Pulmonary vascular disease / Pulmoary hypertension

Pulmonary hypertension / Therapies medical surgical / Grades / secondary pulmonary hypertension / ECG cor pulmonale / Diagnostic testing / Catheterization

1. Pulmonary Vascular Disease
By: Darayus P. Gazder

2. Pulmonary vascular diseases
Pulmonary vascular diseases are a
heterogeneous group of disorders with
multiple causes. Pulmonary vascular
disorders are caused by conditions that
directly affect the pulmonary vessels, as in
idiopathic pulmonary arterial hypertension
(IPAH), or by disorders outside of the lung, as
in pulmonary hypertension associated with
lung disease and hypoxemia.

3. Review of Respiratory CirculationReview of Respiratory Circulation
The pulmonary circulation consists of arteries, capillaries The pulmonary circulation consists of arteries, capillaries
and veins.and veins.
The major role of the pulmonary circulation is to bring blood The major role of the pulmonary circulation is to bring blood
in to close proximity to air, so that gas exchange can occur.in to close proximity to air, so that gas exchange can occur.
The pulmonary vascular bed receives the entire cardiac output.The pulmonary vascular bed receives the entire cardiac output.
-high volume/low pressure system. -high volume/low pressure system.
Pulmonary Circulation:Pulmonary Circulation:
Bronchial Circulation:Bronchial Circulation:
•The bronchial arteries typically originate off aorta and supplyThe bronchial arteries typically originate off aorta and supply
airways (2% of cardiac output).airways (2% of cardiac output).
-low volume/high pressure.-low volume/high pressure.
•1/3 of blood flow through the bronchial circulation empties into 1/3 of blood flow through the bronchial circulation empties into
azygous vein.azygous vein.
•2/3 of blood flow empties into pulmonary capillaries (broncho-2/3 of blood flow empties into pulmonary capillaries (broncho-
–pulmonary anastamoses).pulmonary anastamoses).

4. Pulmonary Hypertension
Which is defined as mean pulmonary artery
pressure over 25 mm Hg at rest or over 30
mm Hg with exercise.
In Pulmonary Hypertension there is:
1. Enlarged proximal pulmonary arteries,
2.Right ventricular hypertrophy
3.Right atrial dilation

5. Chamber Pressures in PAH
55
>30>30
Mean >24 at rest, >30 with exercise
PCWP <15 mmHg

6. Pathophysiology of PHTN
Hypoxemia causes vasoconstriction
Decreased perfusion worsens hypoxemia
Hypoxic alveoli cause arterioles to
vasocontrict further to preserve VQ matching
Pulmonary pressures increase and become
sustained
Right ventricle works harder, develops
hypertrophy and overload

7. Physiologic Effects of PAH

8. Current WHO Classification

9. Group 1 PAH
Connective tissue diseases
12% SSc have PAH
HIV infection
0.5% have PAH
Portal hypertension
2-6% have PAH
Congenital heart disease
Chronic hemolytic anemia
Pulmonary veno-occlusive disease
Pulmonary capillary hemangiomatosis
Idiopathic (BMPR2 gene abnormality)

10. Group 2
Pulmonary Hypertension (PH)
Group 2
Due to left heart
disease
systolic dysfunction
diastolic
dysfunction
valvular heart
disease

11. Group 3 PH
Lung Diseases or Hypoxemia
COPD
interstitial lung disease
(ILD)
sleep-disordered breathing
(OSA 15% have PAH)
alveolar hypoventilation
disorders (OHV)
other causes of hypoxemia
Central
OHV
COPD/ILD
CHF
OSA

12. Group 4 PH
Chronic
Thromboembolic
Disease

13. Group 5 PH
Secondary to unclear
multifactorial causes
hematologic
(myeloproliferative disease)
systemic disorders
(sarcoidosis)
metabolic disorders
(glycogen storage disease)

15. Pathogenesis of Pulmonary HTN

16. Primary pulmonary hypertension
Defined as Pulmonary htn in the absence of
other disease of lungs or heart.
Develops: Young and middle aged women
Characterized by right heart failure, leading to
death in 2-8 yrs.
B/c of low cardiac output:
1.Weakness, fatigue, edema, ascites. Syncope

17. On Examination
Inspection:
1.Prominent JVP
2.Cyanosis
Palpation:
1.Left parasternal heave due to RVH
2.Systolic pulsation in 2
nd
ICS due to dilated
pulmonary artery

18. Auscultation:
1.Loud pulmonary component of 2
nd
heart sound
2.Systolic ejection click and flow murmur in
pulmonary area
3.Right ventricular S4
4.Advanced cases, tricuspid and pulmonary
regurgitation and signs of cor pulmonale are
found

19. Studies in Pulmonary HypertensionStudies in Pulmonary Hypertension
ECG: Right Ventricular Hypertrophy.
CXR: Dilated main pulmonary arteries/pruning of
peripheral vascular markings.
ABG: Hypoxemia with exertion.
PFT’s: Findings c/w underlying disease;
Decreased DLCO.
Echocardiogram:
Right heart catheterization:
Pulmonary Pressure MeasurementPulmonary Pressure Measurement

20. Diagnostic Tests: CXR
Enlarged right
ventricle and
pulmonary arteries.

21. Diagnostic Testing: EKG
Right-axis
deviation
R/S>1 in V1
Deep S-wave in V5,
V6
Tall P-wave in lead
II
Right bundle
branch block

22. Diagnostic Tests
Doppler echocardiogram

23. Diagnostic Tests: CT Scan
Pulmonary emboli
Interstitial lung
disease
PA/Ao > 2/3 can
indicate PHTN

24. Diagnostic Testing
Ventilation perfusion scan (V/Q)
Q: Perfusion V: Ventilation

25. Diagnostic Testing:
Right Heart Catheterization
Heart pressures
RA, RV, PA, LVEDP
Cardiac output
Valve competence
Septal defects

26. Diagnostic Testing
Pulmonary Angiography

27. Risk Factors for Poor Prognosis
Age at presentation > 45 years
Functional class III or IV
Pericardial effusion
Large right atrial size
Elevated right atrial pressure
Septal shift during diastole
Increased BNP level
Failure to improve to a lower functional
class during treatment

28. Diagnostic Approach to Pulmonary HypertensionDiagnostic Approach to Pulmonary Hypertension
History and Physical Exam often suggestive.
ECG and echocardiogram: elevated pulmonary
pressures.
Right heart catheterization ± pulmonary angiography.
Identify treatable causes of secondary pulmonary
hypertension.
Hypoxemia (at rest or at night, with sleep apnea).
Chronic Thromboembolic Disease.

29. Routine Medical Treatments
Continuous oxygen
Pulmonary artery vasodilator
Anticoagulants
Treat and prevent thrombosis
Diuretics / Low Salt Diet
Decrease RA pressures
Treat right heart failure
Digoxin
Increases cardiac output

30. Routine Medical Treatments
Calcium channel blockers
Lowers pulmonary artery pressure
May improve right heart cardiac output
Exercise
Immunization
Prevent pulmonary infections
Opportunistic Infection Prophylaxis
Patients on immunosuppression (ILD)

31. Surgical Therapies
Pulmonary thromboendarterectomy
Correct mitral stenosis
Repair left to right shunt (ASD, VSD)
Atrial septosotomy to relieve RHF
when PA pressure exceeds systemic pressure
Lung transplantation
for advanced PHTN not responsive to medical therapy

~1,000 lung transplants/year in US
Heart / lung transplantation

32. Who Should Receive Advanced Medical
Therapy?
Group 1 (PAH) rarely respond to primary
therapies
Group 2 (cardiac abnormalities) usually
worsen with advanced therapies
Group 3 (COPD) respond to oxygen
therapy
Group 4 (thromboembolic) respond to
thrombectomy and anticoagulation
Group 5 unknown effects of advanced
therapies, primary therapy indicated

33. Treatment of Pulmonary HypertensionTreatment of Pulmonary Hypertension
Treat underlying disease.Treat underlying disease.
Oxygen supplementation- minimize hypoxic Oxygen supplementation- minimize hypoxic
vasoconstriction.vasoconstriction.
Long term anticoagulation (even when not due to chronic PE).Long term anticoagulation (even when not due to chronic PE).
Vasodilators especially for primary pulmonary hypertension:Vasodilators especially for primary pulmonary hypertension:
Calcium channel blockers.Calcium channel blockers.
Prostacyclin.Prostacyclin.
Endothelin receptor blockers (Bosentan).Endothelin receptor blockers (Bosentan).
TransplantationTransplantation

34. Secondary pulmonary HTN
Hypoxia of any cause is the most important
stimulus of Pulmonary arterial vasoconstriction
leading to pulmonary HTN
Chronic lung disease
Chronic bronchitis/ Emphysema /Lung fibrosis
Increased pulmonary blood flow:
Left or right shunt: ASD, VSD, PDA
Increased pulmonary venous pressure:
Mitral stenosis/ Left ventricular failure/ Mitral
regurgitation/ Pulmonary thromboembolic disease

35. Treat the cause
Supplemental Oxygen for atleast 15hrs/ day
in patients with COPD
Inhaled NO
Anticoagulation: To dissolve small pulmonary
emboli

36. Cor Pulmonale
Pulmonary hypertension complicating lung
disease
Right ventricular hypertrophy
Right ventricular dilatation
Right heart failure (swollen legs, congested
liver etc)
Causes:
COPD/ Pneumoconiosis/ Pulmonary fibrosis/
Primary pulmonary hypertension

37. Clinical features
Dyspnea/ Cyanosis/ Clubbing/ Weakness
Features of Right heart failure:
1.Raised JVP
2.Right ventricular heave
3.Murmurs of pulmonary and tricuspid
regurgitation maybe present

38. Investigations:
CXR: Right ventricular enlargement and right
atrial dilation. Prominent pulmonary artery
ECG: Right ventricular hypertrophy
demonstrates right ventricular hypertrophy (right
axis deviation, possibly a dominant R wave in
lead V1, and inverted T waves in right
precordial leads) and a right atrial abnormality
(tall peaked P waves in lead II)
ECHOCARDIOGRAPHY:
Right ventricular dilation

39. Chest radiography
Signs of pulmonary hypertension:
Enlarged right descending pulmonary artery with diameter
≥15mm
The ratio of the diameter of right descending pulmonary
artery to trachea≥1.07
Bulge of pulmonary artery/with the height≥3mm
dilation of the main pulmonary artery and its branches
with concurrent underperfusion of the peripheral branches
Signs of right ventricular enlargement
Signs of underlying diseases and infection

41. Electrocardiography of Chronic Cor Pulmonale

42. Treatment
Treat the cause
Diuretics
Oxygen
Digoxin
ACE inhibitor
Heart and lung transplantation in cases of
young patient

43. THANK-YOU