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Erschienen in: Endocrine 3/2022

04.10.2022 | Original Article

Comparison of plasma metanephrines in patients with cyanotic and acyanotic congenital heart disease

verfasst von: Mojca Jensterle, Ana Podbregar, Andrej Janež, Matej Rakusa, Katja Goricar, Katja Prokšelj

Erschienen in: Endocrine | Ausgabe 3/2022

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Abstract

Purpose

The co-occurrence of cyanotic congenital heart disease (CCHD) and PHEO/PGL has been reported, but the role of the hypoxic environment in the pathogenesis of PHEO/PGL remains unclear. Our aim was to compare plasma metanephrine and normetanephrine levels between patients with CCHD and patients with acyanotic congenital heart disease (ACCHD).

Methods

We performed a cross-sectional study in a prospective cohort of 44 patients with congenital heart disease (CHD) (31 (70.5%) females) with a median age of 37.5 (31.0–55.6) years at the time of evaluation. Thirty-two (73%) patients had CCHD and 12 (27%) patients had ACCHD. Morning blood samples for plasma determination of metanephrine and normetanephrine were collected.

Results

Plasma normetanephrine levels were significantly higher in patients with CCHD compared to ACCHD (p = 0.002). Ten (31.3%) patients with CCHD had plasma normetanephrine levels elevated above the reference range, while all ACCHD patients had normal levels. Patients with lower oxygen saturation and higher proBNP had significantly higher normetanephrine levels (ρ = −0.444, p = 0.003 and ρ = 0.449, p = 0.002, respectively). No chromaffin cell tumors were detected.

Conclusion

Increased plasma normetanephrine levels in patients with CCHD can be explained by the effect of hypoxia. Future research is needed to better understand the impact of chronic hypoxia in CCHD on increased sympathetic outflow, hyperplastic response of chromaffin tissue, and the role of somatic mutations in CCHD-PHEO/PGL pathogenesis related to hypoxia.
Literatur
4.
Zurück zum Zitat G. Eisenhofer, A. Prejbisz, M. Peitzsch, C. Pamporaki, J. Masjkur et al. Biochemical Diagnosis of Chromaffin Cell Tumors in Patients at High and Low Risk of Disease: Plasma versus Urinary Free or Deconjugated O-Methylated Catecholamine Metabolites. Clin. Chem. 64(11), 646–656 (2018). https://doi.org/10.1373/clinchem.2018.291369CrossRef G. Eisenhofer, A. Prejbisz, M. Peitzsch, C. Pamporaki, J. Masjkur et al. Biochemical Diagnosis of Chromaffin Cell Tumors in Patients at High and Low Risk of Disease: Plasma versus Urinary Free or Deconjugated O-Methylated Catecholamine Metabolites. Clin. Chem. 64(11), 646–656 (2018). https://​doi.​org/​10.​1373/​clinchem.​2018.​291369CrossRef
6.
Zurück zum Zitat A.M. Sinclair, C.G. Isles, I. Brown, H. Cameron, G.D. Murray et al. Secondary hypertension in a blood pressure clinic. Arch. Intern Med. 147(7), 1289–1293 (1987)CrossRefPubMed A.M. Sinclair, C.G. Isles, I. Brown, H. Cameron, G.D. Murray et al. Secondary hypertension in a blood pressure clinic. Arch. Intern Med. 147(7), 1289–1293 (1987)CrossRefPubMed
15.
Zurück zum Zitat Ogasawara T., Fujii Y., Kakiuchi N., Shiozawa Y., Sakamoto R., et al. Genetic analysis of pheochromocytoma and paraganglioma complicating cyanotic congenital heart disease [published online ahead of print, 2022 Jun 22]. J. Clin. Endocrinol. Metab. 2022;dgac362. https://doi.org/10.1210/clinem/dgac362 Ogasawara T., Fujii Y., Kakiuchi N., Shiozawa Y., Sakamoto R., et al. Genetic analysis of pheochromocytoma and paraganglioma complicating cyanotic congenital heart disease [published online ahead of print, 2022 Jun 22]. J. Clin. Endocrinol. Metab. 2022;dgac362. https://​doi.​org/​10.​1210/​clinem/​dgac362
39.
40.
Metadaten
Titel
Comparison of plasma metanephrines in patients with cyanotic and acyanotic congenital heart disease
verfasst von
Mojca Jensterle
Ana Podbregar
Andrej Janež
Matej Rakusa
Katja Goricar
Katja Prokšelj
Publikationsdatum
04.10.2022
Verlag
Springer US
Erschienen in
Endocrine / Ausgabe 3/2022
Print ISSN: 1355-008X
Elektronische ISSN: 1559-0100
DOI
https://doi.org/10.1007/s12020-022-03205-6

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