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. 2020 Jan 8;7(1):e001103.
doi: 10.1136/openhrt-2019-001103. eCollection 2020.

Case series, contemporary review and imaging guided diagnostic and management approach of congenital pericardial defects

Mohamed Khayata  1 , Saqer Alkharabsheh  2 , Nishant P Shah  2 , Beni Rai Verma  1 , James L Gentry  2 , Mathew Summers  2 , Bo Xu  2 , Craig Asher  3 , Allan L Klein  2
Affiliations

Case series, contemporary review and imaging guided diagnostic and management approach of congenital pericardial defects

Mohamed Khayata et al. Open Heart. .

Abstract

Objective: Congenital pericardial defect (CPD) is a rare entity with an estimated frequency of 0.01%-0.04%. The recognition of this anomaly is important since it can be associated with serious complications. The aim of this study and review was to describe clinical and imaging features that help in establishing the diagnosis of this condition.

Methods: We retrospectively reviewed all adult patients at the Cleveland Clinic Health System with the diagnosis of CPD between the years 2000 and 2015. Baseline clinical characteristics, clinical manifestations, ECG, transthoracic echocardiography (TTE), cardiac CT and cardiac magnetic resonance (CMR) images were reviewed.

Results: Eight patients were included in the study. Sixty-three percent of patients were males with mean age at diagnosis of 48 years, 63% had a partial pericardial defect on the left side and right ventricular (RV) dilation on TTE. Three patients had CMR. Levocardia was present in all CMRs. One patient had greater than 60° clockwise rotation and none of the CMRs showed ballooning of the left ventricular apex. One patient required surgical pericardioplasty. The remaining seven patients had a median follow-up of 17.3 months (5-144.9 months) and all remained asymptomatic.

Conclusion: CPDs are more likely to be partial on the left side and patients often have RV dilation on the TTE and levocardia on CMR. Most patients remain stable and do not require surgical intervention. TTE and CMR play an important role in making the diagnosis of this anomaly.

Keywords: MRI; ct scanning; echocardiography; pericardial disease.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Typical ECG of patient with absent pericardium showing right axis deviation, incomplete RBBB and right ventricular hypertrophy. RBBB, right bundle branch block.
Figure 2
Figure 2
Chest X-ray showing heart displacement in the left hemithorax and a sharp aortopulmonary window (arrow).
Figure 3
Figure 3
Apical four-chamber view showing RV dilation (A and B) and levoposition of the LV (arrow) (C). LV, left ventricular; RV, right ventricular.
Figure 4
Figure 4
Cardiac magnetic resonance imaging showing significant displacement of the heart into the left hemithorax with complete absence of the pericardium (A) (arrow) compared with normal pericardial position (B) (arrow). LV, left ventricular; RV, right ventricular.
Figure 5
Figure 5
Systematic approach for the diagnosis and management of patients with suspected congenital pericardial defect, incorporating multimodality cardiovascular imaging. CPD, congenital pericardial defect; CMR, cardiac magnetic resonance; CXR, Chest X-ray.
See this image and copyright information in PMC

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