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pulmonary arterial hypertension in pediatric OPD and ICU
- 1. Pulmonary arterial hypertension when to suspect and how to assess Dr Neeraj Aggarwal Pediatric Cardiologist Department of Pediatric Cardiac Sciences Sir Ganga Ram Hospital
- 2. Case 1 • 3 year old child • FTT ,lethargy , early tiredness, NYHA dyspnoea class 2-3 • Not able to play with peers • 2 episodes of near-syncope • Hepatomegaly and mild distress • Single loud S2 –palpable • Saturations 91 % • F/H –not significant
- 3. DD • Congenital Heart disease - obstructive lesions • Chronic disorders –Anaemia,Malnutrition • Chronic lung disease • PAH ?
- 4. CXR
- 5. ECHO • Severe PAH ,TR moderate (PG 80 mmhg) • Dilated RA RV • RV mild dysfunction • No pericardial effusion
- 6. Case 2 • 4 yr old male • History of LRTI - admitted outside • Left pleural effusion, resp distress, bilateral chest pain • Septic markers positive , received IV antibiotics for 7 days but not better • Echo for associated pericardial effusion - severe PAH • CT showed multiple small emboli on both sides
- 7. VQ scan
- 10. Badesch D et al. J Am Coll Cardiol. 2009;54:S55-S66. McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619. Hemodynamic Definition of PH/PAH PH PAH Mean PAP ≥25 mm Hg + PCWP/LVEDP ≤15 mm Hg (absence of LV dysfucntion) Mean PAP ≥25 mm Hg AHA includes PVR >3 Wood Units
- 12. 3. PH Owing to Lung Diseases and/or Hypoxia •COPD ,ILD •Other pulmonary diseases with mixed rest. and obst. pattern •Sleep-disordered breathing •Alveolar hypoventilation disorders •Chronic exposure to high altitude •Developmental abnormalities 4. Chronic Thrombo embolic pulmonary hypertension 5. PH With Unclear Multifactorial Mechanisms Hematologic disorders (Myeloproliferaive disorders, splenectomy) •Systemic disorders -Sarcoidosis,histiocytosis,lymphangio- leiomyomatosis, Thyroid disorders, Renal failure/ dialysis, fibrosing mediastinitis,)
- 13. When to Suspect and Screen for PAH • Family history – 6% - 12% prevalence of positive family history • Connective tissue disease – Scleroderma: 8%- 30% – Raynaud phenomenon : up to 20% - 25% – SLE: 4% - 14% – Rheumatoid arthritis up to 21% • Congenital Heart Disease – Reversal of left-to-right shunt – VSD ,PDA, AP window, Atrial Septal Defect
- 14. When to Suspect and Screen for PAH • Portal hypertension -Nearly 10% have PAH • Deep venous thrombosis/history of pulmonary embolism – Up to 3-4% of survivors • Appetite suppressant or stimulant use • HIV ---- 0.5% pts • Hemolytic anaemia
- 15. When to Suspect and Screen for PAH • PAST HISTORY OF RHD—Mitral stenosis • Poorly controlled HT –Look for LV diastolic dysfunction • Snoring at night –sleep apnoea
- 16. How to suspect • Non specific symptoms • I am a difficult case of asthma • I was fat
- 17. Non specific complaints • Dyspnoea • Syncope • Dizziness • Fatigue • Oedema • Angina
- 18. Infants and young • Takes frequent naps • Poor appetite • Growth restriction • Hesitant and un adventurous • Not interacting with family • Undue fatigue while playing with kids • Dressing /toilet –undue fatigue /syncope • Regression of newly learned activities
- 19. SIGNS • Loud P2 • RV lift • Murmur of TR • Signs of CHF
- 20. ECG-RAD RVH RAE RBBB
- 23. 12 yr old boy ,weight 20 kg and DOE class 1 ,examination revealed loud P2 and CXR done had the clue of disease. Echo had PAH
- 25. ECHO • RV size and RVH • TR,PR,IVS movement to left side • Reduced RVOT acceleration time • IVC dilatation,not collapsing with respiration and flow reversal • Dilated pulmonary arteries • Left sided lesions –LA size dilataion • Pulm veno-occlusive disease,shunts
- 26. ECHO
- 28. ECHO
- 29. Estimating Pulmonary Artery Pressures by Echo SPAP = 4TR Vmax2 + RAP TR Vmax= Peak TR velocity Diast PA = 4PRend Vmax2 + RAP PRend Vmax= End PR velocity TR TRPR Mean Diast 2* Mean PA = TR Vmean+ RAP Or = 4PR Vmax2 + RAP PR Vmax= Peak PR velocity TR Vmean=from VTI Modified from Garvan Kane TR
- 30. limitations of echo • Experience • Images can be limited • The RV, the chamber of highest concern in PAH, is the least emphasized on the “standard” echocardiography exam • TR jet may be absent in some patients, thus precluding PASP assessment • May overestimate or underestimate actual pulmonary arterial pressure • Can estimate LVEDP (PCWP) or CO but may prove impractical
- 31. Missed shunts as PAH
- 34. Cath • ACCURACY • CALCULATION OF PVR • LEFT SIDED FILLING PRESSURES BY WEDGE
- 38. Aim of treatment • Dilate and reverse the abnormal remodeling of the pulmonary vascular bed and to restore endothelial function • by acting on the prostacyclin, endothelin and NO pathways
- 39. Prostacyclin pathway • Potent vasodilator in both the pulmonary and systemic circulations • Anti-proliferative properties • IV prostacyclin (epoprostenol) infusion • Treatment of choice for severely ill patients
- 40. Nitric oxide pathway • Nitric oxide is a potent pulmonary vasodilator, inhibitor of platelet activation and vascular smooth muscle cell proliferation. • The effects of NO are mediated via cGMP in vascular smooth muscle cells regulated by phosphodiesterases • Sildenafil is a PDE-5 inhibitor • Tadafil –long acting
- 41. Endothelin pathway • Endothelin-1 one of the most potent vasoconstrictors and smooth-muscle cell mitogens • Endothelin receptor antagonists (ERAs) • Selective ERA-Sitaxsentan and Ambrisentan (less hepatotoxic) • Nonselective-for the ETA and ETB receptors, (Bosentan)-monthly SGOT/SGPT, quarterly Hb
- 42. Management • NYHA class 1,2 (either of )Sildenafil, bosentan, Ambrisentan • NYHA class 3 combination of sildenafil , bosentan ,ambrisentan , (iv epoprostenol in some cases) • NYHA class 4 iV epoprostenol +others •
- 43. Management • Supportive –controlled diuresis, digoxin, oxygen (Target sats >90) • Avoid exertion, pregnancy, high altitudes • Combination therapy • Inadequate response –Atrial septostomy, lung transplant
- 44. COMPLICATIONS OF PULMONARY VASODILATORS • PAH due to lv dysfunction—pulmonary oedema • Severe interstitial fibrosis /emphysema— worsen V/Q mismatch • Chronic Thromboembolism – delay Treatment
- 45. Evaluation in a PH substrate Family history (gene mutation proved) Annual ECHO Systemic sclerosis Annual ECHO Sickle cell /thal intermedia Annual echo HIV If s/s of PAH- echo Portal HT If liver transplant /symptoms - ECHO Prior appetite suppressant use Echo If s/s present CHD ECHO regularly Recent acute pulm embolism V/Q scan after 3 months
- 47. Thank you Dr Neeraj AggarwalDr Neeraj Aggarwal drneeraj_12@yahoo.co.indrneeraj_12@yahoo.co.in
Editor's Notes
- Doppler echo is the best noninvasive method to evaluate PAP, and should be used in all patients suspected to have PAH. Cardiac cath is mandatory for the final diagnosis of PAH The hemodynamic working definition of PAH listed here is derived from the 2009 Proceedings of the 4th World Symposium on PH. In the new recommendations, exercise and PVR criteria have been eliminated. An accurate PCWP can be difficult to obtain in patients with PH and enlarged pulmonary arteries. If PCWP is elevated despite multiple attempts, especially if blood obtained in the wedge position is not fully saturated, direct measurement of LVEDP should strongly be considered so as not to misdiagnose patients who have PAH. In a recent retrospective study of 4300 patients undergoing simultaneous right and left catheterization, 53% meeting criteria for PAH on the basis of a PCWP <15 had a LVEDP >15 (even among patients being evaluated specifically for PH) Refs: Badesch et al. JACC 2009;126:in press. Halpern SD, Taichman DB. Chest, 2009. Mar 24. [Epub ahead of print].