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Added value of cardiac magnetic resonance in a case of multiple congenital abnormalities.

ABSTRACT: Sub-infundibular stenosis is a rare form of congenital heart disease, difficult to precisely diagnose, especially in adult patients. We report a case of an adult patient, referred to our hospital with long-standing dyspnea on exertion. She was finally diagnosed with sub-infundibular stenosis. During cardiovascular magnetic resonance imaging, right-sided aortic arch and left pulmonary artery agenesis were also identified. .

SUBMITTER: Rangel I

PROVIDER: S-EPMC6281692 | biostudies-literature | 2014 Apr

REPOSITORIES: biostudies-literature

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Added value of cardiac magnetic resonance in a case of multiple congenital abnormalities.

Rangel Inês I Vasconcelos Mariana M Cruz Cristina C Madureira António José AJ Maciel Maria Júlia MJ

Journal of cardiology cases 20140116 4

Sub-infundibular stenosis is a rare form of congenital heart disease, difficult to precisely diagnose, especially in adult patients. We report a case of an adult patient, referred to our hospital with long-standing dyspnea on exertion. She was finally diagnosed with sub-infundibular stenosis. During cardiovascular magnetic resonance imaging, right-sided aortic arch and left pulmonary artery agenesis were also identified. <<b>Learning objective:</b> The case described illustrates that cardiovascu ...[more]

PMID: 30546785

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Project description:ObjectivesFollow-up of congenital lung abnormalities (CLA) is currently done with chest computer tomography (CT). Major disadvantages of CT are exposure to ionizing radiation and need for contrast enhancement to visualise vascularisation. Chest magnetic resonance imaging (MRI) could be a safe alternative to image CLA without using contrast agents. The objective of this cohort study was to develop a non-contrast MRI protocol for the follow-up of paediatric CLA patients, and to compare findings on MRI to postnatal CT in school age CLA patients.MethodsTwenty-one CLA patients, 4 after surgical resection and 17 unoperated (mean age 12.8 (range 9.4-15.9) years), underwent spirometry and chest MRI. MRI was compared to postnatal CT on appearance and size of the lesion, and lesion associated abnormalities, such as hyperinflation and atelectasis.ResultsBy comparing school-age chest MRI to postnatal CT, radiological appearance and diagnostic interpretation of the type of lesion changed in 7 (41%) of the 17 unoperated patients. In unoperated patients, the relative size of the lesion in relation to the total lung volume remained stable (0.9% (range - 6.2 to + 6.7%), p = 0.3) and the relative size of lesion-associated parenchymal abnormalities decreased (- 2.2% (range - 0.8 to + 2.8%), p = 0.005).ConclusionNon-contrast-enhanced chest MRI was able to identify all CLA-related lung abnormalities. Changes in radiological appearance between MRI and CT were related to CLA changes, patients' growth, and differences between imaging modalities. Further validation is needed for MRI to be introduced as a safe imaging method for the follow-up of paediatric CLA patients.Key points• Non-contrast-enhanced chest MRI is able to identify anatomical lung changes related to congenital lung abnormalities, including vascularisation. • At long-term follow-up, the average size of congenital lung abnormalities in relation to normal lung volume remains stable. • At long-term follow-up, the average size of congenital lung abnormalities associated parenchymal abnormalities such as atelectasis in relation to normal lung volume decreases.

Project description:Background and purposeOur goal is to determine the added value of intracranial vessel wall magnetic resonance imaging (IVWI) in differentiating nonocclusive vasculopathies compared with luminal imaging alone.MethodsWe retrospectively reviewed images from patients with both luminal and IVWI to identify cases with clinically defined intracranial vasculopathies: atherosclerosis (intracranial atherosclerotic disease), reversible cerebral vasoconstriction syndrome, and inflammatory vasculopathy. Two neuroradiologists blinded to clinical data reviewed the luminal imaging of defined luminal stenoses/irregularities and evaluated the pattern of involvement to make a presumed diagnosis with diagnostic confidence. Six weeks later, the 2 raters rereviewed the luminal imaging in addition to IVWI for the pattern of wall involvement, presence and pattern of postcontrast enhancement, and presumed diagnosis and confidence. Analysis was performed on per-lesion and per-patient bases.ResultsThirty intracranial atherosclerotic disease, 12 inflammatory vasculopathies, and 12 reversible cerebral vasoconstriction syndrome patients with 201 lesions (90 intracranial atherosclerotic disease, 64 reversible cerebral vasoconstriction syndrome, and 47 inflammatory vasculopathy lesions) were included. For both per-lesion and per-patient analyses, there was significant diagnostic accuracy improvement with luminal imaging+IVWI when compared with luminal imaging alone (per-lesion: 88.8% versus 36.1%; P<0.001 and per-patient: 96.3% versus 43.5%; P<0.001, respectively). There was substantial interrater diagnostic agreement for luminal imaging+IVWI (κ=0.72) and only slight agreement for luminal imaging (κ=0.04). Although there was a significant correlation for both luminal and IVWI pattern of wall involvement with diagnosis, there was a stronger correlation for IVWI finding of lesion eccentricity and intracranial atherosclerotic disease diagnosis than for luminal imaging (κ=0.69 versus 0.18; P<0.001).ConclusionsIVWI can significantly improve the differentiation of nonocclusive intracranial vasculopathies when combined with traditional luminal imaging modalities.

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Added value of cardiac magnetic resonance in a case of multiple congenital abnormalities.

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Added value of cardiac magnetic resonance in a case of multiple congenital abnormalities.

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