Pulmonary hypertension (PH) is an increase of blood pressure in the pulmonary artery, pulmonary vein, or pulmonary capillaries, together known as the lung vasculature, leading to shortness of breath, dizziness, fainting, leg swelling and other symptoms. Pulmonary hypertension can be a severe disease with a markedly decreased exercise tolerance and heart failure. It was first identified by Ernst von Romberg in 1891. According to the most recent classification, it can be one of five different types: arterial, venous, hypoxic, thromboembolic or miscellaneous.

1. Pulmonary Arterial Hypertension
CONGENITAL HEART DISEASE

2. Be sure/No cure/Only care/Till life is there
Although current pharmacological agents have undoubtedly revolutionized
the treatment landscape of this devastating condition, PAH remains a disease
without a cure

3. Define
End-expiratory mean pulmonary artery pressure (PAP) ≥25 mm Hg
Pulmonary artery wedge pressure ≤15 mm Hg
PVR >3 Wood units at rest
Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M, Langleben D, Manes A, Satoh T, Torres F,
Wilkins MR, Badesch DB.Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol.2013;62(25
suppl):D42–D50.

4. 4 types OF PAH related to CHD
1. EISENMENGER SYNDROME
2. PAH ASSOCIATED WITH SYSTEMIC-TO-PULMONARY SHUNTS
3. PAH WITH SMALL DEFECTS
4. PAH AFTER SURGICAL REPAIR:WORSE OUTCOME

5. TWO TREATMENT OPTIONS
1. TREAT-AND-REPAIR
2. REPAIR-AND-TREAT

6. ASSESSMENT OF PAH
CLINICAL SIGNS
ECHOCARDIOGRAPHY
CATHETERIZATION IS GOLD STANDARD:VASOREACTIVITY STUDY
LUNG BIOPSY IS OUTDATED:NOT WITHOUT RISK;NOT RELIABLE;B/L LUNG ALL THE LUNG FIELDS
MAY NOT HAVE SAME CHANGES
PHASE CONTRAST-MRI:DIFFERENTIAL FLOW RATE AND VELOCITIES IN THE ARE OF INTEREST
(r=0.92 when compared with cath data)

7. Biomarkers capable of defining the degree of PVD
ANP,BNP),N-pro-BNP, cardiac troponin T,uric acid,31 urinary PG,metabolites,Enos,
dimethylarginines, ET-1/ET-1:ET3 ratio,circulating VWF,biomarkers of inflammation and oxidative
stress such as cytokines (IL-1a, -2, -4, -6, -8, -10 and 12p70, TNF-b, MCP-1 and osteopontin), C-reactive
protein, urinary F2-isoprostanes and metabolites, pim-1, HbA1c, etc,circulating
endothelial cells and micro-RNA,circulating endothelial cells,pentraxin-3

8. Favourable parameter FOR ICR

9. Outcomes
1. PAH after surgical cardiac defect repair had a far worse outcome than patients with any
other type of PAH with CHD
2. Eisenmenger syndrome survive -93% at 5 years
3. PAH with CHD overall 5yrs survival is 91% is 5 years
4. IDIOPATHIC PAH showed 5 year survival only 63%

10. Surgical Repair Of CHD In Borderline Patients With PAH
1. TREAT-AND-REPAIR
2. REPAIR-AND-TREAT

11. TREAT-AND-REPAIR
HYPOTHESIS END POINTS
1.Despite established, long-standing pulmonary
vascular disease with evidence of significant
vascular remodelling/obstruction, Eisenmenger
syndrome patients often respond favourably to
advanced therapy
2. one-third of Eisenmenger syndrome patients
maintain some degree of pulmonary vasoreactivity
despite the presence of PVD
3.Reverse remodelling may favour surgery(Type B)
4. There is evidence that some of them are effective
in treating PAH-CHD
5.
1.Pretreatment is an increase in shunt volume
(by increasing the compliance of the downstream
chamber or vascular bed) and a consequent
increase in pulmonary blood flow. This may result in
a paradoxical increase in pulmonary
vascular damage if left unguarded and operation is
not done in time may endanger life

12. REPAIR-AND-TREAT
HYPOTHESIS END POINTS
Pre-treating borderline PAH patients with advanced
pulmonary vasodilators has the potential to
demonstrate the reactivity of the pulmonary
vascular bed; however, this comes at the risk of
an increase in shunt volume, pulmonary blood flow
and shear stress. This creates a paradoxical increase
in pulmonary vascular damage if left unguarded,
and might worsen the patient’s
condition before surgical repair
1.It remains unclear if it provides any improvement
in terms of long-term outcomes
2.Sometime Small ASD/VSD with right to left
permission is done
3.The patient is best monitored with optimised
vasodilators

13. Basic care of PAH
1. O2
2. OAC
3. Diuretic
4. CCB Blocker in responder
5. No pregnancy
6. Mental support
7. Rehabilitate under supervision

14. New things
UPFRONT COMBINATION
Start with two or more medications in the
beginning itself if patient is very high risk
TRIALS SUPPORTING
BREATHE 2
AMBITION[Ambrisentan and Tadalafil in
Patients with Pulmonary Arterial
Hypertension]

15. Galiè N, Corris PA, Frost A, Girgis RE, Granton J, Jing ZC,
Klepetko W, McGoon MD, McLaughlin VV, Preston IR, Rubin LJ,
Sandoval J,Seeger W, Keogh A. Updated treatment algorithm of
pulmonary arterial hypertension. J Am Coll Cardiol. 2013;62(25
suppl):D60–D72.

16. Once upon time in Chennai- 2014