Congenital questions Flashcards
Understand how to calculate the estimated left to right shunt for an ASD
Aortic Saturation - SVC saturation/ Pulmonary venous saturation - Pulmonary artery saturation
A 2:1 shunt should be repair with elevated PA pressure
What are two most appropriate or preferred repair techniques for coarctation in the neonatal age group
Extended end to end anastomosis
left subclavian flap
What are the 3 most appropriate or preferred repair techniques for coarctation in the adult age group
Interposition graft
resection and end to end anastomosis
balloon dilation with intralumenal stent
List 5 complications of coarctation surgery
paraplegia chylothorax hemorrhage phrenic nerve injury/palsy Horner's syndrome post operative/residual hypertension recurrent coarctation Postcoartecomy syndrome/abdominal pain/mesenteric arteritis
Describe 3 features that preclude percutaneous ASD device closure
Location of ASD primum defect sinus venosus defect deficient spetal rim Size of ASD a defect over 2.5 cm Associated anomalies azygous continuation partial anomalous venous return pulmonary hypertenion
List 5 complications of ASD closure
Residual shunt migration of shunt obstruction of coronary sinus obstruction of pulmonary veins thromboembolic event cardiac chamber performation
List 6 potential causes of hypoxemia folllowing bidirectional cavopulonary anastomosis
pulmonary artery stenosis stenotic anastomosis elevated pulmonary vascular resistance cardiac tamponade left ventricular dysfunction, pulmonary edema anemia atrioventricular valve dysfunction/mitral regurgitation runoff through venous collaterals- azygos vein hemo/pneumothorax hypovolemia excessive PEEP Vq.Q mismatch, atelectasis
Describe the anatomical features/locations of the following types of ventricular septal defects: Perimembranous subaortic ASD Inlet VSD Muscular VSD Doubly comitted subarterial VSD
Perimembranous VSD: located in perimembranous septum under the commissure of the anterior and septal leaflet of the tricuspid valve.
Inlet VSD: located in the inlet of the tricuspid valve, under the commissure of the septal and posterior leaflets.
Muscular VSD: defect is completely surrounded by muscular rim and can be located anywhere in the interventricular septum
Doubly comitted subarterial VSD: there is fibrous continuity between the leaflets of the pulmonary and aortic valves because of the absence of the infundibular septum
Describe the pathophysiology of subvalvular subaortic stensosis
Fixed fibromuscular discrete membrane. Tunnel diffuse subarotic stenosis Hypertrophic cardiomyopathy (IHSS) Abnormal insertion of mitral valve chordae Hypoplastc aortic valve ring
List 4 complications of subaortic stenosis repair
Residual obstruction
heart block
ventricular septal defect
aortic valve tearing
What is natural history of perimembranous VSD if left unoperated
Progressive RV enlargement Congestive heart failure prolapse aortic valve and aortic valve insufficiency risk of endocarditis adult pulmonary hypertension
Patient wishes to have device closure of VSD . What anatomtomicl structures are importance to assess before closure
Tricuspid valve
aortic valve
size of defect
distance from aortic valve to superior edge of defect
What are complications following closure of perimembranous VSD device closure
Left ventricular outflow tract obstruction
residual shunt
device embolization/migration
tricuspid/mitral/aortic valve regurgitation
Arrhythmia/heart block
List the 5 complications or pitfalls specifically associated with surgery for Ebstein’s anomaly
Injury to the AV node or heart block
Injury to the right coronary or posterior descending coronary artery
residual right ventricular outflow tract obstruction
residual atrial septal defect
tricuspid stenosis
residual tricuspid regurgitation
3 year old presents with a right sided effusion 4 weeks post Fontan procedure
List 4 criteria that would strongly suggest the effusion represents a chylothorax
presence of chylomicrons
WBC > 1000
Lymphocyte > 80%
Triglyceride > 1mM
List 4 treatment options in the sequence that normally would be employed for chylothorax
Pleural drainage NPO or low fat or medium change triglyceride diet total parenteral nutrition surgical ligation of thoracic duct octreotide treatment
List 5 complications of unrepaired congenital ventricular septal defect
Congestive heart failure
Aortic insufficiency
Endocarditis
Pulmonary hypertension (Eisenmenger’s syndrome)
right ventricular outflow tract (RVOT) obstruction
severe failure to thrive
Start CPB and the PA pressure remain high? What is this? what can be done?
Likely a patent ductus arteriosus, so quickly gain control of the ductus and ligate it.
Alternatively, clamp the distal left and right pulmonary arteries and then ligate ductus
Classify interrupted aortic arch
Type A: Interruption distal to left subclavian
Type B: interruption between left subclavian and left common carotid (most common)
Type C: interruption between innominate and left common carotid artery
What are associated lesions with interrupted arch
Isolated VSD (most common) Truncus arteriosus transposition of great arteries with VSD including taussig-bing anomaly aortopulmonary window single ventricle subaortic obstruction
What is DiGeorge Syndrome? What 2 things must be done postop?
No thymus. Watch of this when doing sternotomy.
Monitor Calcium level
Blood products must be irrigated
In a 30 year adult should you close an ASD?
Yes, reduces the risk of paradoxical embolism but this controversial.
Yes, because of mortality benefit if closed before age of 40?
New born presents with extreme cyanosis (arterial saturation 45%) that occurs 2 hours after birth. ET and positive pressure ventilation with an FiO3 of 1.0 results in minimal improvement in oxygenation. What are 4 likley diagnosis?
Transposition of Great Arteries
Pulmonary artery atresia with or without VSD
Obstructed total anomalous pulmonary venous drainage
tricuspid atresia
What is immediate pharmacological treatment for a new born in extreme cyanosis
PGE1 infusion should be started immediately to restore ductal patency. Most important thing regardless of diagnosis!
What is surgical treatment for TGA
Definitive operation Arterial switch is performed early (some in first week but most 2 to 4 weeks of life).
If delayed presentation then primary arterial switch is not likely to succeed as the drop in pulmonary pressure causes involution of the left ventricle making it unlikley to be able to function immediately. The right ventricle cannot sustain the required systemic pressure.
The two options for this patient would be pulmoanry artery banding to prepare the RV for systemic pressure or perform an atrial switch. (Mustard or Senning).
What are classical infow and outflow “from” that constitutes the collateral circulation around a classical coarctation of the aorta in an adult partnre]
Inflow;
Internal mammary artery
vertrbral artery
costocervival artery
scapular vessels
thryocervival trunk
Outflow
superior epigastric/inferior epigastric
first two pair of intercostal arteries distal to coarctation (3rd and 4th intercosteal arteries)
Descending aortaWhat is most common aortic arch congenital anomaly
anomalous origin of the right subclavian artery
List 10 complications of PDA closure
Hemorrhage recurrent laryngeal nerve injury chylothorax ductal recanalization or persistent ductal patency left pulmonary artery occlusion accidental aortic ligation infection esphageal injury pulmonary injury vagus nerve injury phrenic nerve injury pneumothorax
4 week old baby girl presents with cyanosis with oxygen saturation at 68% on room air. Investigations show tricuspid atresia , transposition of great arteries, and severe pulmonary stenosis. List the 3 operations that would be used for her staged repair and the age at which each should occur.
Blalock-Taussig shunt (or central shunt) to control cyanosis in newborn period
Bidirectional Glenn anastomisis at 6 to 12 months of age (SVC onto the Pulmonary artery)
Fontan operation at 3 years of age after the child is allowed to grow (Lateral tunnel or extraanatomic bypass from IVC to PA.
what are the 3 most common cardiac causes of cyanosis in a newborn
Tetralogy of Fallot-particulary with pulmonary stenosis
d-TGA
Tricuspid atresia and pulmonary stenosis
Truncus
list anatomical information needed for repair of TOF
The number, size,and location of VSD
The size and morphology of the pulmonary arteries (confluent, presence of MAPCAS)
presence of anomolous coronary artery (typically LAD from RCA)
size of pulmonary annulus
presence of a PDA/ASD
5 week female with TGA presents post balloon septosotomy. What is main concern for proceeding with arterial switch?
What are two other surgical options?
The ability of an unprepared left ventricle to maintain flow to the high pressure systemic circulation
Atrial repair (mustard or senning)
pulmonary artery banding with or without a BT shunt
List 4 anatomic type of vascular rings
double aortic arch
right aortic arch with left ligamentum artiosum
anomalous innominate artery and compression syndrome
anomalous right subclavian artery (dysphagia lusoria)
pulmonary artery sling
Which type of vascular ring is characterized by an anterior indentation in the esphagus on barium swallow
pulmonary artery sling: the left pulmonary artery passes anterior to the esophagus while all other vascular rings run posterior
what are 3 main components of Rastelli repair for TGA with left ventricular outflow tract obstruction
Tunneling a VSD to aortic root
RV-PA conduit
Closure of main PA
Closure of ASD
15 year old who had TOF with transannular patch. List 4 tests you require to assess cardiac function
EKG Cardiac MRI echocardiogram exercise stress test CT angiogram
List 5 criteria for pulmonary valve implantation
Free or severe Pulmonary insufficiency
symptoms of decreased exercise tolerance
progressive RV dilation
decreased exercise tolerance on exercise test
pulmonary artery bifurcation residual stenosis
arrhymias
new onset of TR in the presence of severe pulmonary insufficiency
QRS > 180 msec
7 day old comes to ER respiratory distress, Systolic ejection murmur, abstent femoral pulses, pulmonary edma, severe metabolic acidosis.
List 4 cardiac manifestations
Critical coarctation with VSD
Critical Aortic stenosis
Interrupted aortic arch
hypoplastic left heart syndrome
List treatment plan for 7 day old presenting with severe metabolic acidosis
PGE1 infusion (0.05 mcg/kg/min to .1 mcg/kg/min) intubation and ventilation inotropic support correction of metabolic acidosis
10 month old with TOF, showing hypoplastic pulmonary valve annulus measuring 6 mm with a main pulmonary artery at 6mm. Describe the main components of the operative procedure
Closure of VSD
resection of infundibular pulmonary stenosis
placement of transannular pericardial patch
Briefly describe the different types of total anomalous pulmonary venous connections
Supracardiac– connected by a vertical vein usually to innominate vein
Cardiac–directly into the coronary sinus
infracardiac- to the hepatic vein or the IVC
mixed type: with some veins draining by a vertical vein to the innonminate vein and the rest into the coronary sinus
How to calculate Pulmonary vascular resistance
PVR = mean PA pressure - Wedge (pressure in left atrium)/ Cardiac output. It then equals woods units
do not close thus shunt as PVR is too high resulting in R to L shunting. ASD closure likely to result in right heart failure.
List 4 characteristics of scimitar syndrome
partial anomalous pulmonary venous connection
systemic pulmonary arterial blood supply
right pulmonary artery hypoplasia
right lung hypoplasia or bronchopulmonary sequestraction
List associated lesions that adversely affect the natural history of atrioventricular canal defects
coarctation of the aorta
patent ductus arteriosus
tetralogy of fallot
ventricular hypoplasia
4 month old undergoes uneventful repair of complete AV canal. 6 hours post there is hypotension and elevated left atrial pressure, oilguria, metabolic acidosis.
List 4 reasons for the patients poor status
cardiac tamponade
acute mitral regurgitation
residual ventricular septal defect
undiagnosed coarctation
List 3 morphological factors that determine the feasibility of biventricular repair in an infant with pulmonary atresia and intact ventricular septum
size and competence of tricuspid valve
size and type of right ventricular cavity (Z score of Tricuspid annulus)
presence (or absence) of right ventricle to coronary artery fistula
size and confluence of pulmonary arteries
What are the types of vascular rings
Complete ring a Double arch: right or left dominant, balanced b. right arch/left ligamentum c. right or left arch dominant Incomplete ring d.left arch aberrant RSCA e. innominant artery compression syndrome f. pulmonary artery sling
Review fetal circulation
Well oxygenated blood returns from placenta to umbilical vein
IVC receives all the blood from the placenta–50% goes to hepatic sinusoids and 50% bypasses the liver through the ductus venosus to the IVC
Then from the IVC—RA—through the PFO and on to the LA-LV–and to systemic flow.
What are types of coarctation
Preductal coarctation (5% of infants with Turner Syndrome)
Ductal (usually occurs when the ductus arteriosus closes)
Post ductal coarctation: The narrowing is distal to the insertion of the ductus. Most common in adults
List the classification for congenital ventricular septal defects
Conoventricular (membranous)
Conal (outlet)
Inlet (AV canal)
Muscular (VSD)
Describe conoventricular defects
Most common, 80% of all VSD
located between conal septum and ventricular septum
exclusively in the membranous septum at the anterior-septal commissure of the tricuspid valve
How do you measure the magnitude and direction of the shunt across a VSD
Large VSD offer little resistance to blood flow and labelled “nonrestrictive”.
Right ventricular pressure equals the left ventricular pressure
Small VSD do offer resistance to flow and labelled “restrictive”
Moderate are Qp:QS of 2.5:1 and 3:1
Natural history and indications for VSD closure
approximately 30% develop severe symptoms of CHF and need surgery in first year
Aggressive medical management is indicated in remained membranous and muscular VSD tend to close spontaneously. Asymptomatic with small restrictive VSD can be followed
Malaliignment and or inlet VSD are unlikely to close and closure at time of diagnosis is recommended.
What are other indications for VSD closure
Development of pulmonary vascular resistance
PA pressure greater than one half the systemic pressure in a child older than 1 year
A small proportion of patients with membranous or outlet VSD develop a prolapse of an aortic cusp into the VSD and need surgery
What are surgical approaches for VSD
For conoventricular VSD—approach through the right atriotomy
retract the anteriorseptal commissure
(can detach the septal or anterior leaflet by making incision that paralles the tricuspid annulus)
Class with patch.
Can do infundibular incision
Transventricular approach for conal septal
What are lesions of Ebstein’s anomaly
- adherence of the triscupid leaflets to the underlying myocardium (failure of delamination
- downward/apical displacement of the functional tricuspid annulus
- dilation of the atrialized portion of the right ventricle
- redundancy, fenstrations, and tethering of anterior leaflet
- dilation of the atrioventricular junction
What is Carpentier classification of Ebsteins
a reproducible classification is not possible because of variability.
There is a Carpentier classifcation in the literature.
What are selection criteria for the Fontan procedure
Initially for tricuspid atresia
- minimal age 4
- sinus rhythm
- normal caval drainage
- right atrium normal pressure
- mean pulmonary pressure < 15 mmhg
- pulmonary artery resistance < 4
- no impairing effects of previous shunts
- competent atroventricular valve
- normal ventricles
- pulmonary-artery-to aorta-diameter ratio of > 0.75
What is Fontan circulation
pulmonary and systemic blood flow are in series with the single ventricle connected to the systemic circulation. Venous drainage bypasses the right heart and enters directly into the pulmonary artery
What are components of complete AV canal defect
Primum ASD
Unrestrictive inlet VSD
Common atrioventricular valve
What cardiac lesions is Down Syndrome associated with
Complete AV canal defect–50% of DS
75% of complete AV Canal defects have DS
Rare for a partial AV canal defect to be associated with DS
What are the 3 types of complete AV canal Defect
Rastelli A (75)
Rastelli B –very rare
Rastelli C -associated with TOF
Appears to be based on the superior common leaflet.
What lesions are associated with Coarc
VSD Bicuspid PDA AS Hypoplasia of arch
List congenital lesions of mitral valve
Cor-Triatriatum
Anomalous pulmonary venous drainage (total or partial)
Mitral stenosis–supravalvular and parachute
Mitral atresia
What is mustard procedure
- pericardial patch is sutured to direct drainage of the pulmonary veins in the pulmonary venous atrium and outflow through the tricuspid valve into the RV and out into the aorta for systemic flow.
- The SVC, IVC, and CS are redirected to drain into the systemic venous atrium and outflow through the mitral valve into the LV which then connects to the pulmonary artery and lungs.
What is congenitally corrected Transposition L-TGA
- The morphologic RV is the systemic ventricle and gives rise to the aorta.
- The morphologic LV is the venous ventricle and gives rise to the PA
- atrioventricular discordance and ventrcul-arterial discordance.
- Aorta is anterior to PA - L-Looping TGA
List all the cyanotic lesions
Tetralogy of fallot Tricuspid atresia Transposition of great arteries Total anomalous pulmonary venous return Truncus arteriosus hypoplastic left heart syndome
Name all the acynaotic lesions
atrial septal defect ventricular septal defect patent ductus arteriosus atrioventricular canal coarctation aortic stenosis pulmonary stenosis
What is natural history of ASD
Spontaneous closure (85-90) within the first few years but rare to close after age 3-4
untreated, the average life expectancy is 40 to 50 years old
cause of death: CHF, PHTN, arrhythmia/sudden death
Investigations for ASD
ECG-Axis deviation, atrial arrhythmia CXR- Vascularity ECHO- ASD location, seixe, RV size, PAP, QP/QS, associated lesions TEE: Device closure/sinus venosus CATH: PVR, Device Closure, CAD
What are class I indications for ASD closure
Hemodynamically significant +/- symptoms (Qp:QS> 1.5)
Large secundum (>38 mm) not amenable to device closure
percutaneous closure
Sinus venosus/primum ASD cannot be closed by devices; should be repaired by congenital CVS
If AF/futter occurs, anticoagulation
atrial arrythmias: rate or rhythm control
When NOT to operate for ASD
PVR > 8 woods units and No response to vasodilators
If the L-R shunt Qp/QS is < 1.5
What are class IIa indications for ASD closure
- paradoxical emboli
- orthodeoxia-platypnea—clinical syndrome characterized by dyspnea and deoxygenation accompanying a change to sitting or standing
- Undergoing TV repair/replacement
- Cath ablation prior to device; Maze at ASD surg closure
- Transvenous pacing avoided with unrepaired ASD
- pHTN present and L-R shunt >1.5 or reactivity; care from a specialist
What are complications of DEVICE placement for ASD
Migration of device Residual shunt thromboembolic event obstruction of coronary sinus obstruction of pulmonary veins
Post op surgical closure of ASD complications
Bradycardia
residual ASD
Arrhytmias–increased rates of SVT (AF/Aflutter)
Pericardial effusion or tamponade
What are associated anomalies for VSD
PDA (25%)
Aortic coartaction (10%)
LVOT obstruction (4%)
PFO/ASD, vascular ring, PLSCA
What is natural history of VSD
Close spontaneously: 24-40% by age 2 and 80% by 10 years of age
If unrepaired the subarterial and perimembranous VSD can lead to
a. Endocarditis b. Aortic insufficiency c. Arrhythmia d. RVOTO and LVOTO e. Pulmonary vascular resistance
What are class I indications for VSD repair (CCS ACDS)
- Significant VSD
deteriorating ventricular function due to volume (LV) or pressure (RV) overload
Qp/Qs > 2
Pulmonary artery systolic pressure > 50mmHg - Significant right ventricular outflow tract obstruction
cath or mean echo gradient > 50 mmHg - PM or subarterial VSD with greater then equal to mild Aortic insufficiency
- Severe Pulmonary hypertension PAP > 2/3 MAP or PVR >2/3 SVR—net L-R shunt > 1.5 or PA reactivity
What are class IIa indications to repair a VSD
Endocarditis Before transvenous pacing Device isolated trabecular muscular remove from TV and aortic valve PM VSD far from aortic valve
What are indications for PA banding
Multiple muscular VSD
VSD/CoA
complex lesion with high pulmonary blood flow
infants with very low body weight who are not fit for CPB
