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