratory crackles (indicative of ILD)
o CV
Parasternal lift at LUSB (pulmonary tap)
Significant pulmonic valve component of S2
TR (holosystolic murmur over the LLSB)
PR (early diastolic murmur at LUSB; Graham Steell murmur)
JVD
S3
o Abdomen
Possible hepatomegaly and ascites in advanced dz
o Extremities
Signs of scleroderma: Telangiectasia, digital ulcers, sclerodactyly, calcinosis
Digital clubbing is typically not a/w PAH
Peripheral edema with cool extremities in advanced disease
Diagnosis
o Suspect PAH in patients with unexplained exertional SOB without S/Sx of respiratory
disease or left heart disease
o Screening test: TEE (often Dx unexpectedly by TTE for a different indication)
Not suitable for ASx or mild PAH
Can determine probability based on Peak TRV (tricuspid regurgitation velocity)
PROBABILITY OF PH
High
Intermediate
Low
PEAK TRV
> 3.4 m/s
2.9 3.4 m/s
2.9 3.4 m/s
2.8 m/s
2.8 m/s
Additional findings
RV to LV diameter ration > 1
Flattening of IVS
ADDITIONAL FINDINGS
Absent
Present
Absent
Present
Absent
Imaging
o CXR: can assist in ruling out lung disease
Central pulmonary Aa dilatation
Pruning of peripheral blood vessels
Advanced disease may show RA and RV enlargement, with loss of retrosternal
airspace on lateral view 2/2 RV enlargement
o CT: consider for all PH
o V/Q scan: recommended for unexplained PH
Labs
o ABG
o PFTs
Treatment
o Supervised exercise rehabilitation in physically deconditioned patients with PAH
Low intensity exercise may increase peak O2 consumption in PAH
Structured exercise training may increase 6-minute walking distance in stable,
chronic PH
o Vasoreactivity testing
Can identify if patients specifically with idiopathic, heritable or drug-induced PAH,
o CCBs: indicated for WHO Functional Classes II and III who have a positive response to
vasoreactivity testing; Start low, go slow
Initial doses
Slow release Nifedipine 30 mg BID
Diltiazem 60 mg TID
Amlodipine 2.5 mg QD
Increase cautiously and progressively to maximum tolerated dose
Nifedipine 120-240 mg per day
Diltiazem 240-720 mg per day
Amlodipine 20 mg per day
For relative bradycardia: nifedipine or amlodipine (peripheral DHP CCBs)
For relative tachycardia: diltiazem (central Non-DHP CCBs)
o If CCB are contraindicated OR Sx persist despite CCB Thx
WHO Functional Classes II III: Ambrisentan + Tadalafil
Prostanoids: WHO Functional Classes III and IV
Continuous IV Epoprostenal/Prostacycline/PGI2: may improve short-term
exercise capacity
Inhaled iloprost: May improve exercise capacity and functional status in
advanced disease
Subcutaneous trepostinil (remodulin): may improve exercise capacity
Oral treprostinil (orenitram) MonoThx: may improve walking distance
Endothelin receptor antagonists: WHO Functional Classes II, III, and IV
Bosentan, ambrisentan, macitentan
Improve exercise capacity and dyspnea; may improve functional class
Phosphodiesterase inhibitors: WHO Functional Classes II, III, and IV
Sildenafil, tadalafil, vardenafil
Improve exercise capacity
Soluble Guanylate Cyclase stimulators: WHO Functional Classes II and II
Complications
o RV Dysfxn/RHF
o Ascites
o Tachyarrhythmias (Afib and Aflutter)
o Comborbidities a/w increased mortality
DM2, COPD, CT Dz (especially systemic sclerosis)
o Biochemical markers associations
Increased serum uric acid a/w increased mortality
Decreased BNP a/w decreased mortality