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Massimo Chessa    Department of Pediatric Cardiology                    &    Adult with Congenital Heart Disease      IRCC...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseScriveva William Osler (MedicoCanadese 1849-1919) ...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease    Extraordinary Management Advances Improved Sur...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                AdvancesDiagnosis                 ...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseThe first cause of such increasing       number of...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                        More than 75.000 ACHD in I...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease It is important to outline thatmost pts with CHD ...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease Surgery is corrective, if….…..ventricular functio...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseCorrective Surgery…….     Atrial Septal defect Ven...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseResiduae                                          ...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseCHD-PAH: anatomical-pathophysiological classificat...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                   Clinical classification of cong...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                               Eritrocytosis and E...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease            Avoid routine phlebotomy in ES patient...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                           Phlebotomy in ES patien...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                                                  ...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseAnticoagulation in ES patients?                   ...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease Cause of death in ES patients“ Death in ES is dif...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseIt is mandatory a correct organization to       fo...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseNational Centre for 5 to 10 milion            peop...
Thank you for your attention       Massimo Chessa    massimo.chessa@grupposandonato.it
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Ph in achd

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Ph in achd

  1. 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. 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. 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. 4. Department of Pediatric Cardiology & Adult with Congenital Heart Disease AdvancesDiagnosis Therapy Survival
  5. 5. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseThe first cause of such increasing number of patients is SURGERY
  6. 6. Department of Pediatric Cardiology & Adult with Congenital Heart Disease
  7. 7. Department of Pediatric Cardiology & Adult with Congenital Heart Disease
  8. 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. 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. 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. 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. 12. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseResiduae Sequelae Mechanical Electrophysiological Functional
  13. 13. Department of Pediatric Cardiology & Adult with Congenital Heart Disease
  14. 14. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseCHD-PAH: anatomical-pathophysiological classification
  15. 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. 16. Department of Pediatric Cardiology & Adult with Congenital Heart Disease
  17. 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. 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. 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. 20. Department of Pediatric Cardiology & Adult with Congenital Heart Disease ESC/ ERS 2009 ESC 2010
  21. 21. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseAnticoagulation in ES patients? 2009 J Am Coll Cardiol 2009;53:733–40
  22. 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. 23. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseIt is mandatory a correct organization to follow this kind of patients
  24. 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. 25. Thank you for your attention Massimo Chessa massimo.chessa@grupposandonato.it

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