Mastocytosis presenting as cardiac emergency
Erminia Ridolo1, Massimo Triggiani, Marcello Montagni
1Department of Clinical and Experimental Medicine, University of Parma, via Gramsci 14, 43100, Parma, Italy, erminia.ridolo@unipr.it.
Internal and Emergency Medicine
|October 19, 2013
Summary
Systemic mastocytosis can present as cardiac emergencies like arrest or ventricular fibrillation, especially without typical skin signs. Elevated serum tryptase after such events may indicate mastocytosis.
Area of Science:
- Cardiology
- Hematology
- Immunology
Background:
- Mastocytosis involves mast cell proliferation and mediator release.
- A subset of patients lack typical skin lesions (urticaria pigmentosa).
- Anaphylaxis can be the initial presentation in these cases.
Observation:
- Three male patients presented with cardiac emergencies (cardiac arrest/ventricular fibrillation).
- None had prior anaphylaxis, mediator symptoms, or significant cardiovascular conditions.
- An eliciting factor for anaphylaxis was identified in one case.
Findings:
- Cardiac emergencies can be the first clinical manifestation of systemic mastocytosis.
- Elevated serum tryptase levels, confirmed basally, can aid in diagnosing mastocytosis following cardiac events.
Implications:
- Highlights cardiovascular emergencies as potential initial signs of systemic mastocytosis.
- Suggests measuring serum tryptase in patients experiencing acute cardiac events to identify underlying mastocytosis.
- Emphasizes the importance of considering mastocytosis in unexplained anaphylaxis presenting with cardiac emergencies.
Related Concept Videos
01:30Introduction Cardiac Emergencies
569
Cardiac emergencies are critical situations involving the heart that require immediate medical intervention to prevent severe complications or death. These emergencies often arise from underlying heart conditions that impair the heart's ability to function correctly.Types of Cardiac EmergenciesThe most common types of cardiac emergencies include Acute Coronary Syndrome (ACS), myocardial infarction (MI), cardiac arrest, and heart failure.Acute Coronary Syndrome (ACS)Acute Coronary Syndrome (ACS)...
569
01:25Cardiomyopathy I: Introduction and Classification
837
Cardiomyopathy, or CMP, is a group of diseases affecting the myocardial structure, impairing its ability to pump blood effectively. This condition can lead to arrhythmias, heart failure, or sudden cardiac death.Cardiomyopathies are classified into primary and secondary categories:Primary Cardiomyopathy refers to conditions involving only the heart muscle that are often idiopathic (of unknown cause) or genetic. They primarily affect the myocardium without the involvement of other systemic...
837
01:29Cardiomyopathy III: Hypertrophic Cardiomyopathy
812
Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
812
01:30Cardiomyopathy II: Dilated Cardiomyopathy
802
Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
802
01:30Acute Coronary Syndrome I: Introduction
2.1K
Acute Coronary Syndrome (ACS) encompasses a spectrum of heart conditions caused by sudden obstruction of coronary arteries, typically resulting from the rupture of an atherosclerotic plaque and subsequent thrombus (blood clot) formation. This obstruction can lead to partial or complete blockage of blood flow, causing varying degrees of myocardial ischemia or infarction.ACS includes the following clinical entities:Unstable Angina (UA)Non-ST-Elevation Myocardial Infarction (NSTEMI)ST-Elevation...
2.1K
01:27Myocarditis II: Clinical Features and Diagnostic Tests
497
Myocarditis is an inflammation of the heart muscle. The symptoms vary widely, encompassing asymptomatic presentations to severe, acute manifestations.Clinical PresentationAsymptomatic cases: In some instances, myocarditis may be asymptomatic, with the infection resolving without intervention. These cases often go undetected unless discovered incidentally through diagnostic imaging or tests conducted for other reasons.General Early Symptoms: Early symptoms of myocarditis are non-specific and can...
497