scholarly journals Noncompaction of left ventricle - a rare cardiomyopathy

2021 ◽  
Vol 9 (1) ◽  
pp. 116
Author(s):  
Parth K. Thakkar ◽  
Mahesh Bhatt ◽  
Sheela Bharani Chawla ◽  
Hitesh Desai

Non-compaction of left ventricular (NCLV) is a rare cardiomyopathy of unknown origin characterized by prominent ventricular trabeculations and deep intertrabecular sinusoids, in communication with the left ventricular cavity. The NCLV results due to failure of compaction during foetal development. In this study, five children were diagnosed with NCLV out of 135 cases of cardiomyopathy and the youngest case was diagnosed at 27th day of life. Congestive cardiac failure presenting as tachycardia and tachypnea were common clinical manifestations. Early detection of NCLV helps patients to get timely treatment. NCLV should be considered as one of the differential diagnosis amongst cases of cardiomyopathy.

PEDIATRICS ◽  
1970 ◽  
Vol 46 (3) ◽  
pp. 464-468
Author(s):  
Karen S. Kuehl ◽  
Lowell W. Perry ◽  
Roma Chandra ◽  
Lewis P. Scott

A newborn infant with cardiac failure resulting from a rhabdomyoma of the left ventricle obstructing the aortic outflow tract is described. Mild subvalvular pulmonary stenosis was also present because of a tumor in the ventricular septum. Tuberous sclerosis occurs in at least half of infants with such tumors and was present pathologically in this child, although not apparent clinically. Rhabdomyomas must be considered in the differential diagnosis of heart disease in the newborn infant and may be associated with tuberous sclerosis even when nervous system and cutaneous signs are not present.


1991 ◽  
Vol 261 (6) ◽  
pp. H1979-H1987 ◽  
Author(s):  
M. Gopalakrishnan ◽  
D. J. Triggle ◽  
A. Rutledge ◽  
Y. W. Kwon ◽  
J. A. Bauer ◽  
...  

To examine the status of ATP-sensitive K+ (K+ATP) channels and 1,4-dihydropyridine-sensitive Ca2+ (Ca2+DHP) channels during experimental cardiac failure, we have measured the radioligand binding properties of [3H]glyburide and [3H]PN 200 110, respectively, in tissue homogenates from the rat cardiac left ventricle, right ventricle, and brain 4 wk after myocardial infarction induced by left coronary artery ligation. The maximal values (Bmax) for [3H]glyburide and [3H]PN 200 110 binding were reduced by 39 and 40%, respectively, in the left ventricle, and these reductions showed a good correlation with the right ventricle-to-body weight ratio in heart-failure rats. The ligand binding affinities were not altered. In the hypertrophied right ventricle, Bmax values for both the ligands were not significantly different when data were normalized to DNA content or right ventricle weights but showed an apparent reduction when normalized to unit protein or tissue weight. Moderate reductions in channel densities were observed also in whole brain homogenates from heart failure rats. Assessment of muscarinic receptors, beta-adrenoceptors and alpha 1-adrenoceptors by [3H]quinuclidinyl benzilate, [3H]dihydroalprenolol, and [3H]prazosin showed reductions in left ventricular muscarinic and beta-adrenoceptor densities but not in alpha 1-adrenoceptor densities, consistent with earlier observations. It is suggested that these changes may in part contribute to the pathology of cardiac failure.


2020 ◽  
Vol 19 (2) ◽  
pp. 18-23
Author(s):  
I. E. Obramenko

Introduction. About 0.2 % of the adult population all over the world suffers from hypertrophic cardiomyopathy. Early and timely diagnosis of the apical form of hypertrophic cardiomyopathy remains an urgent medical problem, since the disease has a wide variability of clinical manifestations and often occurs asymptomatic or with symptoms of other heart diseases. Magnetic resonance imaging is an informative method of radiation diagnosis of hypertrophic cardiomyopathy. The aim is improving of radiology diagnostics in applying to the apical form of hypertrophic cardiomyopathy. Materials and methods. 98 patients with apical hypertrophic cardiomyopathy aged 19 to 76 years were еxamined. There were 48 men and 50 women. All subjects were examined by a cardiologist, all patients underwent electrocardiography and echocardiography, 45 patients underwent magnetic resonance imaging (MRI) of the heart. Results. In our study 13 patients had MRI determined the isolated form of apical form of hypertrophic cardiomyopathy, 32 had combined one. 42.2% of the patients with symmetrical hypertrophy of all apical segments had sawtoothed configuration of the LV revealed by MRI. The symptom of left ventricular cavity obliteration was determined in 19 patients. The symptom of LV cavity sequestration was determined in 5 subjects. 5 patients had an aneurysm on the top of the left ventricle, 1 – on the top of the right ventricle. Signs of left ventricular outflow tract obstruction were visualized in 2 patients, intraventricular obstruction at the level of the middle segments of the left ventricle was determined in 5 cases. Akinesis and hypokinesis were detected in areas of fibrous changes (n=21) or in areas of cardiosclerosis (n=2). In 17.8 % of subjects identified non-compacted myocardium, in 3 cases it was combined with apical form of hypertrophic cardiomyopathy. With contrast enhancement in 29 patients, foci (n=22) or zones (n=7) of pathological accumulation of contrast agent were determined, which indicated the replacement of myocardium with fibrous tissue.


2020 ◽  
Vol 15 (4) ◽  
pp. 100-104
Author(s):  
M.A. Ufimtseva ◽  
◽  
Yu.M. Bochkarev ◽  
A.U. Sabitov ◽  
K.I. Nikolaeva ◽  
...  

We report a case of blueberry muffin syndrome, a rare dermatosis that occurs during the neonatal period. This syndrome is particularly interesting because, despite similar clinical manifestations, it may have various pathological mechanisms and, therefore, requires differential diagnosis. Awareness of doctors of different specialties about clinical manifestations of blueberry muffin syndrome will ensure correct differential diagnosis, timely treatment initiation, no unnecessary therapy, and exclusion of malignant tumors in a child. Key words: neonatal period, blueberry muffin, intrauterine infection of the fetus, TORCH infections, extramedullary hematopoiesis


Folia Medica ◽  
2011 ◽  
Vol 53 (2) ◽  
pp. 5-35 ◽  
Author(s):  
Ivo S. Petrov ◽  
Mariya P. Tokmakova ◽  
Daniel N. Marchov ◽  
Kostadin N. Kichukov

Abstract Introduction: Tako-tsubo syndrome is a novel cardio-vascular disease affecting predominantly postmenopausal women exposed to unexpected strong emotional or physical stress, in the absence of significant coronary heart disease. It is characterized by acute onset of severe chest pain and/or acute left ventricular failure, ECG-changes, typical left ventricular angiographic findings, good prognosis and positive resolution of the morphological and clinical manifestations. First described in 1990 in Japan by Sato, Tako-tsubo cardiomyopathy is characterized by transient contractile abnormalities of the left ventricle, causing typical left ventricular apical ballooning at end-systole with concomitant compensatory basal hyperkinesia. There are also atypical forms, presenting with left ventricular systolic dysfunction which affects the mid-portions of the left ventricle. The etiology of the disease still remains unclear. Many theories have been put forward about the potential underlying pathophysiological mechanisms that may trigger this syndrome among which are the theory of catecholamine excess, the theory of multivessel coronary vasospasm, the ischemic theory, and the theory of microvascular dysfunction and dynamic left ventricular gradient induced by elevated circulating catecholamine levels. Adequate management of Tako-tsubo syndrome demands immediate preparation for coronary angiography. Once the diagnosis is made, treatment is primarily symptomatic and includes monitoring for complications. Patients with Tako-tsubo syndrome most frequently develop acute LV failure, pulmonary edema, rhythm and conductive disturbances and apical thrombosis. Treatment is symptomatic and includes administration of diuretics, vasodilators and mechanical support of circulation with intra-aortic balloon counterpulsation.


2021 ◽  
Author(s):  
Xue-Xue Zou ◽  
Wen-Li Chang ◽  
Heng-Xing Gao

Abstract Background: Primary cardiac osteosarcoma is an uncommon condition, which is challenging to diagnose, and rarely reported.Case presentation: Here, we present a previously healthy 27-year-old patient referred to our hospital with a long-term fever. Echocardiography and thoracic computed tomography (CT) presented two masses in the left atrium (LA) and left ventricle (LV), and surgical excision of the masses revealed cardiac high-grade osteosarcoma. Unfortunately, the left ventricular tumor recurred three months later, and the patient was administered periodic chemotherapy. Then, chest CT showed that the left ventricle was almost occupied by the tumor and also involved the left ventricular outflow tract, the left atrial appendage mass increased significantly, and multiple metastatic small nodules appeared in both lungs.The patient is still in follow-up.Conclusions: The prevalence of primary cardiac osteosarcoma is very low and did not involve LA and LV simultaneously. This patient was hospitalized in our hospital complaining of a long-term fever of unknown origin, which has never been reported in the previous literatures. Our case report findings suggest that primary cardiac osteosarcoma should not be ignored in the differential diagnosis of fever of unknown origin.


2017 ◽  
pp. 94-97
Author(s):  
D. A. Doroshenko ◽  
A. R. Zubarev ◽  
O. B. Lapochkina

The article presents the main pathophysiological mechanisms associated with the development of complications of pregnancy against the background of arterial hypertension in patients without clinical manifestations of left ventricular dysfunction, the possibilities of speckle tracking of echocardiography in revealing latent systolic dysfunction of the left ventricle in pregnant women on the background of preeclampsia not accompanied by proteinuria are shown.Goal. To identify early markers of left ventricular systolic dysfunction in pregnant women with preeclampsia without proteinuria according to speckle tracking echocardiography.Material and methods. 100 pregnant women with hypertension and a gestation period of 29–40 weeks are divided into 2 groups: 1 st group – 50 patients without an arterial hypertension in the anamnesis, 2 nd group – 50 patients with an arterial hypertension in the anamnesis (without the proteinuria during pregnancy). For all patients, echocardiography with evaluation of cardiomyocyte function by speckle-tracking was performed on ultrasonic scanners Aplio ™ 500 and Aplio ™ Artida from Toshiba.Results. Ejection fraction of the left ventricle in the groups was not significantly different, while the longitudinal, radial and circular deformation parameters differed between the groups, with the lowest values characterizing the global longitudinal deformation.Conclusions. In assessing the function of the left ventricle in pregnant women with preeclampsia without diurnal proteinuria, it is not enough to focus on traditional parameters, shifting the emphasis towards derivatives of force, speed and time. Study of myocardium with the help of speckle tracking echocardiography promotes the detection of subclinical left ventricular dysfunction in patients with arterial hypertension without daily proteinuria. 


1970 ◽  
Vol 4 (1) ◽  
pp. 96-98
Author(s):  
SDM Taimur ◽  
SR Khan ◽  
MR Karim ◽  
MH Rahman

Rupture sinus of Valsalva is a relatively rare condition. We report a rare case of ruptured left sinus of valsalva with aneurysm, presenting with acute left ventricular failure. Transthoracic echocardiography showed an aneurysmal dilatation of the base of interventricular septum and part of the aortic root and a marked turbulent flow from the aortic root to the left ventricle with a continuous systolodiastolic shunting. The patient underwent successful repair of ruptured sinus of valsulva with closure of fistula. During Ventriculotomy the defect was repaired using pledgeted 5/ 0 prolene interrupted sutures and pericardial patch. Key Words: Rupture sinus of valsalva; Aneurysm; Left ventricle DOI: http://dx.doi.org/10.3329/cardio.v4i1.9398 Cardiovasc. J. 2011; 4(1): 96-98


2016 ◽  
Vol 22 (1) ◽  
pp. 31-36
Author(s):  
Ol’ga N. Dzhioeva ◽  
E. V Kartashova ◽  
I. I Zakharova ◽  
A. V Melekhov ◽  
G. E Gendlin

The left ventricular non-compaction is a disease characterizing by hyper-trabecularization of myocardium causing development of chronic heart failure. In the development of structural damages under this pathology the main role belongs to genetic disorders and dysfunction of organogenesis in early periods of gestation. The clinical manifestations are associated with development of systolic dysfunction of left ventricle, intricate abnormalities of rhythm and thromboembolic occurrences. In patients with systolic dysfunction of left ventricle phenomenon of hyper-trabecularization of myocardium is quite often found during transthoracic echocardiography. The modern diagnostic criteria permit approaching differentially issues of verification of non-compact myocardium and redundant trabecularization in patients with chronic heart failure with reduced fraction of output of left ventricle.


Sign in / Sign up

Export Citation Format

Share Document