B P9 C82 CHD in the Adolescent and Adult Flashcards
At least __% of these adult congenital heart disease (ACHD) patients have complex cardiovascular anatomy and suffer from multi-system organ involvement.
20%
Transition discussions should begin at the age of __ years, with the expectation of complete transfer to ACHD care by __ years of age.
Begin at 12 years
Complete transfer at 21 years
Anatomic Classification of CHD (Class I)
Class I: Simple
Native disease
* Isolated small ASD
* Isolated small VSD
* Mild isolated pulmonic stenosis
Repaired conditions
* Previously ligated or occluded ductus arteriosus
* Repaired secundum ASD or sinus venosus defect without significant residual shunt or chamber enlargement
* Repaired VSD without significant residual shunt or chamber enlargement
Anatomic Classification of CHD (Class II)
Class II: Moderate Complexity
- Aorto-left ventricular fistula
- Anomalous pulmonary venous connection, partial or total
- Anomalous coronary artery arising from the pulmonary artery
- Anomalous aortic origin of a coronary artery from the opposite sinus
- AVSD (partial or complete, including primum ASD)
- Congenital aortic valve disease
- Congenital mitral valve disease
- Coarctation of the aorta
- Ebstein anomaly (disease spectrum includes mild, moderate, and
severe variations) - Infundibular right ventricular outflow obstruction
- Ostium primum ASD
- Moderate and large unrepaired secundum ASD
- Moderate and large persistently patent ductus arteriosus
- Pulmonary valve regurgitation (moderate or greater)
- Pulmonary valve stenosis (moderate or greater)
- Peripheral pulmonary stenosis
- Sinus of Valsalva fistula/aneurysm
- Sinus venosus defect
- Subvalvar aortic stenosis (excluding HCM; HCM not addressed in
these guidelines) - Supravalvar aortic stenosis
- Straddling atrioventricular valve
- Repaired tetralogy of Fallot
- VSD with associated abnormality and/or moderate or greater shunt
Anatomic Classification of CHD (Class III)
Class III: Great Complexity (or Complex)
- Cyanotic congenital heart defect (unrepaired or palliated, all forms)
- Double-outlet ventricle
- Fontan procedure
- Interrupted aortic arch
- Mitral atresia
- Single ventricle (including double inlet left ventricle, tricuspid atresia, hypoplastic left heart, any other anatomic abnormality with a functionally single ventricle)
- Pulmonary atresia (all forms)
- TGA (classic or d-TGA; CCTGA or
l-TGA) - Truncus arteriosus
- Other abnormalities
of atrioventricular and ventriculoarterial connection
(i.e., crisscross heart, isomerism, heterotaxy syndromes, ventricular inversion)
Physiologic stages of ACHD patient classification
A: NYHA FC I, normal exercise capacity, normal renal, hepatic, and pulmonary function
B: NYHA FC II, mild valvular disease, arrhythmias not requiring treatment, mild hemodynamic sequelae, abnormal objective cardiac limitation to exercise, trivial or small shunt
C: NYHA FC III, significant valvular disease, arrhythmias controlled with treatment, moderate or greater ventricular dysfunction, moderate aortic enlargement, venous or arterial stenosis, mild or moderate hypoxemia/cyanosis, hemodynamically significant shunt, pulmonary hypertension, end-organ dysfunction responsive to therapy
D: NYHA FC IV, arrhythmias refractory to treatment, severe aortic enlargement, severe hypoxemia, severe pulmonary hypertension, Eisenmenger syndrome, refractory end-organ dysfunction
Location of surgical scars will indicate whether a patient has had a lateral thoracotomy, such as with a _____.
Patent ductus arteriosus (PDA) ligation
or
Aortic coarctation repair
Absence of a radial pulse on the ipsilateral arm as a thoracotomy scar may suggest that the subclavian artery was sacrificed in the repair, such as a _____.
Subclavian flap repair for coarctation of the aorta
or
Prior classic Blalock-Taussig-Thomas [BTT] shunt
Anomalous left coronary artery from the pulmonary artery (ALCAPA)
Bland-White-Garland syndrome
Pulmonary hypertension with cyanosis due to right to left shunting
Eisenmenger Syndromer
Septal defect resulting in direct left ventricle to right atrium shunt
Gerbode Syndrome
Double inlet left ventricle with D-looped ventricles and normally related great vessels
Holmes Heart
Coronary sinus septal defect in the presence of a left superior vena cava
Raghib Defect
Partial anomalous pulmonary venous connections of the right lower pulmonary vein to the IVC-RA junction, often accompanied by pulmonary artery hypoplasia and aortopulmonary collateral formation.
Scimitar Syndrome
Syndrome with a series of left-sided obstructive lesions
Shone Syndrome
Form of double outlet right ventricle with D-malposed, side-by-side great vessels, sub-pulmonary VSD, hypoplastic aortic arch
Taussig-Bing Malformation
Early palliative procedure for transposition of the great arteries, with the inferior vena cava directed to the left atrium via homograft
Baffes Procedure
“Classic”—direct end to end anastomosis of subclavian artery to pulmonary artery
“Modified”—tube graft from subclavian artery to pulmonary artery
Blalock-Taussig (-Thomas) Shunt
Closed infundibular resection for relief of pulmonary stenosis
Brock Procedure
Atriopulmonary anastomosis for single ventricle heart disease
Fontan or Fontan-Kreutzer
Includes the right ventricle into the pulmonary circulation, was the unique modification for tricuspid atresia
Fontan-Björk Modification
“Classic”—end to end anastomosis of superior vena cava to right pulmonary artery
“bidirectional”—end to side anastomosis of superior vena cava to right pulmonary artery
Glenn
Bidirectional Glenn in context of interrupted inferior vena cava with azygos continuation to the superior vena cava
Kawashima
Anterior translocation of the pulmonary arteries, so that both branch pulmonary arteries run anterior to the aorta. Most commonly used as part of the arterial switch operation
Lecompte Maneuver
Atrial switch operations for transposition of the great arteries, with atrial baffling using native atrial or pericardial tissue to redirect systemic and pulmonary venous flow
Atrial: Senning
Pericardial: Mustard
In double outlet right ventricle, posterior translocation of the aortic root towards the left ventricle, with baffling of the left ventricle to the aorta in its new position
Nikaidoh
Neonatal palliative procedure for hypoplastic left heart syndrome including aortic arch reconstruction with anastomosis of the native aorta to the pulmonary artery, which becomes the “neo-aorta,” as well as atrial septectomy and a modified BT shunt
Norwood
Direct anastomosis of the left pulmonary artery to the descending aorta
Potts Shunt
Intra-cardiac routing of the left ventricle to the aorta, which arose from the right ventricle. Usually accompanied by a right ventricle to pulmonary artery conduit.
Rastelli
Intrapulmonary baffle of the left coronary artery performed for anomalous left coronary artery from the pulmonary artery
Takeuchi Repair
Direct anastomosis of the right pulmonary artery to the ascending aorta
Waterson shunt
Fixed S2 splitting
ASD
Diminished lower extremity pulse
Aortic coarctation
A classic physical examination finding in a patient with _____ is a systolic ejection click which decreases in intensity with inspiration.
Pulmonary stenosis
This remains the cornerstone of cardiac imaging in the CHD patient
Echocardiography
This is recommended in any ACHD patient with signs of elevated pulmonary artery (PA) pressure to determine pulmonary vascular resistance (PVR)
Cardiac Catheterization
Abnormal lung function is com- mon in ACHD patients,and up to __% of ACHD patients have abnormal pulmonary function tests.
40%
Multiple mechanisms for abnormal pulmonary mechanics in ACHD patients,
RLD: post-op patients
Diaphragmatic paralysis: phrenic nerve injury
Asymmetric pulmonary blood flow due to branch PA abnormalities or acquired conditions
Hemoptysis may occur in up to _____of ACHD patients with Eisenmenger syndrome.
1/3
PH is found in up to __% of ACHD patients and is strongly associated with increased morbidity and mortality
10%
This is common in ACHD patients and has been shown to be a primary driver of high-resource utilization for ACHD hospitalizations,accounting for up to one-third of hospital charges
Renal dysfunction
_____-based estimated glomerular filtration rate (eGFR) more accurately predicts clinical effects in ACHD patients than creatinine-based eGFR.
Cystatin C
The majority of the evidence of hepatic dysfunction in ACHD patients has been focused on _____
Fontan-associated liver disease (FALD)
_____ remains an important cause of liver disease in older ACHD patients, particularly those who received blood transfusions prior to 1992.
Hepatitis C
Abnormalities of the coagulation system are observed in patients with _____ and are associated with bleeding and thrombotic complications.
Fontan physiology
Cancer is the second leading cause of non- cardiovascular death in ACHD patients, with certain malignancies having an increased prevalence in specific CHD conditions, that is, _____.
HCC in adults with Fontan physiology
It is the most common aneuploidy and is usually caused by trisomy 21.
Down Syndrome
It is also the most common chromosome abnormality associated with CHD
Down Syndrome
Fifty percent of children born with Down syndrome have CHD, most commonly defects in the _____
AV canal
Sinus node dysfunction is most commonly related to _____.
Surgical repair
Sinus node dysfunction is commonly encountered in patients with _____.
D-loop TGA who have undergone a Mustard or Senning operation
Repair for ASD or sinus venosus defects
_____ pacing is required for symptomatic sinus node dysfunction.
Atrial pacing
Patients who have had a _____ and those with open atrial shunts typically require epicardial pacing.
Fontan operation with an extracardiac conduit
Heart block is common in patients with _____ as the AV node is superiorly and anteriorly displaced
L-loop TGA
_____ is the most common tachyarrhythmia in CHD, accounting for 62% of atrial arrhythmias.
Interatrial re-entrant tachycardia (IART)
In patients who have undergone the atrial switch operation, IART is a dangerous arrhythmia which may convert to _____ as the noncompliant interatrial baffles and systemic RV perform poorly at high heart rates
Polymorphic VT or PEA
_____ control is usually preferred for adults with CHD and IART.
Rhythm
_____ are often preferred in patients with complex CHD. Amiodarone has considerable cumulative toxicity so is usually not a preferred first-line anti- arrhythmic in younger patients but is sometimes required for patients with ventricular dysfunction.
Sotalol and dofetilide
Patients with _____ and IART should receive long-term anticoagulation.
Fontan circulation
As patients with CHD age, ____ becomes more common and is the most common atrial arrhythmia in adults with CHD older than age 50.
Atril Fibrillation
_____ is the second most common cause of cardiac death in adults with CHD following heart failure,and accounts for approximately 20% of death in patients with CHD, occurring at a rate of ∼0.1% per patient year.
Sudden Death
_____, and complex forms of CHD are at the highest risk for ventricular arrhythmias and sudden cardiac death.
TOF
TGA with a systemic RV
Fontan circulation
Eisenmenger syndrome
Pacemakers should be _____ in patients with intracardiac shunts, single ventricle, or ventricular leads in Fontan circulation.
Epicardial
Class I recommendations for secondary prevention with ICDs in adults with CHD
Class IB:
ICD therapy is indicated in adults with CHD who are survivors of cardiac arrest due to ventricular fibrillation or hemodynamically unstable VT after evaluation to define the cause of the event and exclude any completely reversible etiology
Class IB:
ICD therapy is indicated in adults with CHD and spontaneous sustained VT who have undergone hemodynamic and electrophysiologic evaluation.
Class IC:
Catheter ablation or surgery may offer a reasonable alternative or adjunct to ICD therapy in carefully selected patients
Class I recommendations for primary prevention with ICDs in adults with CHD
Class IB:
ICD therapy is indicated in adults with CHD and a systemic left ventricular ejection fraction ≤ 35%, biventricular physiology, and NYHA Class II or III symptoms
Heart failure is most common in patients with high anatomic or physiologic complexity including:
Single ventricle anatomy
Systemic RV
PH
Cyanosis
The systemic RV is predisposed to systolic dysfunction due to:
Unfavorable myocardial fiber orientation
Nonconical shape
Coronary supply-demand mismatch
Volume loading from tricuspid regurgitation
Fontan failure is characterized by:
Chronically elevated central venous pressure
Low cardiac output
Cyanosis due to collaterals
Ascites
Cirrhosis
Standard guideline-directed medical therapy (GDMT) is likely effective when taken by CHD patients with a _____.
Systemic LV who have heart failure due to left ventricular systolic dysfunction after residual hemodynamic lesions have been addressed
CRT via multi-site pacing is appropriate for patients with a _____.
Systemic LV
Spontaneous or pacing-induced left bundle-branch block
Heart failure
Placing CRT leads is technically more difficult in patients with a systemic RV due to _____. It is reasonable to consider placement of epicardial multi-site pacing leads in selected patients if they are going for cardiac surgery for other indications.
Variability in the location of the CS os
Variant coronary vein anatomy
Presence of surgical interatrial baffles
Patients with CHD and heart failure refractory to medical therapy should be considered for ____.
Heart Transplantation
Compared to patients with acquired heart failure, CHD patients listed for heart transplantation are _____.
Younger
More likely to have a prior sternotomy
Less likely to have coronary artery disease
Less likely to have a left ventricular assist device
The current multimodality imaging guidelines for the thoracic aorta in adults advocate obtaining aortic root measurements at _____ with a leading edge–to–leading edge technique, whereas imaging guidelines for pediatric echocardiograms, which are often used in echocardiography labs specializing in CHD, suggest obtaining aortic root measurements in _____ with an inner edge–to–inner edge technique.
End-diastole
Mid-systole
Aortic dilation is common in adults with CHD, particularly in patients with _____.
BAV
Conotruncal defects
Antibiotic prophylaxis is recommended prior to dental procedures for ACHD patients with high-risk characteristics, which include:
(1) prior episodes of SBE
(2) prosthetic valves (including transcatheter)
(3) valve repair using a prosthetic ring
(4) residual intracardiac shunts adjacent to prosthetic material
(5) cyanotic CHD
(6) any CHD repaired with prosthetic material up to 6 months after the procedure or lifelong if residual shunt or valvular regurgitation remains
(7) for cardiac transplant recipients who develop cardiac valvulopathy
The most common pathogens responsible for SBE include:
Streptococcus viridans
Staphylococcus species
Enterococcus species
The CARPREG 2 investigators reported the mater- nal outcomes of 1938 pregnancies in women with cardiac disease (63% CHD), and 16% of women experienced an adverse cardiac out- come, primarily heart failure and arrhythmias. The highest-weighted risk factors (weight of three points) include:.
(1) A prior history of cardiac events or arrhythmias
(2) Decreased functional status (New York Heart Association, NYHA, Class ≥III)
(3) Presence of a mechanical heart valve
CARPREG2
The predicted risks for cardiac events stratified according to point score were:
≤1 point (5%)
2 points (10%)
3 points (15%)
4 points (22%),
>4 points (41%)
The recommended follow-up for women with WHO risk category II is ____; women with WHO risk category ≥III should be seen _____.
WHO risk category II: every trimester
WHO risk category ≥III: monthly or bimonthly
Women with a high risk of maternal morbidity or mortality, including women with _____ should be counselled against pregnancy and consider termination if they become pregnant
Pulmonary arterial hypertension (PAH)
Eisenmenger syndrome
Severe systemic ventricuar dysfunction
Severe left-sided obstructive lesions
Physiological stage D
Low-risk patients can begin a training program at approximately __% maximal predicted heart rate at least three times per week (totaling 150 minutes/week) and increase intensity or duration over time.
70%
Congenital conditions in which high intensity exercise should be avoided:
Cyanosis
High risk coronary anomalies
Hypoxemia
Severe aortic dilation
Severe outflow tract obstruction
Severe pulmonary hypertension
Severe ventricular dysfunction
Ventricular arrhythmias
The degree of left-to-right shunting determines the amount of chamber dilation and is dictated by the _____.
(1) Size of the defect
(2) Diastolic properties of the heart
(3) Resistance in the great arteries
In general, shunt lesions proximal to the tricuspid valve (such as ASDs and anomalous pulmonary venous return) cause _____ dilation, those below the tricuspid valve (such as VSDs and PDAs) cause left heart dilation
Proximal to TV: Right heart dilation
Below the TV: Left heart dilation
The most common type of ASD is a _____ ASD, which is a true deficiency in the atrial septum, in the region of the fossa ovalis.
Secundum ASD
Secundum ASDs should be differentiated from a _____, which is persistence of patency of the flap valve of the fossa ovalis (not associated with right-sided cardiac dilation) and persists in up to 25% of adults
Patent foramen ovale (PFO)
_____ ASDs may be considered in the spectrum of AVSDs and involve a deficiency in the region of the AV valves and are associated with a cleft in the mitral valve
Primum ASD
Sinus venosus defects occur in the sinus venosus septum posterior to the true atrial septum and usually involve _____.
Anomalous right-sided pulmonary venous return
CS defects (also called unroofed CSs) are rare and involve direct communication of the CS and the LA due to complete or partial unroofing of the CS and are often accompanied by a _____.
Persistent left-sided SVC
PAPVC may be associated with either right- or left-sided pulmonary veins which can have several possible anomalous connections, with the most common being a _____.
LUPV to an ascending vertical vein into the brachiocephalic vein
or
RUPV draining to the SVC
When the right-sided pulmonary veins connect to the inferior vena cava (IVC), this is called a _____.
Scimitar Vein
The presence of cyanosis in patients with ASD should alert one to the possibility of _____.
(1) Shunt reversal and Eisenmenger syndrome
OR
(2) Prominent eustachian valve directing the inferior vena caval flow to the LA via a secundum ASD or sinus venosus defect of the inferior vena caval type
The classic physical examination of an ASD is a _____, which is due to prolonged RV ejection and increased PA capacitance, which, in turn, delay pulmonary valve closure.
Wide, fixed splitting of the S2
The ECG in ASD commonly displays a _____.
Rightward QRS axis and an incomplete RBBB
The classic chest radiograph features are of _____.
Cardiomegaly (from right atrial and right ventricular enlargement)
Dilated central pulmonary arteries with pulmonary congestion
When an ASD is discovered in an adult, if there is any degree of right heart dilation associated with symptoms, closure should be consid- ered.51 It is important to verify the direction of the shunt as left-to-right.
The next step is to verify that the _____.
(1) PVR is less than 1⁄3 the SVR
(2) PA systolic pressure is less than 50% systemic
(3) Right heart enlargement
(4) Qp/Qs is at least 1.5:1
Special attention must be paid to those defects with partial anomalous pulmonary venous drainage, as redirecting the pulmonary venous flow may result in _____
Pulmonary vein stenosis
In patients with primum ASDs, care must be taken in closing the mitral valve cleft to avoid ____.
Mitral stenosis or residual regurgitation
The imaging recommendations for patients following transcatheter ASD closure are dictated by the specific manufacturer, but in general, TTE is performed at _____.
1 week, 1 month, and then annually for at least 5 years following closure
Any patient who has had a transcatheter ASD device placed who presents with chest pain should have an urgent evaluation to rule out _____, which occurs in 1 in 1000 cases.
Device erosion
AVSDs are the result of failed fusion of the _____, and comprise up to 5% of CHD
Endocardial cushions
Complete AVSDs are characterized by both an atrial and ventricular level defect with a common AV valve, usually comprised of ____ leaflets
Five
A partial AVSD does not have a VSD component, and almost always has a _____.
Cleft in the anterior mitral valve leaflet
A unique anatomic feature in patients with AVSD is _____, due to the aortic valve being displaced anteriorly.This results in a scooped-out appearance of the ventricular septum and a shortened LV inlet, creating a “goose neck” appearance.
Elongation of the LVOT
Indications for surgical intervention of AVSD in adults are similar to those for ASD closure. These include _____.
(1) a net left-to-right shunt (Qp:Qs ≥1.5:1)
(2) PA systolic pressure less than 50% systemic
(3) PVR less than one third systemic
For adults with prior AVSD repair, left ____ is the most common reason for later surgical reintervention.
AV valve regurgitation
_____ are the most common form of CHD, and the reported incidence of isolated varies widely, from 1.5 to 53 per 1000 live births.
VSDs
Many _____ VSDs are small and close spontaneously in childhood.
Isolated muscular and perimembranous VSDs
Continuous wave Doppler through these defects reveals a high velocity, often greater than ____ msec, which confirms the pressure-restrictive physiology. In this case, the left-to-right shunt is minimal, and there is usually not left ventricular dilation
5 msec
Angiogram of the left ventricular outflow tract demonstrating the classic “gooseneck” deformity in a patient with a _____. This appearance is the result of the inlet dimension of the left ventricle being shorter than the outlet dimension
Primum ASD
Adults with a VSD and evidence of _____ should undergo VSD closure if PA systolic pressure is less than 50% systemic and PVR is less than one third systemic (AHA/ACC Class I recommendation, level of evidence B-NR).
LV volume overload and hemodynamically significant shunts (Qp:Qs ≥ 1.5:1)
The gold standard for VSD closure remains _____. However, transcatheter device occlusion of selected _____ defects is possible with favorable outcomes.
Surgical direct suture closure or patch
Muscular and perimembranous
The ductus arteriosus, derived from the _____, connects the PA to the descending aorta in fetal life and normally closes within the first day of life.
Distal left sixth primitive arch
Measurement of oxygen saturation should be performed in feet and both hands in adults with a PDA to assess for the presence of right-to-left shunting, as _____ is caused by right-to-left shunting in PDA predominantly downstream from the ductal insertion into the aorta
Cyanosis
On auscultation, a classic murmur of PDA is heard best just below the ____ and typically extends from _____ into diastole and peripheral pulses may be bounding.
Below the left clavicle
Extends from systole past the second heart sound into diastole
PDA closure in adults is recommended if:
Left atrial or LV enlargement is present and attributable to PDA
Net left-to-right shunt
PA systolic pressure is less than 50% systemic, and PVR less than one third systemic
Patients with device occlusion or after surgical closure should be examined periodically for possible _____
Recanalization
Endocarditis prophylaxis is recommended for _____ months following PDA device closure for life if any residual defect persists following device closure
6 months
Ebstein anomaly is characterized by apical displacement of the _____ from the AV junction into the RV resulting from failure of delamination of the tricuspid valve leaflets from the underlying RV endocardium during cardiac development
Septal tricuspid valve leaflets
A distance between insertion sites of the two AV valves greater than _____ mm/m2 is required for the diagnosis of Ebstein malformation of the tricuspid valve, or a maximum displacement of more than ___ mm in an adult.
8mm/m2
Max displacement in an adult: 20 mm
Patients with Ebstein anomaly are typically followed lifelong on a _____ depending on their clinical status
Yearly basis or less frequently
Those with Ebstein anomaly and _____ require closer follow-up.
Impaired RV function
Cyanosis
Cardiomegaly
Recurrent atrial arrhythmia,
Severe tricuspid regurgitation after tricuspid valve repair
Dysfunctional tricuspid valve bioprosthetic or mechanical valves
Patients with Ebstein anomaly with a normal jugular venous pressure is usual at physical examination even with significant tricuspid regurgitation because of the _____.
Large and compliant RA and atrialized RV
ECG findings in Ebstein anomaly:
(1) Low voltage QRS
(2) Peaked tall P waves (>2.5 mm) in leads II and VI due to right atrial enlargement or “Himalayan” p waves (>5 mm height)
(3) Prolonged PR interval
(4) Importantly a short PR interval and delta wave reflective of pre-excitation from an accessory pathway
The chest radiograph in Ebstein anomaly may show cardiomegaly, decreased pulmonary vascular markings, a small aorta and pulmonary trunk shadow, and may classically be described as showing a “____“heart.
Box shaped heart
Use of CMR in Ebstein anomaly
(1) degree of displacement and rotation of the tricuspid valve
(2) quantify tricuspid regurgitation, RV volumes and RV systolic function,LV volumes which may be small, cardiac output which may be low
(3) Qp:Qs for measurement of cardiac shunt
Natural history outcomes for 72 adults with unoperated Ebstein anomaly demonstrated decreased survival due to biventricular failure or sudden death which were predicted by:
Younger age at diagnosis
Male gender
Increased cardiothoracic ratio ≥0.65
Severity of valve displacement
Atrial arrhythmia in Ebstein anomaly increases in prevalence with age. _____is the most common.
Atrioventricular reentrant tachycardia (AVRT)
It is preferable to treat arrhythmia with _____ before surgery as following tricuspid valve surgery access to right-sided accessory pathways and the slow pathway in AV node re-entry tachycardia may be hindered
Ablation
The goal of surgery in Ebstein anomaly is to:
(1) increase pulmonary blood flow
(2) minimize tricuspid regurgitation
(3) eliminate interatrial shunting
(4) improve RV function
Surgery for Ebstein anomaly is recommended for:
(1) symptoms
(2) heart failure attributed to tricuspid valve disease
(3) exercise intolerance demonstrated by exercise testing
(4) progressive systolic dysfunction
____ is favorable for Ebstein anomaly repair.
(1) A large mobile anterior leaflet with a free leading edge and (2) sufficient septal leaflet tissue
Tricuspid valve _____________ is preferred when feasible.
Repair
Pulmonic valve stenosis severity
Mild
Moderate
Severe
Mild stenosis less than or equal to 36 mm Hg (3 msec)
Moderate - 36 to 64 mm Hg (3 to 4 msec)
Severe - greater than 64 mm Hg (4 msec)
Cardiovascular exam of patients with isolated PS will include a crescendo-decrescendo systolic ejection murmur of variable intensity and often a systolic ejection click.
This click becomes softer with _______________.
Inspiration
Give indications for intervention in PS:
This is the preferred intervention for isolated PS
Class I indication for moderate or severe valvar p nary stenosis in the presence of symptoms including heart failure, exercise intolerance, or cyanosis.
For asymptomatic patients with severe valvar pulmonary stenosis, intervention is considered reasonable, as a class IIa indication.
Primary intervention by balloon valvuloplasty is recommended, with surgical repair recommended for patients who have a contraindication to or have failed the transcatheter intervention.
______________ is the most common cyanotic congenital heart defect, comprising
1.
2.
3.
4
TOF is the most common cyanotic congenital heart defect, comprising a “tetrad” of overriding aorta, VSD, pulmonary stenosis, and right ventricular hypertrophy.
Associated anomalies include right aortic arch, present in about 25% of patients; anomalous course of the coronary arteries, the most common with a __________________________ and crosses the RVOT.
Left anterior descending artery that originates from the right coronary artery
_______________________ is a common sequela in repaired TOF patients particularly in patients who received a transannular patch repair
Pulmonary regurgitation
Pulmonary valve replacement in TOF is considered when RV end-systolic volume indexed to body surface area reaches ________ (or end-diastolic volume index reaches ______________)
ESVi 80 ml/m2
EDVi 160 ml/m2
These RV volume thresholds predict normalization of RV volume following intervention
___________________ is the most common cause of death in patients with repaired TOF.
Heart failure is the most common cause of death in patients with repaired TOF
In D-loop TGA, there is ventriculo-arterial discordance, which means
The aorta arises from the RV and the PA arises from the LV
The vast majority of patients with TGA, the atria are normally positioned and the AV connections are normal. Venous return is typically normal. The great arteries are transposed with the aortic valve anterior and rightward to the pulmonic valve (D-loop transposition). In some, the aortic valve is directly anterior to the pulmonic valve or the valves can be side-by-side.
35% of patients with TGA have this associated congenital anomaly
VSD
Type of surgical repair
The SVC and IVC are re-routed leftward toward the mitral valve and the LV via inter-atrial baffles. The pulmonary veins are re-routed anteriorly and rightward, toward the tricuspid valve and the RV.
Atrial Switch Operation
RV is the systemic ventricle, the tricuspid valve is the systemic AV valve, and the LV and mitral valve support the pulmonary circulation
Type of surgical repair
This transects the great arteries above the sinuses and anastomoses them to the contralateral root. The coronary arteries must be removed from the native aortic root and reanastomosed to the neo-aortic root,
Arterual Switch Operation
In the contemporary era the arterial switch operation is the preferred treatment for TGA
Following the arterial switch o ation, the LV supports the systemic circulation and the RV supports the pulmonary circulation
Type of surgical repair
This over-sews the native pulmonic valve and places an angled VSD patch to direct oxygenated blood from the LV, across the VSD, and to the aortic valve. A RV-to-PA conduit provides for pulmonary blood flow
Rastelli Operation
Baffle malfunction such as narrowing commonly involves the __________
____________ communication between the systemic venous pathway and the pulmonary venous pathway
- SVC (IVC obstruction is rare)
- Baffle leak
The most technically difficult aspect of the arterial switch operation is the __________________
Coronary transfer from the native aortic root to the neo-aortic root
Give long term complications post arterial switch operation
- Coronary artery stenosis - within the first year after arterial switch
- Aortic dilation and regurgitation
- Supravalvar PS
L-loop TGA, also called _____________________________
Characterized by a. _______ and b. __________________
True or False - L-loop TGA is a cyanotic heart disease
- Congenitally corrected TGA (ccTGA)
- AV discordance and ventriculoarterial discordance
- False - acyanotic
Deoxygenated blood goes from the vena cavae to the RA, through the mitral valve to the LV, and then to the PA. Oxygenated blood returns via the pulmonary veins to the LA, through the tricuspid valve to the RV, and then is ejected to the aorta
2 procedures in the double switch operation
Atrial switch and Arterial Switch
___________________ is a conotruncal anomaly in which both great arteries are completely or nearly completely aligned with the RV
Double outlet RV (DORV)
Four common physiologic variations of DORV
- VSD physiology: DORV with large subaortic VSD and no pulmonic stenosis
- TOF physiology: DORV with subaortic VSD and pulmonic stenosis
- TGA physiology: DORV with subpulmonary VSD with or without aortic obstruction
- Single ventricle physiology: DORV with mitral atresia, severely unbalanced AV canal defect, or other cause of significant ventricular hypoplasia
This is an uncommon type of congenital heart defect in which a single arterial trunk arises from the heart, giving origin to the coronary arteries, PAs, and systemic arteries, in that order
Truncus arteriosus
Thus is a rare CHD lesion in which a membrane separates the LA (sinister) or the RA (dexter) into two compartments.
Cor triatriatum
The membrane is proximal to the left atrial appendage, so that the pulmonary veins drain into the proximal chamber and the appendage and mitral valve are in the distal chamber.
This consists of a ridge of fibrous or fibromuscular tissue in the LVOT, it is often circumferential extending both onto the septal surface anteriorly and the base of the anterior leaflet of the mitral valve posteriorly. The fibrous tissue may extend onto the aortic valve leaflets contributing to concomitant aortic regurgitation
Subaortic membrane
Symptomatic patients with severe LVOT obstruction (maximum gradient ______ mm Hg, mean gradient _____ mm Hg) should be referred for repair
Max gradient > 50 mm Hg, mean gradient > 30 mm Hg
Repair is also appropriate for patients with severe obstruction and reduced exertional capacity on exercise testing.
Patients with subaortic stenosis and heart failure should also undergo repair, even with moderate gradients (maximum gradient 30 to 50 mm Hg) because the low cardiac output from heart failure may lead to reduced gradients, even if obstruction is severe.
____________________ refers to a narrowing in the ascending aorta. Morphologically it is most commonly an hourglass-shaped narrowing but can also occur from a fibrous ridge, or tubular hypoplasia of the proximal ascending aorta
_________ gene associated in this condition _____________
Supravalvar aortic stenosis (SVAS)
ELN gene, encodes for the elastin protein, on chromosome 7q11.23
Frequently causes stenosis in other proximal arteries - brachiocephalic vessels, branch pulmonary arteries, and ostial coronary arteries
Williams syndrome - SVAS affects 65% of patients with WS
WS - characteristic dysmorphic facial features (broad forehead, stellate irises, upturned nose, pointed chin; often described as “elfin” facies), hypercalcemia, hypothyroidism, diabetes mellitus, intellectual disability, and often an outgoing (“cocktail party”) personality.
This is a localized or tubular narrowing in the aorta or aortic interruption. It is characterized by a generalized arteriopathy with decreased aortic compliance.
Coarctation of the aorta
Coarctation is juxtaductal, located at the junction of the distal aortic arch and the descending aorta beyond the origin of the left subclavian artery
Associated anomalies - BAV 50-85%
Coarctation is common in certain syndromes, including Shone, Turners, Williams-Beuren, Noonan, hypoplastic left heart syndrome (HLHS), and other complex CHD
___________________ is the association with coarctation of the aorta with mitral valve disease (parachute or supramitral ring) and multilevel left-sided outflow tract obstruction (subaortic or aortic valve stenosis)
Shone Syndrome
CXR finding in CoA where there is erosion of the undersurface of the outer third of a posterior 2nd to 9th rib due to collaterals
Rib notching
_______________ refers to the silhouette of aortic dilatation both before (pre-stenotic) and after (post-stenotic) the coarctation site
Figure of 3 sign
_________ is routine in CoA diagnosis. Iy is used for quantification of LV mass, evaluating the entire aorta including for arch hypoplasia or Gothic angulation, severity of coarctation, and quantification of collateral flow. It also identifies complications post repair (e.g., aneurysms, false aneurysms, recoarctation, or residual stenosis), presence of aberrant subclavian arteries, and for the morphology and function of the aortic valve.
CMR
All adults with coarctation should undergo cross-sectional imaging (usually CMR) at least once.
Repair (endovascular or surgical) of coarctation or recoarctation is indicated in hypertensive patients with an invasive peak-to-peak gradient ________________.
> /= 20 mm Hg
Coarctation stenting is also considered when feasible for hypertensive patients with a coarctation diameter ≤50% of the aortic diameter at the diaphragm regardless of peak-to-peak gradient and in normotensive coarctation with peak-to-peak gradient ≥20 mm Hg.
Types of interrupted aortic arch
Interrupted aortic arch is associated with this syndrome ___________
Type A - Interrupted aortic arch occurs distal to the left subclavian artery
Type B - between the left carotid artery and the left subclavian artery
Type C - between the brachiocephalic trunk and the left carotid artery
DiGeorge Syndrome
This is an abnormality of aortic arch development which is defined as complete surrounding of the esophagus and trachea by vascular structures
Give 2 of the most common forms
Vascular ring
Most common forms of vascular ring include double aortic arch and right aortic arch with aberrant left subclavian artery
More common form of anomalous coronary artery from PA
Anomalous left coronary artery from the pulmonary artery (ALCAPA)
Myocardial perfusion is via the coronary artery which arises normally from the aorta and is typically quite dilated.
Diagnosis should be suspected in infants or young children with ventricular dysfunction or secondary mitral regurgitation
Types/forms of single ventricle heart disease
Tricuspid atresia
HLHS
Double inlet LV
Double inlet/outlet RV
In patients with single ventricle, this procedure is generally reached in staged palliation, with the ultimate result of all systemic venous return bypassing the heart to flow passively directly to the pulmonary circulation, while the single functional ventricle acts as the systemic pump.
Fontan procedure/palliation
Versions of Fontan circulation in adults
Extracardiac Fontan
Lateral tunnel Fontan
Direct right atrial to PA anastomosis
