Strangulation of the Left Atrial Appendage through a Congenital Partial Pericardial Defect

Robin, Erwin; Ganguly, Sunilendu N.; Fowler, M.

doi:10.1378/chest.67.3.354

Cited by 54 publications

(21 citation statements)

References 4 publications

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“…Those patients with small, partial left-sided defects have the greatest risk of complications, such as compression, herniation, and strangulation of cardiac chambers, great vessels, or coronary arteries [1]. Strangulation of the left atrial appendage, the left atrium, the right atrium, or a part of the left or right ventricle has been reported [6,7,8]. …”

Section: Discussionmentioning

confidence: 99%

Congenital Absence of the Pericardium: A Systematic Approach to Diagnosis and Management

Asher²,

Rosario³

et al. 2016

Cardiology

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Congenital absence of the pericardium (CAP) is a rare condition. Failure to recognize the clinical features of this condition can lead to incorrect and delayed diagnosis. Limited data are available regarding the optimal approach to diagnose and manage patients with suspected CAP. Due to the rare nature of CAP, this condition can present diagnostic and management dilemmas for clinicians. Using 3 cases of CAP as a framework, a clinically focused review on the diagnosis and management of CAP is presented. Clinicians will be provided with a systematic approach to evaluating patients with suspected CAP, incorporating key history, examination, and multimodality cardiovascular imaging investigations.

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Section: Discussionmentioning

confidence: 99%

Congenital Absence of the Pericardium: A Systematic Approach to Diagnosis and Management

Asher²,

Rosario³

et al. 2016

Cardiology

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“…Despite an extensive work-up, the etiology remained unclear. We considered several hypotheses to establish the relationship between the pericardial defect and myocardial necrosis: transient herniation of the left atrial appendage (4,6,16) or the LV (5), torsion of the great vessels due to cardiac hypermobility or impingement of a coronary artery by the edge of the pericardial defect (17,18). We also considered that these two conditions may be unrelated, and that the pericardial defect was solely an incidental finding.…”

Section: Discussionmentioning

confidence: 99%

Congenital absence of the pericardium presenting as acute myocardial necrosis

Brulotte

Roy

Larose

2007

Canadian Journal of Cardiology

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C ongenital absence of the pericardium is a rare condition (1). With contemporary imaging techniques, an increase of incidental pericardial defects has arisen. Significant concern comes from potential life-threatening complications. We report a case of congenital absence of the pericardium that was discovered in the throes of acute myocardial necrosis. Literature is reviewed, and investigative and therapeutic strategies are discussed. CASE PRESENTATIONA 49-year-old woman required medical attention for an episode of acute respiratory distress leading to syncope. She reported a history of uncontrolled asthma in the prior weeks, which led her to excessively self-medicate with inhaled beta-adrenergic agonists. She nevertheless had excellent exercise capacity. She never felt any chest, jaw or left arm discomfort, or had any palpitations. The patient was an active smoker, but did not have other coronary risk factors. Her past medical history included mild asthma, panic disorder and depression. She also described syncope of an unknown origin, which was investigated when she was 20 years of age. At that time, she was informed that she had a congenital cardiac anomaly, but she refused to undergo investigation and left the hospital against advice. Her physical examination was normal. The initial electrocardiogram (ECG) indicated sinus rhythm with diffuse T wave inversion and a prolonged QT interval (corrected QT 560 ms). Levels of creatine kinase (876 U/L; normal less than 165 U/L) and troponin T (0.17 μg/L; normal 0.0 μg/L to 0.02 μg/L) were elevated. A D-dimer test was normal. The patient was diagnosed with acute non-ST segment elevation myocardial infarction, treated with standard pharmacological therapies and transferred to the Quebec Heart Institute at Laval Hospital (Quebec, Quebec).Urgent selective coronary angiography revealed right dominance and angiographically smooth coronary arteries, with Thrombolysis In Myocardial Infarction (TIMI) grade 3 flow and normal blush. Ventriculography showed normal left ventricular ejection fraction (LVEF) and absence of mitral regurgitation, but a laterally displaced and vertically oriented heart. A follow-up ECG indicated the return of T waves to baseline, with slow R wave progression in the precordial leads; the QT interval also normalized. The chest x-ray ( Figure 1) was notable for leftward displacement of the heart and rectification of the left heart border, suggestive of pericardial absence. Echocardiography was technically challenging because of poor echogenicity, unusual heart orientation within the chest cavity and significant cardiac motion. The patient's regional LVEF was normal (55%).Cardiac magnetic resonance (CMR) imaging was performed to better define cardiac morphology and function. This confirmed the leftward position of the heart and found that the apex was orientated posteriorly (Figure 2). CMR imaging demonstrated the absence of pericardial recesses at the level of the great vessels. A lung was visible between the aorta and pulmonary artery, indicating the abs...

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“…Patients with complete absence of the pericardium are either asymptomatic or have non-specific chest pain and no treatment is needed. 3 Patients with partial absence of the pericardium, on the other hand, are at risk from possible symptomatic herniation of the left atrial appendage 10 or LV, 11,13 leading to fatal myocardial strangulation. Therefore, it is very important to differentiate partial from complete absence of the pericardium.…”

Section: Discussionmentioning

confidence: 99%

“…[2][3][4][5][6][7][8] In partial absence of the pericardium, the characteristic features are quite different from those of complete absence of the pericardium because partial absence is occasionally complicated with herniation of a cardiac structure. [9][10][11] We present a case of partial absence of the left pericardium that was diagnosed by delineating the pericardium with magnetic resonance imaging (MRI), although the chest X-ray and echocardiographic features indicated a complete absence. …”

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confidence: 99%

Magnetic Resonance Imaging Differentiated Partial From Complete Absence of the Left Pericardium in a Case of Leftward Displacement of the Heart

Yamano

Sawada

Sakamoto

et al. 2004

Circ J

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solated congenital absence of the pericardium is a rare malformation and can vary from partial to complete absence, occurring more often on the left than the right side. 1 The features of complete absence of the left pericardium on chest X-ray and echocardiography, which are mainly related to the displacement of the whole heart, have been described by many investigators. [2][3][4][5][6][7][8] In partial absence of the pericardium, the characteristic features are quite different from those of complete absence of the pericardium because partial absence is occasionally complicated with herniation of a cardiac structure. [9][10][11] We present a case of partial absence of the left pericardium that was diagnosed by delineating the pericardium with magnetic resonance imaging (MRI), although the chest X-ray and echocardiographic features indicated a complete absence. Case ReportA 44-year-old man was referred for the evaluation of atypical chest pain. The pain eased spontaneously after several minutes, but was triggered in the left lateral position and he felt no distress. His apical impulse was prominent and displaced leftward to the anterior axillary line in the fifth intercostal space and a grade I/VI systolic murmur was present in the pulmonary valve area. Electrocardiography revealed right-axis deviation (QRS axis: 106°) and Circulation Journal Vol.68, April 2004incomplete right-bundle branch block. The chest X-ray in the standing posteroanterior view demonstrated leftward displacement of the heart without tracheal deviation. The chest X-rays in the right and the left lateral positions showed unusual cardiac mobility in the leftward direction (Fig 1). In transthoracic echocardiography, the apical window was displaced laterally in the usual left lateral position. Each echocardiogram recorded at expiration is shown (Fig 2). The 2-dimensional echocardiogram in the Magnetic Resonance Imaging Differentiated Partial From Complete Absence of the Left Pericardium in a Case of Leftward Displacement of the HeartTetsuhiro Yamano, MD; Takahisa Sawada, MD; Kenzo Sakamoto, MD; Takeshi Nakamura, MD; Akihiro Azuma, MD; Masao Nakagawa, MDA 44-year-old man was referred to hospital for the evaluation of atypical chest pain. His chest X-ray showed leftward displacement of the heart. During echocardiography, the apical window displaced laterally in the usual left lateral position and characteristic motions of the interventricular septum and left ventricular posterior wall were recognized with postural alterations. We presumed a complete absence of the left pericardium. Magnetic resonance imaging (MRI), however, demonstrated a partial left-sided pericardium. The diagnosis was corrected to partial absence of the left pericardium and we have carefully followed up this case without surgical prophylactic intervention. It is very important to differentiate partial from complete absence of the pericardium, because only in patients with partial absence of the pericardium is there a risk of fatal myocardial strangulation. The features of the c...

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Strangulation of the Left Atrial Appendage through a Congenital Partial Pericardial Defect

Cited by 54 publications

References 4 publications

Congenital Absence of the Pericardium: A Systematic Approach to Diagnosis and Management

Congenital Absence of the Pericardium: A Systematic Approach to Diagnosis and Management

Congenital absence of the pericardium presenting as acute myocardial necrosis

Magnetic Resonance Imaging Differentiated Partial From Complete Absence of the Left Pericardium in a Case of Leftward Displacement of the Heart

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