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Massimo Chessa

    Department of Pediatric Cardiology
                    &
    Adult with Congenital Heart Disease


      IRCCS- Policlinico San Donato
      San Donato Milanese – Milano

    massimo.chessa@grupposandonato.it



LE CARDIOPATIE CONGENITE
     Cosa sapere in relazione
    all’ipertensione polmonare
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




Scriveva William Osler (Medico
Canadese 1849-1919) definito a
ragione il padre della medicina
moderna: “... Le cardiopatie congenite
hanno un interesse molto limitato. La maggior
parte si presenta in età neonatale e sopravvive
solo poche settimane o mesi. I casi che
raggiungono un’età adulta sono molto rari…”
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




    Extraordinary Management Advances Improved Survival
                          Rate


Year of birth     Complex                              Moderate                            Simple
                   CHD                                  CHD                                 CHD

1940-1959               10%                                   55%                           90%

1960-1979               50%                                   65%                           95%

1980-1989               80%                                   90%                           95%
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




                Advances

Diagnosis                                                         Therapy


                           Survival
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




The first cause of such increasing
       number of patients is
                    SURGERY
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 Italy
       1200-1600 adolescents enter adulthood every year and
                      require life-long care
   More than 85% of infants are expected to reach adulthood




Chessa M, Cullen S, Deanfield J The care of adult patients with congenital heart defects: a new
challenge. Ital Heart J. 2004 Mar;5(3):178-82.
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




 It is important to outline that
most pts with CHD who have
had their lives transformed by
   surgical intervention, had
          Reparative
                         and not
     Corrective Surgery
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
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




Corrective Surgery…….

     Atrial Septal defect
 Ventricular Septal Defect
 Patent Ductus Arteriosus


…..if treated during
   childhood!!!
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




Residuae                                                                              Sequelae



      Mechanical
                                                     Electrophysiological


                 Functional
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart Disease



CHD-PAH: anatomical-pathophysiological classification
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




                   Clinical classification of congenital
           systemic-to-pulmonary shunts associated with PAH


1) 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.
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart Disease



                               Eritrocytosis and ES


Right-to-left shunt


                       Hypoxemia



                                                    Eritrocytosis



                                                                        Hyperviscosity

VSD with r-l shunt                                                                                        Symptoms
                                                                                                         Complications
                 Courtesy of Dr D’Alto M
Department of Pediatric Cardiology & Adult with Congenital Heart Disease



            Avoid routine phlebotomy in ES patients!
                                                                                                      Ready for
                                                                                                     phlebotomy?
                                  PSD
                            GUCH Unit




Courtesy of Dr D’Alto M
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 stroke
Courtesy of Dr D’Alto M
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




                                                                ESC/ ERS 2009




                            ESC 2010
Department of Pediatric Cardiology & Adult with Congenital Heart Disease



Anticoagulation in ES patients?




                                                                                2009




                              J Am Coll Cardiol 2009;53:733–40
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.  Pregnancy
2.  Cerebral vascular accident
3.  Syncope on effort
4.  RV failure
5.  Fresh haemoptysis at least 100 ml
6.  Increasing hypoxia at rest with decreasing effort
    tolerance
7. Atrial arrhythmias
8. Ventricular runs (>3 ectopics)
9. Operation requiring general anesthesia
10. Need for pacing
11. 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
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




It is mandatory a correct organization to
       follow this kind of patients
Department of Pediatric Cardiology & Adult with Congenital Heart Disease



National Centre for 5 to 10 milion
            people                                                                     Regional Centre
                                                                                         with some
                                                                                        expertise and
                                                                                          facilities




      Primary
     Caregivers


                                                                            Community
                                                                            Cardiologist
Thank you for your attention
       Massimo Chessa
    massimo.chessa@grupposandonato.it

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Ph in achd

  • 1. Massimo Chessa Department of Pediatric Cardiology & Adult with Congenital Heart Disease IRCCS- Policlinico San Donato San Donato Milanese – Milano massimo.chessa@grupposandonato.it LE CARDIOPATIE CONGENITE Cosa sapere in relazione all’ipertensione polmonare
  • 2. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Scriveva William Osler (Medico Canadese 1849-1919) definito a ragione il padre della medicina moderna: “... Le cardiopatie congenite hanno un interesse molto limitato. La maggior parte si presenta in età neonatale e sopravvive solo poche settimane o mesi. I casi che raggiungono un’età adulta sono molto rari…”
  • 3. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Extraordinary Management Advances Improved Survival Rate Year of birth Complex Moderate Simple CHD CHD CHD 1940-1959 10% 55% 90% 1960-1979 50% 65% 95% 1980-1989 80% 90% 95%
  • 4. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Advances Diagnosis Therapy Survival
  • 5. Department of Pediatric Cardiology & Adult with Congenital Heart Disease The 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 adulthood Chessa M, Cullen S, Deanfield J The care of adult patients with congenital heart defects: a new challenge. Ital Heart J. 2004 Mar;5(3):178-82.
  • 9. Department of Pediatric Cardiology & Adult with Congenital Heart Disease It is important to outline that most pts with CHD who have had 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 Disease Corrective Surgery……. Atrial Septal defect Ventricular Septal Defect Patent Ductus Arteriosus …..if treated during childhood!!!
  • 12. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Residuae Sequelae Mechanical Electrophysiological Functional
  • 13. Department of Pediatric Cardiology & Adult with Congenital Heart Disease
  • 14. Department of Pediatric Cardiology & Adult with Congenital Heart Disease CHD-PAH: anatomical-pathophysiological classification
  • 15. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Clinical classification of congenital systemic-to-pulmonary shunts associated with PAH 1) 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 ES Right-to-left shunt Hypoxemia Eritrocytosis Hyperviscosity VSD 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 Unit Courtesy 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 stroke Courtesy 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 Disease Anticoagulation 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. Pregnancy 2. Cerebral vascular accident 3. Syncope on effort 4. RV failure 5. Fresh haemoptysis at least 100 ml 6. Increasing hypoxia at rest with decreasing effort tolerance 7. Atrial arrhythmias 8. Ventricular runs (>3 ectopics) 9. Operation requiring general anesthesia 10. Need for pacing 11. 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 Disease It is mandatory a correct organization to follow this kind of patients
  • 24. Department of Pediatric Cardiology & Adult with Congenital Heart Disease National 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