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Flashcards in Congenital Deck (77):

Physiology of a conal VSD

(Supra cristal, subpulmonary, subarterial, juxtarterial, Infundibular)

Below pulmonic valve

  • intimately related to the right aortic cusp.
  • Associated with right coronary cusp prolapse and AI therefore should be repaired.


Route for surgical closure of Infundibular vsd

Through the pa and the pulmonic valve


Conal VSD aka....

Supracristal Sub arterial Subpulmonary Juxtarterial Infundiular


cardiac anomalies associated with unilateral pulmonary genesis

usually relatively benign - PDA and PFO


congenital lobar emphysema- anatomic location

LUL:  40%

RML: 35%

RUL: 20%


congenital lobar emphysema - Male: female

2.5: 1


CCAM - anatomic location

equal all lobes


Radiographic types of CCAM

Type 1: single cyst type

Type 2: many small cysts 

Type 3: solid mass


CCAM - male to female ratio



CCAM - presenting sx

respiratory distress


Lobar emphysema - sx

Tachypnea and respiratory distress


Radiographic appearance of extra lobar sequestration

wedge shaped mass


Radiographic appearance of intralobar sequestration

cyst or solid


Jeune's syndrome

restrictive chest wall syndrome - causing lung hypoplasia


New born Type 1 Esophageal atresia 6.2 cm gap

  • Most children's medical centers consider a gap between proximal and distal esophagus of 6.0 cm or greater to be too long to be reliably closed within the first weeks of life.
  • Plan:
    • Delay the surgery six weeks. 
    • Placement of a gastrostomy tube within 24 hours of birth.
    • Stabilize the neonate and can be prepared for delayed primary repair.


CDH - mortality rate

Although prenatal diagnosis is accurate, the mortality remains high (80%).


CDH w/ polyhydramnios

Polyhydramnios is both the common prenatal marker for congenital diaphragmatic hernia and a predictor of poor clinical outcome.


Most common type of esophageal atresia

2. atresia with tracheal fistula into the distal segment (85-90%)


Types of esophageal atresia

1. atresia with no tracheal fistula (5-8%)

2. atresia with tracheal fistula into the distal segment (85-90%)

3. atresia with tracheal fistula into proximal segment (rare)

4. atresia with tracheal fistula into both segments (rare)

5. "H-type" tracheal fistula without atresia (5%)


Type 1 esophageal atresia

1. atresia with no tracheal fistula (5-8%)


Type 2 esophageal atresia

2. atresia with tracheal fistula into the distal segment (85-90%)


Type 3 esophageal atresia

3. atresia with tracheal fistula into proximal segment (rare)


Type 4 esophageal atresia

4. atresia with tracheal fistula into both segments (rare)


Type 5 esophageal atresia

5. "H-type" tracheal fistula without atresia (5%)


The underlying mechanism for the development of CCAM?

The underlying mechanism for the development of CCAM is segmental bronchial atresia

perhaps resulting from either:

  1. primary disruption during fetal lung bud development
  2. disruption of the fetal bronchial circulation.


DDx of ring and sling by symptoms:

The focus should be to elucidate symptoms. PA sling will typically have airway symptoms only. Vascular rings will have both respiratory and feeding symptoms.


general definition vascular ring

Abnormality of the aortic arch that results in either a complete or partial encirclement of the trachea or esophagus


general definition of vascular sling

Left PA originates from the right PA and passes between the trachea and esophagus


Anomalies associated with VSD - what % of patients with VSD ? - what are they (8)

Remember that 50% of patients with VSD have associated pathologies

  1. Trisomy 21
  2. Left SVC
  3. TAPVR
  4. Additional VSD (usually posterior and muscular) PDA
  5. Accessory orifice to the AV valve
  6. Aortic arch abnormalities
  7. Conotruncal - TOF, DORV, Transposition


  1. How common is late operation required after repair of TOF?:

The frequency of Late Operation following at TOF Repair: 

  • 10%-15%


Common indications for late operations following TOF repair?  


  1. pulmonary regurgitation or stenosis
  2. residual right ventricular outflow tract obstruction
  3. conduit degeneration
  4. residual VSD. 
  5. arrhythmia 


Abnormality associated with IAA type B 

Type B: Between the left subclavian artery and the left common carotid artery

also associated with DiGeorge syndrome and absence of the thymus).


What is the mortality for surgical management of a device adverse event ?

The mortality for surgical management of a device adverse event (2.6%) is 20-fold higher than for primary elective ASD closure (0.13%, P<0.0001).


what patients are at highest risk of pulmonary HTN crisis 

Patients with complete AV canal operated on later in life 

 (i.e., 9-24 months of age).


What anomaly when occurring with truncus has the highest mortality? 

The combination of:

  • interrupted aortic arch


  • severe truncal valve regurgitation

carries a particularly high risk.


Anomalies associated with truncus arteriosis 

Associated Abrnomaities

IAA (B) or coarcation 10-20%

Right aortic arch 25-30%

Persistent left superior vc 10%

Chromosome 22q11 deleton

            DeGeorge Syndrome


Pathophysiology of truncus arteriosis 


Pulmonary over flow

With increase in PVR  failure




incidence 0.1 - 0.3



when does failure typically present? 




RV failure will occur in the 3rd or 4th decade of life




Is cerebral abscesses an issue? 



 In the absence of severe pulmonary stenosis, these patients do not have right-to-left shunting and hence are not at risk for cerebral abscesses.  




Morphology fo the tricuspid valve  

The morphologic tricuspid valve, which is the systemic atrioventricular valve, is the arteriovenous valve that tends to fail in these patients.  

This valve frequently has an Ebsteinoid morphology and may be displaced into the ventricle.


conduction isues with cTGA 

The conduction system of these hearts is abnormal and typically courses anterior to the mitral valve and posterior to the pulmonary valve.

This long pathway is associated with an elevated risk of developing of third-degree (complete) atrioventricular heart block.  



Epidemiology of conduction issues with ccTGA 


  • Some neonates with ccTGA are actually born with complete heart block,

1/3 of ccTGA patients have third-degree AV block by age 30.  

The risk of requiring an AICD is much lower than patients with more common "uncorrected" transposition of the great arteries (d-TGA) who have had an atrial switch (Mustard or Senning) procedure


on reoperation how should cvp be controlled for intracardiac shunts 

CVP  should be > 6 


pt with large heart on cxr 

think ebstein


symptoms related to Ebstein

  1. cyanosis 
  2. cardiomegaly 
  3. malar  rash 
  4. hepatomegaly 
  5. peradoxical embolus 


of patients born with Ebstein, what % of patients need surgery as a neonate 



Echo findings consistent with Ebstein

  1. Septal leaflet : apical displacement > 8mm/m
  2. Dialated RV 
  3. Delayed closure of the TV (.65ms after the MV ) 


Decision-making process for neonate with Ebstein 

  • if dependent on PGE1:       need surgical intervention 
  • if stable with good SaO2:  BT shunt for palliation 
  • If CHF:                                 TV repair 


RV failure after adult Epstein repair 

Consider bilatteral cavo-pulmonary shunt 

(over ASD fenestration - which would lead to worsening cyanosis ) 


% of Truncus arteriosus associated with IAA (B) or coarcation?

% of Truncus arteriosus associated with 

IAA (B) or coarcation 10-20%



% of Truncus arteriosus associated with Right aortic arch ?




Right aortic arch 25-30%



% of Truncus arteriosus associated with  Persistent left superior vc ?



Persistent left superior vc: 10%



Genetic syndromes associated with Truncus arteriosus? 




  1. Chromosome 22q11 deleto ( DeGeorge Syndrome)


Clinical presentation of DiGeorge Syndrome 

While the symptoms can be variable they often include:

  1. congenital heart problems
  2. specific facial feature
  3. frequent infections
  4. developmental delay
  5. learning problems,
  6. cleft palate.
  7. absent of hyplastic thymus 
  8. Parathyroid issues -- hypocalcemia 

Associated condition include kidney problems, hearing loss, and autoimmune disorders such as rheumatoid arthritis or Graves disease.


Physiologic changes with a Glenn Shunt 

  • reduces the volume load on the ventricle ⇒ improves its diastolic function and provides more efficient flow of blood into the pulmonary circulation.
  • superior vena cava inflow results in decreased total blood volume returning to the single ventricle.  
  • with mild tricuspid valve insufficiency, it is important to reduce the volume load to the heart and to prevent progressive worsening of tricuspid regurgitation.
  • Cerebral perfusion pressure is actually decreased after the Glenn procedure.  



Arrhythmia complication after Mustard procedure 

This is in contrast to up to 80% of patients with atrial arrhythmias late after atrial-type switch operations (Mustard or Senning operations)


Most common complication following arterial switch procedure 

Right ventricular outflow tract (RVOT) stenosis is the most commonly seen complication requiring re-intervention following arterial switch. 


Valvular dysfunction in atrial swtich vas arterial switch procedure 

Ventricular dysfunction is observed in as many as 30% to 40% of patients greater than 10 years following atrial switch operations, but in fewer than 10% of patients following arterial switch. 


Frequency Ventricular dysfunction follwoing atrial switch 

Ventricular dysfunction is observed in as many as 30% to 40% of patients greater than 10 years following atrial switch operations, 


Frequency of ventricular dysfunction following arterial switch 

Ventricular dysfunction is observed  in fewer than 10% of patients following arterial switch. patients greater than 10 years


Results of Tricuspid replacement :

Valve choice:

  • Impact on survival? 
  • Freedom from reoperation ? 

Results of Tricuspid replacement :

1.Valve choice:

  • No survival benefit (approximately 60% actuarial survival at 5 years)
  • No difference in freedom from reoperation (> 90% at 5 years).

Results of Tricuspid replacement :

Biologic Valve choice: 

  • Freedom from structural degeneration @ 9 years? 
  • Non structural dyfunction ? 



  • Freedom from structural degeneration is nearly 100% at 9 years
  • non-structural dysfunction is high (73%) due to pannus formation on the ventricular side of the cusps



Results of Tricuspid replacement :

  • Mortality rate of tricuspid replacement 


prosthetic tricuspid replacement is not complication-free, and mortality approximates 10%.



Results of Tricuspid replacement :


  • Risk of CHB? 
  • %? of patients require a pacemaker ? 
  • %? of patients need a pacemaker in 10 years ?


  • The risk of complete heart block is approximately 6%.
  • 10% of patients require a pacemaker during hospitalization for tricuspid valve replacement
  • up to 25% of patients need a pacemaker within 10 years.



Results of Tricuspid replacement :


% of prosthetic valve rate ? 

more so tissue or mechanical? 


Thrombosis of prosthetic tricuspid valves occurs at a rate of 1% per patient-year.

Mechanical:  Thrombosis was more common with past mechanical prostheses (Smeloff-Cutter, Bjork-Shiley) than with current bileaflet valves.

Bioprosthetic: valve thrombosis is rare.



Results of Tricuspid replacement :

Thrombosis of prosthetic valve - does thrombolytics work? 



If a tricuspid prosthesis does clot, however, thrombolytic therapy has a reported success rate of over 80%.



Repair of pulmonary atresia with VSD 

How to evaluate the conduit size 

 If the ratio of the RV to the LV systolic pressures exceeds 0.85, the likelihood of a successful biventricular repair is low,

and the VSD patch should be fenestrated.  This patient's ratio is 0.72, so additional interventions are not indicated


Components of the TOF 

1.                  RVOT obstruction – at several possible levels

2.                  Anterior malalignment VSD

3.                  Aorta dextroposition

4.                  RV hypertrophy ( compensatory)


treatment of a cyanotic TET patient who does better in the prone position 



Patients with tetralogy of Fallot (TOF) and absent pulmonary valve have rudimentary pulmonary valve leaflets that lack 3-dimensional cusp-like anatomy with central deficiencies. --> pulmonary regurgitation and stenosis 

PA can become aneurysmal --> block the trachea 

- artery falls away when they are prone 

- A LeCompte manuaver can fix this obstruction 



Adult patient with TOF repair as a child 

How to determine if pulmonary valve surgery is necessary 

MRI has proven to be the best tool for serial assessment of RV volumes. 

The size of the RV (usually as measured by RV end-diastolic volume indexed to body surface area) at which pulmonary valve replacement is recommended varies from 140-160 mL/m2.


Current recommendations for pulmonary valve replacement 

Because of this natural history, current recommendations for pulmonary valve replacement include indexed RV end-diastolic volume (RVEDV) >150 mL/m2 and RV systolic pressure >2/3 systemic. 




Qp:Qs  =  (Ao sat - SVC sat) / (PV sat - PA sat)

Hi - Lo O2 sat /Hi - lo O2 sat 


Forms of Sinus venosus repair 


Patch repair to baffle blood flow to the RA and close the VSD 


if drainage is high into the SVC (at the level of the azygous) 

Warden Repair: (1) SVC is transected above the pulmonary veins (2) pulmonary veins are baffled to the RA 


OR: two patch technique - intracardiac patch used to baffle the PV and close the ASD (this is associated with sinus node dysfunction) 


Shone's complex 

  1. a supravalvular mitral ring 
  2. parachute mitral valve,
  3. sub aortic stenosis 
  4. coarctation.


The coarc repair most associated with pseudoaneurysm is 

a patch repair 


Second stage for HLHS 

what needs to be ensured before creating a BDCPS 

  1. Relieve any PA stenosis 
  2. Relive any Arch obstruction 
  3. Deal with any AV valve regurgitation 
  4. Ensure an unobstructed intraatrial communication 
  5. Eliminate any shunts