Associated conditions | ACC/AHA 20102 | ESC 20141 | ASE/EACVI 20153 | Our recommendations |
---|---|---|---|---|
None | No specific recommendations except needing surveillance | No specific recommendations except needing surveillance | Every 1–3 years based on risk after diagnosis | TTE and CTA or MRA at baseline and 6 months; If TAA < 5.0 cm and stable, then yearly If TAA ≥ 5.0 cm or growing > 0.5 cm/year, then every 6 months and refer to surgeon |
Marfan syndrome | Measure dimensions and maximum cross-sectional area divided by height TTE at baseline and 6 months, CTA or MRA at baseline to check TTE If stable and < 4.5 cm, then yearly after, if not then more frequently | TTE and MRA or CTA If no TAA at baseline, TTE yearly, MRA or CTA every 5 years If any aneurysm is above root, MRA or CTA yearly Refer to 2010 ESC adult congenital disease guidelines35 | Dimensions with normative values based on age, body surface area, and Z scores TTE and CTA or MRA If no TAA at baseline, then every 2–3 years First TAA diagnosis: 6 months then yearly if stable, < 4.5 cm and no dissection history; otherwise every 6 months Postoperatively: 6 months, then yearly if stable CTA or MRA at least every 3 years if using TTE | TTE and CTA or MRA at baseline and 6 months If no TAA, then TTE yearly and CTA or MRA every 2 years First TAA diagnosis: TTE and CTA or MRA yearly if stable (< 0.3 cm/year) and < 4.5 cm, other-wise every 6 months and refer to surgeon |
Bicuspid aortic valve | No specific recommendations after initial imaging TTE and CTA or MRA | TTE and CTA or MRA If no TAA at baseline, repeat TTE yearly If TAA > 4.5 cm or growing at > 3 mm/year, then do CTA or MRA to confirm at same time, then yearly | TTE and CTA or MRA If no at baseline, repeat every 3–5 years First TAA diagnosis: 6 months then yearly if stable, < 4.5 cm and no dissection history; otherwise every 6 months Postoperatively: yearly but individualize | TTE + CTA or MRA at baseline and 6 months No TAA: TTE yearly and CTA or MRA every 2 years First TAA diagnosis: TTE and CTA or MRA yearly if stable (< 0.3 cm/year) and < 4.5 cm, other-wise every 6 months and refer to surgeon |
Turner syndrome | Baseline TTE and CTA or MRA If no TAA or dissection risk factors, repeat every 5–10 years | If no TAA: TTE every 3–5 years for low risk, MRA every 3–5 years for moderate risk, and MRI every 1–2 years for high risk | Index dimensions by body surface area; if indexed diameter > 2 cm/m2, repeat yearly | TTE + CTA or MRA at baseline and 6 months Index dimensions by body surface area No TAA: TTE yearly and CTA or MRA every 2 years Indexed diameter > 2 cm/m2: yearly MRA or CTA and refer to surgeon |
Familial TAA | No specific recommendations after initial imaging TTE and CTA or MRA | No specific recommendations after initial imaging TTE and CTA or MRA | Follow plan for Marfan syndrome, but individualize | TTE + CTA or MRA at baseline and 6 months Follow plan for Marfan syndrome but individualize risk |
Loeys-Dietz syndrome | Baseline and 6 months TTE and CTA or MRA, then yearly if stable Whole-body MRA | No specific recommendations after initial imaging TTE and CTA or MRA | Every 1–3 years depending on risk, every 6 months if progression | TTE + CTA or MRA at baseline and 6 months Yearly if low risk, < 4.0 cm and stable (< 0.3 cm/year), otherwise every 6 months and refer to surgeon |
Ehlers-Danlos syndrome | No specific recommendations | No specific recommendations, individualize | No specific recommendations | TTE + CTA or MRA at baseline and 6 months No specific recommendation for surveillance |
ACC = American College of Cardiology; AHA = American Heart Association; ASE = American Society of Echocardiography;
CTA = computed tomography angiography; EACVI = European Association of Cardiovascular Imaging; ESC = European Society of Cardiology;
MRA = magnetic resonance angiography; MRI = magnetic resonance imaging; TAA = thoracic aortic aneurysm; TTE = transthoracic echocardiography