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Pulmonary arterial hypertension in congenital heart disease
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Pulmonary arterial hypertension in congenital heart disease

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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, …

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.

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  • 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ASSESSMENT OF PAH CLINICAL SIGNS ECHOCARDIOGRAPHY CATHETERIZATION IS GOLD STANDARD:VASOREACTIVITY STUDY LUNG BIOPSY IS OUTDATED:NOT WITHOUT RISK;NOT RELIABLE;B/L LUNG ALL THE LUNG FIELDS MAY NOT HAVE SAME CHANGES PHASE CONTRAST-MRI:DIFFERENTIAL FLOW RATE AND VELOCITIES IN THE ARE OF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

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