Pulmonary arterial hypertension in congenital heart disease

Pulmonary Arterial HypertensionCONGENITAL HEART DISEASE

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

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 types OF PAH related to CHD1. EISENMENGER SYNDROME

2. PAH ASSOCIATED WITH SYSTEMIC-TO-PULMONARY SHUNTS

3. PAH WITH SMALL DEFECTS

4. PAH AFTER SURGICAL REPAIR:WORSE OUTCOME

TWO TREATMENT OPTIONS1. TREAT-AND-REPAIR

2. REPAIR-AND-TREAT

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LUNG BIOPSY IS OUTDATED:NOT WITHOUT RISK;NOT RELIABLE;B/L LUNG ALL THE LUNG FIELDS MAY NOT HAVE SAME CHANGES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)

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

Favourable parameter FOR ICR

Outcomes1. 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%

Surgical Repair Of CHD In Borderline Patients With PAH

1. TREAT-AND-REPAIR

2. REPAIR-AND-TREAT

TREAT-AND-REPAIRHYPOTHESIS END POINTS

1.Despite established, long-standing pulmonaryvascular disease with evidence of significant vascular remodelling/obstruction, Eisenmenger syndrome patients often respond favourably to advanced therapy2. one-third of Eisenmenger syndrome patients maintain some degree of pulmonary vasoreactivity despite the presence of PVD3.Reverse remodelling may favour surgery(Type B)4. There is evidence that some of them are effective in treating PAH-CHD5.

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 pulmonaryvascular damage if left unguarded and operation is not done in time may endanger life

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 ofan 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’scondition before surgical repair

1.It remains unclear if it provides any improvement in terms of long-term outcomes2.Sometime Small ASD/VSD with right to left permission is done3.The patient is best monitored with optimised vasodilators

Basic care of PAH1. O2

2. OAC

3. Diuretic

4. CCB Blocker in responder

5. No pregnancy

6. Mental support

7. Rehabilitate under supervision

New thingsUPFRONT 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]

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.

Once upon time in Chennai- 2014