Cardiac Eclipse: Congenital Absence of the Pericardium Manifesting as Atypical Chest Pain

Ignaszewski, Maya; Baturin, Brian; Waldman, Brett; Peters, Priscilla J.

doi:10.1016/j.case.2019.07.005

Cited by 4 publications

(18 citation statements)

References 7 publications

(27 reference statements)

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“…Consistent with our case, left complete CAP is most common 1 with a male predominance. 2 However, the median age at diagnosis is much younger (21 years 4 and 48 years 3 in 2 case series).…”

Section: Discussionsupporting

confidence: 92%

“…Advanced imaging (computed tomography/magnetic resonance imaging) is suggested once CAP is suspected. 1 , 3 The most consistent finding is interposed lung parenchyma between the aorta and the main pulmonary artery ( Fig. 2 B).…”

Section: Discussionmentioning

confidence: 59%

“…CAP is rare, with an estimated prevalence of 0.007%–0.044%. 1 The pericardium may be completely (left, right, or both) or partially (left or right) absent. 2 , 3 During embryogenesis, the pleuropericardial folds fail to fuse, possibly due to an atrophic left common cardiac vein or cardiac enlargement before fusion completion.…”

Section: Discussionmentioning

confidence: 99%

“… 2 However, the median age at diagnosis is much younger (21 years 4 and 48 years 3 in 2 case series). Most patients are asymptomatic, 1 with CAP being detected incidentally during imaging or surgery. 2 Symptoms are nonspecific, including chest pain (most commonly), palpitations, syncope, or dyspnea 2 , 3 and can have confounders, such as COPD as in our case.…”

Section: Discussionmentioning

confidence: 99%

“…Symptoms may be due to the absence of normal pericardial cardiac cushioning, torsion of great vessels, and vascular compression of coronary arteries or pulmonary veins with hemodynamic collapse. 1 , 3 …”

Section: Discussionmentioning

confidence: 99%

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It’s Never Too Late: Spotting Congenital Absence of the Pericardium in an Older Adult

et al. 2020

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A 75-year-old male with a cardiopulmonary history presented with chest pain and dyspnea. He was hypertensive. An electrocardiogram showed paced rhythm. A high-sensitivity test showed his troponin T level was minimally elevated. Coronary angiography results were unremarkable. Chest radiography revealed an elevated cardiac apex, previously attributed to cardiomegaly. Echocardiography revealed a teardrop shaped heart in a nonstandard apical window. Computed tomography confirmed congenital absence of the left pericardium. Challenges of recognizing a rare condition are highlighted. Congenital absence of the pericardium, an often benign but rarely catastrophic condition, can masquerade for decades before diagnosis, underlining the importance of clinical vigilance in evaluating common cardiac complaints.

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“…Consistent with our case, left complete CAP is most common 1 with a male predominance. 2 However, the median age at diagnosis is much younger (21 years 4 and 48 years 3 in 2 case series).…”

Section: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 59%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 3 more Smart Citations

It’s Never Too Late: Spotting Congenital Absence of the Pericardium in an Older Adult

et al. 2020

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Complete Congenital Absence of the Left Pericardium in Elderly Patient: A Case Report

Mekonnen,

Farris,

Azmeraw

2024

IMCRJ

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Congenital pericardial absence is an uncommon heart condition that is usually undiagnosed due to a lack of symptoms and awareness. In this case study, we present a 72-year-old patient who initially presented for medical evaluation due to unexplained weight loss, a displaced cardiac apex, and poor echocardiographic windows. An extracardiac tumor was suspected at first, but it was later determined that the patient had a congenital complete absence of the left pericardium. This case illustrates the possibility for benign pathology to present abnormally and details the clinical features, imaging findings, and management of congenital absence of the pericardium.

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Multimodality imaging and clinical significance of isolated congenital absence of the pericardium

et al. 2021

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Background. Congenital absence of the pericardium is a rare anomaly characterized by variable and non-specific clinical manifestations. The prevalence of this pathology in the population is approximately 0.0001 – 0.044% and it is almost three times more common in men. However, the real prevalence, including asymptomatic and undiagnosed cases, may be higher. Purpose – highlighting the echocardiographic and tomographic signs of isolated congenital absence of the pericardium. Materials and methods. A 42-year-old patient M. Echocardiography did not permit to obtain standard echo images via typical echocardiographic approaches due to poor acoustic window. Magnetic resonance imaging and computed tomography of the heart revealed marked left heart rotation and confirmed the most common variant of congenital absence of the pericardium, that is complete left-sided absence pericardial agenesis. Results. The presented case illustrates the non-specificity of clinical symptoms in patients with congenital absence of the pericardium. The electrocardiogram of the patient showed right axis deviation, right bundle branch block, poor R wave progression in the precordial leads. Chest X-ray in the anterior-posterior projection typically shows a pronounced displacement of cardiac silhouette to the left without deviation of the trachea, elongated left border of the heart and inability to delineate the right border, indicating left heart rotation. Echocardiography revealed an unusual orientation of the heart in the chest, i.e. the displacement of the heart (particularly, of the apical segments) to the left and backwards. In cases when the pericardium is absent on the right and on both sides, the right ventricle can indeed be enlarged, sometimes there is a paradoxical motion of the interventricular septum, severe tricuspid regurgitation as well as the swinging motion of the cardiac apex. Magnetic resonance imaging and computed tomography of the heart enable detection of the displacement of the heart to the left half of the chest with cardiac apex directed backwards, the interposition of lung tissue between the ascending aorta and the pulmonary artery trunk. Conclusions. Congenital absence of the pericardium is a rare pathology and thus it can be challenging in terms of diagnosis, while sometimes it remains undiagnosed. The combination of echocardiography with magnetic resonance imaging and/or computed tomography allows to accurately diagnose congenital absence of the pericardium and assess the risks associated with it. Most patients with complete unilateral or bilateral congenital absence of the pericardium have a favorable prognosis and do not require treatment.

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Cardiac Eclipse: Congenital Absence of the Pericardium Manifesting as Atypical Chest Pain

Cited by 4 publications

References 7 publications

It’s Never Too Late: Spotting Congenital Absence of the Pericardium in an Older Adult

It’s Never Too Late: Spotting Congenital Absence of the Pericardium in an Older Adult

Complete Congenital Absence of the Left Pericardium in Elderly Patient: A Case Report

Multimodality imaging and clinical significance of isolated congenital absence of the pericardium

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