scholarly journals Isolated left ventricular apical hypoplasia with myocardial non-compaction: a case report

2019 ◽  
Vol 4 (1) ◽  
pp. 1-6
Author(s):  
Viktoria I Skidan ◽  
Tatiana Kuznetsova ◽  
Elena N Pavlyukova ◽  
Galina P Nartsissova

Abstract Background Isolated left ventricular apical hypoplasia (ILVAH) is a rare congenital cardiac abnormality, which might result in severe symptomatic heart failure (HF) with pulmonary hypertension, atrial fibrillation (AF), or malignant ventricular tachycardia in adults. Case summary A 32-years-old man presented with exertional dyspnoea New York Heart Association Class II and persistent AF. Echocardiography and cardiac magnetic resonance showed the presence of (i) spherical remodelling of the left ventricle (LV) with impaired contractile function (three-dimensional ejection fraction, EF 32%); (ii) substitution of apical myocardium by fatty tissue; (iii) abnormal origin of a papillary muscle network; and (iv) an elongated right ventricle, compatible with ILVAH. In addition, non-compacted endomyocardial layer of the LV was observed. Because of a high risk of sudden cardiac death in symptomatic HF patients with reduced EF, an implantable cardioverter-defibrillator was placed which followed by pulmonary vein isolation. After the procedures and restoration of sinus rhythm, the patient demonstrated improvement in HF symptoms and exercise tolerance. This was accompanied by an enhancement of left and right ventricular systolic function by echocardiography. At 6-month, 1, and 2-year follow-up the clinical conditions of the patient and echocardiographic findings remained stable. Discussion A rare combination of ILVAH and left ventricular myocardium non-compaction was observed in this young adult who presented with symptomatic HF and persistent AF. The use of consecutive invasive cardiac procedures leads to restoration of sinus rhythm, the improvement of myocardial contractility and clinical manifestation of HF.

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M F Borresen ◽  
E M Blixenkrone-Moeller ◽  
M Tindholdt ◽  
R O B Vogg ◽  
C Pihl ◽  
...  

Abstract Background Pronounced trabeculation of the left ventricular myocardium, or non-compaction, may be accompanied by systolic dysfunction in ventricular non-compaction cardiomyopathy (LVNC). It is unclear whether non-compaction represents a fetal development defect or may develop later in life. Previous studies have estimated the prevalence of non-compaction to 0.01–0.31% in adults referred for echocardiography. The prevalences in unselected populations of adults or children are unknown. Purpose To determine the prevalence of non-compaction of the left ventricle in a large population-based cohort of newborns and to assess the relation to left ventricular systolic function. Methods Transthoracic echocardiography was performed according to a standardized protocol in all newborns included in a regional population-based cohort study in 2016–2018. For the present study, the extent of trabeculations were assessed by one of three primary reviewers in apical long-axis views (4-chamber, 5-chamber, and sinus coronarius views), parasternal short (SAX), and long axis (PLAX) views. Based on the presence of pronounced trabeculations, deep intertrabecular recesses and/or an impression of a ratio of >1 of non-compact:compact myocardium (NC:C), the exam was regarded as either possible or not possible non-compaction: In examinations classified as possible non-compaction, an expert reviewer would determine if diagnostic criteria were fulfilled. A ratio of NC:C myocardium ≥2 was considered as non-compaction in accordance with previously suggested criteria. Results As of January 11, 2019, 6,880 neonate echocardiograms (median age 10 days [IQR 5,14], 48.8% females) had been analyzed. In total, 127 (1.8%) were considered as possible non-compaction. Six newborns (median age 14 days [IQR 8,18], 33.3% females) had a ratio of NC:C myocardium ≥2 corresponding to a prevalence of non-compaction of 8.7 per 10,000 newborns (95% CI 0.04–0.19%) (Figure). One of six neonates had a ratio of NC:C myocardium≥2 in more than one segment. Median fractional shortening (FS %), left ventricular end-diastolic (LVIDd) and end-systolic diameter (LVIDs) were 29.5% (IQR 27.0, 31.0), 1.9 cm (IQR 1.8, 2.1) and 1.3 cm (IQR 1.3, 1.5) respectively in the six newborns with non-compaction. The corresponding mean z-scores (standard deviations away from the age-matched reference mean) according to The Boston Children's Hospital z-score Calculator were FS: r=−3.0 (IQR r=−3.8, r=−2.5), LVIDd: r=−1.3 (IQR r=−2.7, r=−0.2), LVIDs: 0.2 (IQR r=−0.1, 1.6). 10-days-old boy with LVNC (FS 22%) Conclusion Echocardiography of an unselected population-based cohort of 6,880 newborns revealed a prevalence of non-compaction of 8.7 per 10,000 newborns. The prevalence is in accordance with previously described prevalences in adults referred for echocardiography and corroborate the view that non-compaction may be a fetal developmental defect. Non-compaction was not associated with left ventricular dilatation but with systolic dysfunction. Acknowledgement/Funding Danish Heart Association, Danish Children's Heart Foundation, Candy's Found., Toyota Found., Herlev-Gentofte Hospital Research Found., Gangsted Found.


2020 ◽  
Vol 15 (6) ◽  
pp. 813-819
Author(s):  
S. N. Koretskiy ◽  
O. M. Drapkina ◽  
F. B. Shukurov ◽  
D. K. Vasiliev

Stress echocardiography is a modern widely used method of noninvasive diagnosis of coronary heart disease and stratification of the risk of cardiovascular complications. In addition, exercise echocardiography is an important tool to clarify the localization of ischemia and establish a symptomassociated artery for management of patient with known coronary angiography data. This is especially important in multivessel lesions, the presence of an occluded artery or borderline stenosis. Currently, various stress agents are used for stress echocardiography in clinical practice: pharmacological drugs (dobutamine or adenosine), transesophageal or endocardial pacing, treadmill, semi-supine bicycle. To detect signs of ischemia usually used only visual estimation of local contractility in the two-dimensional gray-scale mode. Modern modes of myocardial imaging, such as speckletracking echocardiography or three-dimensional visualization, are practically not used. In the presented clinical case, the possibility of combining standard and modern imaging modes to clarify the localization and quantification of ischemia in multivessel coronary lesions, including chronic artery occlusion, is shown. As a stress agent, a semi-supine bicycle was chosen, the use of which allowed to obtain a qualitative image of the left ventricular myocardium at rest and at peak load, suitable for assessing deformation and threedimensional visualization. Evaluation of left ventricular myocardial deformation by speckle-tracking echocardiography was more accurate than standard diagnosis in detecting signs of ischemia in a patient with multivessel lesions. Three-dimensional imaging was inferior in sensitivity to speckletracking stress echocardiography and, at present, seems to have more research value.


2020 ◽  
Vol 10 (5) ◽  
pp. 27-34
Author(s):  
Eugene V. Timofeev ◽  
Eduard G. Malev ◽  
Nina N. Parfenova ◽  
Eduard V. Zemtsovsky

For many hereditary connective tissue disorders (HCTD), especially Marfan syndrome, remodeling of the heart and main vessels is described, which is manifested by a decrease in the systolic function of the left ventricle and expansion of the thoracic aorta. Evaluation of morphometric characteristics of the heart and main vessels in patients with other HCTD, in particular marfanoid habitus (MH) has not been previously carried out. Materials and methods. Weexamined 90 young men and 74 young women between the ages of 18 to 25 years, 111 patients older age groups with stable over coronary heart disease (mean age 64.66.2 years) and 9 patients with verified Marfan syndrome (mean age 27.99.3years). All survey phenotypic and performed anthropometric survey identifying bone signs of dysembryogenesis as well as Echocardiography study on standard protocol. The results.Patients with MH as compared with control group revealed a relatively larger diameter of aortic root (30.44.7 vs 28.03.6 mm,p= 0.03) and the ascending aorta (26.64.9 vs 24.63.2 mm,p= 0.05). Also young with MH turned out to be significantly thicker myocardium of left ventricular posterior wall (8.30.8 vs 7.71.1 mm,p= 0.02) and interventricular septum (8.81.2vs 8.21.1mm,p= 0.04). When performing correlation analysis identified reliable positive correlation between such highly specialized bone signs as high palate (r= 0.31), infundibular deformation of the chest (r= 0.43), arachnodactyly (r= 0.45) andZ-test (p 0.05 for all). Expansion of the aorta (Z-criterion 2.0) have found 24% of older patients with MH. Conclusion.Inpatients with MH revealed significant structural changes of heart and main vessels which are progredient character thickening of the left ventricular myocardium and expansion of the aortic root.


2016 ◽  
Vol 2016 ◽  
pp. 1-5
Author(s):  
Takashi Mitsui ◽  
Hisashi Masuyama ◽  
Kentaro Ejiri ◽  
Kei Hayata ◽  
Hiroshi Ito ◽  
...  

Hypertrophic obstructive cardiomyopathy (HOCM) is cardiac hypertrophy of ventricular myocardium with left ventricular outflow tract obstruction. We report a pregnancy with HOCM after defibrillator implantation surgery. The patient was a 33-year-old nulligravida and was categorized as New York Heart Association class II. Her brain natriuretic peptide (BNP) level was 724.6 pg/dL at preconception. She received careful pregnancy management. However, because frequent uterine contractions were observed at 25 weeks and 6 days of pregnancy, she was hospitalized, and magnesium sulfate was started as a tocolytic agent. At 27 weeks and 5 days of pregnancy, she had respiratory discomfort and orthopnea with a sudden decrease in peripheral oxygen saturation. Cardiac ultrasonography showed a worsened condition of HOCM and her BNP level was 1418.0 pg/mL. We performed an emergent cesarean section and she delivered a boy weighing 999 g. The Apgar score was 8 and 9 points at 1 and 5 minutes, respectively. The mother’s heart failure quickly improved after birth and she was discharged at 10 days postoperatively. Fluctuations in circulatory dynamics during pregnancy may sometimes exacerbate heart disease. Therefore, the risks should be fully explained and careful assessment of cardiac function should be performed during pregnancy in patients with severe HOCM.


1999 ◽  
Vol 276 (2) ◽  
pp. H595-H607 ◽  
Author(s):  
Kevin D. Costa ◽  
Yasuo Takayama ◽  
Andrew D. McCulloch ◽  
James W. Covell

Previous studies suggest that the laminar architecture of left ventricular myocardium may be critical for normal ventricular mechanics. However, systolic three-dimensional deformation of the laminae has never been measured. Therefore, end-systolic finite strains relative to end diastole, from biplane radiography of transmural markers near the apex and base of the anesthetized open-chest canine anterior left ventricular free wall ( n = 6), were referred to three-dimensional laminar microstructural axes reconstructed from histology. Whereas fiber shortening was uniform [−0.07 ± 0.04 (SD)], radial wall thickening increased from base (0.10 ± 0.09) to apex (0.14 ± 0.13). Extension of the laminae transverse to the muscle fibers also increased from base (0.08 ± 0.07) to apex (0.11 ± 0.08), and interlaminar shear changed sign [0.05 ± 0.07 (base) and −0.07 ± 0.09 (apex)], reflecting variations in laminar architecture. Nevertheless, the apex and base were similar in that at each site laminar extension and shear contributed ∼60 and 40%, respectively, of mean transmural thickening. Kinematic considerations suggest that these dual wall-thickening mechanisms may have distinct ultrastructural origins.


2018 ◽  
Vol 22 (1) ◽  
pp. 185-189
Author(s):  
S.V. Cherniuk

To date, the expediency of immunosuppressive treatment with corticosteroids in patients with myocarditis is one of the most controversial issues of modern cardiology. The purpose of the study was to establish predictors of corticosteroid therapy efficiency in patients with acute myocarditis. 47 patients with acute myocarditis were examined, who received immunosuppressive therapy for 6 months. All patients underwent for immune state assessment, echocardiography and cardiac magnetic resonance imaging. Statistical processing of the results was performed using Excel XP and Statistica for Windows v. 6.0 programming studying Student's criterion, parametric correlation analysis and distribution of the studied indicators by binary scale. It has been established that the appointment of immunosuppressive therapy in patients with high activity of immunopathological reactions of humoral type in the debut of myocarditis after 6 months of treatment leads to a decrease in the activity of autoimmune damage of the myocardium, recovery of left ventricular systolic function and improvement of heart failure functional class. The predictors of heart failure functional class improvement after 6-months of corticosteroid treatment were determined as: concentrations of immunoglobulin M>2,5 g/l, CD19+ lymphocytes >0,23 × 109/l, tumor necrosis factor α>200 pg/ml, interleukin-1β >150 pg/ml, content of circulating immune complexes >75 un. opt. sq., titre of antibodies to myocardium >20.0 conv. un. before the start of immunosuppressive therapy, as well as the absence of fibrotic changes in the left ventricular myocardium on cardiac MRI. Thus, early prognostic markers of the effectiveness of glucocorticoid therapy in patients with acute myocarditis were established. Further research should focus on the search for criteria of immunosuppressive therapy effectiveness and the introduction of clear recommendations for clinical practice that will contain indications and contraindications for its management in patients with myocarditis.


Biology Open ◽  
2021 ◽  
Vol 10 (2) ◽  
pp. bio057059 ◽  
Author(s):  
Jaeike W. Faber ◽  
Jaco Hagoort ◽  
Antoon F. M. Moorman ◽  
Vincent M. Christoffels ◽  
Bjarke Jensen

ABSTRACTThe size and growth patterns of the components of the human embryonic heart have remained largely undefined. To provide these data, three-dimensional heart models were generated from immunohistochemically stained sections of ten human embryonic hearts ranging from Carnegie stage 10 to 23. Fifty-eight key structures were annotated and volumetrically assessed. Sizes of the septal foramina and atrioventricular canal opening were also measured. The heart grows exponentially throughout embryonic development. There was consistently less left than right atrial myocardium, and less right than left ventricular myocardium. We observed a later onset of trabeculation in the left atrium compared to the right. Morphometry showed that the rightward expansion of the atrioventricular canal starts in week 5. The septal foramina are less than 0.1 mm2 and are, therefore, much smaller than postnatal septal defects. This chronological, graphical atlas of the growth patterns of cardiac components in the human embryo provides quantified references for normal heart development. Thereby, this atlas may support early detection of cardiac malformations in the foetus.This article has an associated First Person interview with the first author of the paper.


1999 ◽  
Vol 26 (3) ◽  
pp. 201-207 ◽  
Author(s):  
Stefan Biedenstein ◽  
Michael Schäfers ◽  
Lars Stegger ◽  
Torsten Kuwert ◽  
Otmar Schober

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