Idiopathic Myocardial Helical Calcification.
Ontology highlight
ABSTRACT:
SUBMITTER: Tominaga O
PROVIDER: S-EPMC7932816 | biostudies-literature | 2020 Sep
REPOSITORIES: biostudies-literature
Publications
Idiopathic Myocardial Helical Calcification.
Tominaga Osamu O Teshima Eiichi E Nakashima Atsuhiro A Tominaga Ryuji R
Circulation reports 20200910 10
Similar Datasets
Project description:Myocardial calcification is rare and occurs in previous myocardial infarction, endomyocardial fibrosis, and infections such as tuberculosis, chronic renal failure, or hyperparathyroidism. We present an interesting case of massive myocardial calcification of the left ventricle following prior extensive myocardial infarction, presenting as progressive heart failure.
| S-EPMC5067855 | biostudies-literature
Project description: Not available
| S-EPMC9024093 | biostudies-literature
Project description:Sepsis is a clinical syndrome caused by a dysregulated host response to infection that can lead to multiple organ dysfunction and death. Cardiovascular abnormalities are frequent in sepsis and may result in myocardial injury unrelated to coronary artery disease. Myocardial calcification is a rare complication of sepsis, which shows rapid-onset extensive myocardial calcifications. We present a case of a 67-year-old man who developed severe sepsis complicated with shock, acute renal failure, and acute respiratory distress syndrome. Initial chest computed tomography (CT) on admission showed normal left ventricular (LV) myocardial attenuation. However, serial chest CT demonstrated a gradual increase of the LV myocardial attenuation, which ultimately resulted in extensive myocardial calcification within 6 weeks. Sepsis-related myocardial calcification is usually found in patients with severe sepsis complicated with hemodynamic failure requiring vasopressors, acute renal failure necessitating renal replacement therapy, and acute respiratory distress syndrome. Although the prognostic significance of this pathology is unclear, it may be a precursor to long-term irreversible cardiomyopathy or an arrhythmogenic substrate that induces life-threatening arrhythmias. Therefore, patients who have survived the acute phase of severe sepsis need to be monitored carefully for signs of this complication by an imaging modality such as CT. <Learning objective: Sepsis is common and may cause myocardial injury. Myocardial calcification is an uncommon complication of sepsis, usually found in patients with severe sepsis. Sepsis-related myocardial calcification is classified as dystrophic, and represents the sequelae of local tissue damage and cellular necrosis. With sepsis-related myocardial calcification, rapid-onset extensive myocardial calcifications can develop within the course of 1 to 3 months. Patients who have survived the acute phase of severe sepsis need to be monitored carefully for signs of this complication.>.
| S-EPMC8888720 | biostudies-literature
Project description: Not available
| S-EPMC8471177 | biostudies-literature
Project description: Not available
| S-EPMC3491130 | biostudies-literature
Project description: Not available
| S-EPMC2082956 | biostudies-literature
Project description: Not available
| S-EPMC11329167 | biostudies-literature
Project description:AimsMyocardial calcification is an unusual condition in which excess calcium is deposited in the myocardium. Herein, we report two cases of myocardial calcification from our clinical experience. Furthermore, we conduct a systematic review to examine the clinical course and associated pathologies of myocardial calcification.Methods and resultsThis systematic review was registered in PROSPERO (CRD42023463285). PubMed and Scopus were searched according to the following inclusion criteria: (i) case reports or case series describing patients with myocardial calcification; (ii) diagnosis of myocardial calcification by computed tomography (CT); (iii) adequate description of patients, including their chief complaint, medical history, evaluations, and treatments; and (iv) publication in English. Among the 75 patients, 24 had sepsis, 14 had myocarditis, and 37 had other pathologies. The mortality rate was 33% for patients with sepsis, 14% for patients with myocarditis, and 11% for patients with other pathologies. Follow-up CT findings beyond 2 years were reported in six patients, showing that the CT findings of myocardial calcification persisted but subsided over time. Autopsy was performed in seven patients, and extensive interstitial fibrosis and collection of inflammatory cells were observed in patients with myocarditis, sepsis, and ischaemic heart disease.ConclusionWhile various medical conditions can cause myocardial calcification, accompanying conditions commonly reported with myocardial calcification were sepsis and myocarditis. The CT findings of myocardial calcification tend to regress over time if the underlying disease can be treated.
| S-EPMC11367960 | biostudies-literature
Project description:BackgroundCardiac calcification is a type of ectopic pathologic calcification of unknown etiology and mechanisms. Once diagnosed, the location, extent and morphology of the calcified lesions, as well as their functional significance in the heart, are usually the focus of case reports. Calcification is mostly distributed in myocardium, but rarely reported in atrium. In addition, because of limited sampling and complex pathological mechanisms, the etiology underlying the formation of these calcified lesions also remains unclear.MethodsTwo cardiac calcifications were found in a patient, died of trauma-induced subarachnoid hemorrhage after slightly drinking, during a standard autopsy. The location and morphological characteristics of the calcified lesions were determined by computed tomography (CT) and CT-based 3D reconstruction. The specific histopathological characteristics of the lesions were determined by multi-staining. The concentration of free calcium and inorganic pyrophosphate (PPi) in plasma reflected the change of calcium metabolism. The expression and membranal localization of the ATP Binding Cassette Subfamily C Member 6 (ABCC6) in hepatocytes were detected by immunofluorescence. The variants of the ABCC6 were detected by Sanger sequencing and potential pathogenic variants were further identified by in silico analysis.ResultsThe present study describes a patient with idiopathic calcification with two pear-shaped and irregularly hollow lesions symmetrically distributed in the patient's atrium. Massive accumulation of calcium salts was identified by multi-staining. For this patient, the plasma concentration of free calcium was higher than the control, indicating that calcium metabolism was disturbed. Furthermore, the plasma PPi of the patient was lower than the normal. By using immunofluorescence, the expression and membranal localization of ABCC6 was decreased and impaired in hepatocytes, respectively. Combined with Sanger sequencing and in silico analysis, 7 variants were identified.ConclusionsThis study described a novel patient with symmetrically distributed idiopathic atrial calcifications. Furthermore, all the results indicated that these pathologic calcifications may be secondary to reduced plasma PPi content due to ABCC6 dysfunction in hepatocytes. Moreover, these findings provided novel clues to the pathogenesis, clinical diagnosis and treatment of idiopathic atrial calcification in future.
| S-EPMC8978529 | biostudies-literature
Project description:Inogranic pyrophosphate (PPi) inhibits hydroxyapatite deposition, and mice deficient in the PPi-generating nucleoside triphosphate pyrophosphohydrolase (NTPPPH) Plasma cell membrane glycoprotein-1 (PC-1) develop peri-articular and arterial calcification in early life. In idiopathic infantile arterial calcification (IIAC), hydroxyapatite deposition and smooth muscle cell (SMC) proliferation occur, sometimes associated with peri-articular calcification. Thus, we assessed PC-1 expression and PPi metabolism in a 25-month-old boy with IIAC and peri-articular calcifications. Plasma PC-1 was <1 ng/ml by enzyme-linked immunosorbent assay in the proband, but 10 to 30 ng/ml in unaffected family members and controls. PC-1 functioned to raise extracellular PPi in cultured aortic SMCs. However, PC-1 was sparse in temporal artery lesion SMCs in the proband, unlike the case for SMCs in atherosclerotic carotid artery lesions of unrelated adults. Proband plasma and explant-cultured dermal fibroblast NTPPPH and PPi were markedly decreased. The proband was heterozygous at the PC-1 locus, and sizes of PC-1 mRNA and polypeptide, and the PC-1 mRNA-coding region sequence were normal in proband fibroblasts. However, immunoreactive PC-1 protein was relatively sparse in proband fibroblasts. In conclusion, deficient extracellular PPi and a deficiency of PC-1 NTPPPH activity can be associated with human infantile arterial and peri-articular calcification, and may help explain the sharing of certain phenotypic features between some IIAC patients and PC-1-deficient mice.
| S-EPMC1850320 | biostudies-literature