Dataset Information

Congenital coronary artery-left ventricular multiple micro-fistulas and hypertrophic cardiomyopathy: a case report and literature review.

ABSTRACT:

Background

Coronary artery-left ventricular multiple micro-fistulas (CA-LVMMFs) is a rare congenital vascular anomaly that may present with no obvious clinical symptoms or a typical angina attack. CA-LVMMFs is usually found unexpectedly during coronary angiography (CAG).

Case presentation

We report a case of a 65-year-old man admitted to the hospital with acute coronary syndrome. CA-LVMMFs was found during coronary angiography. Echocardiography showed apical hypertrophy and blood flow signals were seen in the apical myocardium, connected with the left ventricle. We searched the MEDLINE database and found 39 relevant reports. We made statistics on the clinical characteristics of these patients and found half involved hypertrophy or perfusion defects in the ventricular septum or apex.

Conclusion

As a rare congenital anomaly, the effect of CA-LVMMFs on patients is unclear. By reporting a case and summarizing literature reports, we found that CA-LVMMFs may be associated with myocardial hypertrophy, especially ventricular septal and apical hypertrophy.

SUBMITTER: Liu Y

PROVIDER: S-EPMC9652968 | biostudies-literature | 2022 Nov

REPOSITORIES: biostudies-literature

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Publications

Congenital coronary artery-left ventricular multiple micro-fistulas and hypertrophic cardiomyopathy: a case report and literature review.

Liu Yue Y Wang Zhiyuan Z Zeng Hong H Yang Sibao S Li Xiangdong X

BMC cardiovascular disorders 20221112 1

<h4>Background</h4>Coronary artery-left ventricular multiple micro-fistulas (CA-LVMMFs) is a rare congenital vascular anomaly that may present with no obvious clinical symptoms or a typical angina attack. CA-LVMMFs is usually found unexpectedly during coronary angiography (CAG).<h4>Case presentation</h4>We report a case of a 65-year-old man admitted to the hospital with acute coronary syndrome. CA-LVMMFs was found during coronary angiography. Echocardiography showed apical hypertrophy and blood ...[more]

PMID: 36371156

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Project description:BackgroundApical hypertrophic cardiomyopathy (ApHCM) is recognized for its associated cardiovascular morbidity. Herein we describe left ventricular (LV) function and mechanics over long-term follow-up in ApHCM.MethodsA retrospective study of 98 consecutive ApHCM patients was performed (mean age: 64±15 years, 46% female) using 2D and speckle-tracking echocardiography. LV function and mechanics were characterized by global longitudinal strain (GLS), segmental strain, and myocardial work indices. Myocardial work was calculated by integrating longitudinal strain and blood pressure as estimated by the brachial artery cuff pressure, to generate an LV pressure-strain loop with adjusted ejection and isovolumetric periods. Composite complications were defined as all-cause mortality, sudden death, myocardial infarction, and/or stroke.ResultsMean LV ejection fraction measured 67%±11% and GLS was -11.7%±3.9%. Global work index (GWI) was 1,073±349 mmHg%, constructive work was 1,379±449 mmHg%, wasted work was 233±164 mmHg%, and work efficiency was 82%±8%. In 72 patients with follow-up echocardiography, at a median of 3.9 years there was progressive impairment in GLS (-11.9% vs. -10.7%; P=0.006), GWI (1,105 vs. 989 mmHg%; P=0.02), and global constructive work (1,432 vs. 1,312 mmHg%; P=0.03), without change in wasted work or work efficiency. Atrial fibrillation (β=0.37; P<0.001), mitral annular e' velocity (β=-0.32; P=0.001), and glomerular filtration rate (β=-0.2; P=0.03) were independently associated with follow-up GLS; atrial fibrillation (β=-0.27; P=0.01) and glomerular filtration rate (β=0.23; P=0.04) were also associated with follow-up GWI. Global wasted work >186 mmHg% was predictive of composite complications (AUC =0.7, 95% CI: 0.53-0.82, sensitivity 93%, specificity 41%).ConclusionsApHCM is associated with preserved LV ejection fraction but abnormal LV GLS and work indices, with progressive impairment. Important clinical and echocardiographic measures are independently predictive of long-term follow-up LV GLS, GWI and adverse events.

Project description:BackgroundThe right ventricle (RV) in hypertrophic cardiomyopathy (HCM) tends to be neglected, as previous efforts have predominantly focused on examining the prognostic value of left ventricular (LV) abnormalities. The objectives of this study were to assess RV function in HCM, changes over time, and association with clinical outcomes.MethodsTwo hundred and ninety HCM patients with preserved LV ejection fraction (LVEF ≥ 55%) and 30 age- and sex-matched controls underwent cardiovascular magnetic resonance (CMR). All patients were followed up for clinical events for a median duration of 4.4 years. Sixty-three patients had a follow-up CMR undertaken at a median interval of 5.4 years. Main study measures and outcomes were RV function (RV ejection fraction (RVEF) and RV strain) at baseline, temporal changes in RV function over time and prognostic value of RV dysfunction for predicting cardiovascular outcomes in HCM.ResultsWhen compared to controls, HCM patients exhibited lower RV and LV peak global longitudinal systolic strains on feature-tracking analysis of cine images, while RVEF and LVEF were within the normal range. On follow-up CMR, both RV and LV strain parameters decreased over time. RVEF decreased at follow-up (65 ± 7% to 62 ± 7%, P < 0.001) but the change in LVEF was not significant (68 ± 10% to 66 ± 8%, P = 0.30). On clinical follow up, reduced RVEF was an independent predictor of non-sustained ventricular tachycardia (NSVT) [HR 1.10 (95% CI 1.06-1.15), P < 0.001] and composite cardiovascular events (NSVT, stroke, heart failure hospitalisation and cardiovascular death) [HR 1.07 (95% CI 1.03-1.10), P < 0.001]. RV longitudinal strain was an independent predictor of NSVT [HR 1.05 (95% CI 1.01-1.09), P = 0.029]. Patients with RVEF < 55% showed an increased risk of NSVT and composite cardiovascular events. In contrast, LVEF and LV global longitudinal strain were not predictive of such events on multivariable analysis.ConclusionsIn HCM, RV function, including RV strain, and LV strain decrease over time despite preserved LVEF. Reduction in RV but not LV function is associated with adverse cardiovascular outcomes. Assessing RV function in early HCM disease might have a role in risk stratification to prevent future cardiovascular events.

Dataset Information

Congenital coronary artery-left ventricular multiple micro-fistulas and hypertrophic cardiomyopathy: a case report and literature review.

Background

Case presentation

Conclusion

Publications

Congenital coronary artery-left ventricular multiple micro-fistulas and hypertrophic cardiomyopathy: a case report and literature review.

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