Dataset Information

Cardiac magnetic resonance imaging findings of isolated left ventricular apical hypoplasia.

ABSTRACT: Isolated left ventricular apical hypoplasia (ILVAH) is an uncommon and likely congenital cardiac abnormality that has been described as relatively new. ILVAH is characterized by a truncated, globular-shaped left ventricle (LV) with bulging of the interventricular septum toward the right ventricle (RV), wrapping of an elongated and lengthened RV around the absent LV apex, thinning and fat replacement of apical myocardium of the LV, and abnormalities in the papillary muscle arrangement of the LV. In this report, we present the cardiac magnetic resonance imaging findings of a 22-year-old female patient with non-specific cardiac complaints that were compatible with ILVAH. Recognition of this rare cardiomyopathy is important for clinicians and radiologists in order to follow up on patients with ILVAH, as it may lead to severe complications, and to distinguish it from other cardiomyopathies.

Learning objective

Isolated left ventricular apical hypoplasia (ILVAH) is a rare congenital cardiomyopathy that has some serious complications, such as left-sided heart failure, severe pulmonary hypertension, and fatal arrhythmias. By recognizing and identifying the cardiac magnetic resonance imaging findings of ILVAH, clinicians and radiologists can take appropriate measures to manage and treat patients with this condition, potentially improving outcomes and reducing the risk of complications.

SUBMITTER: Sahin S

PROVIDER: S-EPMC10658309 | biostudies-literature | 2023 Nov

REPOSITORIES: biostudies-literature

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Cardiac magnetic resonance imaging findings of isolated left ventricular apical hypoplasia.

Şahin Sercan S Özbülbül Nilgün Işıksalan NI

Journal of cardiology cases 20230816 5

Isolated left ventricular apical hypoplasia (ILVAH) is an uncommon and likely congenital cardiac abnormality that has been described as relatively new. ILVAH is characterized by a truncated, globular-shaped left ventricle (LV) with bulging of the interventricular septum toward the right ventricle (RV), wrapping of an elongated and lengthened RV around the absent LV apex, thinning and fat replacement of apical myocardium of the LV, and abnormalities in the papillary muscle arrangement of the LV. ...[more]

PMID: 38024111

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Project description:BackgroundCardiac magnetic resonance (CMR) has been used to diagnose and risk-stratify patients with left ventricular noncompaction (LVNC). The prognostic value of CMR parameters for LVNC, especially feature tracking (CMR-FT), is not well known in LVNC patients with left ventricular dysfunction. The present study aimed to investigate whether the combination of CMR-FT with traditional CMR parameters can increase the prognostic value of CMR for LVNC patients with reduced left ventricular ejection fraction (LVEF).MethodsA total of 123 candidates were retrospectively included in this multicenter study and 55 LVNC patients (mean age, 45.7 ± 16.2 years; 61.8% men) remained after applying the exclusion criteria. Clinical features, left ventricular (LV) function parameters, global and segment myocardial strain, and late gadolinium enhancement (LGE) were evaluated. The outcomes include the composite events of cardiovascular death, heart transplantation, hospitalization for heart failure, thromboembolic events, and ventricular arrhythmias.ResultsAfter a median follow-up of 5.17 years (interquartile range: 0.17 to 10.58 years), 24 (36.8%) patients experienced at least one major adverse cardiovascular event (MACE). The myocardial strain parameters of patients with events were lower than those of patients without events. In the univariable Cox analysis, LVEF, the presence of LGE, global longitudinal strain (GLS) and segmental strains, including longitudinal strain at the apical level and radial and circumferential strain at the basal level, were significantly associated with MACEs. In the multivariate analysis, LGE (hazard ratio (HR) 3.452, 95% CI 1.133 to 10.518, p = 0.029) was a strong predictor of MACEs and significantly improved the predictive value (chi-square of the model after adding LGE: 7.51 vs. 13.47, p = 0.009). However, myocardial strain parameters were not statistically significant for the prediction of MACEs after adjusting for age, body mass index, LVEF and the presence of LGE and did not increase the prognostic value (chi-square of the model after adding GLS: 13.47 vs. 14.14, p = 0.411) in the multivariate model.ConclusionsThe combination of CMR-FT with traditional CMR parameters may not increase the prognostic value of CMR in LVNC patients with reduced LVEF, while the presence of LGE was a strong independent predictor of MACEs and significantly improved the predictive value.

Dataset Information

Cardiac magnetic resonance imaging findings of isolated left ventricular apical hypoplasia.

Learning objective

Publications

Cardiac magnetic resonance imaging findings of isolated left ventricular apical hypoplasia.

Similar Datasets

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