Scholarly article on topic 'Comprehensive characterization of cardiac morphology and function in adult patients with phenylketonuria using CMR'

Comprehensive characterization of cardiac morphology and function in adult patients with phenylketonuria using CMR Academic research paper on "Medical engineering"

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Similar topics of scientific paper in Medical engineering , author of scholarly article — Jan-Hendrik Hassel, Nikolaus Tilling, Lenka Bosanska, Bernhard Schnackenburg, Daniel Messroghli, et al.

Academic research paper on topic "Comprehensive characterization of cardiac morphology and function in adult patients with phenylketonuria using CMR"

Hassel et al. Journal of Cardiovascular Magnetic Resonance 2014, 16(Suppl 1):P246 http://www.jcmr-online.com/content/16/S1/P246

Journal of Cardiovascular Magnetic Resonance

POSTER PRESENTATION

Open Access

Comprehensive characterization of cardiac morphology and function in adult patients with phenylketonuria using CMR

Jan-Hendrik Hassel1, Nikolaus Tilling2, Lenka Bosanska2, Bernhard Schnackenburg3, Daniel Messroghli1,

11 11 O 1 ^

Alexander Berger , Rolf Gebker, Christopher Schneeweis , Eckart Fleck , Ursula Plöckinger, Sebastian Kelle

From 17th Annual SCMR Scientific Sessions New Orleans, LA, USA. 16-19 January 2014

Background

Phenylketonuria (PKU) is one of the most common inherited metabolic disorders. The molecular pathway of neurological damage is not yet sufficiently understood. To date, there is a lack information about cardiac involvement related to the disease. This study aims to characterize cardiac morphology and function in adult patients with PKU using cardiovascular magnetic resonance (CMR).

Methods

28 patients with PKU (age 30 ± 9 years/mean ± SD) underwent a comprehensive CMR protocol at a 1.5T CMR scanner (Philips, Achieva) including assessment of left ventricular (LV) volume and mass. In addition, T1 measurements pre- and post-administration of gadolinium for evaluation of extra corpuscular volume (ECV) and tagging for quantitative analysis of left ventricular circumferential strain (Ecc) were performed. 8 healthy age-matched volunteers underwent a similar protocol and served as

controls for the ECV values. LV parameters and Ecc were compared to reference values from previous studies with similar data setup [1-3].

Results

CMR exams were successfully performed in all patients. As shown in Figure 1 LV mass index was reduced to the lower 95% confidence interval of the reference values [4,5] in each subgroup. ECV showed no significant difference between PKU patients (0.27 ± 0.03/mean ± SD) and the control group (0.28 ± 0.02/mean ± SD) (Figure 2) p = 0.15. PKU patients had higher Ecc values (= -0.22%) compared to reference values (Ecc = -0.20%) with similar segmental patterns.

Conclusions

The results of this study indicate that in PKU patients compared to healthy controls LV mass indexed to BSA is reduced to the reference values; we found increased

F 20-29 6 F 30-39 S F 40-49 3

M 20-29 M 30-39 M 40-49

F=fernal

Mámale

EDVI LV [mlfm1]

SB.S3 ±23.32 74.96 ±11,19 90.31 ±2,43

86.69 ±13,40 87.90 ±17.62 75.90 ±7,45 mean±S0

Reference Range

82 (65-69) 79 (S2-96) 7B (59-93)

ESVILV [ml/m]

35.74 ±11.50 33.39 ±4,43 34.63 ±6,29

Reference Range

28 (19-37) 27 (17-36) 25 (16-34)

SVI LV [m lfm2]

52.79 ±1 3.40 41.59 ±7,59 55.SB ±3.B6

Reference Range

54 (42-68) 53 (40-65) 51 (39-63)

Mass Index Ltf [gfm£]

44.25 ±7,43 35.05 ±643 42.99 ±6,73

8B (BS-103) 83 (66-101) 81 (B4-99) mean (95% confidence interval)

42.28 ±5,53 40.40 ±10,64 38.44 ±4,53 rnean±SD

30 (19-41) 29 (18-3S) 27 (17-38) mean (95% confidence interv al)

46.41 ±8,91 47.51 ±8,06 37.4S ±2,92 rnean±SD

56 (44-68) 55 (43-67) 54 (42-66) mean (95% confidence interval)

50.05 ±11,27 41.47 ±10,35 33.08 ±11,05 mean±SD

Reference Range

62 (47-7?)

62 (47-77)

63 (48-77)

76 (59-93) 76 (59-92) 75 (58-91) mean (95% confidence interval)

Figure 1 left ventricular mass index (LVMI) in patients with phenylketonuria separated after age and sex.

Cardiology, German Heart Institute Berlin, Berlin, Germany Fulllist of author information is available at the end of the article

© 2014 Hassel et al.; licensee BioMed Central Ltd. This is an Open Access article distributed underthe terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http:// creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

BioMed Central

Hassel et al. Journal of Cardiovascular Magnetic Resonance 2014, 16(Suppl 1):P246 http://www.jcmr-online.com/content/16/S1/P246

Page 2 of 2

Figure 2 ECV in patients with phenylketonuria and healthy controls.

average Ecc parameters and normal ECV values. Further investigations in larger patient groups and older PKU patients are necessary for evaluation of cardiac involvement of the disease over time and its consequences.

Funding

Authors' details

Stoffwechsel-Centrum, Charite-Universitatsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany. 3Philips Healthcare Systems, Hamburg, Germany.

Published: 16 January 2014

References

1. Kuijer, et al: Three-dimensional myocardial strains at end-systole and during diastole in the left ventricle of normal humans. J Cardiovasc Magn Reson 2002, 4(3):341-351.

2. Moore, et al: Three-dimensional systolic strain patterns in the normal human left ventricle: characterization with tagged MR imaging.

In Radiology 2000, 214(2):453-466.

3. Young, et al: Three-dimensional left ventricular deformation in hypertrophic cardiomyopathy. In Circulation 1994, 90(2):854-867.

4. Maceira, et al: Normalized left ventricular systolic and diastolic function by steady state free precession cardiovascular magnetic resonance. In J Cardiovasc Magn Reson 2006, 8(3):417-426.

5. Maceira, et al: Reference right ventricular systolic and diastolic function normalized to age, gender and body surface area from steady-state free precession cardiovascular magnetic resonance. In Eur Heart J 2006, 27(23):2879-2888.

doi:10.1186/1532-429X-16-S1-P246

Cite this article as: Hassel et al.: Comprehensive characterization of cardiac morphology and function in adult patients with phenylketonuria using CMR. Journal of Cardiovascular Magnetic Resonance 2014 16(Suppl 1):P246.

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Cardiology, German Heart Institute Berlin, Berlin, Germany. Interdisziplinäres