1. Pulmonary Hypertension (PH)
By: Ahmad Al-Masri

2. Definition
defined as mean pulmonary artery pressure of ≥ 25 mmHg at rest, as measured at right heart catheterisation.
Normal mean pulmonary artery pressure?!
15 mm Hg

3. Classifications Pulmonary arterial hypertension
Pulmonary venous hypertension
PH caused by respiratory disorders & /or hypoxaemia
PH caused by chronic thromboembolic disease
Miscellaneous

4. Pulmonary arterial hypertension
found in:
75% of the familial cases
25% of sporadic cases
Primary (Idiopathic) :
sporadic & familial… (BMPR2 gene)!!
Typically young female (20-30)
Secondary:
connective tissue disease (systemic sclerosis)
congenital systemic to pulmonary (left to right) shunts (VSD,ASD,PDA)
portal hypertension
HIV infection
various drugs or toxins
persistent pulmonary hypertension of the newborn

5. Worsening the Pulmonary hypertension
Cont.
Pulmonary arterial hypertension will cause damage to Endothelial cells in pulmonary a.
release
(Less production)
Endotheline-1
Serotonin
Thromboxan
Nitric Oxide
Prostacyclin
Pulmonary artery dilatation
Inhibit smooth muscle Hypertrophy
Pulmonary artery constriction
Smooth muscle Hypertrophy
Worsening the Pulmonary hypertension
Vicious cycle

6. Pulmonary venous hypertension
Left-sided atrial or ventricular heart disease (eg. hypertrophy, atrial myxoma ,etc..)
Left-sided valvular heart disease (eg. Mirtal stenosis)
Pulmonary veno-occlusive disease
Pulmonary capillary haemangiomatosis (very rare)

7. Cont. Left Heart Disease
(Pulmonary BVs are normal & undamaged)
Left-side of heart unable to pump blood efficiently
Backup of blood in pulmonary veins and capillary beds
↑ Pressure in pulmonary artery

8. PH caused by respiratory disorders & /or hypoxaemia
COPD
Diffuse parenchymal lung disease
Sleep-disordered breathing
Alveolar hypoventilation disorders
Chronic exposure to high altitude
Neonatal lung disease
Alveolar capillary dysplasia
Severe kyphoscoliosis

9. Cont. respiratory disorders & /or hypoxaemia
Hypoxic vasoconstriction of pulmonary arterioles
↑ Pulmonary vascular resistance
(harder for Rt. Ventricle to pump blood)

10. PH caused by chronic thromboembolic disease
Thromboembolic obstruction of the proximal pulmonary arteries
In situ thrombosis
Sickle cell disease
Emboli BLOCK pulmonary vessels
↑ resistance to blood flow
Endothelial cells will release
↑ pulmonary BP
Constrict pulmonary arterioles
Histamine
Serotonin

11. Miscellaneous Inflammatory conditions
Extrinsic compression of central pulmonary veins… eg. Tumour

12. Pathological features
hypertrophy of the media of the vessel wall
Intimal fibrosis
plexiform lesions?!
(group of capillaries placed together, that arise as complication of long standing pulmonary hypertension)

13. Consequences Lung  pulmonary edema Heart  Rt. Ventricle hypertrophy
O2 Demand exceeds O2 supply
Rt. Sided heart failure
(Cor Pulmonule)
Blood backup in venous system:
↑JVD
Hepatomegaly
Ascites
Legs edema
Left ventricle receive less blood
To compensate it will pump harder & faster

14. Clinical features Symptoms: - Breathlessness - chest pain
- Fatigue palpitation
- Syncope (with severe disease)
Signs:
accentuation of pulmonary component of the second heart sound (S2) m.c.
Right ventrical heave (right ventricular hypertrophy)
elevation of the jugular venous pulse (prominent ‘a’ wave if in sinus rhythm)
right ventricular third heart sound.
Tricuspid regurgitation

15. Investigations CXR?! ECG?! Echocardiogram?!
Enlarged pulmonary arteries
ECG?!
right axis deviation due to Rt. Ventricle hypertrophy
Echocardiogram?!
1) Dilated pulmonary artery
2) Dilatation/hypertrophy of RA and RV
3) Abnormal movement of IV septum (due to increased right ventricular volume)
Right heart catheterization?!
increased pulmonary artery pressure
V/Q scan… when?!
If the cause is not revealed (neither heart nor lung), to know is it due PE or is it primary pulmonary hypertension

16. Cont. PFT?! What are the 1st tests you order when you suspect PH?!
most pulmonary function parameters are normal in PH (if it’s not caused by respiratory disorders) , but the DLCO (Diffusing capacity of the lungs for carbon monoxide) decreases. Know that a very low DLCO is associated with a poor prognosis in patients with PH.
What are the 1st tests you order when you suspect PH?!
What follow-up test is done if the 1st tests are suggestive?!
ECG & an echocardiogram.
Right heart catheterization to confirm the diagnose

17. Management
1) Exercise : improve the functional class of patients greatly but does not change the hemodynamics. 2) Unless there is an increased risk of bleeding anticoagulants mainly warfarin. Our goal is to reach INR of ) Diuretics are recommended for fluid retention. 4) Oxygen for those with chronic hypoxaemia. 5) Vasodilators 6) Lung transplantation

18. Vasodilator agents
1) Oral Calcium Channel Blockers.. vasodilators of choice (eg. ifedipine, amlodipine & diltiazem) 2) Oral Endothelin Receptor Antagonists (eg. Bosentan) 3)IV Prostacyclin (eg. Epoprostenol) 4) Inhaled PDE (phosphodiesterase) Inhibitors type 5

19. Questions ??