Top Things to Know: Cardiovascular Management of Aortopathy in Children
Published: August 12, 2024
Prepared by Barbara Entl, MD
- Aortopathy encompasses a range of conditions predisposing to aortic and other blood vessel dilation, aneurysm, dissection, or rupture. Pediatric cases, primarily affecting the thoracic aorta, often result from factors like bicuspid aortic valve (BAV) and connective tissue disorders.
- Pediatric aortopathy management faces challenges due to diverse causes and limited evidence, with existing guidelines mainly tailored for adults. This scientific statement introduces a flexible framework accommodating pediatric-specific needs.
- Diagnosing aortopathy in children requires a thorough evaluation, considering both syndromic and subtle manifestations. A comprehensive assessment encompassing various bodily systems is crucial, emphasizing craniofacial features and cardiovascular, ocular, skeletal, integumentary, and neurologic examinations.
- Guidance on imaging frequency and suggested measures for children with aortopathy is also provided. While transthoracic echocardiography (TTE) remains primary for diagnosis and monitoring, transesophageal echocardiography (TEE) is preferred for suspected abscesses and an alternative for dissection when computed tomography angiography (CTA) is inconclusive.
- In emergent situations, such as aortic dissection evaluation, CTA is the optimal modality, particularly when pacemakers or other implanted devices impede magnetic resonance imaging.
- A z-score >2 standard deviations (SD) may indicate aortic dilation, but accurate confirmation is crucial due to various influencing factors. These include height, weight, population norms, and measurement methods.
- The vertebral artery and aortic tortuosity indexes are prognostic indicators, correlating higher values with increased disease severity and complication risks.
- Genetic diagnosis significantly influences clinical management. Testing is advised for suspected heritable thoracic aortic disease or when a specific genetic diagnosis could inform treatment decisions.
- Individualized pharmacologic therapy tailored to each patient's characteristics, diagnosis, and risk profile is crucial. Early initiation delays aortic growth, potentially reducing the need for surgery and mitigating the risk of aortic dissection.
- Prior to participating in sports and activities, it is imperative clinicians conduct a thorough evaluation for pediatric aortopathy, encompassing cardiovascular and non-cardiovascular screening, along with genetic and subspecialty consultations as warranted.
Citation
Morris SA, Flyer JN, Yetman AT, Quezada E, Cappella ES, Dietz HC, Milewicz DM, Ouzounian M, Rigelsky CM, Tierney S, Lacro RV; on behalf of the American Heart Association Council on Cardiovascular and Stroke Nursing; Council on Peripheral Vascular Disease; Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation; and Council on Cardiovascular Surgery and Anesthesia. Cardiovascular management of aortopathyin children: a scientific statement from the American Heart Association. Circulation. Published online August 12, 2024. doi: 10.1161/CIR.0000000000001265