Congenital Heart Disease I and II Flashcards
Congenital Heart Disease
- General
- Etiology
- Genetic factors
- Environmental factors
- General
- Leading cause of neonatal mortality
- Etiology
- Cardiac malformations arise during the development of the heart tube morphogenesis at 4-7 weeks gestation
- Genetic factors
- Critical role
- > 10% have an identified chromosomal abnormality or single gene defect
- Environmental factors
- Maternal health (ex. rubella infection)
- Infection (ex. diabetes
- Drug exposure (ex. lithium, alcohol)
Down Syndrome
- Genetics
- Other
- Cardiac abnormalities
- Trisomy 21
- Hypotonia & developmental delay
- AV or ventricular septal defects

Turner’s Syndrome
- Genetics
- Cardiac abnormalities
- Female XO karyotype
- Coarctation of the aorta & bicuspid aortic valve

William’s Syndrome
- Genetics
- Other
- Cardiac abnormalities
- Abnormality in elestan gene (7q11)
- Developmental delay, loquacious personality, & facial dysmorphology (flat nasal bridge, long philtrum, wide mouth)
- Supravalue aortic stenosis, pulmonary artery stenosis, & renal artery stenosis

Noonan’s Syndrome
- Genetics
- Other
- Cardiac abnormalities
- Autosomal dominant genetic abnormality (12q)
- Turner’s phenotype, short stature, webbed neck, hypertelorism, & developmental delay
- Pulmonary valve stenosis & hypertrophic cardiomyopathy

Diagnosis & Classification of Congenital Heart Disease
- Diagnosis
- Classification
- Acyanotic lesions
- Cyanotic lesions
- Diagnosis
- History & physical exam
- Lab tests: ECG, chest x-ray
- Analysis: echo, MRI, CT, angiography
- Therapeutic interventional procedures: cardiac catheterization
- Classification
- Acyanotic lesions
- Septal defects
- ASD
- VSD
- AV septal defect
- Patent ductus arteriosus
- Obstruction
- Smilunar valve (pulmonic & aortic stenosis)
- Coarctation of the aorta
- Septal defects
- Cyanotic lesions
- Abnormal connection
- Transposition of the great arteries
- Total anomalous pulmonary venous return
- Septal defect + obstruction to pulmonary blood flow
- Tricuspid atresia
- Pulmonary atresia
- Tetralogy of Fallot
- Complete mixing
- Aortic atresia / hypoplastic left heart syndrome
- Truncus arteriosus
- Single ventricle
- Abnormal connection
- Acyanotic lesions
Atrial Septal Defect
- Defect
- Types
- Physiology
- Clinical findings
- Lab findings
- Chest x-ray
- ECG
- Echo
- Natural history & complications
- Management
- Defect
- Acyanotic septal defect
- Types
- Secundum defect: in the fossa ovalis region (most common)
- Sinus venosus defect: adjacent to the SVC or IVC
- Ostium primum (part of AV septal defect)
- Physiology
- Left to right shunt
- Volume overload of RA & RV
- Clinical findings
- Increased parasternal impulse
- Fixed splitting of S2
- Grade II-III ejection murmur mid to upper left sternal border
- Mid diastolic murmur low left sternal border
- Due to flow across the tricuspid & pulmonic valves resulting in tricsupid & pulmonic stenosis
- Not due to flow across the atrial septum b/c this is low velocity w/ minimal turbulence
- Lab findings
- Chest x-ray
- Cardiomegaly
- Increased pulmonary vascularity
- ECG
- Right axis deviation
- RVH
- Echo
- Defines the type & size of the defect
- Provides indirect evidence of the degree of left to right shunting by the degree of RA & RV enlargement
- Chest x-ray
- Natural history
- Children: rarely symptomatic
- Adults: complications
- Right heart failure from chronic volume overload
- Atrial arrhythmias from chronic atrial stretching
- Pulmonary hypertension from excessive pulmonary blood flow over a long period of time
- Paradoxical emboli related to intermittent right ot left shutnign across the defect
- Management
- Surgical closure
- Transcatheter closure (avoid open heart surgery)

Ventricular Septal Defect
- Defect
- Anatomy
- Physiology
- Clinical implications dependent on…
- Clinical findings
- Small VSD
- Large VSD
- Large VSD shunt variability
- Newborn
- First few weeks of life
- Tetralogy of Fallot
- Lab findings
- Chest x-ray
- Small defect
- Large defect
- ECG
- Small defect
- Large defect
- Echo
- Catheterization
- Chest x-ray
- Natural history & complications
- Small defects
- Large defects
- Management
- Defect
- Acyanotic septal defect
- Anatomy
- Perimembranous region
- Muscular septum
- Doubly committed or supracristal (immediately subjacent to the aortic & pulmonary valves)
- Physiology
- Left to right shunt
- Volume overload of LV, LA, & RV
- Pressure &/or volume overload to right heart
- Clinical implications dependent on…
- Size of the hole
- Downstream resistance to flow
- Pulmonic stenosis
- Pulmonary vascular resistance
- Clinical findings
- Small VSD
- Left to right shunt
- Normal intracardiac pressures
- No symptoms
- High pitched, pansystolic murmur at LLSB
- Normal S2
- Large VSD
- Pulmonary hypertension
- CHF
- Failure to thrive in infancy
- Ejection murmur due to equal pressures in the two ventricles
- Loud, single S2
- Small VSD
- Shunt is variable in large VSD due to presence & degree of pulmonary stenosis & level of pulmonary vascular resistance
- Newborn
- Very small shunt b/c neonatal pulmonray vascular resistance is high, providing almost as much resistance to flowa s the systemic vascular bed
- First few weeks of life
- As pulmonary resistance falls, left to right shunt increases w/o changing the defect size
- Tetralogy of Fallot
- Predominant right to left shunt due to severe pulmonary stenosis
- Newborn
- Lab findings
- Chest x-ray
- Small defect
- Normal
- Large heart
- Cardiomegaly & increased vascularity
- Small defect
- ECG
- Small defect
- Normal
- Large defect
- LVH +/- RVH
- Small defect
- Echo
- Diagnostic
- Catheterization
- Rarely necessary
- Chest x-ray
- Natural history
- Spontaneous closure or decrease in size common
- Small defects
- Don’t require any intervention
- Large defects
- CHF & failure to thrive
- Pulmonary HTN & vascular disease
- Bacterial endocarditis
- Management
- Surgical repair
- Medical therapy for CHF
- Prevention of pulmonary vascular disease

AV Septal Defect
- Defect
- Anatomy
- Physiology
- General
- Partial defect
- Complete defect
- Clinical findings
- Common in…
- Partial defect
- Complete defect
- Lab data
- ECG
- Chest x-ray
- Echo
- Catheterization
- Complications
- Management
- Defect
- Acyanotic septal defect
- Anatomy
- Deficiency of the AV (lower atrial & ventricular) septum
- Affects the atrial septum, AV valves, & ventricular septum
- Physiology
- General
- ASD + VSD
- Left to right shunts w/ volume overload of RA, RV, & LV
- Partial defect: physiology of an atrial defect
- Atrial defect only & cleft (abnormal) AV/mitral valve
- No intraventricular communication
- Volume overload of RA & RV
- Cleft AV valve –> mitral regurgitation
- Complete defect: physiology of a ventricular defect
- Atrial & ventricular defects
- Large left to right shunt
- Volume overload of the RA, RV & LV
- General
- Clinical findings
- Common in…
- Down syndrome
- Partial defect
- Findings of an ASD
- Murmur of mitral regurgitation
- Complete defect
- Findings of a large VSD
- Pulmonary HTN & CHF
- Common in…
- Lab data
- ECG
- Superior or left axis deviation
- Chest x-ray
- Large heart w/ increased markings / vascularity
- Echo
- Diagnostic
- Catheterization
- Rarely needed
- ECG
- Complications
- AV valve regurgitation
- CHF
- Pulmonary HTN & eventual vascular disease
- Endocarditis
- Management
- Surgical repair / closure

Patent Ductus Arteriosus
- Defect
- Anatomy
- Physiology
- Clinical findings
- Complications
- Management
- Lab findings
- Management
- Defect
- Acyanotic septal defect
- Anatomy
- Normal component of fetal circulation: extension of main pulmonary artery to descending aorta
- Failure of the normal process of spontaneous closure of the ductus arteroisus immediately after birth
- Physiology
- Left to right shunt due to run off from aorta to pulmonary artery
- Volume overload of LA & LV
- Increased pulmonary blood flow
- Clinical findings
- Continuous murmur at LUSB
- Bounding pulses due to diastolic run-ff from the aorta to the pulmonary artery
- If duct is large: CHF & pulmonary HTN
- Complications
- Endocarditis
- CHF
- Pulmonary HTN & pulmonary vascular disease
- Management
- Surgical ligation
- Transcatheter coil embolization
- Lab findings
- Chest x-ray
- Large heart & increased vascularity
- Hemodynamics
- CHF
- Pulmonary HTN
- Chest x-ray
- Management
- Most are successfully closed in the cath lab w/ transcatheter coil or device placement

Left to Right Shunt Summary
- Result in…
- Chest x-ray
- If the degree of shunting is hemodynamically significant…
- Eisenmenger’s syndrome
- Result in…
- Increased pulmonary blood flow –> volume overload of various heart chambers
- Chest x-ray
- Cardiomegaly
- Increased pulmonary vascularity
- If the degree of shunting is hemodynamically significant…
- CHF + pulmonary HTN
- Eisenmenger’s syndrome
- Long standing increased pulmonary blood flow & pulmonary HTN
- –> damaged pulmonary vasculature at the arterioles
- –> increased pulmonary vascular resistance to the point where it exceeds systemic vascular resistance
- –> reversed direction of the shunt from left-to-right to right-to-left
- –> cyanosis
Pulmonic Stenosis
- Defect
- Anatomy
- Physiology
- Clinical findings
- Lab data
- ECG
- Chest x-ray
- Echo
- Catheterization
- Complications
- Management
- Defect
- Acyanotic obstruction defect
- Anatomy
- Fusion &/or thickening of valve leaflets
- Dysplastic pulmonic vavle leaflets (seen in Noonan syndrome)
- Physiology
- Obstruction to RV outflow
- Elevated pressure in the RV
- RVH
- Clinical findings
- SEM at the LUSB radiating to the back
- Ejection click
- Lab data
- ECG
- RVH
- Chest x-ray
- Dilated main pulmonary artery due to post-stenotic dilation
- Echo
- Diagnostic
- Quantitates the degree of stenosis
- Catheterization
- For intervention
- ECG
- Complications
- Right heart failure
- Cyanosis if right to left atrial shunt
- Endocarditis (rare)
- Management
- Surgery
- Transcatheter balloon valvuloplasty

Aortic Stenosis
- Defect
- Anatomy
- Physiology
- Clinical findings
- Lab data
- ECG
- Echo
- Catheterization
- Complications
- Management
- Defect
- Acyanotic obstruction defect
- Anatomy
- Thickened leaflets, fused commissures
- Bicuspid aortic valve
- Physiology
- Obstruction to LV outflow
- LVH
- Clinical findings
- SEM at the RUSB
- Apical ejection sound
- Decreased upstroke of the carotid & peripheral pulses (if severe)
- Commonly associated w/ coarctation of the aorta
- Lab data
- ECG
- LVH
- Echo
- Diagnostic
- Quantitates the degree of stenosis
- Catheterization
- For intervention
- ECG
- Complications
- CHF
- Sudden death
- Exertional syncope
- Endocarditis
- Management
- Transcatheter balloon valvuloplasty (initial palliative procedure)
- Surgical replacement of their aortic valve (eventually later in childhood or adulthood)

Coarctation of the Aorta
- Defect
- Anatomy
- Etiology
- Clinical presentation
- Clinical findings
- ECG
- Chest x-ray
- Echo
- MRI
- Defect
- Acyanotic obstruction defect
- Anatomy
- Obstruction of aortic arch adjacent to insertion of the ductus arteriosus
- Etiology
- Closure of hte ductus arteriosus shortly after birth
- –> migration of ductal tissue into the descending aorta
- –> precipitous manifestation of arch obstruction from coarctation
- –> severe CHF & cardiogenic shock
- Clinical presentation
- Cardiogenic shock in the newborn period
- Asymptomatic
- Upper extremity HTN
- Decreased lower extremity pulses
- Clinical findings
- Discrepancy in blood pressure: normal or hypertensive upper extremity pulses w/ weak or absent femoral pulses
- Systolic murmur at the LUSB, axilla, & back
- Associatd lesions: bicuspid aortic valve, VSD
- Lab data
- ECG
- Infants: RVH (related to increased fetal pulmonary HTN)
- Children/adults: LVH
- Chest x-ray
- Figure “3” in the descending aorta
- If unrepaired for >5 years: rib notching due to collateral flow through dilated intercostal arteries
- Echo
- Diagnostic in infants
- MRI
- Diagnostic in older children
- ECG
- Management
- Surgical repair
- Transcatehter balloon dilation
- Stent placement for therapy of post-operative recurrent coarctation

Cyanotic Heart Disease
- Pathophysiology
- Hyperoxia test
- Three mechanisms of cyanotic heart disease
- Pathophysiology
- Desaturated venous blood enters systemic circulation through intracardiac mixing
- Normal saturation = 95%
- Depressed pO2 (to 60 mmHg) = 90% saturation
- Cyanotic saturation < 85-90%
- Hyperoxia test
- Expose patient to 100% O2 to differentiate cardiac & pulmonary causes of desaturation
- Airway or pulmonary etiology: pO2 will rise above 100 mmHg
- Cyantoic heart disease: pO2 barely or doesn’t increase due to fixed right-to-left shunt that’s not exposed to alveolar oxygen
- Three mechanisms of cyanotic heart disease
- Abnormal connections of the great vessels to the heart
- Septal defect + obstruction to pulmonary blood flow
- Complete mixing of desaturated ysstemic venous & saturated pulmonary venous return

Transposition of the Great Arteries
- Defect
- Pathophysiology
- Clinical findings
- Lab data
- ECG
- Chest x-ray
- Echo
- Catheterization
- Natural history
- Management
- Defect
- Cyanotic abnormal great vessel connection
- Most common cyanotic anomaly in newborns
- Pathophysiology
- Normal atrial & ventricular connections
- Reversal of the great vessel relationships so that the aorta arises from the RV & the pulmonary artery from the LV
- Parallel systemic & pulmonary circuits
- Systemic venous blood goes from the RA to the RV & out the aorta w/o exposure to oxygenated blood
- Requires intracardiac or great vessel mixing to be compatible w/ life
- All newborns have some interatrial connection through the foramen ovale
- Foramen ovale allows enough satured pulmonary venous blood from the LA to mix w/ the systemic venous return in the RA to achieve a survivable level of systemic saturation
- Inherently unstable situation: infants become critically hypoexmic within the first hours after birth
- Clinical findings
- Extreme cyanosis w/o labored respirations in a newborn
- Single S2
- Unimpressive or no murmur
- Lab data
- ECG
- Normal for age
- Chest x-ray
- Narrow base
- Due to the aorta being directly anterior to the pulmonary artery
- Minimizes the usual shadow from the pulmonary artery which is normally positioned to the left of the aorta
- Narrow base
- Echo
- Diagnostic
- Catheterization
- For atrial septostomy if the patient doesn’t have a reasonable large atrial communication
- ECG
- Natural history
- High mortality if untreated due to severe hypoxemia
- Management
- Prostaglandins to maintain ductal patency
- Increase pulmonary venous return to the LA
- Facilitate left-to-right shunting
- Balloon atrial septostomy (Rashkin procedure)
- Catheter w/ a deflated balloon is advanced across the atrial septum
- Inflate balloon –> pull back from the LA to the RA –> enlarge atrial communication
- Allows systemic arterial saturations to increase to a level high enough to maintain homodynamic stability
- Allows recovery from the initial stress until stable enough to under go open heart surgery
- Arterial switch operation
- Done in the first week of life
- Removes the aortic & pulmonary trunks from their semilunar valves & attaches them to the appropriate ventricle
- Transposes coronaries from the original aortic root to the posterior pulmonary root which will become the aortic outflow track
- Prostaglandins to maintain ductal patency

Total Anomalous Pulmonary Venous Return
- Defect
- Pathophysiology
- Blood mixing
- Pulmonary venous return
- RA flow
- 3 main avenues of drainage of the pulmonary venous confluence into the systemic veins
- All avenues
- Clinical presentation
- Depends…
- Descending vein to the portal circulation
- Other forms of pulmonary venous return
- Opperative approach
- Defect
- Cyanotic abnormal great vessel connection
- Pathophysiology
- Complete mixing of pulmonary & systemic venous return
- All pulmonary veins return to a systemic vein
- Appropriately connected atria, ventricles, & great vessels
- Pulmonary veins return to a confluence behind the LA & don’t enter the heart directly
- Abnormal vein forms from this confluence & enters the systemic venous circulation
- Results in total mixture of the pulmonary venous return w/ the sytemic venous return when flow reaches the RA
- RA flow
- Most will pass through the tricuspid valve into the RV & pulmonary artery
- Some will pass across the atrial septum w/ right-to-left shunting entering the LA, LV, & aorta to provide systemic cardiac output
- 3 main avenues of drainage of the pulmonary venous confluence into the systemic veins
- Vertical vein –> innominate vein –> SVC
- Vein that descends below the diaphragm –> portal circulation
- Almost associated w/ severe obstruction to pulmonary venous return
- Confluence –> coronary sinus
- All avenues
- Increase pulmonary blood flow
- Pulmonary HTN
- Obligatory right-to-left hsunt across the atrial communication providing cardiac output
- Complete mixing of pulmonary & systemic venous return
- Clinical presentation
- Depends on degree of obstruction to pulmonary venous return
- Descending vein to the portal circulation
- Almost always –> pulmonary venous obstruction –> severe pulmonary edema immediately after birth –> urgent need to surgically repair
- Other forms of pulmonary venous return
- Eventually cause severe heart failure & pulmonary HTN –> need for early surgical intervention
- Opperative approach
- Oppose the pulmonary venous confluence to the back wall of the LA
- Create a wide anastomosis b/n these chambers
- Ligate the anomalous vein that connects the confluence to the systemic venous circuit

Tricuspid Atresia
- Defect
- Pathophysiology
- Clinical presentation
- Degree of cyanosis
- Small VSD
- Large VSD
- Lab data
- Management
- Defect
- Cyanotic septal defect + obstruction to pulmonary blood flow
- Pathophysiology
- Obligatory right to left atrial shunt across foramen ovale or ASD
- Total mixture of systemic & pulmonary venous return in the LA
- Flow goes from the LA to the LV out into the aorta
- VSD allows some flow from the LV to the RV to provide a source of pulmonary blood flow
- Hypoplastic RV b/c it doesn’t receive the normal amount of inflow during fetal life to promote normal development of the chamber
- Obligatory right to left atrial shunt across foramen ovale or ASD
- Clinical presentation
- Cyanosis
- Systolic murmur related to pulmonary stenosis or VSD
- Single 2nd heart sound
- Degree of cyanosis
- Inversely related to amount of pulmonary blood flow
- Small VSD
- Pulmonary outflow track obstruction
- Most of the flow from the LV will go out the aorta
- Pulmonary blood flow will be diminished –> significant hypoxemia
- Large VSD
- Modest pulmonary stenosis
- Enough pulmonary blood flow –> mild cyanosis
- Prominent systolic murmur from pulmonic stenosis or VSD & a single S2
- Lab data
- ECG
- LAD (unusual in a newborn)
- LVH due to under-developed of the RV
- Chest x-ray
- Decreased pulmonary vascularity
- Echo
- Diagnostic
- ECG
- Management
- Prostaglandins if cyanosis is severe & ductal dependent
- Allows opening of the ductus arteriosus to provide adequate pulmonary blood flow to achieve a sustainable systemic arterial saturation
- Aorta to pulmonary artery shunt if cyanosis is severe
- Prostaglandins if cyanosis is severe & ductal dependent

Pulmonary Atresia
- Defect
- Pathophysiology
- Lab data
- EKG
- Chest x-ray
- Management
- Defect
- Cyanotic septal defect + obstruction to pulmonary blood flow
- Pathophysiology
- Complete obstruction to outflow from the RV –> tricuspid regurgitation
- Right to left shunt across the atrial septum
- Systemic venous return mixes w/ pulmonary venous return in the LA & flows into the LV & out into the aorta
- Patent ductus arteriosus provides some pulmonary blood flow
- Otherwise condition would be letal
- Hypoplastic RV b/c blood can’t flow easily into or out of the RV
- Lab data
- EKG
- LV predominance due ot RV hypoplasia
- Chest x-ray
- Variable heart size
- Decreased pulmonary vascularity
- EKG
- Management
- Prostaglandins maintain ductal patency
- Aorta to pulmonary shunt eventually needed

Tetralogy of Fallot
- Defect
- 4 features
- Physiology
- Clinical findings
- Lab data
- ECG
- Chest x-ray
- Echo
- Catheterization
- Management
- Defect
- Cyanotic septal defect + obstruction to pulmonary blood flow
- 4 features
- (1) VSD
- (2) Pulmonic stenosis
- (3) Overriding aorta
- (4) RVH
- Physiology
- Pulmonary stenosis + VSD –> right ot left shunt across VSD
- Anterior deviation of outlet septum
- –> aorta overrides the VSD & partially arises from the RV
- –> narrowed RV outflow tract –> subvalvular & valvular pulmonic stenosis
- Right aortic arch in 25%
- Main variability: degree of pulmonary stenosis determines…
- Severity of right-to-left ventricular shunting
- Degree of cyanosis
- Clinical findings
- Variable cyanosis
- SEM from pulmonary stenosis
- Single S2
- Flow across non-restrictive VSD is silent b/c the defect is large & RV systolic pressure = LV systolic pressure
- Murmur inversely related to severity of pulmonary stenosis
- Severe pulmonary stenosis –> increased right-to-left shunt across VSD w/ small amount of flow across the pulmonary stenosis –> soft murmur
- Moderate pulmonary stenosis –> large amount of flow across the outflow tract –> turbulent flow –> loud murmur
- Lab data
- ECG
- RAD
- RVH
- Chest x-ray
- Small, “boot” shaped heart due to absent main pulmonary artery shadow at LUSB
- Right aortic arch may be present
- Decreased pulmonary vascularity
- Echo
- Diagnostic
- Catheterization
- Rarely needed prior to intervention
- ECG
- Management
- Almost always complete repair w/ open heart surgery to close the VSD & relieve pulmonray stenosis (repair)
- In low birth weight infants or infants w/ severe anatomy, pulmonary artery shunt (palliative)
- Repair: close VSD & relieve the pulmonary stenosis

Septal Defect + Obstruction to Pulmonary Blood Flow Summary
- May be ductal dependent
- Prostaglandins provide dramatic improvement in oxygenation
- Aorta to pulmonary shunt for palliation
Aorta to Pulmonary Shunts
- Classic Blalock Taussig Shunt
- Modified Blalock Taussig Shunt
- Waterston Shunt
- Potts Shunt
- Central Shunt
- Classic Blalock Taussig Shunt
- Division of left subclavin artery with end to side anatomosis of the proximal subclavisn artery to the pulmonary artery
- Modified Blalock Taussig Shunt
- Gore-tex tube connecting the subclavian artery to the pulmonary artery with bilateral end to side anastomoses
- Waterston Shunt
- Creation of an aorto-pulmonary window connecting the posterior ascending aorta to the anterior right pulmonary artery
- Potts Shunt
- Creating an aorto-pulmonary window by connecting the anterior wall of the descending aorta to the posterior wall of the left pulmonary artery
- Central Shunt
- Gore-tex tube connecting any portion for the aorta to the pulmonary artery
Septal Defect + Obstruction to Pulmonary Blood Flow Summary
- Lesions
- Prostaglandins
- Aorta to pulmonary shunts
- Lesions
- May be dependent on ductal flow in the newborn period to maintain adequate levels of systemic arterial saturation in the setting of greatly diminished pulmonary blood flow
- Prostaglandins
- Provide dramatic improvement in oxygenation by increasing ductal flow to the pulmonary artery
- Aorta to pulmonary shunts
- May be required for palliation proir to complete repair
Aortic Valve Atresia
- Defect
- Pathophysiology
- Clinical findings
- Lab data
- EKG
- Chest x-ray
- Echo
- Management
- Defect
- Cyanotic complete mixing of systemic venous & pulmonary venous return at atrial, ventricular, or great vessel level
- Pathophysiology
- Underdeveloped left heart –> hypoplatic left heart syndrome
- Obstruction to outflow from the LV
- –> blood returning to the LA is divered across the atrial septum
- –> complete mixing of pulmonary & systemic venous return in the RA
- All of the venous return crosses the tricuspid valve –> RV –> main pulmonary
- –> branch pulmonary arteries
- –> shunt right-to-left across the patent ductus out into the systemic circulation
- Abnormal fetal flow –> under-developed LV, mitral valve, & ascending aorta
- Systemic perfusion is completley dependent on ductal patency
- Clinical findings
- Constricting ductus –> cardoigenic shock within first several days of life
- Hyperactive precordium w/ weak or absent pulses
- Lab data
- EKG
- RVH
- Chest x-ray
- Cardiomegaly
- Increased pulmonary vascularity
- Echo
- Diagnostic
- EKG
- Management
- Prostaglandins maintain ductal patency & allow adequate systemic perfusion
- Norwood operation
- After the infant is stable, redirect the outflow across the pulmonary artery to the aorta to provide an alternate source of pulmonary blood flow via an aorta to plumonary shunt
- Heart transplant

Truncus Arteriosus
- Defect
- Pathophysiology
- Clinical findings
- Management
- Defect
- Cyanotic complete mixing of systemic venous & pulmonary venous return at atrial, ventricular, or great vessel level
- Pathophysiology
- A single arterial trunk arises from the heart overriding a large VSD & receives outflow from both the RV & LV
- Pulmonary arteries arise directly from the root of the “truncus” (ascending aorta)
- No pulmonary valve –> ungarded pulmonray arteries –> icnreased pulmonary blood flow from the aorta
- Complete mixing of systemic & pulmonary venous return
- Clinical findings
- Increased pulmonary blood flow
- Pulmonary HTN
- CHF
- Management: Rastelli procedure (surgical)
- Closure of the VSD such that only the LV outflow reaches the aorta
- B/c there’s no outlet form the RV after closure, a conduit is placed from the RV to the pulmonary artery

Single Ventricle
- Defect
- Pathophysiology
- Clinical presentation w/ pulmonary stenosis
- Clinical presentaiton w/o pulmonary stenosis
- Management
- Defect
- Cyanotic complete mixing of systemic venous & pulmonary venous return at atrial, ventricular, or great vessel level
- Pathophysiology
- One dominant ventricle + hypoplastic chamber via a ventricular communication
- Both the pulmonary artery & aorta arise from a “functionally common” chamber
- Both right 7 levt AV valves empty into the common chamber –> “double inlet ventricle”
- Clinical presentation w/ pulmonary stenosis
- Blood diverts out into the aorta –> pulmonary blood flow will be insufficient –> systemic desaturation –> cyanosis
- Sometimes, just enough pulmonary stenosis allows balanced pulmonary & aortic flow
- Clinical presentation w/o pulmonary stenosis
- Pulmonary over-circulation
- Lower pulmonary vascular resistance compared to systemic resistance –> pulmonary HTN
- CHF
- Management
- Early surgical intervention

Congenital Heart Disease Summary
- Timing of presentation
- Modes of presentation
- Management
- Timing of presentation
- Presents from fetal life to adulthood
- Severe cyanosis or cardiogenic shock in newborn period (aortic atresia, transposition)
- Asymptomatic w/ murmur in an adult (ASD)
- Modes of presentation
- Murmur
- Abnormal pulses or BP
- CHF
- Cyanosis
- Management
- Surgery
- Interventional cath
- Prevent endocarditis