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

https://doi.org/10.25040/lkv2021.03-04.082

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

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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.

References

  1. Dudnik SV, Koshelya II. Diseases of the circulatory system as a socio-medical problem. Ukraine. Health of the Nation. 2017;3:320-321. (Ukrainian)
  2. Ipatov AB, Moroz OM, Gondulenko NO, Khanyukova IYa, Mamatiev AO, Sanina NA et al. The main indicators of disability and the activities of medical and social expert commissions of Ukraine for 2018: analytical and information guide. Dnipro: Accent of PE; 2019. 180 p. (Ukrainian)
  3. Kovalenko VM, Lutai MI, Sirenko UM, Sychov OS, ed. Cardiovascular diseases. Classification, standards of diagnosis and treatment. 4th edition, revised. and ext. : Morion; 2020. 247 p. (Ukrainian)
  4. Lazoryshynets VV, Kovalenko VM, Fedkiv SV, Potashev SV, Rudenko AV, Vitovsky RM et al. The use of multimodal cardiovascular imaging in patients with pericardial disease: practical recommendations. Ukrainian Journal of Cardiovascular Surgery. 2020;3(40):107-126. (Ukrainian) https://doi.org/10.30702/ujcvs/20.4009/107-126.18.09.2020
  5. Malinovsky NN, Platov AI. Pericardial malformations. Surgery. 2001;5:4-9. (Ukrainian)
  6. Ringach NO, Lushchik LV. Regional features of loss of years of potential life due to premature mortality from the main causes in Ukraine. Demography and Social Economy. 2018;3(34):39-55. (Ukrainian) https://doi.org/10.15407/dse2018.03.039
  7. Sidorenko PI, Bondarenko GO, Kuts SO. Human anatomy and physiology. 4th ed., ed. K.: Medicine; 2012. 199 p. (Ukrainian)
  8. Terenda NO. Death rate from cardio-vascular diseases as a state problem. Bulletin of Scientific Research. 2015;4:11-13. (Ukrainian)
  9. Cherkasov VG, Kravchuk SY. Human anatomy: textbook. way. Vinnytsia: New Book; 2011. 639 p. (Ukrainian)
  10. Avoidable mortality: OECD/Eurostat lists of preventable and treatable causes of death. 2019.
  11. Abbas AE, Appleton CP, Liu PT, Sweenet JP. Congenital absence of the pericardium: case presentation and review of literature. Int J Cardiol. 2005;98(1):21-25. https://doi.org/10.1016/j.ijcard.2003.10.021
  12. Adler Y, Charron P, Imazio M, Badano L, Barón-Esquivias G, Bogaert J et al. 2015 ESC Guidelines for the diagnosis and management of pericardial diseases. The Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology (ESC) Endorsedby: The European Association for Cardio-Thoracic Surgery (EACTS). Eur Heart J. 2015;36(42):2921-2964. https://doi.org/10.1093/eurheartj/ehv318
  13. Hundley WG, Bluemke DA, Finn JP, Flamm SD, Fogel MA, Friedrich MG et al. ACCF/ACR/AHA/NASCI/ SCMR 2010 expert consensus document on cardiovascular magnetic resonance: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents. J Am Coll Cardiol. 2010;55(23): 2614-2662. https://doi.org/10.1016/j.jacc.2009.11.011
  14. Bogaert J, Francone M. Pericardial disease: valueof CT and MR imaging. Radiology. 2013;267(2):340-356. https://doi.org/10.1148/radiol.13121059
  15. Bremerich J, Reddy GP, Higgins CB. Magnetic resonance imaging of cardiac structure. In: Imagingin Cardiovascular Disease. Pohost GM, O’Rourke RA, Berman D, Shah PM, editors. USA: Lippincott Williams&Wilkins, Philadelphia, PA; 2000.
  16. Cipriani A, Brunetti G, BernardinelloV, Baritussio A. Congenital Pericardial Agenesis. Expert Analysis. Am Col Cardiol. 2020. Availableat: https://www.acc.org/Latest-in-Cardiology/Articles/2020/07/27/09/25/Congenital-Pericardial-Agenesis
  17. Boxall R. Incomplete pericardial sac; escape of hear tin to left pleural cavity. Trans Obstet Soc Lond. 1887;28:209-210.
  18. Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R. Isolatednon-compaction of left ventricular myocardium: a study of eight cases. Circulation. 1990;82(2):507-513. https://doi.org/10.1161/01.CIR.82.2.507
  19. Connolly HM, Click RL, Schattenberg TT, Seward JB, Tajik AJ. Congenital absence of theperi cardium: echocardiography as a diagnostic tool. J Am Soc Echocardiogr. 1995;8(1):87-92. https://doi.org/10.1016/S0894-7317(05)80362-1
  20. Elbadawi A, Baig B, Alotaki E, Khalid H, Khan M. Congenital absence of the pericardium Arareand challenging diagnosis. JAAPA. 2019;32(5);37-39. https://doi.org/10.1097/01.JAA.0000554223.33382.c0
  21. Ellis K, Leeds NE, Himmelstein A. Congenital deficiencie sinthe parietal pericardium: a review with 2 new cases including successful diagnosis by plain rentgenography. Am J Roentgenol Radium Ther Nucl Med. 1959;82(1):125-137.
  22. Ekeke CN, Daniels C, Raman SV, Hitchcock C, Katz SE, Crestanello JA. Annulo aortice ctasiain a patient with congenital absence of the left pericardium. Ann Thorac Surg. 2013;96(6):2234-2235. https://doi.org/10.1016/j.athoracsur.2013.04.106
  23. Foo JS, Koh CH, Sahlén A,Tang HC, Lim CP. Congenital Partial Absence of Pericardium: A Mimic of Arrhythmogenic Right Ventricular Cardiomyopathy. Case Rep Med. 2018;2018:4297280. https://doi.org/10.1155/2018/4297280
  24. Hirono K, Hata Y, Nakazawa M, Momoi N, Tsuji T, Matsuoka T et al. Clinical and Echocardiographic Impactof Tafazzin Variantson Dilated Cardiomyopathy Phenotypein Left Ventricular Non-Compaction Patientsin Early Infancy. Circ J. 2018;82(10):2609-2618. https://doi.org/10.1253/circj.CJ-18-0470
  25. His W. Arch Fur Anatu. Entwick lungs gesch. Leipzig; 1881. P. 303-392.
  26. Ichida F, Hamamichi Y, Miyawaki T, Ono Y, Kamiya T, Akagi Tetal. Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background. J Am Coll Cardiol. 1999;34(1):233-240. https://doi.org/10.1016/S0735-1097(99)00170-9
  27. Imazio M, Cecchi E, Demichelis B, Chinaglia A, Ierna S, Demarie D et al. Myopericarditis versus viral or idiopathic acute pericarditis. Heart. 2008;94(4):498-501. https://doi.org/10.1136/hrt.2006.104067
  28. Imazio M, Spodick DH, Brucato A, Trinchero R, Adler Y. Controversial issues in the management of pericardial diseases. Circulation. 2010;121(7):916-928. https://doi.org/10.1161/CIRCULATIONAHA.108.844753
  29. Kenneth R. Congenital foramen of the left pericardium. Ann Thorac Surg. 2000;70(3):993-998. https://doi.org/10.1016/S0003-4975(00)01287-X
  30. Khan IA, Biddle WP, Najeed SA, Abdul-Aziz S, Mehta NJ, Salaria V et al. Isolatednon-compaction cardiomyopathy presenting with paroxysmal supraventricular tachycardia: case report and literature review. 2003;54(2):243-350. https://doi.org/10.1177/000331970305400216
  31. Klein AL, Abbara S, Agler DA, Appleton CP, Asher CR, Hoit B et al. American Society of Echocardiography Clinical Recommendations for Multimodality Cardiovascular Imaging of Patients with Pericardial Disease: Endorsed by the Society for Cardiovascular Magnetic Resonance and Society of Cardiovascular Computed Tomography. J Am Soc Echocardiogr. 2013;26(9):965-1012. https://doi.org/10.1016/j.echo.2013.06.023
  32. Kumor M, Lipczyńska M, Biernacka EK, Klisiewicz A, Wójcik A, Konka M et al. Cardiac arrest and ventricular arrhythmia in adult swith Ebsteinanomal yand left ventricular non-compaction. J Cardiol. 2018;71(5):484-487. https://doi.org/10.1016/j.jjcc.2017.11.012
  33. Ling LH, Oh JK, Tei C, Click RL, Breen JF, Seward JB et al. Pericardial thickness measured with trans esophageal echocardiography: feasibility and potential clinical usefulness. J Am Coll Cardiol. 1997;29(6):1317-1323. https://doi.org/10.1016/S0735-1097(97)82756-8
  34. Lubiszewska B, Hoffman P, Ruzyllo W. Isolated non-compaction of ventricular myocardium: case report and review of literature. Kardiol Pol. 2001;55:443-450.
  35. Maisch B, Seferovic PM, Ristic AD, Erbel R, Rienmuller R, Adler Y et al. Guidelines on the diagnosis and management of pericardial diseases executive summary; the Task Force on the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology. Eur Heart J. 2004;25(7):587-610. https://doi.org/10.1016/j.ehj.2004.02.002
  36. Nasser WK. Congenital diseases of the pericardium. Cardiovasc Clin. 1976;7(3):271-286.
  37. Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R. Long-termfollow-upof 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol. 2000;36(2):493-500. https://doi.org/10.1016/S0735-1097(00)00755-5
  38. Parekh JD, Iguidbashian J, Kukrety S, Guerins K, Millner PG, Andukuri V. A Rare Case of Isolated Left Ventricular Non-compactioninan Elderly Patient. Cureus. 2018;10(6):e2886. https://doi.org/10.7759/cureus.2886
  39. Richard P, Ader F, Roux M, Donal E, Eicher JC, Aoutil N et al. Targeted panels equencing in adult patients with left ventricular non-compaction reveals a large genetic heterogeneity. Clin Genet. 2019;95(3):356-367. https://doi.org/10.1111/cge.13484
  40. Ritter M, Oechslin E, Sutsch G, Schneider J, Jenni R. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc. 1997;72(1):26-31. https://doi.org/10.4065/72.1.26
  41. Robin E, Ganguly S, Fowler MS. Strangulation of the left atrial appenda get hrough a congenital partial pericardial defect. 1975;67(3):354-355. https://doi.org/10.1378/chest.67.3.354
  42. Roth GA, Mensah GA, Johnson CO, Addolorato G, Ammirati E, Baddour LM et al. Global Burden of Cardiovascular Diseases and Risk Factors, 1990-2019: Update Fromthe GBD 2019 Study. J Am Coll Cardiol. 2020;76(26):2982-3021. https://doi.org/10.1016/j.jacc.2020.11.010
  43. Shah AB, Kronzon I. Congenital defects of the pericardium: a review. Eur Heart J Cardiovasc Imaging. 2015;16(8):821-827. https://doi.org/10.1093/ehjci/jev119
  44. Sechtem U, Tscholakoff D, Higgins CB. MRI of the normalpericardium. AJR Am J Roentgenol. 1986;147(2):239-244. https://doi.org/10.2214/ajr.147.2.239
  45. Singh DP, Patel H. Left Ventricular Non-compaction Cardiomyopathy. 2020.
  46. Sunderland S, Wright-Smith RJ. Congenital pericardial defects. Br Heart J. 1944;6(4):167-175. https://doi.org/10.1136/hrt.6.4.167
  47. Troughton RW, Asher CR, Klein AL. Pericarditis. Lancet. 2004;363(9410):717-727. https://doi.org/10.1016/S0140-6736(04)15648-1
  48. Vizzardi E, Nodari S, Metra M, Dei Cas L. Non-compaction of the ventricular myocardium. Heart Int. 2006;2(3-4):178. https://doi.org/10.4081/hi.2006.178
  49. Wilson SR, Kronzon I, Machnicki SC, Ruiz CE. A constrained heart: a case of sudden on setun relenting chest pain. 2014;130(18):1625-1631. https://doi.org/10.1161/CIRCULATIONAHA.114.011410
  50. Xu B, Betancor J, Asher C, Rosario A, Klein A. Congenital absence of the pericardium: a systematic approach to diagnosis and management. Cardiology. 2017;136(4):270-278. https://doi.org/10.1159/000452441
  51. Yared K, Baggish AL, Picard MH, Hoffmann U, Hung J. Multimodality imaging of pericardial diseases. JACC Cardiovasc Imaging. 2010;3(6):650-660. https://doi.org/10.1016/j.jcmg.2010.04.009