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PULMONARY HYPERTENSION

PULMONARY HYPERTENSION. Prof. S. Shanmuga Sundaram K.S. Hospital, Chennai. PULMONARY HYPERTENSION AT SYSTEMIC LEVEL, DUE TO HIGH PULMONARY VASCULAR RESISTANCE ( > 800 dynes sec cm -5 ) WITH REVERSED OR BIDIRECTIONAL SHUNT…..

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PULMONARY HYPERTENSION

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  1. PULMONARY HYPERTENSION Prof. S. Shanmuga Sundaram K.S. Hospital, Chennai

  2. PULMONARY HYPERTENSION AT SYSTEMIC LEVEL, DUE TO HIGH PULMONARY VASCULAR RESISTANCE ( > 800 dynes sec cm-5 ) WITH REVERSED OR BIDIRECTIONAL SHUNT….. 8% (1950) → → → → 4%

  3. DEATH: Sudden death 30% , Heart failure 23% , Hemoptysis 11% Death during noncardiac surgery & pregnancy

  4. DISSECTION OF PULMONARY ARTERY PROXIMAL PA THROMBOSIS

  5. PULMONARY ARTERIAL HYPERTENSION IN SHUNT LESIONSmPAP > 25 mmHg at rest / > 30 mm Hg post exercisePAWP < 15 mm Hg ; PVR > 3 Wood Units • TRANSMISSION OF SYSTEMIC ARTERIAL PRESSURE • VASOCONSTRICTION • VASCULAR OBLITERATION – MEDIAL HYPERTROPHY INTIMAL PROLIF + FIBROSIS ARTERIAL THROMBI HYPERKINETIC OBLITERATIVE PVR < 5 W.U > 5 W.U PA PP/PA SP > 60% < 40%

  6. ASD VSD PDA CA, APVC TGA VSD, DORV APWINDOW SINGLE VENTRICLE TRUNCUS > 2 cm > 1 cm > 1 cm

  7. PULMONARY CIRCULATION - STRUCTURAL REMODELING Elastic > Fully muscular > Partially muscular > Non muscular • At birth the smallest muscular arteries dilate with medial thinning • By 4 months, this process involves larger arteries & get completed • Alveoli and Arteries grow both in number & size Al : Art = 20:1 > 8:1 • With shunt lesions resulting in increased flow ± pressure, proximal arteries dilate, distal arteries reduce in number and size bcause of extension of muscle in media of partially or non muscular arteries

  8. NORMAL VSD

  9. MATURATION OF PULMONARY VASCULAR BEDLucas R. Am J Dis Child

  10. PAH IN L > R SHUNTS • NONRESTRICTIVE VSD = 15 % < 2 yrs of life MODERATE DEFECTS = 3% ; LARGE DEFECTS (1.5cm) = 50% • LARGE PDA = similar incidence • LARGE ASD = 6-10% > 3rd decade Frequent in SVC, partial AV Canal defects & in Lutembacher’s • TGA = 8% (intact IVS) 40% ( VSD/PDA ) < 1 yr 75% at 2 yrs • COMMON AV CANAL all develop PAH • TRUNCUS ARTERIOSUS by 1-2 yrs • SYSTEMIC - PA SHUNTS: BT Shunt (<10%) Waterston / Pott’s ~ 30%

  11. MECHANISMS OF PAH IN L>R SHUNTS LESION ↑Qp ↑PAP ↑PVP ↓ pH ↑ Ht ASD + - - - - VSD + + + - - PDA + + + - - AV CANAL + + ++ - - TGA, TA + + + + +

  12. ENDOTHELIAL DYSFUNCTION ↑ ET, TXA2 , SEROTONIN ↓ NO ,PGI2,VIP PLATELET ADHESION + THROMBUS

  13. GENETIC SUSCEPTIBIILITY BMPR2 MUTATION = 6 % 26%(IPAH) 50% (FPAH)

  14. MORPHOMETRIC GRADING Rabinovitch M Grade A : Extension of muscle into small peripheral arteries Wall thickness increased but < 1.5 times the normal ↑ PBF ↑ PA PP + NORMAL MEAN PAP Grade B : Mild : medial thickness 1.5 – 2.0 times the normal Severe : medial thickness > 2 times the normal PAH - MEAN PAP > 50 % OF SYSTEMIC LEVEL Grade C : Size and number of arteries reduced PAH - PVR > 3.5 - 6.0 u.m2

  15. CLINICAL RECOGNITION • Apparent improvement of neonatal HF • Reduction of frequency of respiratory infections • Precordium becomes less tumultous • Flow murmur decreases > disappears • Shunt murmur decreases in intensity & duration • S2 split decreases and P2 increases in intensity

  16. EISENMENGER’S SYNDROME SYMPTOMS: 1) Low C.O + Hypoxia > DOE, Dizziness, Syncope, Fatigue 2) Hemoptysis: Rupture of plexiform, dilatation lesions, pulmonary arterioles, Broncho Pulmonary connexions, Pulmonary Embolism / in situ thrombosis 3) Hyperviscosity: Headache, dizziness, Visual sx 4) Right Heart failure : Edema, RHC pain 5)CVA :Hyperviscosity, Parad. emboli, Cerebral abscess 6) Sudden cardiac death: Arrhythmia

  17. EISENMENGER’S SYNDROME SIGNS : 1) Cyanosis and Clubbing 2) JVP inconspicuous 3) Pulmonary Ejection Sound 4) 2-3/6 Ejection Systolic Murmur 5) Loud P2 6) Murmurs of TR and PR

  18. EISENMENGER’S SYNDROME FEATURE ASD VSD PDA Neonatal HF - + + Age 30-40 2-12 2-12 Syncope ± ± - Cyanosis Uniform Uniform Differential Cardiomegaly,PSH + - - Wide pulse pressure - - ± Prominent ‘a’ JVP + - - S2 split Fixed Single Normal Long PR murmur - - +

  19. PDA DOPPLER PATTERNS PAH GROWING PULSATILE CLOSING CLOSED

  20. DOPPLER IN PDA

  21. SHUNT LESIONS - OPERABILITY • Qp : Qs = > 2:1 No or mild PAH • Qp : Qs = < 1.5:1 Severe PAH - INOPERABLE Qp = O2 Consumption / PV – PA O2 content Qs = O2 consumption / SA - MV O2 content O2 content = O2 saturation x O2 carrying capacity x Hb Qp : Qs = SA – MV O2 sat / PV – PA O2 sat

  22. Why to assess operability ?

  23. CHD PAH – REVERSIBILITY TESTING HIGH SURGICAL RISK ( 20% ) RIGHT VENTRICULAR FAILURE ( IPAH like ! ) PROGRESSION OF PAH AGENTSCRITERIA 100% OXYGEN (10 mts) ↓Rp /Rs > 20% NITRIC OXIDE (10-80ppm) Rp:Rs < 0.33 02 + N.O (Se 97% Sp 90%) Rp < 8 u.m2 ADENOSINE (50-500µg/kg/mt) EPOPROSTENOL (2-10 ng/kg/mt) ILOPROST (2.5-5.0 µg )

  24. ASSESSING OPERABILITY BASED ON PVRMISTAKES & MISCONCEPTIONSExpecting PAP to decline ( ↓ PVR > ↑ FLOW )Assuming O2 consumptionIgnoring dissolved O2 in calculating PVRO2 sat x 1.36 x Hb = 60 x 1.36 x 12 = 98 ml/L ( 0.03 x 55 = 1.7ml ) 98 x 1.36 x 12 = 160 ml/L ( 0.03 x 95 = 2.9ml ) PVR = 60 – 8 = 52 / 3.2 = 16 units ( 16.5 units ) After 100% oxygen : 72 x 1.36 x 12 = 118 ml/L ( 0.03 x 100 = 3 ml ) 98 x 1.36 x 12 = 160 ml/L ( 0.03 x 500 = 15 ml) PVR = 55 – 8 = 47 / 4.8 = 9.8 units ( 12.7 units ) 22 to 44% 40 to 60% 60 to 100%

  25. PVR INDEXED TO BODY SURFACE AREA A child of BSA of 0.5 m2 has a PBF of 2 l/mt PA mean pressure = 20 mmHg ; mean LAP = 8 mmHg PVR absolute value = 20-8/2 = 6 units If corrected for BSA = 6/0.5 = 12 units PBF corrected to BSA = 2/0.5 = 4 l/mt/m2 PVR indexed to BSA = 20-8/4 = 3 u.m2

  26. ROLE OF ECHOCARDIOGRAPHY • Qp/Qs by doppler, PAcT not reliable • PA peak velocity > 1.0 m/s predictive • PVR = TR Velocity/ TVI RVOT x 10 + 0.16 • Vp > 18 cm/s = PVR < 6 units 8.8 W.U 4 WU 16.4 W.U 12.4 cm/s 23.1 cm/s

  27. PULMONARY WEDGEANGIO

  28. PREDICTION OF PVODWilson NJ CCVD 1993;28:22 PREDICTING HEATH EDWARDS Grade III - IV Sensitivity Specificity PVR > 6 units 100% 94% Monopedial count<3 83% 100% Abnormal blush 83% 69% Combination of all 100% 100%

  29. LUNG BIOPSYMORPHOMETRIC GRADING Rabinovitch M Grade A : Extension of muscle into small peripheral arteries Wall thickness increased but < 1.5 times the normal ↑ PBF ↑ PA PP + NORMAL MEAN PAP Grade B : Mild : medial thickness 1.5 – 2.0 times the normal Severe : medial thickness > 2 times the normal PAH - MEAN PAP > 50 % OF SYSTEMIC LEVEL Grade C : Size and number of arteries reduced PAH - PVR > 3.5 - 6.0 u.m2

  30. CARDIAC MR • DEFECT SIZE & LOCATION • PA SIZE ↑ WITH PAH • RV FUNCTION • Qp/Qs RATIO Phase contrast velocity mapping • MR OXIMETRY ( T2 relaxation time) • DEGREE OF PAH

  31. BALLOON OCCLUSION IN HYPERTENSIVE DUCTUSRoy A IHJ 2005;57:332 Fall in m/d PAP > 20 mmHg

  32. TRIAL OCCLUSION OF PDA Yan C Heart 2007;93:514 Trial occlusion for 30 mts with ADO Reduction of mPAP 78 ± 19.3 to 41 ± 13.8 mm Hg FU for 3 to 6 months – clinical improvement

  33. PAH IN ATRIAL SEPTAL DEFECT • 6% ( Mayo clinic); 9% - half were below 20 yrs(CMC) • PAH (mPAP>30 mmHg) 26% SVC (9% FO) ↑PVR 16% SVC (4% FO ) ; at younger age • 85 % were women ( overall F:M = 2:1 ) • PVR > 15 units do poorly – death / progression of PAH • PVR < 10 units do well with surgery • PVR 10 – 15 units – if SPO2 is < 90% surgery not useful

  34. DEVICE CLOSURE IN ASD + PAHBalint OH Heart 2008;94:1189 PAH Moderate Severe PASP 50-59 >60 At 3 m PASP ↓ 57± 11 to 51±17 At 3 yrs PASP ↓ to 44 ±16 Only in 43.6% PAP normalised 15.4% had persistent severe PAH

  35. EISENMENGER’S SYNDROMEMANAGEMENT ISSUES • Avoid dehydration, living at high altitude • Air travel safe (supplemental O2) • Avoid pregnancy ( No OCP – tubal ligation/vasectomy) • Treat Iron deficiency ( MCV < 82 ) ; hyperuricemia • Vensection for hyperviscosity syndrome • Antiplatelet / Anticoagulants ? • Disease targeting therapies : Prostacyclin & analogues, sildenafil, bosentan • Surgery: Correction after PA banding, prolonged vasodilator therapy, Heart Lung Transplant

  36. BOSENTAN IN CHD + PAHDiller GP Heart 2007;93:974

  37. BOSENTAN IN CHD + PAHAlto MD, Heart 2007;93:621

  38. PROGNOSIS EISENMENGER SYNDROME ~ IPH ACTUARIAL SURVIVAL E.S IPAH 1 yr 97 % 77 % 2 yr 89 % 69 % 3 yr 77 % 35 %

  39. MORPHOMETRIC GRADING Rabinovitch M Grade A : Extension of muscle into small peripheral arteries Wall thickness increased but < 1.5 times the normal ↑ PBF ↑ PA PP + NORMAL MEAN PAP Grade B : Mild : medial thickness 1.5 – 2.0 times the normal Severe : medial thickness > 2 times the normal PAH - MEAN PAP > 50 % OF SYSTEMIC LEVEL Grade C : Size and number of arteries reduced PAH - PVR > 3.5 - 6.0 u.m2

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