PHT in Indi Agend PHT in Indi Bckground: Indin Subcontinent Epidemiologicl insights: Wht diseses re likely to be responsible for PHT in Indin children in the yer 2009? Likely mgnitude of the problem? Our most significnt chllenges? Our most importnt priorities? Solutions: Are there ny? Numerous strk contrdictions Generliztions re impossible Chos is the most pproprite term for most situtions Helth Cre: Extreme vribility in ccess to bsic services, qulity of services helth indices 3/14/2009 AIMS,Cochin 1 3/14/2009 AIMS,Cochin 1 PHT in Indi PHT in Indi 3/14/2009 AIMS,Cochin 2 3/14/2009 AIMS,Cochin 2 1
PHT in Indi Bckground: Indin Helth Cre Where nd why re 10 million children dying every yer? Rnk by no. of child deths No. of child deths Under 5-yer mortlity rte rnk Indi 2,402,000 54 Nigeri 834,000 17 Chin 784,000 88 Pkistn 565,000 43 D R Congo 484,000 9 3/14/2009 AIMS,Cochin 1 The Lncet, Volume 361, Issue 9376, Pges 2226-2234, 28 June 2003 Interstte Differences in IMR in Indi Ech dot represents 5000 deths 2
Humn Development Indices PHT in Indi Epidemiologicl Insights Etiology of Pulmonry Hypertension in Indin Children Rheumtic Hert Disese Congenitl Hert Disese IPAH Other cquired conditions 3/14/2009 AIMS,Cochin 1 Rheumtic Hert Disese PHT results from bckpressure (PVH) Juvenile mitrl vlve stenosis Severe mitrl regurgittion Juvenile MS: Subvlvr deformity Sever PVH nd PAH (incresed PVR) Rheumtic MR: often sstd. With MS; significnt PAH in children 3
Rheumtic Hert Disese Declining trends in mny prts of Indi Hospitl dmissions: Proportion of ptients with RHD Number of procedures (Blloon Mitrl Vlvotomy) Systemtic surveys Proportion of Vrious Crdic Conditions t the Crdic Clinic of AIIMS 1966-70 1976-80 1981-8585 1991 1998 RHD (%) 28.5 25.4 23.8 16.6 12.4 CHD (%) 18.4 15.2 19.7 21.2 21.1 HTN (%) 13.0 12.7 13.9 12.6 10.9 Others (%) 41.1 46.3 42.6 49.9 55.2 16 4
Rheumtic Hert Disese RF / RHD : School Surveys o 1972 75 (133,000): 0.8 to 11/1000; overll 5.3/1000. o 1984-87 (52, 793): 1.0 to 5.7 / 1000 overll 2.97 / 1000. o 2002-2005 (100,269): 0.43 1.47 / 1000 overll 0.9 / 1000 Indin Council of Medicl Reserch project on Rheumtic Fever nd Rheumtic Hert Disese, 10 million popultion registry nd ~200,000 school children surveyed Cochin 3 centers 3 centers Registry Component Pssive Surveillnce Prevlence Rte 0.7 0.6 0.5 0.4 0.3 0.2 0.1 0 RHD RF/RHD Prevlence of RF- RHD/1000 RF Chndigrh Vellore Kochi Indore Rheumtic Hert Disese Definite decline in mny prts of Indi Lrge res hve still not been surveyed Disese documented to be very much prevlent in Pkistn (Sdiq et l 2008) 3/14/2009 AIMS,Cochin 21 5
Estimtes of Congenitl Hert Disese (CHD) Prevlence Among Live-born Infnts in Indi* Totl CHD t birth ~130-270,000 Criticl CHD (requiring intervention in infncy): ~ 80,000 CHD mortlity s frction of infnt mortlity: 3-20% *Bsed on vilble dt of CHD prevlence t birth in developed countries nd present birth rtes in Indi Worldwide Estimtes of Congenitl Hert Disese (CHD) Prevlence Among Live- born Infnts in the Developing World Totl CHD t birth ~10,00,000 (one million) Criticl CHD (requiring intervention in infncy): ~ 300,000 CHD mortlity s frction of infnt mortlity: 1-30% *Bsed on vilble dt of CHD prevlence t birth in developed countries nd present birth rtes in the Individul countries of the developing world; Dt from Children s Hertlink Survey Opertions for CHD (Globl Sitution): Dt from Yer 2000 Totl Open Hert Procedures: 1,211,624 Totl Congenitl opertions: 159,482 Adult Congenitl: 25,556 Peditric Congenitl: 81,506 Infnt/Newborn: 52,419 Prts of The World Where the Averge Child in the Region hs Access to Congenitl Hert Surgery? 6
Indin Sitution (2004) Criticl CHD: Untreted- n ~ 80,000 Criticl CHD receiving Tretment (3.04%) Totl CHD opertions: 9750 Infnt nd Newborn opertions: 2437 Centers performing congenitl hert opertions regulrly in the Indin subcontinent (2005) 3 centers Belgum 3 centers Cochin Tretment N~2500 Peditric Hert Cre in 2005: US vs. Indi Detection N =? Totl CHD popultion Timely referrl N =? US: 280 Million Lrge proportion of CHD dignosed in-utero Most ptients operted in infncy One center per < 5 million popultion > 2500 peditric crdiologists > 200 dedicted peditric hert surgeons Indi: 1.029 billion > 95% missed in infncy < 2-3% operted in infncy One center per 100 million popultion 35 peditric crdiologists 15-20 dedicted peditric hert surgeons N = 80,000 7
Trining in Peditric Crdiology Indi Vs. US Section on peditric crdiology in AAP: 1957 1961: First qulifying exmintion instituted by subbord in US Peditric Crdic Society of Indi: 1997 2003: First qulifying exmintion of ntionl bord of exm for fellowship in ped. Crdiology Lte Dignosis of CHD: Consequences Survivors selected by nturl history Shunt lesions: Most children with lrge shunts with Qp would not survive beyond 2 yers ge Reltively lrge proportion of PVR mong survivors Lte Dignosis of CHD: Consequences 100% Defect vs. PVOD Risk Likelihood of operbility Single ventricle vrints TGA VSD/PDA Infncy Truncus Erly childhood Foss ovlis ASD SV ASD Unrestrictive VSD or PDA Adolescence Adulthood Age 8
PHT in the Indin Subcontinent 3/14/2009 Clinicl spectrum of post-tricuspid shunts with PAH Operble Cler clinicl /noninvsive evidence of lrge left right shunt Filure to thrive, precordil ctivity, mid distolic murmur t pex, Crdic enlrgement, pulmonry blood flow Q in lterl leds on ECG, good LV forces LA/LV enlrgement, exclusively L-R flows cross the defect Borderline clinicl non-invsive dt: uncertin operbility Blue Cynosis, quiet precordium, no MDM Cler evidence of shunt reversl resulting from high PVR. Inoperble 26 yer old Norml hert size, peripherl pruning No Q in lterl leds, predominent RV forces Single loud S2 No LA LV enlrgement, significnt R-L flows cross the defect 7 month old with lrge VSD 10 yer old with lrge VSD 9
No dt IPAH nd Fmilil forms Absolute numbers likely to be enormous given the popultion of the region? 20% of the world s disese burden Others Relted to Collgen Vsculr Disese Portl hypertension HIV infection Drugs/toxins Persistent pulmonry hypertension of the newborn Pulmonry hypertension ssocited with disorders of the respirtory system or hypoxemi Pulmonry hypertension cused by chronic thrombotic or embolic disese Pulmonry hypertension cused by disorders directly ffecting the pulmonry vsculture Peditric Pulmonry Hypertension in Indi: Chllenges Mgnitude Referrl Estblishing specilized centers Dignostic work up Tretment: Affordble medictions Follow up Reserch Timely Detection nd Referrl: Brriers Qulity of primry helth cre Culturl brriers in helth seeking behviour Symptom onset Locl Helth Fcility Avilbility Access Affordbility Locl expertise Referrl Fcility Logistics Distnce Expertise Expense 1-3 yers Tretment 10
Previling Helth-Cre Culture in Indi Declining qulity of teching institutions Declining role of the government sector Very little helth insurnce coverge for the verge citizen Extrordinry (unregulted) growth of the privte sector- exploittive Disproportionte remunertion from procedures Dignostic work up Tretment of Pulmonry hypertension: Cost of Medictions Drug Cost per tblet ($) Cost per month ($) Cost / Averge per cpit income Sildenfil (Revtio) 9.5 285 4.4 Sildenfil (Indin brnd) 0.22 6.73 0.1 Bosentn 55.7 3342 49.8 Sitxentn??? Imtinib Ptented version costs 103$ (4.5$ in Indi for generic versions) 3090 (135) 46.1 (2) 11
Tretment of Pulmonry hypertension: Cost of Medictions Drug Cost per month Cost / Averge per cpit income Inhled prostcycline Intrvenous Prostcycline 2960$ 44.1 5000$ 74.6 Solutions: Wish List Development of mny more qulity institutions (one for every 5-10 million: 100-200) with fcilities for comprehensive peditric hert cre Estblishment of dedicted centers for mngement of PHT (one for every 50 million?) with fcility for bsic reserch Wish List: Specilized Centers Multiple peditric specilties under one roof Acdemic centers preferbly s prt of university Strong emphsis on reserch Effective tem of dedicted helth professionls Continued cre in the community Solutions: Wish List Improved qulity of primry nd secondry cre to ensure erly dignosis nd prompt referrl Avilbility of drugs for pulmonry hypertension t ffordble costs Estblishment of community bsed services for follow-up cre nd rehbilittion 12
Solutions: Wish List The Rurl-Urbn Divide Epidemiologicl studies on disese prevlence Rheumtic Hert Disese Congenitl Hert Disese Trining Cregivers Developing Qulity Institutions Indigenous Technology Cost Effective Strtegies Solutions: PVRI South Est Asi, A beginning Others Defining Disese Burden Delivering Cre Resource genertion Chnges in Curriculum Adult Crdiologists Peditricins Other Cre Givers OB Gyn. Peditric Hert Cre: The Big Picture Improving Awreness Through Eduction Generl popultion Policy mkers Understnding the unique ptient popultion Professionl bodies 13
Epidemiology, nturl history studies through registries Bsic nd Clinicl reserch, Qulity clinicl trils To estblish effective therpies, ccessible to ptients First Pulmonry Hypertension Symposium: prepcsi, Nov 7, 2008, AIMS, Kochi PVRI South Est Asi: Vision nd Mission To spred wreness bout PVD, mong helthcre professionls nd the public Consulttive services to helth gencies, nd the industry, regrding PVD Coordinte the PVRI Indi ctivities with the other globl subsidiries of the PVRI. Conclusions The profile nd priorities of the developing world re completely different from tht of developed ntions The gp between knowledge (wht is possible) nd prctice (wht ctully hppens) is constntly widening nd needs to be bridged through conscious effort 14