Congenital Flashcards
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
1:1
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
- atresia with tracheal fistula into the distal segment (85-90%)
Types of esophageal atresia
- atresia with no tracheal fistula (5-8%)
- atresia with tracheal fistula into the distal segment (85-90%)
- atresia with tracheal fistula into proximal segment (rare)
- atresia with tracheal fistula into both segments (rare)
- “H-type” tracheal fistula without atresia (5%)
Type 1 esophageal atresia
- atresia with no tracheal fistula (5-8%)
Type 2 esophageal atresia
- atresia with tracheal fistula into the distal segment (85-90%)
Type 3 esophageal atresia
- atresia with tracheal fistula into proximal segment (rare)
Type 4 esophageal atresia
- atresia with tracheal fistula into both segments (rare)
Type 5 esophageal atresia
- “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:
- primary disruption during fetal lung bud development
- 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
- Trisomy 21
- Left SVC
- TAPVR
- Additional VSD (usually posterior and muscular) PDA
- Accessory orifice to the AV valve
- Aortic arch abnormalities
- Conotruncal - TOF, DORV, Transposition
- 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?
Indications:
- pulmonary regurgitation or stenosis
- residual right ventricular outflow tract obstruction
- conduit degeneration
- residual VSD.
- 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
- and*
- 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
Pathophysiology
Pulmonary over flow
With increase in PVR failure
AAOCA
incidence
incidence 0.1 - 0.3
cTGA
when does failure typically present?
ccTGA
RV failure will occur in the 3rd or 4th decade of life
.
cTGA
Is cerebral abscesses an issue?
No
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.
cTGA
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
- cyanosis
- cardiomegaly
- malar rash
- hepatomegaly
- peradoxical embolus
of patients born with Ebstein, what % of patients need surgery as a neonate
50%
Echo findings consistent with Ebstein
- Septal leaflet : apical displacement > 8mm/m2
- Dialated RV
- 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?
- Chromosome 22q11 deleto ( DeGeorge Syndrome)
Clinical presentation of DiGeorge Syndrome
While the symptoms can be variable they often include:
- congenital heart problems
- specific facial feature
- frequent infections
- developmental delay
- learning problems,
- cleft palate.
- absent of hyplastic thymus
- 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 ?
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- 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
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Results of Tricuspid replacement :
- Mortality rate of tricuspid replacement
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prosthetic tricuspid replacement is not complication-free, and mortality approximates 10%.
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Results of Tricuspid replacement :
.Arrhythmia
- Risk of CHB?
- %? of patients require a pacemaker ?
- %? of patients need a pacemaker in 10 years ?
Arrhythmia
- 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.
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Results of Tricuspid replacement :
Thrombosis
% 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
- RVOT obstruction – at several possible levels
- Anterior malalignment VSD
- Aorta dextroposition
- RV hypertrophy ( compensatory)
treatment of a cyanotic TET patient who does better in the prone position
pathology?
treatment?
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
calculation
Qp:Qs = (Ao sat - SVC sat) / (PV sat - PA sat)
Hi - Lo O2 sat /Hi - lo O2 sat
Forms of Sinus venosus repair
Typical:
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
- a supravalvular mitral ring
- parachute mitral valve,
- sub aortic stenosis
- 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
- Relieve any PA stenosis
- Relive any Arch obstruction
- Deal with any AV valve regurgitation
- Ensure an unobstructed intraatrial communication
- Eliminate any shunts
