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Implication of Aortic Root Dilation and Stiffening in Patients with Tetralogy of Fallot

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Abstract

Progressive aortic root dilation and stiffening have been reported in patients with tetralogy of Fallot (TOF), even after repair. However, the implications of such aortic dilation and stiffening have not been fully assessed. A stiff aorta has a negative effect on the coronary circulation. 15 patients with TOF after repair were enrolled in this study. Using a left ventriculogram and pressure waveform recorded by a pressure sensor-mounted catheter, we analyzed the diameter and distensibility of the ascending and descending aorta. We also calculated the subendocardial viability ratio (SEVR), which measures the cardiac blood supply–workload balance. These values were compared with those in one-to-one age-matched controls. Moreover, the correlation between the diameter ratio (aortic diameter of the patient/aortic diameter of the matched control subject) and the distensibility ratio (distensibility of the patient/distensibility of the matched control subject) was analyzed. The mean age of patients was 3.9 ± 1.6 years. In patients with TOF, the indexed diameter was larger (0.20 ± 0.02 vs. 0.15 ± 0.02 mm/cm, p < 0.0001) and the distensibility was lower (5.1 ± 2.2 vs. 7.7 ± 2.0 cm2 dynes−1 10−6, p = 0.0009) in the ascending aorta than in control subjects. There was no difference in the SEVR between patients and control subjects. The distensibility ratio was significantly negatively correlated with the indexed diameter ratio in the ascending aorta (r2 = 0.35, p = 0.019). Regardless of the aortic stiffening, the cardiac blood supply–workload balance was maintained. We speculate that the aortic root dilation might be a mechanism to functionally complement aortic stiffness in patients with TOF.

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Acknowledgements

We thank Ellen Knapp, PhD, from Edanz Group (http://www.edanzediting.com/ac) for editing a draft of this manuscript.

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Authors and Affiliations

  1. Department of Pediatrics, Hokkaido University Hospital, Sapporo, Japan

    Kohta Takei, Tomoaki Murakami & Atsuhito Takeda

  2. Department of Cardiology, Chiba Children’s Hospital, 579-1 Heta-cho, Midori-ku, Chiba, 266-0007, Japan

    Tomoaki Murakami

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  1. Kohta Takei
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Correspondence to Tomoaki Murakami.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study.

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Takei, K., Murakami, T. & Takeda, A. Implication of Aortic Root Dilation and Stiffening in Patients with Tetralogy of Fallot. Pediatr Cardiol 39, 1462–1467 (2018). https://doi.org/10.1007/s00246-018-1917-5

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  • DOI: https://doi.org/10.1007/s00246-018-1917-5

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