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Prof. S. Shanmuga Sundaram K.S. Hospital, Chennai.

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Presentation on theme: "Prof. S. Shanmuga Sundaram K.S. Hospital, Chennai."— Presentation transcript:

1 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 LESIONS mPAP > 25 mmHg at rest / > 30 mm Hg post exercise PAWP 3 Wood Units TRANSMISSION OF SYSTEMIC ARTERIAL PRESSURE VASOCONSTRICTION VASCULAR OBLITERATION – MEDIAL HYPERTROPHY INTIMAL PROLIF + FIBROSIS ARTERIAL THROMBI HYPERKINETIC OBLITERATIVE PVR 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 BED Lucas R. Am J Dis Child

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11 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% > 3 rd 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%

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

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

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

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16 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 > u.m2

17 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 S 2 split decreases and P 2 increases in intensity

18 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

19 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

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

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25 PDA DOPPLER PATTERN S PAH PULSATILECLOSING CLOSED GROWING

26 DOPPLER IN PDA

27 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

28 Why to assess operability ?

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

30 ASSESSING OPERABILITY BASED ON PVR MISTAKES & MISCONCEPTIONS Expecting PAP to decline ( ↓ PVR > ↑ FLOW ) Assuming O2 consumption Ignoring dissolved O2 in calculating PVR O2 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%

31 PVR INDEXED TO BODY SURFACE AREA A child of BSA of 0.5 m 2 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/m 2 PVR indexed to BSA = 20-8/4 = 3 u.m 2

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

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34 PULMONARY WEDGE ANGIO

35 PREDICTION OF PVOD Wilson 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%

36 LUNG BIOPSY 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 > u.m2

37 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

38 BALLOON OCCLUSION IN HYPERTENSIVE DUCTUS Roy A IHJ 2005;57:332 Fall in m/d PAP > 20 mmHg

39 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

40 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

41 DEVICE CLOSURE IN ASD + PAH Balint OH Heart 2008;94:1189 PAH Moderate Severe PASP >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

42 EISENMENGER’S SYNDROME MANAGEMENT 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

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46 BOSENTAN IN CHD + PAH Diller GP Heart 2007;93:974

47 BOSENTAN IN CHD + PAH Alto MD, Heart 2007;93:621

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

50 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 > u.m2

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53 VENTRICULAR SEPTAL DEFECT

54 PATENT DUCTUS ARTERIOSUS

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