Fanaroff Chapter 75
most likely occurs secondary to an abnormality in conotruncal development that happens between weeks 4-5 of gestation
TGA
the heart tube loops to the right during organogenesis, yielding normal ventricular orientation and great vessel formation but the aorta and pulmonary artery fail to rotate resulting in reversal of these two vessels
TGA
The most important associated defect with DORV is subpulmonary or pulmonary stenosis because it looks like?
TOF
These neonates require a palliative systemic-to-pulmonary shunt (e.g., Blalock-Thomas-Taussig or central shunt) or complete repair in the newborn period to provide pulmonary blood flow; Balloon dilation or stenting of the stenotic pulmonary valve or RVOT has been favored
TOF
a systolic ejection murmur along the left sternal border secondary to pulmonary stenosis/RVOT obstruction; May be a single second heart sound due to marked diminishment of its pulmonary component and a palpable right ventricular impulse
TOF
T/F? At a minimum, an atrial level defect with balanced bidirectional shunting is essential for survival in transposition of the great arteries
TRUE
T/F? Forward pulmonary blood flow is dependent on the severity of tricuspid valve displacement and regurgitation and RV atrialization and dysfunction in ebstein anomaly
TRUE
T/F? Isolated small to moderate secundum ASDs often close spontaneously over time
TRUE
T/F? Mild forms of Ebstein anomaly require no specific treatment, and these infants in general do well
TRUE
T/F? Most VSDs require no intervention and undergo spontaneous closure within the first few months to years of life; Hemodynamically insignificant and generally remain asymptomatic
TRUE
T/F? Neonates with pulmonary venous obstruction in TAPVR are profoundly hypoxic and require significant respiratory and metabolic support
TRUE
T/F? Persistent or more sizable secundum ASDs with left-to-right shunting must be longitudinally monitored for intracardiac effects - right heart dilation or evidence of increasing right ventricular pressures, indicative of developing pulmonary vascular disease
TRUE
T/F? TOF can range from mild pulmonary stenosis with excellent saturations to pulmonary atresia with very low saturations
TRUE
T/F? fetuses exposed to pre gestational maternal diabetes are considered at a higher risk for developing TGA
TRUE
T/F? in PA/IVS, ventriculocoronary connections (sinusoids and fistulae) and coronary artery abnormalities must be well defined prior to surgical or transcatheter intervention; needed for survival or MI, infarction, and death
TRUE
T/F? in TAPVR, in the absence of pulmonary venous obstruction, pulmonary blood flow is increased and hypoxia is mild; If the anomalous pulmonary venous connection is obstructed at any point in its course, results in pulmonary venous hypertension with pulmonary edema and decreased pulmonary blood flow causing profound systemic hypoxemia
TRUE
T/F? in abstain anomaly, repair or replacement of the tricuspid valve is feasible in older children, but this is not useful in neonates; Either surgical exclusion of the right ventricle, with plans for a long-term, single-ventricle palliation, or transplantation might be the only alternatives for the neonate with persistent cyanosis
TRUE
T/F? in truncus arteriosus, both pulmonary and systemic blood flows are determined by the relative systemic and pulmonary vascular resistances; As the PVR falls, there is increased pulmonary blood flow, resulting in pulmonary overcirculation and increased left atrial and ventricular size
TRUE
T/F? in truncus arteriosus, systemic oxygen saturations in the mid-80s, with higher saturations indicating falling PVR and increasing pulmonary blood flow resulting in signs and symptoms of pulmonary overcirculation; Lower saturations indicate increased PVR, branch pulmonary artery stenosis, or pulmonary dysfunction from edema
TRUE
T/F? large VSD are often hemodynamically significant and present with signs of pulmonary over circulation with or without CHF
TRUE
T/F? small to moderate VSD that do not encourage significant pulmonary over circulation are associated with a harsh pan-systolic mumur along left sternal border
TRUE
Large VSDs causes pulmonary overvcirculation- what are symptoms of this?
Tachypnea, increased work of breathing, poor feeding, and suboptimal weight gain
DORV variant with subpulmonary VSD and transposition of the great arteries
The Taussig-Bing malformation
Characteristic holosystolic murmur and location on exam, generally best heard along the left sternal border with localization to the left lower sternal border
VSD
Most infants with isolated VSDs are discharged home with no symptoms and not diagnosed until initial follow up visits with PCP
VSD
_______ such as accessory pathways (e.g., Wolff Parkinson-White syndrome) are found in up to 20% of patients with ebstein anomaly
conduction system abnormalities
Severe form of obstructed pulmonary venous connection in TAPVR can lead to?
cyanosis and respiratory distress
group of congenital malformations that lead to low circulating blood oxygen levels
cyanotic heart defects
these type of defects involve the systemic recirculation of desaturated venous blood which occurs by 1) mixing of deoxygenated and oxygenated blood at the atrial, ventricular, or ductal levels or 2) a structural diversion redirecting deoxygenated blood returning to the heart away from the lungs and back to the systemic circulation; this shunting of blood from R to L results in hypoxemia, low arterial saturations, and cyanosis
cyanotic heart defects
why should supplemental oxygen be avoided in truncus arteriosus?
decreases PVR, increasing pulmonary blood flow
____ have L to R shunting given their higher LV (systemic) pressures compared to RV (pulmonic) pressures under normal circumstances; Increases after birth as the PVR decreases
isolated VSD
type of VSD that arise between the pulmonic and aortic valves at the infundibular septum
junta-arterial VSD
which VSDs typically do not undergo spontaneous closure?
juxta-arterial (subpulmonary/subarterial)
pulmonary overcirculation can become considerable with resulting clinical sequelae, as well as intracardiac effects (left atrial and ventricular dilation)
large VSD
in PA/IVS, unless there is severe tricuspid regurgitation or a restrictive atrial communication, the ______ generally is not enlarged while ductal patency is maintained
liver
type of VSD that occurs in the upper, thinner, membrane-like portion of the ventricular septum as a result of fibrous deficiencies
perimembranous VSD
in transposition of the great arteries, you have to have ______ to maintain PDA - improves arterial oxygenation by increasing shunting from the oxygen-rich pulmonary artery to the deoxygenated aorta
prostaglandin
in TOF, infants with severe pulmonary stenosis or atresia may have severely hypoplastic or absent _______ with major aortopulmonary collateral arteries serving as the primary source of pulmonary blood flow
pulmonary arteries
in ebstein anomaly, the anterior tricuspid leaflet can also become markedly enlarged and redundant ("sail-like"), causing RVOT obstruction with decreased blood flow and functional or anatomic _____
pulmonary atresia
Pulmonary valve may well-formed with a plate that obstructs outflow to complete absence of the valve; All forms may lead to severe RV hypertrophy with RV dysfunction and varying RV hypoplasia; ventricular septum is intact
pulmonary atresia with intact ventricular septum
in tricuspid atresia, those with a large VSD and no pulmonary stenosis have high ___________; When PVR falls in the postnatal period, pulmonary flow dramatically increases with resultant pulmonary overcirculation and risk for respiratory issues and feeding/growth failure
pulmonary blood flow
A restrictive atrial level defect can produce significant _____ in HLHS
pulmonary edema
in TOF, _____ results in right ventricular outflow obstruction which leads to ________ and this leads to increased ventricular pressure which causes the perimembranous portion of the ventricular septum to rupture leading to the _____; the aorta is enlarged and displaced to the right straddling the ventricular septum which permits aortic uptake of blood from ______
pulmonary stenosis right ventricular hypertrophy large VSD both ventricles
The _________ may indicate the severity of RVOT obstruction in TOF on CXR
pulmonary vascular markings
Urgent neonatal surgery is essential for those with TAPVR with ______
pulmonary venous obstruction
the major defect in TOF is _______ which limits pulmonary blood flow and impedes adequate oxygenation
right ventricular outflow obstruction
in TOF, cyanosis is proportional to the degree of __________, with severe obstruction limiting pulmonary blood flow and leading to very low saturations
right ventricular outflow tract (RVOT) obstruction
in transposition of the great arteries, the patient's _______ is loud and persistently single because of the anterior position of the aorta and the posterior position of the pulmonary artery
second heart sound
to goal of the staged surgeries of HLHS is to ?
separate the systemic and pulmonary circulation in step wise fashion while ensuring adequate systemic blood flow at each stage and protecting the pulmonary vasculature from excessive flow
in PA/IVS, severe tricuspid and RV hypoplasia or RV-dependent coronary circulation is an indication for ____; Should also be considered candidates for superior cavopulmonary (Glenn) anastomosis combined with closure of the atrial level defect; This results in direction of inferior vena cava flow across the tr tricuspid valve into the RV, promoting potential RV growth
single ventricular palliation
clinical manifestations of VSD are contingent on ?
size of the defect severity of the defect
why do patients with TOF benefit from fluid loading?
subpulmonary obstruction is somewhat dynamic, and a pre-loaded right ventricle can help to keep these patients from having hypercyanotic spells. A volume-loaded RV will physically stretch or distend the subpulmonary
______- acts as a pulmonary vasodilator, further increasing pulmonary blood flow = acidosis and diminished end organ perfusion- do not use in single ventricular anatomy
supplemental oxygen
A vertical vein emanates from the pulmonary venous confluence posterior to the LA and courses superiorly to the left innominate vein, which then drains to the SVC and RA
supra cardiac TAPVR
what if you do not have a shunt with transposition of the great arteries?
systemic hypoxia and often lethal
in tricuspid atresia, if ductal-dependent or if hypoxia increases in the first month of life, a _______ is necessary to provide adequate pulmonary blood flow and oxygenation
systemic to pulmonary shunt
RV is reactive to circulating catecholamines, which increase with increasing patient agitation, resulting in increased gradient across the RV outflow tract forcing deoxygenated blood across the ventricular septal defect into the aorta, resulting in cyanosis
tet spells
infants with "blue" TOF may manifest with paroxysmal hyper cyanotic spells known as?
tet spells
in PA/IVS, the ventricular septum is intact and pulmonary blood flow is ductal-dependent; Can only occur via shunting at ?
the atrial level and PDA
The ratio of ________ has been a useful measure of severity and outcome of ebstein anomaly
the right atrial plus atrialized RV size to the functional RV plus left heart (left atrium and ventricle) size
CXR shows a classic egg-shaped heart with increased pulmonary vascularity
transposition of the great arteries
Deoxygenated blood entering the aorta recirculates to the body, while the richly oxygenated pulmonary venous return is recirculated to the lungs
transposition of the great arteries
Most common cyanotic heart defect in the first week of life - should be considered in any cyanotic neonate; most common cyanotic heart defect that manifests during the neonatal period
transposition of the great arteries
The aorta arises from the RV, which receives systemic venous deoxygenated blood, and the pulmonary artery arises from the LV, which receives pulmonary venous oxygenated blood; this results in two independent and parallel circulations
transposition of the great arteries
Electrocardiogram is usually diagnostic; CXR tends to be nonspecific, although severely cyanotic neonates show decreased pulmonary vascularity; Catheterization - balloon atrial septostomy in those with a restrictive atrial septuM
tricuspid atresia
Platelike tissue in place of the tricuspid valve with no direct communication between the RA and RV; SVR from the IVC enters the RA and crosses the atrial septum with resultant complete mixing of the systemic and pulmonary venous return in the LA
tricuspid atresia
Typical presentation is cyanosis at or before ductal closure in an infant with a murmur; Precordial activity is normal, and the second heart sound is often single; There may be a systolic ejection murmur if there is RVOT obstruction; If the VSD is large, tachypnea, a left ventricular impulse, and a third heart sound may develop during the first few days of life as the PVR falls
tricuspid atresia
in PA/IVS, _______ is rarely normal, ranging from severely hypoplastic and stenotic to regurgitant
tricuspid valve
Chromosomal microarray testing should be done on all infants with?
truncus arteriosus
Infants may have bounding pulses and increased pulse pressures secondary to decreased diastolic pressure caused by runoff into the branch pulmonary arteries
truncus arteriosus
RV-PA conduit replacement is generally required by age 5 years in about 20% of patients, and the conduit must always be replaced at some point in the child's life, sometimes multiple times
truncus arteriosus
The aorta and pulmonary artery originate from a single arterial trunk; This single trunk overrides a large VSD that allows mixing of systemic and pulmonary venous return
truncus arteriosus
The single semilunar truncal valve demonstrates anywhere from one to six leaflets and differing degrees of valvar stenosis and regurgitation; The truncal valve leaflets also tend to be thickened and dysplastic
truncus arteriosus
typically repaired in the first months of life; Includes VSD closure and placement of a right ventricle to pulmonary artery (RV-PA) conduit
truncus arteriosus
which type of truncus arteriosus- there is a short main pulmonary artery segment arising from the truncus, which then gives rise to branch pulmonary arteries?
type I
which type of truncus arteriosus- both the branch pulmonary arteries have a direct origin from the truncus?
type II
which type of truncus arteriosus- the pulmonary arteries arise from opposite sides of the truncus and are remote from each other?
type III
unlike cyanotic defects that usually present in the first day of life, acyanotic defects may go _____ or become apparent _____
undetected days to weeks after birth
infants are generally asymptomatic; diagnosed at an older age secondary to recurrent pneumonias or are identified by echocardiogram in evaluation for a murmur; Volume overload and increased pulmonary blood flow can gradually develop
unobstructed TAPVR
infants with HLHS must have an ________ - increased mortality without it
unrestrictive atrial level shunt
in a VSD, postnatally, as the L side of the heart becomes dominant, blood flow via the VSD permits persistent L to R shunting and pulmonary over circulation increasing risk of ?
CHD
HLHS prediposes the infant to _____ given that the RV modulates both pulmonary and systemic circulations
CHF
If the echo cannot provide all the necessary information needed for diagnosing TAPVR, what should you get?
CT angiography, cardiac cath, or MRI
_____ or _____ can also occur in association with D-TGA with VSD
Coarctation of the aorta or aortic arch interruption
Sites of venous obstruction can be illustrated via spectral and color flow Doppler imaging on echo
TAPVR
L to R shunting at the ventricular or great vessel level results in overload in _____
LV
stage I surgical palpation of HLHS in the immediate postnatal period
Norwood procedure
CXR shows cardiomegaly, owing to right atrial enlargement; Echocardiography is diagnostic
PA/IVS
Cyanosis becomes apparent within hours of birth and is progressive; Tachypnea may be prominent; The first and second heart sounds are single, and there may be a holosystolic murmur heard best at the left lower sternal border consistent with tricuspid regurgitation
PA/IVS
four types of TAPVR?
Supracardiac type (most common), Infracardiac type, Cardiac type, and Mixed type (least common)
Which VSDs typically have spontaneous closure?
perimembranous muscular
A group of defects in which all the pulmonary veins carrying oxygenated blood drain into the systemic venous circulation rather than normally to the LA
TAPVR
CXR is very helpful in ______ since it may demonstrate pulmonary edema which is highly suggestive of pulmonary venous obstruction
TAPVR
in TOF, severe pulmonary stenosis is associated with ____ and is responsible for the audible murmur heard during early disease states; blood is preferentially shunted from R to L across the VSD resulting in varying degrees of cyanosis depending on the severity of RVOT and patency of PDA
"blue" TOF
in TOF, involves minimal ventricular outflow obstruction, no significant directional shunt across the VSD, and no cyanosis; may remain subclinical for years
"pink" TOF
Dilated mediastinal veins give an appearance of fullness to the superior mediastinum -this is usually masked by the thymus in neonates
"snowman" sign in TAPVR
tetralogy of fallot consists of?
1. Large VSD 2. pulmonary stenosis 3. right ventricular hypertrophy 4. large aorta overriding the ventricular septum
four major types of VSD?
1. perimembranous 2. muscular 3. inlet 4. juxta-arterial (sub arterial)
indications for surgical closure of VSD?
1. persistent respiratory symptoms or suboptimal feeding and growth despite maximal medical therapy 2. evidence of right ventricular hypertension 3. deleterious intracardiac changes such as left heart dilation or aortic valve leaflet prolapse/insufficiency 4. defect types with minimal likelihood of spontaneous closure
Treat tet spells by finding ways to decrease the subpulmonary obstruction, encourage more pulmonary blood flow, and increase the relative degree of left to right shunting at the ventricular level- how can you do this?
100% O2, knee to chest, fluid bolus, meds to decrease catecholamines/infundibular RV spasm (morphine, ketamine, fentanyl, propranolol)
in TAPVR, pulmonary vein diameters less than _____ have been associated with a poor prognosis
2 mm
in tricuspid atresia, some infants with well-balanced systemic and pulmonary flows can proceed directly to a bidirectional Glenn operation or a total cavopulmonary (Fontan) operation at about ____
2-3 years of age
Elective repairs of TOF occur when infants are between _______; in the absence of marked pulmonary artery hypoplasia or unfavorable coronary artery anatomy
4-6 months of age
TGA parallel circulation is incompatible with life; therefore, early identification is critical to establishing an open communication between the two parallel circuits by way of an?
ASD VSD PDA
treatment options for PA/IVS?
Balloon pulmonary valvuloplasty is feasible in infants with even a tiny orifice in the pulmonary valve Radiofrequency perforation of the valve may be considered If interventional catheterization is unsuccessful, surgical valvotomy is indicated unless there is clear RV-dependent coronary flow
what does the repair of TOF consist of?
Consists of relief of pulmonary stenosis and RVOT obstruction (often with a transannular patch) and patch closure of the VSD
treatment of tricuspid atresia?
Conversion to a bidirectional cavopulmonary (Glenn) anastomosis - between 3 and 6 months of age
The aorta and the pulmonary artery both arise from the right ventricle; There are four distinct subtypes based on the location of the VSD and its relationship to the great arteries
DORV
There is arrested delamination of the tricuspid leaflets during valve development; The septal leaflet can become tethered to the interventricular septum to varying degrees with apical displacement of its annular attachment, potentially leading to significant tricuspid regurgitation
Ebstein anomaly
The most severe manifestation of pulmonary vascular disease due to persistent shunting is __________, with shunt reversal (right-to-left), cyanosis, and irreversible pulmonary hypertension
Eisenmenger syndrome
T/F? the VSD in TOF is generally large and unrestrictive and produces a significant murmur
FALSE- it does not produce a murmur
Classically associated with abnormalities of the mitral and aortic valves - ranging from mitral stenosis and aortic stenosis to mitral atresia and aortic atresia (most severe); There is resultant LV hypoplasia and varying degrees of ascending aorta and transverse arch hypoplasia
HLHS
In utero, when PVR is high, providing excellent organ perfusion, and fetal growth; Postnatally, even as PVR falls and pulmonary blood flow increases, right ventricular output remains adequate to supply both the systemic and pulmonary circulations; However, ductal closure leads to profound systemic hypoperfusion with acidosis and multisystem organ failure
HLHS
Tachypnea and mild cyanosis in the postnatal period; There can be a moderately increased right ventricular impulse on exam with a single second heart sound and variable murmurs; With ductal patency, pulses can be nearly normal; Pulse oximetry 70-90% depending on pulmonary blood flow and edema
HLHS
a spectrum resulting from underdevelopment of the left heart-aorta complex that involves the atresia, stenosis, or hypoplasia of the mitral and aortic valves and hypoplasia of the left ventricle, preductal aorta, and aortic arch
HLHS
aberrant development of the mitral valve leads to reduced preload into the LV and blood aggregates within the left atrium which increased after load within in the LA and encourages blood flow across the PFO and over time this permits ongoing reductions in blood flow into LV encouraging hypoplasia
HLHS
incompetence of the aortic valve inhibits evacuation of blood from the left ventricle and into the ascending aorta which leads to increased pressure/afterload in LV which over time leads to LV hypertrophy and the constant distending pressure created by the increased after load inhabits normal flow into the LV which over time inhibits growth and leads to LV hypoplasia
HLHS
involves parallel pulmonary and systemic circulations both modulated by the right ventricle
HLHS
right sided compensation leads to enlargement and inhibits normal development of the left side of the heart; increased LA after load encouraged L to R shunting across PFO and back into R side and results in compensatory expansion of right side of heart and over time the right sided enlargement competes spatially with the left side of the heart and contributes to left sided hypoplasia
HLHS
In transposition of the great arteries, a ______ allows for additional shunting from the aorta (deoxygenated blood) to the pulmonary artery (oxygenated blood), resulting in increased pulmonary venous return and L to R shunt at the atrial level
PDA
the only mechanism by which gas exchange occurs in HLHS
PDA
common acyanotic CHD?
PDA ASD VSD AV canal
in tricuspid atresia, pulmonary blood flow is supplied by L to R shunting through either _____ or _____; Associated RV hypoplasia and pulmonary artery hypoplasia are proportional to the size of the _____ and the degree of subpulmonary and pulmonary stenosis
PDA or VSD VSD
Severe Ebstein anomaly with severe tricuspid valve displacement and regurgitation that are ductal dependent with minimal or no anterograde flow across the pulmonary valve - _______ can be useful in the short term
PGE infusion
_____ is critical for survival in HLHS in the immediate postnatal period to maintain ductal patency
PGE infusion
______ is essential for maintaining ductal patency in PA/IVS
PGE infusion
Evidence of significant L to R shunting with a VSD and resultant pulmonary overcirculation may not become apparent until _____ after the immediate neonatal period
PVR drops
most common type of VSD
Perimembranous VSD
in TAPVR, systemic blood flow is dependent on shunting from _____
RA to LA through an ASD
L to R shunting at the atrial level results in overload in ______
RV
the severity of TOF presentation is directly related to the severity of _____
RVOT obstruction
Preferred method for ASD closure if there is continued shunting with intracardiac sequelae?
Transcatheter ASD device closure
anatomical defect caused by abnormal ventricular septation; single or multiple openings at various locations within the ventricular septum result; as a consequence, oxygenated blood mixes with deoxygenated blood during fetal and postnatal development
VSD
in tricuspid atresia, ______ mixing to supply the pulmonary circulation may be adequate in the first months of life as long as the atrial level defect remains nonrestrictive
VSD
in TOF, shunting across the _____ is determined by an intricate balance between the SVR and PVR; endogenous ______ increases PVR more than SVR (from acute narrowing) resulting in R to L shunting and worsening systemic desaturations
VSD catecholamines
a murmur is not heard in transposition of the great arteries unless: holosystolic murmur, when present, suggests an associated _____; Systolic ejection murmur with _______
VSD pulmonary stenosis
Most common congenital heart defect
Ventricular septal defect (VSD)
the increased pulmonary blood flow and obstructed outflow from LA in HLHS leads to increased pulmonary venous pressure which often causes the neonate to manifest with ________; may precede or present alongside cardiogenic shock
a "gasping" respiratory effort
classic CXR finding of TOF?
a boot-shaped heart caused by RV hypertrophy and consequent upturned cardiac apex
central nervous system anomalies such ______ are common in HLHS
absent corpus callosum
are there respiratory symptoms in transposition of the great arteries?
absent or limited to hyperpnea or tachypnea without dyspnea
The most common obstructive arch lesion is ?
aortic coarctation
what does the Norwood procedure consist of?
aortic reconstruction systemic to pulmonary arterial shunt placement atrial septecomy
closure of a perimembraneous VSD may be the result of _____ and therefor must be carefully followed
aortic valve prolapse
Unilateral or bilateral branch pulmonary artery stenosis occurs in about 10% of truncus arteriosus cases = ________ supplying the lungs
aortopulmonary collaterals
An abnormal connection between the ascending aorta and the main pulmonary artery; The defects tend to be large, allowing high-pressure shunting from the systemic to the pulmonary circulation and resulting in considerable pulmonary overcirculation
aortopulmonary window
Mimics that of a large VSD or PDA with significant L to R shunting that increases as the PVR falls
aortopulmonary window
what can happen long term after surgical correction of TAPVR?
arrhythmias and poor chronotropic response to exercise; Recurrent or persistent pulmonary venous obstruction at the anastomotic site can happen
surgical fix of transposition of the great arteries?
arterial switch operation
Balloon atrial septostomy is rarely necessary in tricuspid atresia unless there is significant restriction ______
at the atrial level
in transposition of the great arteries, the shunts are PFO, ASD, PDA, VSD- what has been shown to be the best site of blood mixing?
at the atrial level between PFO or ASD
If functional or anatomic pulmonary atresia occurs in ebstein anomaly, an obligatory R to L shunt must exist at the ______; The degree of this shunt is proportional to the severity of the tricuspid valve abnormality
atrial level
All neonates with tricuspid atresia must have an _____ for postnatal survival
atrial level defect
An ________ is essential for postnatal survival with TAPVC
atrial level defect
in single ventricle anatomy, there must be adequate mixing of systemic and pulmonary venous return to support single ventricular output; This mixing occurs most effectively through?
atrial level defect - ASD or PFO
in ebstein anomaly, secondary to apical tricuspid annular displacement into the right ventricle, a portion of the RV becomes ________ detrimentally affecting its contractile efficiency
atrialized
in between the blue and pink extremes are cases of TOF with _______; equivocal pressures protect the pulmonary arteries from over circulation and reactive pulmonary hypertension ; the amount of RVOT is proportionate to the degree to which the aorta overrides the ventricular septum
balanced pulmonary stenosis
if prostaglandin management fails in transposition of the great arteries, ________ is needed to enlarge atrial level defect and improved mixing
balloon septostomy
a small tube that connects the arterial circulation to the pulmonary circulation in order to get more blood to the lungs. This is the first in a series of operations required to correct complex congenital (present at birth) heart defects; a temporary fix, as it is only a certain size, but it allows the baby to grow to better prepare for his or her next operation; mimics the role of the ductus arteriosus, meaning it allows blood to flow from a major artery through a connection to the pulmonary artery. Not only does this allow more blood to be oxygenated by the lungs, it also encourages the pulmonary arteries to grow, making the next surgery easier.
blalock-traussig shunt (systemic to pulmonary shunt)
in PA/IVS, _____ is necessary to assess coronary flow, the presence (including size and location) or absence of coronary sinusoids and fistulae, and pulmonary blood flow sources and for consideration of pulmonary valve intervention
cardiac catherization
________ remains an option but is greatly limited by donor heart availability in HLHS
cardiac transplantation
This procedure involves disconnecting the superior vena cava from the right atrium and connecting it to the right pulmonary artery. This lets the blue, deoxygenated blood flow directly to both lungs, bypassing the heart. This procedure is usually one in a series of operations
cavopulmonary (glenn) anastomosis
in transposition of the great arteries, the peripheral pulses are normal unless ______ is present
coarctation of the aorta
two types of TGA?
dextro-transposition (D-TGA) - most common levo-transposition (L-TGA)
a consistent diversion of systemic blood flow away from the descending aorta and back across the PDA in a left to right direction is commonly referred to as ____; can lead to inadequate oxygen delivery to the cerebral arteries, mesentery, and renal systems and risks associated with ischemic damage
diastolic steal phenomenon
all patients with truncus arteriosus should be screened for what chromosomal abnormality?
digeorge syndrome (22q11 chromosomal micro deletion) **Should also be screened for the presence of a thymus and serum calcium derangements
Hemodynamically significant VSDs often require _____ to treat pulmonary circulation; as such, these infants incur increased risk for nutritional deficiencies and poor linear growth
diuretic therapy
Increased pulmonary blood flow and resultant symptomatology with truncus arteriosus can generally be treated with?
diuretics and digoxin
Neonates with tricuspid atresia, a small VSD, and severe pulmonary stenosis have severely limited pulmonary blood flow and are?
ductal dependent
The systemic circulation is ______ in HLHS
ductal dependent
May present with profound cyanosis, tachypnea, and cardiovascular collapse; Cyanosis is dependent on the extent of atrial right-to-left shunting; A low-frequency holosystolic murmur of tricuspid regurgitation is often heard on exam, and the first heart sound may be widely split
ebstein anomaly
gold standard for diagnosing TAPVR
echo
gold standard for diagnosing truncus arteriosus?
echo
how do you diagnose transposition of the great arteries?
echo
diagnosis of TFO?
echo **may see associated coronary artery anomalies, additional VSDs, or collateral vessels
Preventing complications from ______ is essential for good long-term results in HLHS
excessive pulmonary blood flow
in tricuspid atresia, ______ is an indicator of a restrictive ASD and elevated right atrial pressures
hepatic enlargement
Neonates with _______ commonly have associated TAPVC
heterotaxy
in transposition of the great arteries, profound hypoxia caused by a ________ can lead to rapid deterioration and death within the first hours of life
highly restrictive atrial level defect
leads to increased pulmonary stenosis and reduced blood flow to the pulmonary artery which leads to increased R to L shunting and an acute reduction in oxygen content of the blood; with severe hypoxemia, a switch from aerobic to anaerobic metabolism occurs and leads to lactic and metabolic acidosis; can be fatal if left untreated
hyper cyanotic tet spells
factors favoring postnatal persistence of PDA?
hypoxemia acidosis PGE administration postnatal glucocorticoid exposure
in HLHS, The combo of pulmonary edema and restricted pulmonary blood flow produces profound ________
hypoxia
the arterial switch operation is optimally performed _______ for infants with D-TGA
in first week of life
type of VSD that arise inferior to the perimembranous region and slightly below the tricuspid and mitral valves
inlet VSD
in TOF, severe pulmonary stenosis or atresia with markedly diminished pulmonary blood flow and R to L shunting across the VSD will result in?
intensely cyanotic newborn
The most severe form of arch obstruction is ______ with complete aortic discontinuity; classified according to the location of the discontinuity in relation to the head and neck vessel origins
interrupted aortic arch
Uncomplicated truncus can be subtle with ______ and ______ being the only symptom
mild tachypnea and cyanosis
in TOF, lesser degree of pulmonary stenosis with mainly L to R shunting across the VSD will result in?
minimally cyanotic or acyanotic newborn
type of VSD located in the lower, thicker trabecular portion of the ventricular septum; occur secondarily to excessive excavation of myocardial tissue during ventricular growth
muscular VSD
in severe Ebstein anomaly with severe tricuspid valve displacement and regurgitation, measures to lower the PVR such ______ might be necessary before anterograde flow across the pulmonary valve can be established
nitric oxide several attempts to wean the PGE infusion
the pathophysiology of HLHS relates to the severity of the _______ caused by the constellation of anatomic defects and associated hypoplasia
obstruction
large VSD with pulmonary over circulation symptoms is an indication to initiate medical management which consists of?
oral diuretics to help reduce pulmonary symptoms and improve feeding and growth
type of ASD that is located in the center of the atrial septum in the area of the foramen oval; arises secondary to defective development of the septum primum
ostium secundum ASD
four types of ASD?
ostium secundum ASD (most common) ostium primum ASD sinus venosus (superior and inferior) ASD coronary sinus ASD