Aortopathy

• MM 2024 (Marfan): Lopez 2017 (PHN/Boston) Z may be considered the method of choice; if leading edge in diastole is used, use the Campens nomograms; Haycock BSA favoured in young children; same method at every follow-up.
• Z may be wrong at extremes of height and weight (AHA 2024). ACC/AHA 2022: several BSA formulas and Z calculators that perform differently; Z not typically used in adults. ESC 2024 (adults): index to BSA with nomograms, Z scores or other methods, especially for small body size (IIa, B); in extreme BSA and age, use Z scores (text); same modality and method for serial studies (I, C).
• ACC/AHA 2022 adult cut-offs assume height and BSA within 1–2 SD of the mean.
• Adult ESC 2024 (text): root dilation suspected at >40 mm (men), >36 mm (women) or ASI >22 mm/m²; AHI >32.1 mm/m linked to 12% yearly aortic events.
• Sports (AHA/ACC 2025): Z ≥3 prompts HTAD work-up; >42 mm (male) or >40 mm (female) is rare in young athletes with a tricuspid valve and should prompt work-up.

• Early-onset Imaging every 1–3 months, may extend to every 6 months if stable (AHA 2024 Table 2).
• Child TTE at diagnosis (root, ascending, mitral valve); annual TTE is adequate for most (AHA 2024). MM 2024: yearly; “biannual” (term not defined) if root ≥45 mm or growth >5 mm/yr with Z increase >1 SD. Intervals by dilation grade: AHA 2024 Table 2 (colour-coded; not reproduced).
• Adolescent MRI preferred, once in adolescence when no sedation is needed; neck to pelvis at the first study (MM 2024). Sedation is typically needed below 9 y (AHA 2024).
• Adult ACC/AHA 2022: TTE at diagnosis and 6 months later, then annually if stable (I, C-EO); CT or MRI after the first TTE (IIa, C-EO). After root replacement: MRI or CT annually, every other year if unchanged after 2 years (I, C-LD). Distal arch, descending or abdominal aneurysm ≥5.0 cm: surgery reasonable (IIa, C-EO). Near threshold, valve-sparing candidate, very low surgical risk: may be reasonable (IIb, C-LD). ESC 2024: TTE at least yearly if root <45 mm without risk factors, at least every 6 months if <45 mm with risk factors (as defined in its footnote, whose wording is ambiguous: see Sources), at least every 6–12 months if ≥45 mm without (I, C). No previous aortic surgery: complete imaging of the thoracoabdominal aorta and peripheral vessels by CMR or CT and duplex ultrasound at the first evaluation, then every 3–5 years if stable (I, C). After root replacement: CMR or CT of the thoracic aorta at least every 3 years (I, C). Ascending, arch, descending or abdominal aneurysm ≥50 mm: replacement by an expert surgeon should be considered (IIa, C).

• Child MM 2024: beta-blocker or ARB at maximally tolerated dose with dilation, started at Z ≥2 (text); may treat without dilation if risk factors; combined therapy if growth 5 mm/yr, diameter >40 mm or Z >5 (table; text says ≥40 mm or Z ≥5); ACE inhibitor if both contraindicated; avoid calcium-channel blockers.
• AHA 2024: start even in the youngest with dilation; optimise the first drug before adding a second; avoid fluoroquinolones, decongestants, excess caffeine and triptans unless no alternative; ADHD stimulants not restricted for most (monitor heart rate and BP). Medication level by grade: AHA 2024 Table 3 (not reproduced).
• Adult ACC/AHA 2022: beta-blocker or ARB at maximally tolerated doses (I, A); both (IIa, C-LD). ESC 2024: either (I, A); both (IIa, A); caution with calcium-channel blockers (text). Aortic aneurysm: fluoroquinolones generally discouraged; may be considered with a compelling indication and no reasonable alternative (IIb, B).
• PHN trial, 608 patients 6 months to 25 y with root Z >3.0: atenolol vs losartan Z slope −0.139 vs −0.107 SD/y (P=0.08); no difference in surgery, dissection or death at 3 y abstract.

• Child TTE and head-to-pelvis MRA or CTA at diagnosis (may be delayed in milder TGFB3, SMAD2, SMAD3); CTA often used in young children to avoid sedation. Intervals: AHA 2024 Table 2 (not reproduced).
• AHA 2024: an ARB, a beta-blocker or both is reasonable; with moderate to severe dilation, high-risk features or a severe family history, start at diagnosis, even in infancy. Dissection has been reported as young as 3 months (MacCarrick 2014).
• Adult ACC/AHA 2022: TTE at baseline, 6 months, then annually if stable (I, C-EO); annual MRI or CT of dilated or dissected segments (I, C-EO); baseline head-to-pelvis MRI or CT (I, C-LD); chest-to-pelvis every 2 years if no distal disease (IIa, C-EO); cerebral imaging every 2–3 years if first normal (IIa, C-EO). Beta-blocker, ARB or both (IIa, C-EO). TGFBR1, TGFBR2 or SMAD3 with arch, descending or abdominal aorta ≥4.5 cm: surgery may be considered (IIb, C-EO). ESC 2024: TTE at baseline, then every 6–12 months by diameter and growth (I, C); head-to-pelvis CMR or CT at baseline, then CMR, CT or duplex every 1–3 years (I, C).
• Genes: ACC/AHA 2022 lists 5; AHA 2024 and ESC 2024 add SMAD2. TGFB2 is not TGFBR2.

• High risk (AHA 2024 Table 1): glycine substitutions or splice variants (glycine to valine lower risk); height-adjusted vertebral tortuosity index ≥15.5; frequent haematomas or stitches; preterm delivery history; prior major arterial or organ event in patient or family.
• Child Head-to-pelvis cross-sectional imaging, may wait until after age 3 years to avoid sedation; normal imaging does not exclude events (AHA 2024). A third-generation beta-blocker or an ARB is reasonable, a beta-blocker even without dilation; celiprolol is not available in the US.
• ESC 2024 (written for adults): celiprolol should be considered (IIa, B); no consensus about the age to start it, after 10 years of age is considered reasonable by many experts (text); regular surveillance of the aorta and peripheral arteries by duplex, CT or CMR (I, C), annual in the text. Celiprolol trial: 53 patients, events 20% vs 50%, HR 0.36 (95% CI 0.15–0.88), up to 400 mg/day abstract.
• Adult ACC/AHA 2022 text: baseline head-to-pelvis MRI or CT, annual imaging of dilated or dissected segments, every 2 years when normal. Pregnancy: maternal mortality 4%–25%.

• ACC/AHA 2022 (all ages): multigenerational family history, genetic testing with HTAD risk factors, cascade testing and TTE of variant carriers (all I, B-NR); imaging of first-degree relatives even without family history (I, C-LD). If negative, repeat in 5 years (younger) or 10 years (older) relatives.
• AHA 2024: child of a gene-negative parent with an aneurysm and normal imaging: repeat in 5–10 years. ACTA2 and PRKG1: medical therapy, preferably beta-blockers, from the early teens even without enlargement. PRKG1 dissection can occur in adolescence with little or no dilation.
• Unexplained dilation in children: manage as classic Marfan if family data are unavailable (AHA 2024).
• ESC 2024 (written for adults; ages as printed), root or ascending aneurysm or thoracic dissection: family history over at least three generations (I, B); with HTAD risk factors, genetic counselling at an expert centre and testing if indicated (I, B). Pathogenic or likely pathogenic variant: cascade testing of relatives irrespective of age (I, C); management guided by the gene when known (IIa, B). HTAD risk factors and no variant found: TTE screening of first-degree relatives when the family history of TAD is negative (I, B); imaging of family members from age 25, or 10 years below the youngest case, whichever is younger, then every 5 years to 60 if normal (IIa, C). Additional risk features (Figure 39 box): syndromic features of Marfan, LDS or vEDS; family history of TAD, peripheral or intracranial aneurysm, or unexplained sudden death <60 years. Care in an experienced centre; individualised management with shared decisions (both I, C).

• Child Root dilation (Z >2) in about 20%, ascending about 50%; no reported dissection in children with isolated BAV. AHA 2024 authors suggest medical therapy at aortic Z ≥4 is reasonable, on limited data (one retrospective study and a survey; Table 3 footnote). Image all first-degree relatives.
• Adult ACC/AHA 2022: TTE for valve, aorta and coarctation (I, B-NR); ≥4.0 cm: lifelong surveillance (I); dilated aorta: TTE of all first-degree relatives (I, C-LD); screening relatives of any BAV patient (IIa, B-NR). ACC/AHA 2025 ACHD: aorta >4 cm or risk factors: lifelong surveillance (I, B-NR); first-degree relatives: one-time echo (IIa, B-NR); cross-sectional imaging within 0.1–0.2 cm of the threshold. ESC 2024: TTE at diagnosis (I, B); CT or CMR of the thoracic aorta at diagnosis, when TTE measurements disagree or above 45 mm (I, C); above 40 mm, TTE after 1 year, then every 2–3 years if stable (I, C); TTE screening of first-degree relatives of patients with a root phenotype or isolated aortic regurgitation (I, C), and of all BAV patients (IIa, B).

• Adult After arterial switch: echo and CMR periodically (I, B-NR); CMR every 3–5 years if neoaorta <5 cm, every 1–2 years if >5 cm; no reported dissection at any diameter. Truncus: intervene only with rapid progression or valve disease (text).
• Non-HTAD causes of Z ≥2 (AHA 2024): congenital heart block, renal disease or hypertension, truncus, d-TGA, TOF, PA-VSD, Fontan, very low weight for height or age, measurement error.

Child Pregnancy is a high-risk feature in children (AHA 2024 Table 1). ACC/AHA 2022 rows are written for patients of any age; ESC 2024 rows for adults (Turner: women ≥15 y).

• ACC/AHA 2022, all aortopathies: genetic counselling and imaging before pregnancy (I, C-LD); beta-blocker in pregnancy and postpartum for syndromic and nonsyndromic HTAD (I, C-EO); TTE each trimester and several weeks postpartum (I, C-LD); ARBs and ACE inhibitors contraindicated.
• Adult ESC 2024, Marfan: pre-conception evaluation and follow-up with a pregnancy heart and vessel team; whole-aorta CMR or CT before pregnancy; follow-up frequency in pregnancy set by diameter and growth; beta-blockers during pregnancy (all I, C). Pre-conception genetic counselling when a partner has or is at risk of HTAD (I, C). ARBs not recommended in pregnancy (III, B).
• Delivery (ACC/AHA 2022): diameter <4.0 cm vaginal (I, C-EO); ≥4.5 cm cesarean reasonable (IIa, C-EO); 4.0–4.5 cm vaginal with regional anaesthesia and expedited second stage (IIb, C-EO).
• vEDS: maternal mortality 4%–25% (text); most deliver by cesarean. The 2025 ESC pregnancy guideline is not covered on this page.

• 0–5 y: encourage play (≥1–3 h/day), full PE and recreational sport. 5–10 y: moderate activity 30–60 min/day, 5 days/week. From 5 y: competitive sport may be considered with regular risk/benefit discussions; avoid contact sports (tackle football, hockey, wrestling) and high-intensity training, leagues and competitions.
• 10–18 y: peak-exertion PE activities discouraged; recreational sport with contact sports avoided; avoid strenuous training, heavy weightlifting with Valsalva and exertion to exhaustion. 14–18 y: lower-intensity varsity or club sport; not highly select travel teams. Adult rules over-restrict youth. High-risk features: more aggressive exercise modification (Table 4 footnote).
• Condition notes: Marfan lens and joints; LDS atlantoaxial instability; vEDS avoid trampolines; BAV moderate to severe valve disease.
• Marfan (MM 2024): ≤10 y any recreational sport; >10 y no power sports; >10 y and Z <3: skill, mixed or endurance sport at recreational level, any intensity; Z ≥3 or ≥40 mm: moderate intensity (able to hold a conversation).

• HTAD, normal aorta: participation can be considered with shared decision-making, but for high-intensity strength sports risks likely outweigh benefits; with dilation: risks likely outweigh benefits; at surgical threshold or after dissection: should not participate.
• BAV: 40–44 mm without risk factors: can be considered with shared decision-making; ≥45 mm: risks likely outweigh benefits (select cases with shared decision-making). These are the statement's terms, not classes.
• ACC/AHA 2022: avoid intense isometric exercise, burst exertion and collision sports (I, C-EO); 30–60 min mild-to-moderate aerobic activity ≥3–4 days/week when BP is controlled (IIa, C-LD).
• Adult ESC 2024: exercise and sport should be considered after risk stratification by aneurysm extent, dissection risk and BP control (IIa, C). Marfan: individualise by diameter, family history of dissection and fitness (I, C); regular moderate aerobic exercise, intensity set by aortic diameter (I, C); cardiac rehabilitation after dissection or aortic surgery (IIa, B); competitive sport contraindicated (text).

• AHA 2024 (pediatric statement, consensus, no class): yes, Table 5 by gene and age; Z for surveillance and medical therapy, cm for surgery.
• MM 2024 (European pediatric Marfan consensus, % agreement): yes, Marfan surgery 50 mm, 45 mm with risk factors.
• ACC/AHA 2022: no pediatric thresholds; Z “routinely used” in children. Turner <15 y: Turner Z named, no number.
• ESC 2024: no; recommendations are intended for adults unless stated. Ages it does state: cascade testing irrespective of age; family imaging from 25 y or 10 y below the youngest case, whichever is younger; Turner from 15 y; celiprolol after 10 y (text).
• AHA/ACC 2025 sports: absolute mm, deliberately not Z; no separate pediatric numbers. ACC/AHA 2025 ACHD: adults only.

• Marfan, adult: ACC/AHA 2022 ≥5.0 cm (I), ≥4.5 cm with risk features (IIa); ESC 2024 sinus ≥50 mm (I, B), ≥45 mm with risk factors (IIa, C); AHA 2024 “5.0” (no sign); MM 2024 50 mm. Beta-blocker plus ARB: IIa in both (ACC/AHA level C-LD, ESC level A).
• LDS, adult: ESC 2024: root >45 mm, no gene specified (IIa, C); threshold may be adjusted to gene and high-risk features (IIb, C); its text gives ≥40 mm with high-risk features. ACC/AHA 2022: by gene, TGFBR1/2 ≥4.5 cm (I). AHA 2024 text says “generally >4.0–4.5 cm” (TGFB3 >5.0) where its Table 5 prints ≥.
• BAV, adult: ESC 2024 ≥55 mm (I, B) and root phenotype ≥50 mm (I, B); ascending phenotype at low surgical risk >52 mm (IIa, B), ≥50 mm with a risk factor (IIa, C); area/height 13 cm²/m for the ascending phenotype (text) vs ≥10 cm²/m (ACC/AHA 2022, IIa).
• Turner, ≥15 y: ESC 2024 ASI >23 mm/m² with risk factors or planning pregnancy (IIa, C), >25 mm/m² without (IIb, C), and adds AHI and Z; its text recommends surgery before pregnancy at ASI 25 mm/m². ACC/AHA 2022: ASI ≥2.5 cm/m² (IIa with risk factors, IIb without).
• Sporadic dilation, adult: ACC/AHA 2022 ≥5.5 cm (I), ≥5.0 cm at an aortic team (IIa); ESC 2024 (tricuspid valve) ≥55 mm (I, B), tubular ascending >52 mm at low risk (IIa, B), ≥50 mm at low risk with a risk factor (IIb, B).
• Rapid growth: see the table above; MM 2024 needs a Z increase as well.
• Early-onset Marfan FBN1 exons: AHA 2024 23–32, MM 2024 24–32.
• Annulus for adult-size grafts: AHA 2024 about 20 mm (valve-sparing limited below about 19 mm); MM 2024 ≥22 mm.
• Adult BP target: ACC/AHA 2022 ≤130/≤80 (systolic <120 if tolerated); ESC 2024 systolic towards 120–129 mmHg if tolerated (I, A; arterial or aortic disease with hypertension), while its aneurysm text says <140/90, 120/80 if tolerated.