Congenital absence of pericardium: a nomad heart

Liliana Marta1, António M Ferreira1, Katya R Santos1

  • 1Hospital da Luz,Lisbon,Portugal.

Cardiology in the Young
|November 11, 2014
PubMed

Insights

Congenital pericardial absence, a rare heart defect, is often asymptomatic but can lead to serious complications. Early diagnosis through imaging is crucial for managing this condition.

Area of Science:

  • Cardiology
  • Medical Imaging
  • Genetics

Background:

  • Congenital pericardial absence is a rare cardiac anomaly.
  • While often asymptomatic, it carries risks of severe complications.

Purpose of the Study:

  • To report a case of congenital pericardial absence diagnosed in an asymptomatic individual.
  • To highlight the importance of diagnostic imaging for this rare condition.

Main Methods:

  • Case report of a 33-year-old woman.
  • Diagnostic investigation prompted by family history of sudden cardiac death.
  • Review of imaging modalities for diagnosis.

Main Results:

  • Diagnosis of congenital absence of the pericardium confirmed.
  • The patient was asymptomatic at the time of diagnosis.

Conclusions:

  • Congenital pericardial absence requires clinical recognition despite asymptomatic presentation.
  • Imaging plays a vital role in diagnosing this rare cardiac malformation.

Related Concept Videos

Pericarditis I: Introduction01:22

Pericarditis I: Introduction

Pericarditis is defined as the inflammation of the pericardium, the thin, sac-like membrane surrounding the heart. This condition can cause significant chest pain and other symptoms, often necessitating medical intervention. The pericardium has two layers: the inner visceral layer and the outer parietal layer, separated by a small amount of fluid that reduces friction during heartbeats.Types of PericarditisPericarditis can be classified into several types based on the duration and nature of the...
714
Location and Orientation of the Heart01:13

Location and Orientation of the Heart

The human heart, despite its modest size and weight, is an organ of remarkable strength and endurance. Roughly the size of a fist, the heart weighs between 250 and 350 grams and is nestled within the mediastinum, the medial cavity of the thorax. It extends obliquely for about 12 to 14 cm, resting on the superior surface of the diaphragm. The heart is positioned anterior to the vertebral column and posterior to the sternum, with two-thirds of its mass lying to the left of the midsternal line.
15.3K
Development of the Heart01:27

Development of the Heart

The development of the human heart, a crucial organ, commences from the mesoderm on the 18th or 19th day after fertilization. This process initiates in the cardiogenic area, a group of mesodermal cells at the embryo's head end, which evolves into elongated strands known as cardiogenic cords. These cords undergo a transformation to form hollow-centered endocardial tubes.
As the embryo undergoes lateral folding, these paired tubes approach each other, merging into a single primitive heart...
4.0K
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

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
Pericarditis II: Clinical Features and Diagnostic Tests01:19

Pericarditis II: Clinical Features and Diagnostic Tests

Pericarditis is distinguished by inflammation of the pericardium, the fibrous sac that encases the heart. It can be acute, lasting less than six weeks, or chronic, persisting for over three months. Understanding its clinical manifestations and diagnostic findings is crucial for timely and effective management.Clinical ManifestationsWhile pericarditis can be asymptomatic, it usually presents with characteristic symptoms such as:Chest Pain: The most characteristic symptom of pericarditis is chest...
599
Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
999