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Aortic coarctation: guidelines mismatch across the ocean.

ABSTRACT: Pseudocoarctation is a rare congenital anomaly characterized by aorta elongation and kinking, without significant obstruction. We report the case of an elderly patient with history of congestive heart failure (CHF) and aortic regurgitation (AR) who was referred for progressive exertional dyspnoea. After multimodal imaging evaluation, aortic coarctation with significant trans-stenosis gradient but mild luminal narrowing was diagnosed; this borderline patient was not addressed to repair, according to ESC guidelines and in spite of AHA ones. He rather met the criteria for pseudocoarctation diagnosis. An integration of functional and anatomical data is essential for a reliable diagnostic process in similar cases.

SUBMITTER: Pepe M

PROVIDER: S-EPMC3936898 | biostudies-other | 2014

REPOSITORIES: biostudies-other

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Aortic coarctation: guidelines mismatch across the ocean.

Pepe Martino M Iacovelli Fortunato F Masi Filippo F Marangelli Vito V Scardapane Arnaldo A De Santis Alessandro A Sgarra Luca L Quagliara Donato D Favale Stefano S

Journal of cardiothoracic surgery 20140220

Pseudocoarctation is a rare congenital anomaly characterized by aorta elongation and kinking, without significant obstruction. We report the case of an elderly patient with history of congestive heart failure (CHF) and aortic regurgitation (AR) who was referred for progressive exertional dyspnoea. After multimodal imaging evaluation, aortic coarctation with significant trans-stenosis gradient but mild luminal narrowing was diagnosed; this borderline patient was not addressed to repair, according ...[more]

PMID: 24555691

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Project description:ObjectivesThis study aimed to explore aortic morphology and the associations between morphological features and cardiovascular function in a population of patients with bicuspid aortic valve, while further assessing differences between patients with repaired coarctation, patients with unrepaired coarctation and patients without coarctation.MethodsThis is a single-centre retrospective study that included patients with available cardiovascular magnetic resonance imaging data and native bicuspid aortic valve diagnosis (n = 525). A statistical shape analysis was performed on patients with a 3-dimensional magnetic imaging resonance (MRI) dataset (n = 108), deriving 3-dimensional aortic reconstructions and computing a mean aortic shape (template) for the whole population as well as for the 3 subgroups of interest (no coarctation, repaired coarctation and unrepaired coarctation). Shape deformations (modes) were computed and correlated with demographic variables, 2-dimensional MRI measurements and volumetric and functional data.ResultsOverall, the results showed that patients with coarctation tended towards a more Gothic arch architecture, with decreased ascending and increased descending aorta diameters, with the unrepaired-aortic coarctation subgroup exhibiting more ascending aorta dilation. Careful assessment of patients with repaired coarctation only revealed that a more Gothic arch, increased descending aorta dimensions and ascending aorta dilation were associated with reduced ejection fraction (P ≤ 0.04), increased end-diastolic volume (P ≤ 0.04) and increased ventricular mass (P ≤ 0.02), with arch morphology distinguishing patients with and without recoarctation (P = 0.05).ConclusionsA statistical shape modelling framework was applied to a bicuspid aortic valve population revealing nuanced differences in arch morphology and demonstrating that morphological features, not immediately described by conventional measurements, can indicate those shape phenotypes associated with compromised function and thus possibly warranting closer follow-up.

Dataset Information

Aortic coarctation: guidelines mismatch across the ocean.

Publications

Aortic coarctation: guidelines mismatch across the ocean.

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