Congenital Heart Disease and Vascular anomalies

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Congenital Heart Disease and Vascular anomalies
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Normal Fetal Circulation

• The routes of oxygenated blood through the fetal
  heart and great vessels are
• IVC?RA?foramen ovale?LA? LV?aorta
• IVC ? RA? RV? PA? ductus arteriosus? aorta

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Congenital Heart Disease

• Though cardiac catheterization is the definitive
  method of diagnosing congenital heart disease,
  echocardiography and MRI are playing an
  increasingly important role. A key role of the
  radiologist is plain film diagnosis.

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APPROACH TO CONGENITAL HEART DISEASE

• CLINICAL QUESTIONS TO CONSIDER
• Patient's age
• Cyanosis or Acyanosis
• Murmur (optional)

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Congenital Heart disease

• Acyanosis
• nonspecific.
• Murmur
• Often nonspecific - false positives and false
  negatives.

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Symptoms with respect to Patient's Age

• Onset of symptoms at birth.
• With congestive heart failure signs consider
  hypoplastic left heart lesions.
• Cyanosis - variable onset (5 T's).
• Cyanosis suggests right to left shunting (i.e.,
  gt5 gms deoxygenated hemoglobin sent
  systemically).
• Think "5 T's"
• Total anomalous pulmonary venous return (TAPVR).
• Tetralogy of Fallot (T. of F.).
• Truncus arteriosus.
• Tricuspid atresia.
• Transposition of the great vessels (TGV).

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Symptoms with respect to Patient's Age

• Onset of symptoms at approximately six weeks.
• Time required to reduce pulmonary vascular
  resistance to adult levels.
• Think left to right shunt.
• Ventricular septal defect (VSD).
• Atrial septal defect (ASD).
• Patent ductus arteriosus (PDA).

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Radiographic Approach

• Heart size and shape
• Enlarged, normal, small (rare)
• Specific chamber enlargement
• Characteristic configurations
• Pulmonary Vascularitymost important helps to
  know if patient is cyanotic.
• Increasedactive (left to right shunt) or passive
  (congestive heart failure)
• Decreasedsuggests blood not reaching lungs
• Normal

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Increased Pulmonary Vascularity without cyanosis

• Active Congestion with L to R shunts
• Atrial Septal Defect (ASD)foramen secundum near
  foramen ovale and rarer sinus venosus (higher and
  more posterior) are functionally the same
• Enlargement of the RA and RV
• LA not enlarged because of rapid shunting of
  blood from LA to RA
• With larger shunts, PA dilatation occurs
• Rarely symptomatic in childhood

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Increased Pulmonary Vascularity without cyanosis

• Active Congestion with L to R shunts
• Ventricular Septal Defect (VSD)
• Significant shunts (membranous or bulbar defects)
  lead to excessive pulmonary venous return.
  Consequently there is diastolic overloading and
  enlargement of the LA and LV.
• In small defects, L?R shunting occurs during
  systole and no RV enlargement occurs
• With larger defects, both ventricles dilate and
  enlarge present at 2-3 moa with CHF

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Ventricular septal defect
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Increased Pulmonary Vascularity without cyanosis

• Active Congestion with L to R shunts
• Patent Ductus Arteriosus (PDA)
• arises near origin of the LPA and joins aorta
  just distal to L subclavian artery essential in
  the fetus L?R from the aorta to PA when it
  persists after birth.
• Functions during diastole and systole causing
  inc. flow through lungs that returns to the LA
  and LV causing sag due to LV enlargement. Inc.
  flow through aorta results in normal size or
  slight enlargement.
• Large shunts?RV enlargement also

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Increased Pulmonary Vascularity

• Combine Active and Passive congestion
• Occurs in young infants with a congenital lesion
  where there is voluminous intracardiac mixing,
  voluminous L?R shunting or L side obstruction
  with an associated large L?R shunt such as
  coarctation of the aorta and a VSD.

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Increased Pulmonary Vas-cularityPassive
congestion

• Occurs whenever pulmonary venous hypertension
  exists
• Left side obstructive lesions (MS or AS) or left
  side myocardial dysfunction in older infants and
  children
• In the neonate usually due to Total Anomalous
  Pulmonary Venous Return, pulmonary atresia, or
  hypoplastic L heart in the neonate.

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Increased Pulmonary Vascularity with cyanosis

• Active Congestion with L to R shunts
• Total Anomalous Pulmonary Venous Returnresults
  from persistent connection of the pulmonary veins
  to the right side of the heart. Type ISVC or
  azygos vein. Type IIRA or coronary sinus. Type
  IIIsystemic or portal vein.
• In types I and II, blood from the lungs returns
  to the RA and RV causing enlargement. ASD
  necessary to sustain life.
• Type I have snowman or figure 8 heart
  configuration

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Increased Pulmonary Vascularity with cyanosis

• Persistent Truncus Arteriosus
• Failure of division of common truncus into aorta
  and PA. Single vessel drains both ventricles and
  supplies the systemic, pulmonary and coronary
  circulations. Most have high bulbar VSDs.
• Cardiac enlargement is biventricular causing an
  oval shape
• Main Pulmonary Artery prominent in type I, but
  not II, or III.
• Dilated aorta that is R sided in 30-35

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Increased Pulmonary Vascularity with cyanosis

• Active Congestion with L to R shunts
• Transposition of the Great Vessels
• Most common cause of cyanosis due to congenital
  heart disease in newborns
• Aorta always drains the RV but the PA can arise
  1) solely from the LV (complete transposition),
  2) from the RV and LV (Tausig-Bing or double
  outlet, or 3) from the RV with the aorta.

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Increased Pulmonary Vascularity with cyanosis

• Complete Transposition of the Great Vessels
• venous blood from the RV is delivered to the
  aorta, circulated systemically and returned to
  the right side of the heart. On the left,
  oxygenated blood is recycled from lungs to the
  left heart and back to the lungs. Various intra-
  and extra-cardiac shunts are necessary so that
  oxygenated blood can reach the systemic
  circulation.
• Characteristic cardiac configuration is oval or
  egg-shaped with a narrow base causing the egg on
  a string appearance.

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Increased Pulmonary Vascularity with cyanosis

• Atrioventricular Canal Defect (ACD)
• Is the most common congenital heart disease
  lesion in patients with Downs syndrome.

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Decreased Pulmonary Vascularity with cyanosis

• R Outflow Tract Obstruction (with R?L shunt and
  cyanosis)
• Tetralogy of Fallothigh VSD, pulmonary
  stenosis, R ventricular hypertrophy, and
  overriding of the aorta
• Hemodynamically--gtPulmonary stenosis?RV
  hypertrophy?R to L shunting-gtcyanosis
• Classic coeur en sabot (boot-shaped heart) 2ndary
  to RV hypertrophy combined with concave and
  shallow main pulmonary artery

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Decreased Pulmonary Vascularity with cyanosis

• Hypoplastic R heart syndromesmall RV, large LA
  and LV, varying degrees of RA enlargement, and
  atrial R?L shunting
• Tricuspid Atresia (TA)
• tricuspid valve obliterated causing complete
  obstruction to flow of blood from the RA. R?L
  shunt via ASD necessary for blood to reach L side
  of heart. PDA or VSD also usually present.
• Dec. pulm. vascularity and flat or concave PA
• RA enlargement varies with size of ASD
• LV enlargement

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Decreased Pulmonary Vascularity with cyanosis

• Hypoplastic R heart syndrome cont.small RV,
  large LA and LV, varying degrees of RA
  enlargement, and atrial R?L shunting (causes
  cyanosis)
• Tricuspid stenosissimilar to TA
• Pulmonary atresia (usually of pulm. valve)
• Dec. pulm. vascularity and shallow or concave PA
  segment
• Oval heart due to enlarged RA and LV

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Decreased Pulmonary Vascularity with cyanosis

• Hypoplastic R heart syndrome cont.
• Ebsteins Anomaly
• Downward displacement of the septal and
  frequently the posterior leaflets of the
  tricuspid valve causing RV to be functionally and
  anatomically incorporated into RA and obstruction
  of the RA causing inc. RA pressure and R?L
  inter-atrial shunt. Insufficient tricuspid valve
  usually present
• Uhls Diseasesimilar to Ebsteins

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Causes of massive cardiac enlargement

• Congenital Heart Diseases
• Ebsteins Anomaly
• Pulmonary Atresia
• Other Cardiac
• Pericardial Effusion
• Cardiomyopathy
• Cardiac Tumors

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Normal Pulmonary Vascularity

• In Normal patients
• In uncomplicated valvular or vascular lesions
• Aortic stenosis
• Coarction of the Aorta
• Pulmonary stenosis
• Endocardial fibroelastosis

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Normal Pulmonary Vascularity

• L sided valvular or vascular obstructing lesions
• Coarctation of the Aortatwo types
• Postductal or adult type
• narrowing just distal to ductus arteriosus
  produces systolic overloading and hypertrophy of
  the LV
• Rib notching is seen in the 3rd - 9th ribs
  because the 1st and 2nd ribs intercostal arteries
  come from the thyrocervical trunk which is above
  the coarctation
• the "3" sign is created by a prominent left
  subclavian artery, the coarctation and post
  stenotic dilation of the descending aorta.

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L sided valvular or vascular obstructing lesions
cont.

• Coarctation of the aortaPreductal or infantile
  type
• Proximal to the ductus between the left
  subclavian and the ductus
• Longer segment of narrowing
• VSD sometimes present and PDA always present
• The infantile type usually presents with
  congestive heart failure and cardiomegaly.

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Normal Pulmonary Vascularity

• L sided valvular or vascular obstructing lesions
• Aortic Stenosisbicuspid valve
• LV hypertrophy

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L sided valvular or vascular obstructing lesions
cont.

• Hypoplastic L heart syndromemost marked cases
  have severe stenosis or atresia of the aortic and
  mitral valves with marked underdevelopment of the
  LA, LV and aorta causing impairment of blood flow
  from the L side of heart, pulmonary venous
  hypertension, L?R shunt via ASD, and R?L shunt
  via persistent PDA causing cyanosis.
• Heart size is normal at birth but within a few
  hours, cardiomegaly and vascular congestion
  become apparent.
• Cardiomegaly is due to RA and RV enlargement.
• Most common cause of heart failure in first week
  of life

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Normal Pulmonary Vascularity

• Right sided valvular lesions unless R?L shunt
  present, then dec. vascularity
• Pulmonary valve stenosisprominence of MPA but
  very little effect on pulmonary vascularity

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Situs

• Situs solitus is normallevocardia trilobed
  lung, IVC, and systemic venous atrium are on the
  R bilobed lung, pulmonary venous atrium, stomach
  and spleen are on the L.
• Situs inversusdextrocardia abdominal contents
  inversed also?no congenital heart disease but
  associated with Kartageners syndrome.
• Situs ambiguousdextrocardia situs of the
  abdominal viscera and the atria are uncertain or
  indeterminate asplenia and polysplenia exist
  indicating complex congenital heart disease

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L
R
R
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Vascular Anomalies

• Aberrant R subclavian arterymost common vascular
  anomaly arises just distal to the L subclavian
  travels posterior to esophagus indenting it
  rarely causes symptoms but may cause dysphagia.

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Vascular Anomalies cont.

• R aortic arch and mirror-image branching of the
  brachiocephalic vesselsass. with congenital
  heart disease, Tetralogy and Truncus arteriosus
  most common Tricuspid atresia occasionally.

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Vascular Anomalies cont.

• R aortic arch with R Descending Aorta and
  Aberrant L Subclavian arterymost do not have
  congenital heart disease mirror image lesion to
  aberrant R subclavian artery

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Congenital Pericardial Defect

• Partial Defects on L most commoneither
  herniation of a portion of the heart or
  pronounced shift of the heart to the left.

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Superior and Inferior Vena Cava abnormalities

• Persistence of the L SVCwidening of the superior
  mediastinum can occur alone or with congenital
  heart disease and may drain into the LA.

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Summary I

• Increased Pulmonary Vascularity without cyanosis
• ASD
• VSD
• PDA
• Increased Pulmonary Vascularity with cyanosis
• TAPV
• Truncus
• Transposition

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Summary II

• Decreased Pulmonary vascularity with cyanosis
• Tetralogy
• Hypoplastic R Heart
• Tricuspid Atresia
• Pulmonary Atresia
• Ebsteins
• Normal Pulmonary Vascularity
• AS
• Coarctation
• Heart Failure in Newborn
• Hypoplastic L Heart

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References

• Wimpfheimer O, Boxt LM MR Imaging of adult
  patients with congenital heart disease. Rad Clin
  North Am 37421-438,1999
• Baron MG Plain film diagnosis of common cardiac
  anomalies in the adult. Plain film diagnosis of
  common cardiac anomalies in the adult. Rad Clin
  North Am 37401-420, 1999
• Strife JL, Sze RW Radiographic evaluation of the
  neonate with congenital heart disease. Rad Clin
  North Am 371093-1107
• Swischuk L Congenital Heart Disease, Williams
  and Wilkins.
• http//bubbasoft.org/index.htm