From the clinical spectrum of myocardial calcification to a cardiovascular imaging perspective.

We herein report the case of a 74-year-old man with recurrent transient ischemic attacks and falls who was found to have an unusual large mass of the interventricular septum. After extensive workup, isolated myocardial calcification was confirmed using multimodality cardiovascular imaging. This rare finding might be a reflecting cause of the patient's clinical presentation.

Myocardial calcification is associated with endocardial ablation failure of post-myocardial infarction ventricular tachycardia.

AIMS: In patients with post-myocardial infarction (post-MI) ventricular tachycardia (VT), the presence of myocardial calcification (MC) may prevent heating of a subepicardial VT substrate contributing to endocardial ablation failure. The aims of this study were to assess the prevalence of MC in patients with post-MI VT and evaluate the impact of MC on outcome after endocardial ablation. METHODS AND RESULTS: In 158 patients, the presence of MC was retrospectively assessed on fluoroscopy recordings in seven standard projections obtained during pre-procedural coronary angiograms. Myocardial calcification, defined as a distinct radiopaque area that moved synchronously with the cardiac contraction, was detected in 30 patients (19%). After endocardial ablation, only 6 patients (20%) with MC were rendered non-inducible compared with 56 (44%) without MC (P = 0.033) and of importance, 8 (27%) remained inducible for the clinical VT [compared with 9 (6%) patients without MC; P = 0.003] requiring therapy escalation. After a median follow-up of 31 months, 61 patients (39%) had VT recurrence and 47 (30%) died. Patients with MC had a lower survival free from the composite endpoint of VT recurrence or therapy escalation at 24-month follow-up (26% vs. 59%; P = 0.003). Presence of MC (HR 1.69; P = 0.046), a lower LV ejection fraction (HR 1.03 per 1% decrease; P = 0.017), and non-complete procedural success (HR 2.42; P = 0.002) were independently associated with a higher incidence of VT recurrence or therapy escalation. CONCLUSION: Myocardial calcification was present in 19% of post-MI patients referred for VT ablation and was associated with a high incidence of endocardial ablation failure.

Myocardial Calcification and the Demise of an Infant With Surgically Treated Hypoplastic Left Heart Syndrome.

A term female infant with hypoplastic left heart syndrome underwent Norwood palliation including aortic and pulmonary amalgamation with arch reconstruction, atrial septectomy, and right ventricle to pulmonary artery conduit. Postoperatively, she experienced hypoxemia and lactic acidosis although echocardiogram showed adequate conduit function. She was placed on veno-arterial extracorporeal membrane oxygenation (ECMO) on postoperative day two with improvement. ECMO decannulation was attempted with subsequent cardiac arrest and ultimate failure to resuscitate, eleven days after surgery. Autopsy confirmed clinical findings and evidence of surgical intervention with a patent conduit and neo-aorta. Multiple subendocardial right ventricular dystrophic calcifications involving the outflow tract were identified grossly and histologically with foci of associated myonecrosis. Myocardial calcification may lead to abnormal heart wall motion by increasing rigidity and compromising myocyte function or compromising the conduction system. In this patient, right ventricular turbulence caused by systolic and diastolic flow patterns, including mild tricuspid regurgitation, may have played a role in inducing dystrophic calcification along with surgery and ECMO dependence. Compromised myocyte function from calcifications, right ventricular hypertrophy, lung immaturity, and persistent pulmonary hypertension were likely sources of cardiac strain leading to the patient's demise. This case represents a previously unreported complication of hypoplastic left heart syndrome treatment.

A case of bi-ventricular extensive calcification caused by multiple factors.

BACKGROUND: Extensive myocardial calcification has a low incidence rate, but when the patients do have extensive myocardial cases, the prognosis is usually poor. Several sepsis-related extensive myocardial calcification cases have been reported, but there are cases of biventricular calcifications that are caused by multiple cases besides bacteremia and the treatment for it has a low percentage of success. CASE PRESENTATION: A 9 year old girl had an extensive biventricular calcification which is caused by multiple factors including multiple organ failure (heart, lung, liver, and kidney), aseptic cardiomyopathy, systemic inflammatory response syndrome, pulmonary hemorrhage, viral encephalitis. In this case study, the massive myocardial calcification present in the patient was classified as dystrophic. After the patient was transferred to the Intensive care unit, a series of rescue treatments such as anti-inflammatory factor storm were implemented to protect the organs. In the end, the patient was rescued from the rescue treatment procedure. After 18 months of follow-up, it was observed that the patient's heart function returned to normal and it was observed that there was no change in myocardial calcification in the patient. CONCLUSION: In this case study, it showcased a case of the diffused biventricular calcification that caused by multiple factors. Furthermore, the precise role of calcification on cardiac function was largely unknown and there has to be further follow-up observation on the patient.

Diffuse myocardial calcification in a drug addict, complicating advanced life support.

Myocardial calcification is a rare echocardiographic finding, which is often found in patients who have suffered from a myocardial infarction. Rarely, myocardial calcification may be present in individuals without a significant medical history. Until today, there has not been a published case report emphasising the relation between myocardial calcification and cocaine usage. Herein, we report a case of a young male with diffuse calcification of the left ventricular myocardium, who had an addiction to cocaine. This case highlights an uncommon etiology for myocardial calcification and it may lead to further studies about cardiotoxic effects of cocaine.

Myocardial calcification: case reports and a systematic review.

Aims: Myocardial 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 results: This 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. Conclusion: While 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.

An Autopsy Case of Fulminant Myocarditis with Massive Left Ventricular Calcification.

Myocardial calcification in myocarditis is rare and may be linked to poor outcomes. We herein report a case of fulminant myocarditis with massive myocardial calcification and its pathological outcomes at autopsy. A 49-year-old man experienced chest pain and was diagnosed with acute myocarditis. His cardiac function did not recover despite mechanical circulatory support in combination with V-A extracorporeal membrane oxygenation and IMPELLA CP®. He eventually developed sepsis and gastrointestinal bleeding and died on day 27. Diffuse myocardial calcification was observed on computed tomography at autopsy. The pathological autopsy depicted that calcification filled every myocardial cell in the left ventricle.

Hearts of stone: Rapidly progressive left ventricular myocardial calcifications in severe sepsis: Case series.

Rapidly progressive left ventricular myocardial calcification (RPLVMC) is a rare phenomenon of severe sepsis which is associated with long-term complications like irreversible cardiomyopathy and arrhythmias. To date, only 19 cases have been reported in literature. We present a case series of two patients with RPLVMC which manifested within a period of days in the setting of severe sepsis. Unique to previous case reports, the patients in the current case series had no pre-existing systemic risk factors such as end-stage kidney disease or endocrinological dysfunction. This case series aims to increase awareness of RPLVMC in severe sepsis, improve its opportunistic detection on routine medical imaging (namely chest x-ray and computed tomography), and spur future research to develop potential prevention strategies.

Myocardial Calcification: An Unusual Complication of Remote Trauma With Coidentified Amyloidosis.

We present the heart of a 38-year-old man with paraplegia due to a remote traumatic spinal cord injury. In the 20 years following his injury, he experienced chronic decubitus ulcers, osteomyelitis, neurogenic bladder, malnutrition, and urinary tract infections. He was admitted to the hospital with septic shock secondary to multiple decubitus ulcers and osteomyelitis and expired after a two-month hospitalization. At autopsy, there was marked replacement of left ventricle and interventricular septal myocardium by gritty, firm, yellow-white tissue. Microscopic examination demonstrated a remote infarct with marked dystrophic calcification and unexpected amyloid deposition. This example demonstrates the extraordinary extent to which dystrophic calcifications can replace myocardium and highlights multiple potential etiologies of myocardial calcifications. Of note, this is the first report documenting myocardial calcification as a complication of remote, non-iatrogenic trauma. The role of the amyloidosis in the development of calcification is unclear, but a contributory effect cannot be excluded.

An Intriguing Case of Myocardial Deposits.

Cardiac calcification refers to calcium deposits in the coronary arteries, heart valves, pericardium, or myocardium. Calcium deposition within the myocardium is unique and can be secondary to metastatic or dystrophic calcification. Both forms are linked to cardiac abnormalities and poor prognosis. The most common causes include myocardial infarction, sepsis, myocarditis, renal failure, and hyperparathyroidism. Here, we report the case of a 74-year-old male who was found to have gallbladder adenocarcinoma with subsequent preoperative workup indicating possible metastases to the myocardium. With the use of multimodality imaging, particularly cardiac MRI, the differentiation between metastatic disease and intramyocardial calcification was made. The case aims to highlight the complexity of diagnosing and managing myocardial calcifications and underscores the need for further research into their etiology and implications.

Novel echocardiographic pixel intensity quantification method for differentiating transthyretin cardiac amyloidosis from light chain cardiac amyloidosis and other phenocopies.

AIMS: Differentiating cardiac amyloidosis (CA) subtypes is important considering the significantly different therapies for transthyretin (ATTR)-CA and light chain (AL)-CA. Therefore, an echocardiographic method to distinguish ATTR-CA from AL-CA would provide significant value. We assessed a novel echocardiographic pixel intensity method to quantify myocardial calcification to differentiate ATTR-CA from phenocopies of CA and from AL-CA, specifically. METHODS AND RESULTS: 167 patients with ATTR-CA (n=53), AL-CA (n=32), hypertrophic cardiomyopathy (n=37), and advanced chronic kidney disease (n=45) were retrospectively evaluated. The septal reflectivity ratio (SRR) was measured as the average pixel intensity of the visible anterior septal wall divided by the average pixel intensity of the visible posterior lateral wall. SRR and other myocardial strain-based echocardiographic measures were evaluated with receiver operator characteristic analysis to evaluate accuracy in distinguishing ATTR-CA from AL-CA and other forms of left ventricular hypertrophy. Mean septal reflectivity ratio (SRR) was significantly higher in the ATTR-CA cohort compared to the other cohorts (p <0.001). SRR demonstrated the largest AUC (0.91, p<0.0001) for distinguishing ATTR from all other cohorts and specifically for distinguishing ATTR-CA from AL-CA (AUC=0.90, p<0.0001, specificity 96%, sensitivity 63%). There was excellent inter- and intra-operator reproducibility with an ICC of 0.91 (p <0.001) and 0.89 (p <0.001), respectively. CONCLUSION: The SRR is a reproducible and robust parameter for differentiating ATTR-CA from other phenocopies of CA and specifically ATTR-CA from AL-CA.

Extensive myocardial calcifications: a systematic literature review of a rare pathological phenomenon.

Introduction: Myocardial calcifications (MC) represent a relatively rare pathological process, which may accompany different cardiovascular conditions and can be broadly categorized as dystrophic or metastatic. Myocardial infarction (MI) has been traditionally regarded as the main cause of MC overall; however, no updated comprehensive data on the relative incidence of different forms of MC is available. The purpose of this systematic review of the literature is to analyze the currently available evidence on MC in terms of pathophysiology, diagnosis, and clinical presentation. Methods and results: A total of 241 studies including a total of 368 patients affected by extensive MC were included in the final review. The majority of patients (69.8%) presented with dystrophic MC. Endomyocardial fibrosis (EMF) represents the single most common etiology of MC (24.2%), while sepsis/acute systemic inflammatory syndrome (SIRS) and chronic kidney disease were identified as the second and third most common causes respectively. The relative incidence of etiologies also varies across the years, with MI being more represented before 1990, and sepsis/SIRS becoming the single most common cause of MC after 1990. Multimodality imaging was used in the work-up of MC in 42.7% of cases. The most commonly employed imaging modality overall was echocardiography (51.9%), while after 1990 computed tomography scan became the most widely used tool (70.1%). Conclusion: The present systematic review provides new insights into the pathophysiology of MC. Previously thought to be mainly a consequence of ischemic heart disease, our data indicate that other diseases, namely EMF and sepsis/SIRS, are indeed the main conditions associated with MC. The importance of multimodality imaging in the work-up of MC is also highlighted.

Ultra-early myocardial calcification secondary to fulminant myocarditis with 4 years of follow-up: a case report.

Background: Early myocardial calcifications secondary to fulminant myocarditis (FM) are rare, and their natural evolution and effects on cardiac function are poorly understood. Here, we followed the patient for 4 years to observe the development of cardiac calcification and its impact on heart function. Case summary: A 16-year-old man was hospitalized with a fever and cough for 1 day. The patient was previously healthy and had no history of heart disease or specific family conditions. The patient was positive for anti-Epstein-Barr virus IgG and IgM. The computed tomography (CT) scan showed no coronary lesions. Cardiogenic shock and recurrent ventricular fibrillation developed on the third day after admission, and the patient received rescue therapy such as endotracheal intubation, defibrillation, extracorporeal membrane oxygenation, and corticosteroids. On the 13th day of admission, a CT scan revealed significant calcification in the left ventricular wall. The patient was discharged after 30 days in the hospital. After discharge, his left ventricular calcification peaked at 6 months and gradually subsided after that, and his left ventricular function slowly returned to normal at 12 months. Discussion: In younger patients, myocardial calcifications secondary to FM may occur as early as 13 days and affect cardiac function. After proper treatment and rehabilitation, the patient's myocardial calcification can gradually subside and the cardiac function can gradually recover. For FM patients, timely and comprehensive intensive treatment, including heart, lung, and kidney replacement therapy and early administration of hormone preparations, may be beneficial to the early recovery of patients.

Case report: Catheter ablation for persistent atrial fibrillation in a patient with heart of stone.

Myocardial calcification is a rare condition, with only a few reports in the literature. For the first time, we report a case of diffuse myocardial calcification who underwent successful catheter ablation for persistent atrial fibrillation (AF). In this case, catheter ablation was recommended due to repeated hospitalization for palpitation and heart failure, but preoperative computed tomography showed massive myocardial calcification. Electroanatomic mapping of the atrium was performed with a Pentaray catheter before ablation, which showed areas of low voltage in the calcified region. As the persistent AF was terminated after circumferential pulmonary vein isolation and posterior wall isolation, and no further ablation was performed. The patient recovered well, with no recurrence of palpitation or heart failure during the one-year follow-up.

Pacemaker lead insertion sites contribute to abnormalities of myocardial function and histopathology.

BACKGROUND: Ventricular pacing can cause myocardial dysfunction, but how lead anchoring to the myocardium affects function has not been studied. OBJECTIVE: The purpose of this study was to evaluate patterns of regional and global ventricular function in patients with a ventricular lead using cine cardiac computed tomography (CCT) and histology. METHODS: This was a single-center retrospective study with 2 groups of patients with a ventricular lead: (1) those who underwent cine CCT from September 2020 to June 2021 and (2) those whose cardiac specimen was analyzed histologically. Regional wall motion abnormalities on CCT were assessed in relation to lead characteristics. RESULTS: For the CCT group, 122 ventricular lead insertion sites were analyzed in 43 patients (47% female; median age 19 years; range 3-57 years). Regional wall motion abnormalities were present at 51 of 122 lead insertion sites (42%) in 23 of 43 patients (53%). The prevalence of a lead insertion-associated regional wall motion abnormality was higher with active pacing (55% vs 18%; P < .001). Patients with lead insertion-associated regional wall motion abnormalities had a lower systemic ventricular ejection fraction (median 38% vs 53%; P < .001) than did those without regional wall motion abnormalities. For the histology group, 3 patients with 10 epicardial lead insertion sites were studied. Myocardial compression, fibrosis, and calcifications were commonly present directly under active leads. CONCLUSION: Lead insertion site-associated regional wall motion abnormalities are common and associated with systemic ventricular dysfunction. Histopathological alterations including myocardial compression, fibrosis, and calcifications beneath active leads may explain this finding.

Rapidly progressive myocardial calcification following sepsis.

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. .

Petrified heart in a patient with Wolfram Syndrome.

Myocardial calcification is a rare complication of severe systemic sepsis. The pathophysiological mechanisms underlying calcium accumulation in cardiomyocytes in generalized sepsis are complex and still under investigation. It is a serious and life-threatening condition, usually diagnosed by autopsy, rarely with imaging techniques. We describe the case of a 38-year-old woman with Wolfram Syndrome who developed diffuse myocardial calcification during an episode of generalized sepsis.