Mid‐aortic syndrome: a rare cause of heart failure in infants

Zhao, Lu; Zhu, Li; Zhao, Qiyi; Liang, Xue‐cun; Wang, Lai-Shuan; Lin, Wenxiong

doi:10.1002/ehf2.14066

Cited by 3 publications

(2 citation statements)

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“…They also observed a higher prevalence of LV dysfunction in infants, although the difference was not statistically signi cant (p = 0.054). Furthermore, in previous reports, cases of LV dysfunction in young patients have been frequently documented, often with a favorable prognosis [5,13,14]. Similarly, in our study, most patients with LV dysfunction were under the age of 5, and their cardiac function improved rapidly.…”

Section: Discussionsupporting

confidence: 84%

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Computed Tomography Measurement of the Aorta in Midaortic Syndrome in Children and Adolescents and Their Clinical Manifestations and Outcomes

Baek,

Lee,

Song

et al. 2024

Pediatr Cardiol

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BackgroundMidaortic syndrome (MAS) presents challenges in diagnosis due to the absence of well-de ned diagnostic criteria, and its clinical features remain uncertain. In this retrospective study, we aimed to aid in the diagnosis of MAS by employing computed tomography (CT) to measure the z-score of the aorta as well as to identify and understand its clinical features. MethodsCT images, echocardiography ndings, and medical records of 17 patients diagnosed with MAS between 1997 and 2023 were reviewed, and z-scores were calculated. Aortic size on follow-up CT, blood pressure, and left ventricular function at the last follow-up were analyzed, and possible prognostic factors were examined. ResultsWith the exception of one patient, all individuals exhibited a z-score below − 2 at the level corresponding to stenosis. Left ventricular dysfunction occurred more frequently in patients aged < 5 years (p = 0.024). Patients with idiopathic MAS showed a better prognosis in terms of blood pressure and follow-up aortic size (p = 0.051 and 0.048, respectively). ConclusionsCT-measured aortic z-scores may be useful for the diagnosis and follow-up of MAS. A more conservative management plan should be considered for selective idiopathic patients.

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Section: Discussionsupporting

confidence: 84%

“…LV dysfunction due to MAS may be misdiagnosed as primary dilated cardiomyopathy [13]. Therefore, particularly in young patients with severe LV dysfunction and relatively normal or elevated blood pressure compared to LV function, MAS should be considered as a potential underlying cause of hypertension.…”

Section: Discussionmentioning

confidence: 99%

Computed Tomography Measurement of the Aorta in Midaortic Syndrome in Children and Adolescents and Their Clinical Manifestations and Outcomes

Baek,

Lee,

Song

et al. 2024

Pediatr Cardiol

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Mid‐aortic syndrome: a rare cause of heart failure in infants

et al. 2022

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This case reports describe a rare disease, mid‐aortic syndrome (MAS), that can cause severe heart failure and hypertension in infancy. The typical images, key points of diagnosis, and therapy methods of the disease have also been presented. We report two critical thoracoabdominal aortic coarctation cases in infants aged 2 and 11 months with severe heart failure. The patients were initially misdiagnosed as dilated myocardiopathy, with the correct diagnosis confirmed through imaging. Both patients underwent balloon angioplasty; one patient also had bare‐metal stents implanted. The patient treated with balloon angioplasty alone died after the procedure, whereas the other patient recovered well. In conclusion, careful physical examinations, especially upper and lower extremity blood pressure differences and palpation of upper and lower limb pulses, are critical in unexplained infant heart failure cases. Stent implantation may be a safer and more effective treatment than simple balloon angioplasty in infants with MAS.

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Computed Tomography Measurement of the Aorta in Midaortic Syndrome in Children and Adolescents and Their Clinical Manifestations and Outcomes

Baek

Lee

Song

et al. 2023

Preprint

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Background Midaortic syndrome (MAS) presents challenges in diagnosis due to the absence of well-defined diagnostic criteria, and its clinical features remain uncertain. In this retrospective study, we aimed to aid in the diagnosis of MAS by employing computed tomography (CT) to measure the z-score of the aorta as well as to identify and understand its clinical features. Methods CT images, echocardiography findings, and medical records of 17 patients diagnosed with MAS between 1997 and 2023 were reviewed, and z-scores were calculated. Aortic size on follow-up CT, blood pressure, and left ventricular function at the last follow-up were analyzed, and possible prognostic factors were examined. Results With the exception of one patient, all individuals exhibited a z-score below − 2 at the level corresponding to stenosis. Left ventricular dysfunction occurred more frequently in patients aged < 5 years (p = 0.024). Patients with idiopathic MAS showed a better prognosis in terms of blood pressure and follow-up aortic size (p = 0.051 and 0.048, respectively). Conclusions CT-measured aortic z-scores may be useful for the diagnosis and follow-up of MAS. A more conservative management plan should be considered for selective idiopathic patients.

show abstract

Mid‐aortic syndrome: a rare cause of heart failure in infants

Cited by 3 publications

References 9 publications

Computed Tomography Measurement of the Aorta in Midaortic Syndrome in Children and Adolescents and Their Clinical Manifestations and Outcomes

Computed Tomography Measurement of the Aorta in Midaortic Syndrome in Children and Adolescents and Their Clinical Manifestations and Outcomes

Mid‐aortic syndrome: a rare cause of heart failure in infants

Computed Tomography Measurement of the Aorta in Midaortic Syndrome in Children and Adolescents and Their Clinical Manifestations and Outcomes

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