AHA Guidelines - Adults with Congenital Heart Disease 2018 Flashcards
Physiological Variables as Used in ACHD AP Classification:
Artopathy?
Mild aortic enlargement is defined as maximum diameter 3.5–3.9 cm
Moderate aortic enlargement is defined as maximum diameter 4.0–4.9 cm
Severe aortic enlargement is defined as maximum diameter ≥5.0 cm
NYHA Functional classification system?
I Patients with cardiac disease but resulting in no limitation of physical activity. Ordinary physical activity does not cause undue fatigue, palpitation, dyspnea, or anginal pain.
II Patients with cardiac disease resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity results in fatigue, palpitation, dyspnea, or anginal pain.
III Patients with cardiac disease resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes fatigue, palpitation, dyspnea, or anginal pain.
IV Patients with cardiac disease resulting in inability to carry on any physical activity without discomfort. Symptoms of HF or the anginal syndrome may be present even at rest. If any physical activity is undertaken, discomfort increases.
Define hypoxia, severe hypoxia, and discuss when patients may have visible cyanosis?
Hypoxemia is defined as oxygen saturation measured by pulse oximetry at rest ≤90%.
Severe hypoxemia is defined as oxygen saturation at rest <85%.
In patients with normal or high hemoglobin concentrations, severe hypoxemia will be associated with visible cyanosis (which requires ≥5 g/L desaturated hemoglobin to be appreciated).
Define pulmonary hypertension and pulmonary arterial hypertension. Are they the same?
Pulm HTN is a broad term that encompasses PAH, which is Pulm HTN with increased PVR.
Pulm HTN: Mean PA pressure by RHC ≥25 mm Hg.
PAH:
- Mean PA by RHC ≥25 mm Hg AND…
- PCWP ≤15 mm Hg
- PVR ≥3 Wood units
What defines a hemodynamic intracardiac shunt?
An intracardiac shunt is hemodynamically significant if:
There is evidence of chamber enlargement distal to the shunt
And/or evidence of sustained Qp:Qs ≥1.5:1
An intracardiac shunt not meeting these criteria would be described as small or trivial
Which ACHD patients are considered “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 (ie, crisscross heart, isomerism, heterotaxy syndromes, ventricular inversion)
Which ACHD are considered Phsiologic Stage D?
NYHA FC IV symptoms
Severe aortic enlargement
Arrhythmias refractory to treatment
Severe hypoxemia (often assd w/ cyanosis)
Severe pulmonary hypertension
Eisenmenger syndrome
Refractory end-organ dysfunction
Use of ECGs in ACHD Evaluation: What specific disease processes are you looking for and why?
Identification of sinus bradycardia or junctional rhythm in patients at risk of sinus node dysfunction (especially after the Mustard, Senning, Glenn, or Fontan procedure)
Identification of clinically inapparent intra-atrial re-entry tachycardia in patients who have had atriotomy
Identification of atrioventricular block in patients at risk for progression of atrioventricular conduction system disease (especially CCTGA)
Evaluation of rhythm in patients with pacemakers
Measurement of QRS duration in patients after repair of TOF and as part of CRT evaluation
Preoperatively to compare with postoperative ECGs in patients undergoing heart surgery and noncardiac surgery
Postoperatively to identify arrhythmias (eg, atrial ectopic tachycardia, atrial flutter, AF, junctional ectopic tachycardia, atrioventricular block)
Diagnosis of Wolff-Parkinson-White Syndrome in patients with Ebstein anomaly
Initial evaluation of suspected acute coronary syndromes
ACHD Circumstances Where Cardiac MRI, Cardiac CT, TEE, and/or Cardiac Catheterization May be Superior to TTE?
Assessment of RV size and function in repaired TOF, systemic right ventricles, and other conditions associated with RV volume and pressure overload.
Identification of anomalous pulmonary venous connections.
Serial assessment of thoracic aortic aneurysms, especially when the dilation might extend beyond the echocardiographic windows.
Accurate assessment of PA pressure and pulmonary vascular resistance.
Assessment for recoarctation of the aorta.
Sinus venosus defects.
Vascular rings.
Evaluation of coronary anomalies.
Quantification of valvular regurgitation.
In ACHD patients, when would ambulatory electrocardiographic monitoring be performed?
In CHD patients at risk for tachyarrhythmia, bradyarrhythmia, or heart block, or when symptoms possibly of arrhythmic origin develop.
Does every adult undergoing surgery for CHD require intraop TEE?
COR1 recommendation states intraop TEE is recommended to guide surgical repair of CHD in adults.
- A large retrospective study has shown that the routine use of intraoperative TEE has a substantial impact on patient care, leading to alteration of planned procedure or revision of the initial repair in 14% of cases and was also determined to be cost-effective.
TTE preop for initial assessment, with further or repeat studies as necessary.
What specific ACHD populations benefit from Cardiac MRI?
ACHD pts who have or are at risk of developing RV enlargement and dysfunction. Serial CMR is recommended for quantitative assessment of RV size and function.
Should patients with ACHD be evaluated for depression and anxiety? What level of recommendation?
Yes. COR 1.
Patients with conotruncal cardiac defects can have what genetic association?
22q11.2 deletion. DiGeorge Syndrome.
Genetic testing is reasonable. COR IIa.
