The following are key points to remember from a scientific statement on cardiovascular management of aortopathy in children:

1. Clinical evaluation for suspected aortopathy in a child should include a detailed history, physical examination, and family history of ≥3 generations. Normal physical examination results or negative family history does not rule out a genetic aortopathy.
2. Z-scores adjusted for body surface area allow for appropriate interpretation of aortic size and growing children. The authors propose a new grading system. For children <16 years old, a Z-score <2 cm is normal, mild aortic dilatation is ≥2 and <3.5, moderate dilatation is ≥3.5 and <5, and severe dilation is ≥5. For individuals ≥16 years old, a maximum dimension of <3.5 is normal, mild aortic dilatation is ≥3.5 and <4, moderate dilatation is ≥4 and <4.5, and severe dilation is ≥4.5 cm.
3. Magnetic resonance imaging or computed tomography angiography should use double-oblique techniques, measuring maximum sinus-to-sinus dimensions in systole, inner edge-to-inner edge.
4. Genetic testing impacts aspects of clinical management. Genetic testing should be considered when there is a reasonable likelihood of hereditary thoracic aortic disease (HTAD), or when a specific genetic diagnosis might affect management.
5. Medications that cause vasoconstriction, hypertension, or tachycardia should be avoided in patients with HTAD. These would include decongestants, and triptans for migraine headaches. Fluoroquinolone antibiotics should be avoided. Stimulant medications for attention deficit disorder may be used with close monitoring of heart rate and blood pressure.
6. Both angiotensin receptor blockers and beta-blockers have been shown to be safe and effective in slowing the rate of aortic growth. Medical therapy may be of greatest benefit when initiated at younger ages.
7. Age-appropriate, healthy exercise practices should be encouraged for all children with aortopathy starting in early childhood. Very young children require no restrictions.
8. Routine ongoing counseling regarding exercise should involve shared decision-making, and should consider the underlying diagnosis and cardiovascular size along with the proposed activity type and level of effort.
9. Timing of elective aortic surgery is based on one’s dimensions, genetic diagnosis, and presence of high-risk factors. The document includes specific intervention dimensions based on these factors. Surgical management should be directed by a specialized multi-disciplinary aortic center.
10. Genetic testing should be performed in children suspected to have Marfan syndrome. Variants in FBN1 are found in >95% of children with Marfan syndrome.
11. Clinical presentation of Loeys-Dietz syndrome is highly variable in children. Transthoracic echocardiogram and cross-sectional imaging from head to pelvis should be performed at baseline and during surveillance. The surgical threshold for aortic dimensions tends to be lower in Loeys-Dietz syndrome than for Marfan syndrome.
12. Clinical presentation of vascular Ehlers-Danlos syndrome is highly variable in children. A pathogenic variant or likely pathogenic variant in COL3A1 is necessary for diagnosis. Normal imaging results do not rule out the risk of an arterial event.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Vascular Medicine, Congenital Heart Disease

Keywords: Aortic Diseases, Genetic Testing, Heart Defects, Congenital

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