Term
| epidemiology of congential heart defects |
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Definition
• Present at birth, may present later
• Most common birth defect ~1% of live births
• About 1/3 of those with CHD require intervention, either with surgery or catheterization in first year of life
• CHD – leading cause of mortality from birth defects in developed countries
• EtiologyofCHD–poorly understood
• Majority of CHD secondary to genetic mutations.
• Some studies have suggested population- specific genetic contributions |
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Term
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Definition
Parallel circulation 3 circulatory shunts :
• DuctusVenosus(DV) • ForamenOvale(FO) • Ductusarteriosus(DA)
Deliver most oxygenated blood to the brain, and coronaries. |
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Term
| post natal transition steps |
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Definition
• Begins with the first breath as spontaneous respiration is begun
• ↓ in pulmonary vascular resistance and ↑ systemic vascular resistance
• ↑oxygen content in the pulmonary vascular bed → pulmonary vasodilation → increase in pulmonary blood flow
• Increase in left atrial pressure →closure of the flap of foramen ovale
• Expansion of lungs with air – increased oxygen tension ..> closure of the ductus arteriosus |
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Term
| overall classifications of congenital heart defects |
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Definition
• Left to right shunts (Non cyanotic )
• Right to left shunts (Cyanotic)
• Left sided obstructive lesions
• Other complex CHD – single ventricle, Double outlet right ventricle, etc. |
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Term
| 3 types of non cyanotic lesions |
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Definition
• Atrial septal defects (ASD)
• Ventricular septal defects (VSD)
• Patent ductus arteriosus (PDA) |
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Term
| atrial septal defect incidence and types |
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Definition
• Second most common
• Types
-Secundum ASD – most common among ASD (middle)
- Primum ASD – usually associated with AV canal defect (closer to AV node)
- Sinus venosus (closer to SA node) |
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Term
| physiology of atrial septal defect |
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Definition
• Low pressure shunt
• Flow dictated by difference in relative diastolic compliance of RV and LV.
• Shunt : RA→LA
• Chamber enlargement RA, RV, PA
• Pulmonary hypertension |
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Term
| presentation of atrial septal defect |
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Definition
• Usually late childhood, adulthood
• SOB, exercise intolerance
• Exam:
• Systolic ejection murmur (relative PS)
• Diastolic murmur
• Wide and fixed split S2
• EKG : RA enlargement, RV conduction delay.
• CXR : Mild cardiomegaly, increased pulmonary vascularity
• Echocardiogram |
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Term
| diagnostic work up of atrial septal defect, EKG findings, CXR findings, other testing |
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Definition
• EKG : RA enlargement, RV conduction delay, RVH
• CXR : mild cardiomegaly, increased pulmonary vascularity
• Echo
• Cardiac catheterization |
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Term
| management of atrial septal defect |
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Definition
• Observation for spontaneous closure
• Elective closure ~ 3 years of age
• Surgical:
- Patch closure
- Primary closure
• Trans-catheter device closure:
- Location: only secundum defects
- Presence of sufficient rims |
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Term
| ventricular septal defects incidence, associated w |
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Definition
• Most common form of CHD ~ 20 %-30%
• Different types based on location
• Frequently associated with various syndrome (eg. Trisomy 21) |
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Term
| parts of ventricular septum and types of ventricular septal defects |
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Definition
• Parts of the ventricular septum:
• Inlet septal
• Muscular or trabecular septum
• Outlet septum
• Types of VSDs:
• Conoventricular
• Inlet
• Muscular
• Supracristal (outlet) |
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Term
| perimembranous ventricular septal defect location, can lead to, findings |
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Definition
• Underneath the aorta in LV and the tricuspid valve in the RV
• Aneurysmal tricuspid tissue may form→ spontaneous closure
• Aortic cusp prolapse → Aortic regurgitation |
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Term
| muscular ventricular septal defects |
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Definition
• Single or multiple
• Spontaneous closure |
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Term
| inlet ventricular septal defects |
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Definition
• Commonly seen with atrioventricular defects
• Most posteriorly located
• Superior frontal QRS axis on EKG
• Would need surgical closure |
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Term
| supra-cristal ventricular septal defects |
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Definition
• OutflowregionoftheRV
• Underbothsemi-lunar valves
• Aorticvalveprolapseand aortic regurgitation
• Associatedwithmorbidity
• Wouldneedsurgical closure |
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Term
| picture w location of ventricular septal defects |
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Definition
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Term
| physiology of ventricular septal defects |
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Definition
• Vascular resistances ( Blood flow from high pressure – low pressure chamber)
• Shunting LV→ RV
• For large VSDs :
-Chambers dilated – PA, pulmonary vascular bed LA, LV
- Pulmonary Vascular disease – exposure to systemic pressure |
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Term
| presentation of ventricular septal defects for large vs small |
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Definition
- Large defects: around 4-6 weeks of age
• Poor feeding, tachypnea, diaphoresis with feeds (CHF)
• Tachypnea, tachycardic, active precordium,
• Murmur- holosystolic + diastolic rumble
• Gallop
-Small defects : asymptomatic
• Normal growth
• Quiet precordium
• Louder murmur – harsher +/- thrill |
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Term
| diagnostic work-up of ventricular septal defects |
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Definition
• CXR
• EKG – LVH, LA enlargement
• Echo
• Cardiac cath:
- Qp:Qs
- Pulmonary hypertension |
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Term
| management/treatment of ventricular septal defects |
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Definition
• Observe and follow up spontaneous closure
• Medical treatment with anti-congestive medications, optimization of nutrition
• Surgical – Patch closure
• Trans-catheter closure- muscular defects |
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Term
| outcomes and long term findings with ventricular septal defects |
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Definition
-Good overall outcomes
- No major long term deficits
- Bacterial endocarditis prophylaxis
• Unrepaired – none
• Repaired VSD • 6 months post repair • Residual shunt
-No exercise restrictions |
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Term
| Patent Ductus Arteriosus is what, found when |
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Definition
• Connects aorta to pulmonary artery • Normal structure during fetal life
• Closes spontaneously after birth
• Premature babies- delayed closure |
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Term
| patent ductus arteriosus physiology |
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Definition
• Flow – difference in vascular resistances
• Aorta → PA
• increased flow to PA, pulmonary capillaries, pulmonary veins, LA, LV
• exposure of the pulmonary vasculature to high pressure–
vascular disease over time |
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Term
| presentation of patent ductus arteriosus |
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Definition
• If small (restrictive), continuous murmur, no symptoms
• If large, continuous murmur with signs of congestive heart failure (tachypnea, slow growth at 3 to 6 weeks of age)
• CHF earlier in premature infants |
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Term
| management of patent ductus arteriosus |
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Definition
- Medical management
• Anti congestive medications
• Premature babies-indomethacin
- Surgical management
• PDA ligation for Premature infants, newborns with CHF
-Trans-catheter
• Coilordeviceclosure
• Also a possibility in premature babies |
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Term
| types of cyanotic congenital heart defects |
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Definition
• Tetralogy of Fallot
• Transposition of great arteries
• Pulmonary stenosis
• Tricuspid atresia
• Total anomalous pulmonary venous return |
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Term
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Definition
• Most common cyanotic CHD
• 0.25 per 1000 live births
• Entails :
1. Ventricular Septal defect
2. Stenotic pulmonary valve, pulmonary arteries
3. Over-riding aorta
4. Right Ventricular hypertrophy
• Commonly associated with 22 q 11 del |
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Term
| physiology of tetralogy of fallot |
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Definition
• Obstruction to blood flow in the right ventricular outflow tract, across PV →Right to left flow across VSD→ cyanosis
• Degree of pulmonary stenosis will detect degree of cyanosis |
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Term
| presentation of tetralogy of fallot |
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Definition
• Cyanosis – birth to early months
• Exam
-Prominent right ventricular impulse/ RV heave
- Systolic ejection murmur
- Single S2 |
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Term
| diagnostic workup for tetralogy of fallot |
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Definition
• CXR - “ boot” shaped heart
• EKG- RA enlargement, RVH
• Echo |
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Term
| management for tetralogy of fallot |
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Definition
-Mild pulmonary stenosis – observe and elective surgery ~ 4-6 months
- Severe stenosis
• Palliative surgery : BT shunt or PDA stent
• Complete repair : VSD closure + relief of pulmonary obstruction. •-Transannular patch – no PV – results in severe pulmonary regurgitation, • Pulmonary valve sparing – pulmonary valvotomy. • RV-PAconduit |
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Term
| long term outcomes for tetralogy of fallot |
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Definition
• Significantly improved survival in infancy
• Risk of RV dilation, RV dysfunction, arrhythmias, sudden cardiac death in adulthood
• Need for subsequent surgeries or interventions – replacement of pulmonary valve – surgical or trans –catheter
• Need for SBE ppx 6 month after surgery
• Life long cardiology follow up – MRI, Holter monitoring |
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Term
| transposition of great arteries for tetralogy of fallot |
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Definition
• Left ventricle – Pulmonary artery
• Right ventricle – aorta |
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Term
| physiology of tetralogy of fallot |
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Definition
-Parallel circulations
•-Mixing is essential to maintain tissue oxygenation
• Atrial level – PFO, ASD
• Ductus arteriosus - PDA |
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Term
| presentation of tetralogy of fallot |
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Definition
• Extreme cyanosis at birth
• Mottled appearance – tissue hypoxia, acidosis
• Exam : No murmur |
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Term
| diagnostic workup for tetralogy of fallot |
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Definition
• CXR – “Egg on string”
• EKG- usually normal
• Echo
• Cardiac catheterization
-Balloon septostomy
-Coronary artery anatomy |
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Term
| Post natal management for tetralogy of fallot |
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Definition
• Immediately at birth - PGE1 infusion to maintain patency of ductus arteriosus
• Atrial balloon septostomy
• Surgical repair – neonatal period
-Arterial switch - Current
- Atrial switch – Old |
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Term
| long term outcomes from atrial switch |
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Definition
• Excellent survival outcome post repair
• Long term morbidities and need for intervention
• Risk of sudden cardiac death
• Healthy life style modification. |
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Term
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Definition
• “Doming pulmonary valve” – Fusion of commissures
• “Dysplastic pulmonary valve”
• Fourth most common cyanotic CHD
• Incidence of 25 per 100000 live births
• Commonly associated syndromes – Noonan, Williams, Congenital rubella, Alagille |
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Term
| physiology of pulmonary valve stenosis |
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Definition
• Depends on the degree of obstruction
• Obstruction at outflow ..> Elevation of systemic RV pressure ..> Elevation of RA pressure ...> right to left shunting at the PFO ..> cyanosis |
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Term
| presentation of pulmonary valve stenosis |
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Definition
• Presentation depends on degree
• Mild – usually later due to a murmur • Moderate
• Severe
• Critical
• Can progress ..unusual beyond first year of life
• Examination : Right ventricular impulse, early systolic click, ejection systolic murmur
• EKG- RVH , ? RA enlargement |
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Term
| management of pulmonary valve stenosis |
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Definition
• Critical- PGE1 infusion
• Intervention:
• Pulmonary valvuloplasty - transcatheter
• PDA stent
• Surgical pulmonary valvotomy
-balloon valvuloplasty |
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Term
| left sided obstructive lesions |
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Definition
• Coarctation of aorta
• Aortic valve stenosis
• Hypoplastic left heart Syndrome |
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Term
|
Definition
• Obstruction in aortic arch
• Usually at the aortic isthmus, after take off of the left subclavian artery
• Discreet or long segment
• 5–7%ofCHD
• 12% incidence with Turner Syndrome |
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Term
| presentation of coarctation of aorta |
|
Definition
• SevereobstructionpresentsasCHFprogressingtoshockat1 to 2 weeks of age
• Milderobstructionpresentsasupperextremityhypertension with decreased femoral pulses
• May have a murmur at the left back–formation of collaterals |
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Term
| diagnostic workup for coarcation of aorta |
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Definition
• 4 extremity Blood pressures
• EKG – may be normal
• CXR – “3” sign
• Echo
• Cardiac catheterization |
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Term
| management for coarctation of aorta |
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Definition
• Neonates – need PGE1 therapy to keep the ductus arteriosus patent.
• Surgical
- End-to-endanastomosis - Arch augmentation
• Trans-cath eter balloon dilation:
- 6 months- 7 years
- Stent placement > 7 y of age |
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Term
| long term outcomes of coarctation of aorta |
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Definition
• Excellent neonatal surgical outcomes
• Recurrence or risk of re-coarctation – amenable to trans catheter interventions
• Systemic hypertension
• Healthy lifestyle modification |
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Term
|
Definition
- Bicuspid aortic valve
• AS,AR
• Aortic root and ascending aorta dilation
-Varying degrees of obstruction
• Mild
• Moderate
• Severe
• Critical (PDA dependent) |
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Term
| presentation of aortic valve stenosis |
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Definition
• Depends on degree of obstruction
• Severe or critical– present within hours of life - CHF , shock
• Examination : early systolic click , systolic ejection murmur
• EKG- LVH |
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Term
| management of aortic valve stenosis |
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Definition
• Mild – observation with serial EKG, echocardiogram – can progress
• Critical AS - PGE1initiation
• Intervention: Trans-catheter balloon valvuloplasty
• Surgical repair
-ROSS procedure
- Prosthetic valve replacement–usually wait until adolescents |
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Term
| aortic valve stenosis long term anticipatory guidance |
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Definition
• Risk for need of re- intervention → AVR, aortic root/ ascending aorta replacement
• Avoid contact sports , heavy weight lifting but aerobic exercise is highly encouraged
• Blood pressure control – treatment for hypertension |
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Term
| Hypoplastic Left Heart Syndrome |
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Definition
• Atresia or severe stenosis of the aortic valve and/or mitral valve(MS+AS, MS+AA, MA+AA)
• Severely hypoplastic LV
• Ductal dependent
• Essentially single functional ventricle. |
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Term
| hypoplastic left heart syndrome management |
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Definition
• PGE1 for ductal patency
• Single ventricle palliation through 3 stages
- Stage 1: Norwood (reconstruction of aortic arch, BT shunt or Sano shunt, DKS – anastomosis of the main pulmonary artery to aorta)• Unobstructed systemic outflow• Adequate mixing at atrial level• Adequate pulmonary blood flow
- Bidirectional Glenn • Gradual unloading of the ventricle
- Fontan • Complete separate of systemic and pulmonary circuits |
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Term
| norwood with BT shunt physiology (hypoplastic left heart syndrome) |
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Definition
• Balance between PVR and SVR
• ↑ PVR → desaturations
• ↑SVR → less systemic output and hypoperfusion
• Diastolic steal →coronary hypoperfusion |
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Term
| norwood with sano physiology (hypoplastic left heart syndrome) |
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Definition
• RV is the single pumping chamber →less dependent on PVR and SVR
• No diastolic steal |
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Term
| Cavo-pulmonary connection physiology for glenn and fontan (hypoplastic left heart syndrome) |
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Definition
• Glenn : SVC – PA connection
• Fontan: IVC – PA connection
• Passive flow from systemic circuit to pulmonary bed ..> trans- pulmonary gradient (PA pressure – Pulmonary Capillary Wedge Pressure)
- low PVR
- Good systolic and diastolic function of the ventricle
- AV valve competence |
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Term
| Long term outcomes for hypoplastic left heart syndrome |
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Definition
• One of the most complex and highest morbidity
• Long term issues – arrhythmias, sudden cardiac death, plastic bronchitis, liver failure, need for pacemakers, cardiac transplantation
• Avoid contact sports – aspirin
• Healthy lifestyle modification |
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