Lviv clinical bulletin 2021, 3(35)-4(36): 82-90

Absence of Pericardium in Combination with Myocardial Noncompactness (Literature Review; Clinical Case Description)

N. Ilenkiv1, Z. Bilous2, O. Abrahamovych2, M. Abrahamovych2, N. Mazur1, R. Ivanochko2

1Clinical Hospital of Lviv Railway

2Danylo Halytsky Lviv National Medical University

Introduction. Cardiovascular diseases (CVD) in all epidemiological indicators continue to hold a leading position not only in Ukraine but also around the world and are a global medical and social problem. In the first place, such positions are provided by coronary heart disease (CHD) – the most common variant of SSC, despite the significant advances in modern clinical medicine.

At the same time, sometimes the verification of the diagnosis is delayed or it is not possible to establish it during life, which contributes to the hyper- or hypodiagnosis of the most common nosologies, forgetting about diseases that are extremely rare.

The aim of the study. To make the review of the literature and the description of a clinical case for the purpose of clarification of features of a clinical condition and diagnostics at patients with congenital absence of a pericardium in combination with noncompactness of a myocardium.

Materials and methods. Content analysis, method of system and comparative analysis, bibliosemantic method of studying current scientific researches concerning studying of congenital absence of pericardium in combination with noncompactness of myocardium are used. Sources were searched in scientometric databases: PubMed, Medline, Springer, Google Scholar, Research Gate by keywords: congenital absence of pericardium, not myocardial compactness. 51 sources in English and Ukrainian were selected and analyzed, which covered the epidemiology of congenital absence of the pericardium and myocardial compactness, their clinical and diagnostic features; described a clinical case.

Results. Congenital absence of the pericardium is a rare congenital anomaly of the pericardium, which, depending on the extent of the defect is left-handed (from 0.0001 % to 0.044 % in the population and in 70.0 % of all cases of congenital absence of the pericardium), right-handed and total (9.0 % of all cases) is more common in men than in women, respectively, as 3.0:1.3.

During embryonic development, both the cardiac and pulmonary rudiments, the beginning of the formation of which begins in 3-4 weeks, are displaced from the cervical region into the thoracic cavity, going to the pericardial and pleural cavities, respectively. Premature atrophy of the left cuvier duct leads to non-separation of the pericardial cavity from the left pleural cavity. Due to these reasons, the absence of the left half of the pericardium is the most common. If pleuropericardial folds are not formed, the rudiments of the heart and lungs are in a single pleuropericardial cavity.

Quite often, congenital absence of pericardium is associated with other congenital heart defects, for example, with a defect of the atrial septum (MPP), open ductus arteriosus, tetrad E.-L. Fallot, mitral valve stenosis, with defects of the diaphragm, lungs, kidneys.

Most cases of this defect are asymptomatic and may not be diagnosed for life, so they can often be confused with other diseases such as heart aneurysm, coronary heart disease, mitral valve or atrial septal defects.

Some informative signs may appear during X-ray diagnosis (radiograph may show convexity of the left upper border of the heart, high position of the heart), but the main emphasis in the diagnosis is on echocardiography (Echo-CG) (enlarged pancreas and right atrium), significant regurgitation tricuspid valve), magnetic resonance imaging (MRI), and the gold standard is the so-called multimodal imaging using multislice computed tomography (MSCT) (no visualization of the pericardial layer, rotation of the heart to the left, interposition of the pulmonary artery and lung tissue). At the same time, any of these techniques may have more or less pronounced shortcomings, which sometimes make it difficult to diagnose pericardial abnormalities.

Myocardial noncompactness is a genetic malformation whose prevalence is not high, although its final prevalence cannot be established, as it is not uncommon for such individuals to have an asymptomatic course.

On the ECG, myocardial noncompactness may be accompanied by supraventricular and ventricular arrhythmias, blockades of varying degrees, and one of the most accurate methods of visualizing myocardial noncompactness is Echo-CG. During this examination, two layers of the myocardium are visualized: subepicardial with a compact myocardium and subendocardial with a non-compact myocardium, and an important diagnostic and prognostic feature is the ratio of these layers at the end of systole.

Conclusions. A review of the literature and described a clinical case of pericardial absence in combination with myocardial infarction.

Elucidation of clinical features of absence of a pericardium in combination with incompatibility revealed that this anomaly is usually asymptomatic, however at patients with a left defect can be disguised under an ischemic heart disease, followed by ventricular arrhythmias (ventricular tachycardia), lengthening of a Q interval.

Of particular value for the diagnosis of congenital absence of the pericardium in combination with myocardial infarction is Echo-CG and MRI, and the gold standard is considered to be MSCT, but these methods can sometimes have limited diagnostic capabilities.


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