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Adult Congenital Heart Disease

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Adult Congenital Heart Disease Powered By Docstoc
					   Adult Congenital
    Heart Disease
“Common” Complex Lesions

     Jeffrey D. Solomon, MD

    November 14 & 19, 2008
                 Objectives
• Describe the changing profile of ACHD
• Describe the challenges in the transfer of care
  from pediatric to adult practitioners
• Review the development of the heart
• Describe the anatomy, natural history, surgical
  repair, and common adult presentations of
  tetralogy of Fallot, transposition of the great
  arteries, and hypoplastic left heart syndrome
                     ACHD
• A gross structural abnormality of the heart or
  intrathoracic great vessels that is actually or
  potentially of functional significance
• Overall incidence: 75 of every 1000 live births
                     ACHD
• A gross structural abnormality of the heart or
  intrathoracic great vessels that is actually or
  potentially of functional significance
• Overall incidence: 75 of every 1000 live births
• Incidence of moderate and severe CHD: 8 of
  every 1000 live births
                    ACHD
• A gross structural abnormality of the heart or
  intrathoracic great vessels that is actually or
  potentially of functional significance
• Overall incidence: 75 of every 1000 live births
• Incidence of moderate and severe CHD: 8 of
  every 1000 live births
• Over 90% of children survive to adulthood
• 20,000 operations for CHD in the US each year
• Over 1 million adults in the US with CHD
   Transfer/Transition of Care
• Challenges
  – Complex CHD is seldom cured
  – Patients have a poor understanding
  – Timing of transition
  – Lack of exposure among adult
    cardiologists
   Transfer/Transition of Care
• 32nd Bethesda Conference (2000)
   – Regular evaluation at an ACHD center
   – Health care passport
   – Structured transfer/transition plan
   – Co-management with cardiologists
     having undergone advanced training
     Tetralogy of Fallot (TOF)
• Named by Etienne-Louis Arthur Fallot in 1888
• Approximately 10% of all complex CHD
• Single developmental error of the terminal
  portion of the spiral truncoconal septum
• Four distinct components: subpulmonic stenosis,
  VSD, overriding aorta, and RV hypertrophy
• Often accompanied by other anomalies
              Natural History
• Depends on severity of RVOT obstruction
• Cyanosis and exertional dyspnea
• “Tet spell”
   – profound cyanotic episode secondary to
     augmented right-to-left shunt
• Without surgical correction, patients do poorly
            Surgical Repair
• Longest surgical history/most studied outcomes
• Palliative surgical shunts
             Surgical Repair
• Longest surgical history/most studied outcomes
• Palliative surgical shunts
• Classic/complete repair
  – Infundibular muscle resection, VSD patch, &
    RVOT repair
  – Typically preformed between 4-6 months of age
  – Surgical risk: < 5%
• Survival rates: 85% at 30+ years
          Adult Presentation
• Repaired
  – RVOT obstruction
  – Pulmonary or tricuspid regurgitation
          Adult Presentation
• Repaired
  – RVOT obstruction
  – Pulmonary or tricuspid regurgitation
  – LV/RV dysfunction
  – Atrial/ventricular arrhythmias
• Unrepaired
  – Significant morbidity
  – Consider later repair
         Transposition of the
         Great Arteries (TGA)
• Complete TGA or D-TGA
  – Embryological inversion of the great arteries
  – Ventriculoarterial discordance
         Transposition of the
         Great Arteries (TGA)
• Complete TGA or D-TGA
  – Embryological inversion of the great arteries
  – Ventriculoarterial discordance
• Congenitally corrected TGA or L-TGA
  – Embryological inversion of the ventricles
  – Atrioventricular & ventriculoarterial
    discordance (double discordance)
              Surgical Repair
• Balloon atrial septostomy
  – Developed by Rashkind (1965)
  – Enlarges the atrial communication
• Atrial switch
  – Performed first by Senning (1958) and later
     modified by Mustard (1964)
  – Atrial baffle is created to direct venous return
     to the contralateral ventricle
             Adult Presentation

•   RV dysfunction
•   Tricuspid insufficiency
•   Bradyarrhythmias
•   Atrial tachycardias
•   Obstructed/leaky atrial baffle
             Surgical Repair
• Arterial switch
  – Performed first by Jatene (1976)
  – Great arteries transected and reattached to
    appropriate AV valve
  – Coronary ostia also transplanted
  – Surgical treatment of choice
  – Excellent outcomes so far
             Adult Presentation

•   Coronary perfusion issues
•   Supravalvar aortic and pulmonic stenosis
•   Aortic root dilatation and valvular insufficiency
•   Branch pulmonary stenosis
   Single Ventricle Physiology
• Hypoplastic left-heart syndrome
• Hypoplastic right-heart syndrome (pulmonary
  atresia with intact ventricular septum)
• Tricuspid valve atresia
• Mitral valve atresia
• Double-inlet ventricle
• Atrioventricular canal defects
• Double outlet right ventricle
             Surgical Repair
• Staged rerouting of the systemic venous return
• Step One
  – Complete mixing of systemic and pulmonary
    venous return
  – Unobstructed outflow tract to the systemic
    arterial circulation
  – Reliable source of pulmonary blood flow
          Adult Presentation

• RV dysfunction
• Cyanosis
• Protein losing enteropathy
                Key Points
• The majority of children born with CHD survive
  into adulthood
• Infants born with complex CHD are seldom
  cured; residual problems are common and
  predictable
• There are many challenges in the transition of
  care from pediatric to adult practitioners
• Adults with moderate and complex CHD should
  undergo regular evaluation at a regional ACHD
  center
             Selected References
Bashore TM. Adult congenital heart disease: right ventricular outflow
  tract lesions. Circulation 2007 115: 1933-1947.
Gewillig M. The Fontan circulation. Heart 2005 91: 839-846.
Hoffman JIE, Kaplan S. The incidence of congenital heart disease. J
  Am Coll Cardiol 2002 39: 1890-1900.
Sommer RJ, Hijazi ZM, Rhodes JF. Pathophysiology of congenital
  heart disease in the adult: part III: complex congenital heart disease.
  Circulation 2008 117: 1340-1350.
Warnes CA. Transposition of the great arteries. Circulation 2006 114:
  2699-2709.
Warnes CA, Liberthson R, Danielson GK, Dore A, Harris L, Hoffman
  JIE, Somerville J, William RG, Webb GD. Task force 1: the
  changing profile of congenital heart disease in adult life. J Am Coll
  Cardiol 2001 37: 1170-1175.

				
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posted:1/14/2012
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