B P9 C82 CHD in the Adolescent and Adult Flashcards
(170 cards)
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