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Assessment for operability of CHD with PAH R. Tandon.

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Presentation on theme: "Assessment for operability of CHD with PAH R. Tandon."— Presentation transcript:

1 Assessment for operability of CHD with PAH R. Tandon

2 CONGENITAL HEART DISEASE  8 to 10 / 1000 live births  Uncorrected – 30 % PAH  PPH of new born  Idiop. Ped. PAH

3 L > R Shunt with PAH  Acyanotic CHD-PDA -VSD -AVSD -AP Window - ASD  Cyanotic CHD with ↑ Qp (Transposition physiology)

4 Cyanotic CHD with ↑ Qp (Transposition physiology)  Complete TGA  DORV, ↑ Qp  TA, ↑ Qp  PTA  SV, ↑ Qp  TAPVC  Miscellaneous malp. without obstr. to pulm. blood flow

5 Congenital Heart Disease Pulm. Circ: PAH  CHD - ↑ QP, ↑ Pr, ↑ O 2 sat, ??  Hypoperf, 2° to under development  Blood Viscosity  Thrombo–embolic obstr.  Aorto–pulm. Collat.

6 Congenital Heart Disease Pulm. hypertension : PA Pr.=Pulm flow x PVR Hyperkinetic= flow Obstructive= PVR

7 Principles Low resistance pulmonary circuit 1) Qp 2) Vol. overload High resistance pulmonary circuit 1) Curtailment of Qp 2) Loss of volume loading

8 CONGENITAL HEART DISEASE PAH :  Hyperkinetic -PVR normal  Obstructive -PVR ↑  Due to PVH -PVR normal, ± ↑

9 CONGENITAL HEART DISEASE Pulmonary Hypertension : dx  PA (m) ↑ 25  PAW/LAm/LVedp 15 mm or ↓  PVR ↑ 3u (Incorrect)

10 Factors determining development of PVOD  Type of intracardiac defect  Age   PA Pressure   PV Pressure   PBF, PAO 2  Hypoxia  Acidosis  Unexplained - ? Genetic suscept. ? Endoth. dysf.

11 Congenital Heart Disease Pulmonary hypertension : Operability AgeAge LesionLesion Physical findingsPhysical findings Systemic O2 saturationSystemic O2 saturation InvestigationsInvestigations oNon-invasive oInvasive

12 Congenital Heart Disease PVOD : Age  Unknown 3 m  Rare 6 m  Uncommon 1 yr Except in TGA physiology

13 Congenital Heart Disease  in PVR :  Improving symptoms   in CCF  Improved exercise capacity  Cyanosis : Absent, intermittent, persistent.

14 Congenital Heart Disease PVOD : Lesion  Very early -  3 m TGA Physio., AP window, Comp. AVC defects  Large PDA earlier than VSD ( ↓ 1 yr).  ASD – rare

15 HyperkineticObstructive Heart sizelargenormal (except in ASD) Parasternalhyperkineticforcible or heaving in ASD impulsemild in VSD & PDA Click of PAHabsentpresent Second soundASD ‑ wide & fixedASD - wide and fixed (P2 accent ‑ VSD ‑ wide & variableVSD - single uated in both)PDA ‑ paradoxicallyPDA - normal split Shunt murmurloudshort or absent Flow murmurpresentabsent Pulmonary hypertension

16 Congenital Heart Disease PVOD : Syst. O 2 saturation  L  R shunt -  93% - PVR   95%,  PVR not excluded  TGA physiol. -  85% high flow and low PVR

17 Congenital Heart Disease PVOD  ECG: Increasing RVH - Not helpful  X-ray: * Heart size  * Pulm. Vasculature Characteristics changes appear too late.

18 Congenital Heart Disease Pulm. hypertension : Echo assessment : PA syst. pr-Good PA diast. pr-Reasonable PA mean pr-Poor For assessment of PVR – cath is gold standard and essential.

19 Congenital Heart Disease Invasive evaluation Invasive evaluation  Hemodynamic study.  Pulmonary wedge angiography.  Biopsy – Pulmonary histology.  Pulmonary vascular compliance. (MRI & intravascular ultrasound)

20 CONGENITAL HEART DISEASE PAH Cath study:  Pressures – RA,RV,PA, PAW, LA/LVED,SA  Saturations – SVc, RA,RV,PA,SA  VO 2, CI, SVR, PVR  HR  pO 2, pH  Effect of intervention.

21 Cardiac Catheterisation  Measurement of PAP  Measurement of PVR  Vascular Reactivity PVR = PAm - LAm Qp If PVR & PAH  - pO 2 and pH essential.

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23 Congenital Heart Disease Flow: (Poiseuille) ΔP. π r 4 Q= 8ŋL

24 Congenital Heart Disease PAH: PA (m) – LA (m). 8L η PVR= Qp π r 4 Assumes constant steady flow

25 Congenital Heart Disease Assessment for reactivity :  Oxygen  Isuprel infusion  Nifedipine  Prostaglandin  SNP, NO. PVR ↓ 6 units – operable.

26 Congenital Heart Disease Flows: VO 2 Qp= PVO 2 – PAO 2 VO 2 Qs= PVO 2 (SA) – MVO 2 Qp=Qs

27 Congenital Heart Disease Pulmonary hypertension : VO 2  Must be measured  Assumed value cannot be used For PVR calculation to determine operability.

28 Congenital Heart Disease Flows:Qp/Qsratio SAO 2 – MVO 2 Qp/Qs= PVO 2 – PAO 2 Eliminates need to measure VO 2

29 Congenital Heart Disease Flows:Qp/Qsratio SAO 2 – MVO 2 PV (SA)O 2 – PAO 2 Echo:SV LV SV X HR = Qs Qp from Qp/Qs

30 CONGENITAL HEART DISEASE PAH – PVR  Fixed : PVOD  Variable: Vasoconstr - 20% pts.

31 Congenital Heart Disease PAH:  Operable – PVR ↓ 6u Rest or ac.  Inoperable – PVR ↑ 8u testing. Grey zone 7u ±1.

32 CONGENITAL HEART DISEASE PAH Vasodilat testing : Identifies -Prognosis -Responders (utility of CCB)

33 PULMONARY HYPERTENSION AC. VASODILAT TESTING All PATIENTS  Contraindications : RV failure Unstable haemodynamics + Ve resp. : PA (m) → ↓ by 10 mms absolute level ↓ 40 mms (no ↓ in Co.) 20% ↓ in PApr & PVR

34 Congenital Heart Disease Pulm. hypertension : Reversibility  Qp on the basis of assumed VO 2 - not reliable  100% : O 2 –VO 2 not known, hence all calculation will be incorrect.  Dissolved O2 -  (modifies calculations)

35 Congenital Heart Disease PVOD : O 2 study  PA saturation increases abnormally giving increased calculated QP and low PVR.  Fall in PA pressure more reliable. Fall in PA diast. pr may result in lower mean pressure.

36 Problems with vasodilators Oxygen -fick’s principle NA - Failure to respond in patients with reactive airway disease Tolazoline,PG, Ca Block -Fall in SVR - Arterial hypotension - VP mismatch Isoprenaline -Very high heart rates

37 CONGENITAL HEART DISEASE PAH : Nitric oxide:  Vasodilator  Inhibits -Platelet activation -SMC proliferation  ↓ levels in PAH  NO → cGMP (inactivated by PDE-5, large amounts in lungs)

38 NO Advantages - No ↑ in syst. sat. - No systemic hypotension - No arterial hypoxemia Disadvantages - Methaemoglobinemia - Pulmonary edema - Lung injury

39 NO as Pulmonary vasodilator - Results  NO at 40 ppm more effective than 5 ppm  3/15 (20 %) pt with PVR > 8 u fall in PVR < 6 after NO  Response same in those with Down Syndrome Yasuda T et al,Paediatric Cardiol,2000

40 NO vs NO + Oxygen combination  Number of responders with combination significantly > than when NO/Oxygen used alone.  Combination of NO + 02 provides add’nal pulmonary vasodilatation & can rapidly identify patients with pulmonary vasoreactivity.  Good postoperative results Lock et al, JACC 2000

41 NO + Oxygen combination : PVR 10 u  O2 alonePVR 8 u  O2 + NOPVR 6 u Does not guarantee operability Wilkinson heart 2001 : 85:113

42 Lung biopsy  Open lung specimen is taken  General anaesthesia Biopsy those with borderline haemodynamics (Heath. Edwards classification)

43 CONGENITAL HEART DISEASE PAH Creating ASD -RVF not relieved  R → L shunt - ↓ RA pr.  QP ↓, Syst. O2 sat. ↓ CO more stable – syncope relieved. Change in life span - ?

44 Congenital Heart Disease PVOD : Defect occlusion PDA & some VSDs Significant fall in PA pressure is helpful in decision making.

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46 CONGENITAL HEART DISEASE PAH Treatment  Prostanoids -EpoprostenolIV  E.R.A. -BosentanPO  PDE-5-I -SildenafilPO  CCB -Diltiazem, Amlodip.PO

47 CONGENITAL HEART DISEASE PAH: Breathe – 5 : ASD & VSD Bosentan Vs Placebo – 54 pts., class III, 16 weeks  Syst. O 2 Sat. maintained.  PVR, PAm ↓  Ex. Cap. ↑, funct. class ↑  Time to deterioration ↓  Placebo – PVR worsened (?)

48 CONGENITAL HEART DISEASE PAH : Bosentan : Long FU (12 m – 29 m)  PVR/ SVR ratio ↓ (N- 0.15 to ↓ 0.3)  Qp & Q s ↑, R → L shunt ↓  PAp & SAp ↓ (NS)  RV after load ↓ more than LV Initial improvement returns to baseline in 2 years.

49 CONGENITAL HEART DISEASE PAH : Bosentan  Hepatotoxicity  Potentially teratogenic  Testicular atrophy & infertility  Hormonal birth control - ↓ effectiveness.

50 CONGENITAL HEART DISEASE PAH : Sildenafil  PAp (m) ↓ 3-5 mms  Ex. capacity ↑  Side effects – minor  Functional class ↑

51 CONGENITAL HEART DISEASE PAH : Eisenmenger ASD  40 pts., FU – 4 years, PVR ↑ 7u  ASD closed 26, Med. Rx -14 PVR 9-14 -No ↑ in PVR 7-9 -PVR ↓  No vasoreactivity check pre op  PVR ↑ in all unopted. Steele etal Circ. 1987 : 76 : 1037 - 42

52 CONGENITAL HEART DISEASE PAH : Eisenmenger Pts : O 2 ± No  124 Pts, 1 mt 47 yr. (m 28)  74 op or transplant  PVR/ SVR – 0.33 or ↑ O2 → Rp/Rs ↓ 0.42 O2 + NO Rp/Rs ↓ 0.27 (indicates operable defect) Balzer etal Circ. 2002 : 106:1-76-81

53 Operability & Reversibility  Not Synonymous  Patients with severe intimal proliferation may not show decrease in PAP post-op  PAH can progress after repair

54 Congenital Heart Disease PVR : mistakes in assessment  Presence of A.V. valve regurg.  Obligatory shunts  Pulm. infection, acidosis, anemia, polycythemia, hypoxia

55 SUMMARY  Knowledge of determinants of progression of PVOD is poor  Remember confounding factors affecting PVR in assessing L → R shunts.  Clinical auscultation- fading art.

56 SUMMARY  Age of patient probably THE most important determinant *Pre tricuspid shunt except TAPVC- Longer waiting period * Post tricuspid shunt without cyanosis 6 months to 1 year *Post tricuspid shunt with cyanosis before 3 months

57 Message Timely surgical intervention is THE KEY for good immediate & long term results.

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