1 / 61

Assessment for operability of CHD with PAH R. Tandon

Assessment for operability of CHD with PAH R. Tandon. CONGENITAL HEART DISEASE. 8 to 10 / 1000 live births Uncorrected – 30 % PAH PPH of new born Idiop. Ped. PAH. L > R Shunt with PAH. Acyanotic CHD - PDA - VSD - AVSD - AP Window - ASD Cyanotic CHD with ↑ Qp

dvaughn
Download Presentation

Assessment for operability of CHD with PAH R. Tandon

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

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, ↑ O2 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, PAO2 • Hypoxia • Acidosis • Unexplained - ? Genetic suscept. ? Endoth. dysf.

  11. Congenital Heart Disease Pulmonary hypertension : Operability • Age • Lesion • Physical findings • Systemic O2 saturation • Investigations • Non-invasive • Invasive

  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. Pulmonary hypertension HyperkineticObstructive Heart size large normal (except in ASD) Parasternal hyperkinetic forcible or heaving in ASD impulse mild in VSD & PDA Click of PAH absent present Second sound ASD‑wide & fixed ASD - wide and fixed (P2 accent‑ VSD‑wide & variable VSD - single uated in both) PDA‑paradoxically PDA - normal split Shunt murmur loud short or absent Flow murmur present absent

  16. Congenital Heart Disease PVOD : Syst. O2 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 DiseaseInvasive 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 • VO2, CI, SVR, PVR • HR • pO2, pH • Effect of intervention.

  21. Cardiac Catheterisation • Measurement of PAP • Measurement of PVR • Vascular Reactivity PVR = PAm - LAm Qp If PVR & PAH  - pO2 and pH essential.

  22. Congenital Heart Disease Flow: (Poiseuille) ΔP. π r4 Q= 8ŋL

  23. Congenital Heart Disease PAH: PA (m) – LA (m) . 8Lη PVR= Qp π r4 Assumes constant steady flow

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

  25. Congenital Heart Disease Flows: VO2 Qp= PVO2 –PAO2 VO2 Qs= PVO2 (SA)–MVO2 Qp= Qs

  26. Congenital Heart Disease Pulmonary hypertension : VO2 • Must be measured • Assumed value cannot be used For PVR calculation to determine operability.

  27. Congenital Heart Disease Flows: Qp/Qs ratio SAO2 – MVO2 Qp/Qs= PVO2 – PAO2 Eliminates need to measure VO2

  28. Congenital Heart Disease Flows: Qp/Qs ratio SAO2 – MVO2 PV (SA)O2 – PAO2 Echo: SVLV SV X HR = Qs Qp from Qp/Qs

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

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

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

  32. PULMONARY HYPERTENSIONAC. 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

  33. Congenital Heart Disease Pulm. hypertension : Reversibility • Qp on the basis of assumed VO2 - not reliable • 100% : O2–VO2 not known, hence all calculation will be incorrect. • Dissolved O2 -  (modifies calculations)

  34. Congenital Heart Disease PVOD : O2 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.

  35. 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

  36. 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)

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

  38. 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

  39. 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

  40. NO + Oxygen combination : PVR 10 u • O2 alone PVR 8 u • O2 + NO PVR 6 u Does not guarantee operability Wilkinson heart 2001 : 85:113

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

  42. 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 - ?

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

  44. CONGENITAL HEART DISEASE PAH Treatment • Prostanoids - Epoprostenol IV • E.R.A. - Bosentan PO • PDE-5-I - Sildenafil PO • CCB - Diltiazem, Amlodip. PO

  45. CONGENITAL HEART DISEASE PAH: Breathe – 5 : ASD & VSD Bosentan Vs Placebo – 54 pts., class III, 16 weeks • Syst. O2 Sat. maintained. • PVR, PAm ↓ • Ex. Cap.↑, funct. class ↑ • Time to deterioration ↓ • Placebo – PVR worsened (?)

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

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

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

More Related