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  • 1. Massimo Chessa Department of Pediatric Cardiology & Adult with Congenital Heart Disease IRCCS- Policlinico San Donato San Donato Milanese – Milano massimo.chessa@grupposandonato.itLE CARDIOPATIE CONGENITE Cosa sapere in relazione all’ipertensione polmonare
  • 2. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseScriveva William Osler (MedicoCanadese 1849-1919) definito aragione il padre della medicinamoderna: “... Le cardiopatie congenitehanno un interesse molto limitato. La maggiorparte si presenta in età neonatale e sopravvivesolo poche settimane o mesi. I casi cheraggiungono un’età adulta sono molto rari…”
  • 3. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Extraordinary Management Advances Improved Survival RateYear of birth Complex Moderate Simple CHD CHD CHD1940-1959 10% 55% 90%1960-1979 50% 65% 95%1980-1989 80% 90% 95%
  • 4. Department of Pediatric Cardiology & Adult with Congenital Heart Disease AdvancesDiagnosis Therapy Survival
  • 5. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseThe first cause of such increasing number of patients is SURGERY
  • 6. Department of Pediatric Cardiology & Adult with Congenital Heart Disease
  • 7. Department of Pediatric Cardiology & Adult with Congenital Heart Disease
  • 8. Department of Pediatric Cardiology & Adult with Congenital Heart Disease More than 75.000 ACHD in Italy 1200-1600 adolescents enter adulthood every year and require life-long care More than 85% of infants are expected to reach adulthoodChessa M, Cullen S, Deanfield J The care of adult patients with congenital heart defects: a newchallenge. Ital Heart J. 2004 Mar;5(3):178-82.
  • 9. Department of Pediatric Cardiology & Adult with Congenital Heart Disease It is important to outline thatmost pts with CHD who havehad their lives transformed by surgical intervention, had Reparative and not Corrective Surgery
  • 10. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Surgery is corrective, if….…..ventricular function is normal …..life expetancy is normal!…..there is no need for therapeutic measures during f-up
  • 11. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseCorrective Surgery……. Atrial Septal defect Ventricular Septal Defect Patent Ductus Arteriosus…..if treated during childhood!!!
  • 12. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseResiduae Sequelae Mechanical Electrophysiological Functional
  • 13. Department of Pediatric Cardiology & Adult with Congenital Heart Disease
  • 14. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseCHD-PAH: anatomical-pathophysiological classification
  • 15. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Clinical classification of congenital systemic-to-pulmonary shunts associated with PAH1) Eisenmenger syndrome: Large defects and reversed(pulmonary-to-systemic) or bidirectional shunts (eritrocytosis,cyanosis: O2 sat <90%). 2) PAH associated to systemic-to-pulmonary shunts: Moderate to large systemic-to-pulmonary shunts (largely prevalent) (O2 sat >90%). 3) PAH con small defects: similar to idiopathic PAH. 4) Pulmonary arterial hypertension after corrective cardiac surgery.
  • 16. Department of Pediatric Cardiology & Adult with Congenital Heart Disease
  • 17. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Eritrocytosis and ESRight-to-left shunt Hypoxemia Eritrocytosis HyperviscosityVSD with r-l shunt Symptoms Complications Courtesy of Dr D’Alto M
  • 18. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Avoid routine phlebotomy in ES patients! Ready for phlebotomy? PSD GUCH UnitCourtesy of Dr D’Alto M
  • 19. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Phlebotomy in ES patients “Therapeutic phlebotomy, however, is a ‘two-edged sword’ since erythropoietin may stimulate the bone marrow to produce more red cells”. repetitive phlebotomies deplete iron stores iron-deficient red blood cells reduced O2- carrying and deformability of RBC (microcytes) increased risk of strokeCourtesy of Dr D’Alto M
  • 20. Department of Pediatric Cardiology & Adult with Congenital Heart Disease ESC/ ERS 2009 ESC 2010
  • 21. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseAnticoagulation in ES patients? 2009 J Am Coll Cardiol 2009;53:733–40
  • 22. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Cause of death in ES patients“ Death in ES is difficult to predict.The following factors suggest it may be imminent (within next 1-2 y):1. Pregnancy2. Cerebral vascular accident3. Syncope on effort4. RV failure5. Fresh haemoptysis at least 100 ml6. Increasing hypoxia at rest with decreasing effort tolerance7. Atrial arrhythmias8. Ventricular runs (>3 ectopics)9. Operation requiring general anesthesia10. Need for pacing11. Dangerous lifestyle habits (alcohol, disco, Somerville J. Int J Cardiol 1998;63:1-8 hot bats, sauna, drug abuse)”. Courtesy of Dr D’Alto M
  • 23. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseIt is mandatory a correct organization to follow this kind of patients
  • 24. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseNational Centre for 5 to 10 milion people Regional Centre with some expertise and facilities Primary Caregivers Community Cardiologist
  • 25. Thank you for your attention Massimo Chessa massimo.chessa@grupposandonato.it