by cardiologist ODAY ABDOW (damascus university)

1. Pulmonary hypertension
Dr ODAY ABDOW
19/12/2018

5. Pulmonary hypertension
definition:
PH is defined as an increase in mean pulmonary
arterial pressure(PAPm) >25 mmHG at rest as
assessed by right heart catheterization(RHC)

9. Classification of pulmonary
hypertension
•pulmonary arterial hypertension(PAH)
•PH due to left heart disease
•PH due to lung disease and or hypoxia
•Chronic thromboembolic PH
•PH with unclear multiple mechanisms

10. PAH (group1)
1-idiopathic
2-Heritable(BMPR2 mutation,other mutations)
3-drug and toxin induced
4-associated with
Connective tissue disease
HIV infection
Portal hypertension
Congenital heart disease
Schistosomiasis
•Pulmonary veno-oclusive disease and or pulmonary capillary
hemangiomatosis
•Persisitent pulmonary hypertension in newborn

11. Pulmonary hypertension due to left
heart disease(group2)
1-Left ventricular systolic dysfunction
2-Left ventricular diastolic dysfunction
3-Valvular disease
4-Congenital acquired left heart inflow outflow tract
obstruction and congenital cardiomyopathies
5-Congenital acquired pulmonary veins stenosis

12. PH due to lung diseases and or
hypoxia(group3)
1-copd
2-Interstitial lung disease
3-Pulmonary diseases with mixed obstructive and
restrictive pattern
4-Sleep disordered breathing
5- alveolar hypoventilation disorders
6-Developmental lung disease

13. Chronic thromboembolic PH and
other pulmonary artery
obstructions (group4)
1-Chronic thromboembolic PH
2-Other pulmpnary artery obstructions:
•Angiosarcoma
•Other intravascular tumors
•Arteritis
•Congenital pulmonary artery stenosis
•parasitis (hydatidosis)

14. PH with unclear and or
multifactorial mechanisms(group5)
1-Hematological disorders(haemolytic anemia
,myeloproliferative disorders,splenectomy)
2-Metabolic disorders(glycogen storage disorders,Gaucher,
thyroid disorders)
3-Systemic disorders(sarcoidosis,pulmonary histiocytosis
,lymphangioleiomyomatosis)
4-others:Pulmonary tumoral thrombotic microangiopathy,
fibrosing mediastinitis, chronic renal failure with or without
dialysis ,segmental pulmonary hypertension)

15. Updated risk level of drugs and toxins
known to induce PAH

16. Clinical symptoms
-Exertional dyspnea
-Fatigue,lethargy
-Exertional chest pain(angina)
-Exertional syncope(inability to
increase cardiac output during
exercise)
-Peripheral edema due toRVfailure
-Dry cough.haemoptysis,hoarseness

18. Physical examination
- Accentuated pulmonary component of S2(audible at
the apex)
- Early systolic click (increases with expiration)
- Pansystolic murmur of TR (increases with inspiration)
- Diastolic murmur of PR(in more severe disease)
- Left parasternal heave
-RV S4
-Prominent A wave (jugular venous pulse)
- Hepatojugular reflux

19. Advanced PH with right
ventricular failure
RV S3
 Distension of jugular veins
Prominent V wave (jugular venous pulse)
 Hepatomegaly,pulsatile liver
 Peripheral edema
 Ascites
 Low BP, narrow pulse pressure, cool extrimeties

20. ECG
ECG is neither sensitive nor specific but can provide
supportive evidence of PH
Normal ECG doesn’t exclude the diagnosis
ECG abnormalities can include:
 P pulmonale
 Right axis deviation
 RVH(sensitivity 55%specificity70%)
 RV strain pattern(more sensitive than RVH)
 RBBB
 QT prolongation
SVT in advanced disease in particular atrial flutter, AF

22. Typical appearance of
RVH:
RAD(+150 degree)
Dominant R wave in V1 (R/s>1)
Dominant S wave inV6(R/S <1)
RS strain pattern ST depression and T wave inversion
in V1-V4

24. RAD
P pulmonale (p wave in lead II >2.5mm)
Incomplete RBBB
RV strain pattern(T wave inversion and st depression
in the right pericordial V1-V3 and inferior II, III, avf
leads

25. Chest radiograph
-In 90% of patients with PAH the chest radiograph is
abnormal at the time of diagnosis
-As forECG normal chest radiograph doesn’t exclude PH
-Findings include:
Central pulmonary arterial dilatation
Pruning of the peripheral blood vessels(oligemic lung
fields)
RA and RV enlargement may be seen
Pulmonary venous congestion (left sided heart disease)
Lung disease(copd , ILD)may be seen

29. Echocardiogram
-TTE should always be performed when PH is
suspected
-The estimation of systolic PAP is based on the peak
TRV(Bernoulli equation)
-RAP can be estimated by TTE based on the diameter
and respiratory variation in diameter of the IVC
-When treatment of PH itself is being considered TTE
alone is not sufficient and RHC is required

31. Echocardiographic probability of PH in
symptomatic patients with suspected PH

32. Echocardiographic signs suggesting PH used to
asses the probapility of PH

34. Recommenadions for right heart catheterization
in pulmonary hypertension
-RHC is recommended to confirm the diagnosis of PAH
(group1)and to support treatment decisions class1c
-RHC is recommended in congenital cardiac shunts to support
decision on correction class1c
-RHC is recommended in PH due to left heart disease (group2)
or lung disease (group3)if organ transplantation is considered
class1c
-RHC is indicated in patients with CTEPH (group4) to confirm
the diagnosis and support treatment decisions class1c
-RHC may be considered in patients with suspected ph due to
lung or heart disease to assisit the differential diagnosis and
treatment decisions classIIb

35. Recommendations for
vasoreactivity testing
-Vasoreactivity testing is recommended in patients with
IPAH,HPAH,PAH associated with drug use to detect patients
who can be treated with high doses of CCB classIc
-Vasoreactivity testing is not recommended in PH groups
2,3,4 and 5 classIII
-A positive response to vasoreactivity testing is defined as a
reduction of mPAP≥ 10 mmHG to reach an absolute of
mPAP≤40 mmHG with an increased or unchanged cardiac
output

37. Risk assesment in pulmonary
arterial hypertension
Evaluation of severity:
-Clinical parameters
-Imaging and haemodynamics
-Exercise capacity
-Biochemical markers

38. Risk assesment in pulmonary arterial
hypertension

39. Management of pulmonary
arterial hypertension
1- general measures
2-supportive therapy
3-specific drug therapy
4-combination therapy
5-transplantation

41. Supportive therapy for PAH
Diuretic therapy is recommended in PAH patients with signs of
RV failure and fluid retention(class1c)
Continuous long term O2 therapy is recommended in PAH
patients when arterial blood O2 pressure is <60mmHG(class1c)
Oral anticoagulant treatment may be considered in patients
with IPAH,HPAH and PAH due to use of anorexigenes(classIIb c)

43. Specific drug therapy
•Calcium channel blockers
•Endothelin receptor antagonists
•Phosphodiesterase type5 inhibitors and
guanylate cyclase stimulators
•Prostacycline analogues and prostacycline
receptor agonists

44. Calcium channel blockers
-High doses of CCB are recommended in patients with
IPAH,HPAH,and DPAH who are responders to
vasoreactivity testing classIc
-High doses CCB are not indicated in patients without
vasoreactivity study or nonresponders unless standard
doses are prescribed for other indications (e.g Raynaud’S
phenomenon)classIII

45. Phosphodiesterase type 5
inhibitors
-Inhibit phosphodiesterase type 5 enyme
resulting in vasodilation through cGMP
pathway
-Sildenafil,tadalafil,vardenafil cause
significant pulmonary vasodilatation>>and
have favourable results on exercise
capacity,symptoms and haemodynamics
-Side effects are related to
vasodilation(headache,flushing,epistaxis)

46. RIOCiGUAT is an oral guanylate cyclase
stimulator and enhance cGMP production
-Benificial in patients with inoperable
CTEPH or persistent/recurrent CTEPH
after surgry
-Can cause hypotension or syncope
-The combination of Riociguate and
PDE-5i is contraindicated due to
hypotension

47. Prostacyclin analogues and
prostacyclin receptor agonists
-Prostacycline is produced predominantly by
endothelial cells and induce potent vasodilatation
of all vascular beds,and a most potent
endogenous inhibitor of platlet aggregation and
have both cytoprotective and antiproliferative
activities
-Dysregulation of prostacycline pathwayshave
been shown in patients withPAH
-These drugs include ((Beraprost -Epoprostenol
Iloprost –Treprostinil- Selexipag))

48. -Intravenous epoprostenol
improves haemodynamics,
functional capacity and survival in
patients with IPAH,
-the only treatment shown to
reduce mortality in IPAH in a single
RCT study
-it has a short half life 3-5 minute it
requires cooling and continuos
infusion by an infusion pump
-side effects include flushing,
headache, diarrhea,leg pain,pump
malfunction,catheter infection,local
site infection
-abrupt interruption of
epoprostenol should be avoided
because it may lead to PH rebound
and deterioration and even death

49. Endothelin receptor antagonists
-Endotheline system has a prominent role in the
pathogenesis of PAH,and activation of the
endotheline systym has been demonstrated in plasma
and lung tissue of PAH patients
-these drugs Improve symptoms .excercise capacity
and haemodynamics
Ambrisentan(elevated liver enzymes up to 3%)
Bosentan( elevated liver enzymes 10%)
Macitentan(no liver toxicity but low haemogloboline
was noted)

50. Drug monotherapy forPAH according to who class

51. Combination therapy

52. Transplantation
-Transplantation should continuo to be an
important option for those who remain WHO-
FC III or IV in spite of receiving specific drug
therapy
-More recent data show that survival is
increased to 52-75% at 5 year and to 45-66%at
10 years

54. The use of PAH approved therapies is not
recommended in PH due to left heart disease or due to
lung disease
Lifelong anticoagulation is recommended in all patients
withCTEPH
It is recommended that in all patients with CETPH the
assesment of operability should be made by a
multidisciplinary team of experts
Surgical PEA in deep hypothermia circulatory arrest is
recommended for patients with CTEPH

55. references
-ESC GUIDELINES 2015 PULMONARY
HYPERTENSION
-BRAUNWALD’S HEART DISEASE
-MNANUAL OF CARDIOVASCULAR MEDICINE
-- MEDSCAPE