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A Quick Tour of Congenital Heart Disease. Chris Longhurst, MD Monday, March 10, 2014. Introduction. Present in 0.8% of North American and European children Most common category of congenital structural malformation

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a quick tour of congenital heart disease

A Quick Tour of Congenital Heart Disease

Chris Longhurst, MD

Monday, March 10, 2014

  • Present in 0.8% of North American and European children
  • Most common category of congenital structural malformation
  • Commonly divided into noncyanotic (L  R) and cyanotic (R  L) categories based on direction of shunting
relative frequency of lesions
Relative Frequency of Lesions
  • Ventricular septal defect 25-30
  • Atrial septal defect (secundum) 6-8
  • Patent ductus arteriosus 6-8
  • Coarctation of aorta 5-7
  • Tetralogy of Fallot 5-7
  • Pulmonary valve stenosis 5-7
  • Aortic valve stenosis 4-7
  • Transposition of great arteries 3-5
  • Hypoplastic left ventricle 1-3
  • Hypoplastic right ventricle 1-3
  • Truncus arteriosus 1-2
  • Total anomalous pulm venous return 1-2
  • Tricuspid atresia 1-2
  • Double-outlet right ventricle 1-2
  • Others 5-10
noncyanotic chd l r
Noncyanotic CHD (L R)
  • Atrial septal defects (ASD)
  • Ventricular septal defects (VSD)
  • Patent ductus arteriosus (PDA)
  • Obstruction to blood flow
    • Pulmonic stenosis (PS)
    • Aortic stenosis (AS)
    • Aortic coarctation
atrial septal defect
Atrial Septal Defect
  • Most commonly asymptomatic
  • Essentials of diagnosis:
    • Right ventricular heave
    • S2 widely split and usually fixed
    • Grade I-III/VI systolic murmur at the pulmonary area
    • Widely radiating systolic murmur mimicking PPS in infancy
    • Cardiac enlargement on CXR
atrial septal defect7
Atrial Septal Defect
  • Three major types
    • Ostium secundum
      • most common
      • In the middle of the septum in the region of the foramen ovale
    • Ostium primum
      • Low position
      • Form of AV septal defect
    • Sinus venosus
      • Least common
      • Positioed high in the atrial septum
      • Frequently associated with PAPVR
atrial septal defect8
Atrial Septal Defect
  • Treatment
    • Closure generally recommended when ratio of pulmonary to systemic blood flow (qP/qS) is > 2:1
    • Operation performed electively between ages 1 and 3 years
  • Previously surgical; now often closed interventionally
ventricular septal defect
Ventricular Septal Defect
  • Single most common congenital heart malformation, accounting for almost 30% of all CHD
  • Defects can occur in both the membranous portion of the septum (most common) and the muscular portion
ventricular septal defect12
Ventricular Septal Defect
  • Three major types
  • Small, hemodynamically insignificant
      • Between 80% and 85% of all VSDs
      • < 3 mm in diameter
      • All close spontanously
        • 50% by 2 years
        • 90% by 6 years
        • 10% during school years
      • Muscular close sooner than membranous
ventricular septal defect13
Ventricular Septal Defect
  • Moderate VSDs
    • 3-5 mm in diameter
    • Least common group of children (3-5%)
    • Without evidence of CHF or pulmonary hypertension, may be followed until spontaneous closure occurs
ventricular septal defect14
Ventricular Septal Defect
  • Large VSDs with normal PVR
    • 6-10 mm in diameter
    • Usually requires surgery, otherwise…
    • Will develop CHF and FTT by age 3-6 months
ventricular septal defects
Ventricular Septal Defects
  • Clinical findings
    • Grade II-IV/VI, medium- to high-pitched, harsh pansystolic murmur heard best at the left sternal border with radiation over the entire precordium
ventricular septal defect16
Ventricular Septal Defect
  • Treatment
    • Indicated for closure of a VSD associated with CHF and FTT or pulmonary hypertension
    • Patients with cardiomegaly, poor growth, poor exercise tolerance, or other clinical abnormalities and a qP/qS > 2:1 typically undergo surgical repair at 3-6 mo
patent ductus arteriosus
Patent Ductus Arteriosus
  • Persistence of normal fetal vessel joining the pulmonary artery to the aorta
  • Closes spontaneously in normal term infants at 3-5 days of age
  • Epi facts
    • Accounts for about 10% of all cases of CHD
    • Higher incidence of PDA in infants born at high altitudes (> 10,000 feet)
    • More common in females
patent ductus arteriosus18
Patent Ductus Arteriosus
  • Accounts for about 10% of all cases of CHD
  • Higher incidence of PDA in infants born at high altitudes (over 10,000 feet)
  • More common in females
patent ductus arteriosus20
Patent Ductus Arteriosus
  • Clinical findings and course depend on size of the shunt and the degree of associated pulmonary hypertension
patent ductus arteriosus21
Patent Ductus Arteriosus
  • Pulses are bounding and pulse pressure is widened
  • Characteristically has a rough “machinery” murmur which peaks at S2 and becomes a decrescendo murmur and fades before the S1
patent ductus arteriosus22
Patent Ductus Arteriosus
  • Treatment consists of surgical correction when the PDA is large except in patients with pulmonary vascular obstructive disease
  • Transcatheter closure of small defects has become standard therapy
  • In preterm infants indomethacin is used (80-90% success in infants > 1200 grams)
cyanotic chd r l
Cyanotic CHD (R L)
  • Tetralogy of Fallot (TOF)
  • Tricuspid atresia (TA)
  • Total anomalous pulmonary venous return (TAPVR)
  • Truncus arteriosus
  • Transposition of the great vessels
  • Hypoplastic left heart syndrome (HLH)
  • Pulmonary atresia (PA) / critical PS
  • Double outlet right ventricle (DORV)
tetralogy of fallot
Tetralogy of Fallot
  • “Cyanosis, especially in the adult, is the result of a small number of cardiac malformations well determined…. One…is much more frequent than the others…. This malformation consists of a true anatomopathologic type represented by the following tetralogy: (1) Stenosis of the pulmonary artery; (2) Interventricular communication; (3) Deviation of the origin of the aorta to the right; and (4) Hypertrophy, almost always concentric in type, of the right ventricle. Failure of obliteration of the foramen ovale may occasionally be added in a wholly accessory manner.”
    • Fallot, Ètienne-Louis-Arthur. Contribution to the pathologic anatomy of morbus caeruleus (cardiac cyanosis). Marseilles Med. 1888; 25:418-20.
tetralogy of fallot26
Tetralogy of Fallot
  • Most common cyanotic lesion (7 to 10% of all CHD)
  • Typical features
    • Cyanosis after the neonatal period
    • Hypoxemic spells during infancy
    • Right-sided aortic arch in 25% of all patients
    • Systlic ejection murmur at the upper LSB
tetralogy of fallot27
Tetralogy of Fallot
  • Clinical findings vary depending on degree of RVOFT obstruction
  • Most patients are cyanotic by 4 months and it is usually progressive
  • Hypoxemic spells (“tet spells”) are one of the hallmarks of severe tetralogy
tetralogy of fallot29
Tetralogy of Fallot
  • Tet spells most commonly start around 4 to 6 months of age and are charcterized by
      • Sudden onset or deepening of cyanosis
      • Sudden onset of dyspnea
      • Alterations of consciousness
      • Decrease in intensity of systolic murmur
tetralogy of fallot30
Tetralogy of Fallot
  • Repair may be staged (modified BT shunt) or complete