Methodist Journal

FEATURED GUEST EDITOR

ISSUE INTRO

The Burgeoning Field of Cardio-Oncology

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RECOGNITIONS

Barry H. Trachtenberg Leads Issue on Cardio-Oncology

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REVIEW ARTICLES See More

Heart Failure in Relation to Anthracyclines and Other Chemotherapies

Heart Failure in Relation to Tumor-Targeted Therapies and Immunotherapies

The Role of Cardiovascular Imaging and Serum Biomarkers in Identifying Cardiotoxicity Related to Cancer Therapeutics

Prevention and Treatment of Chemotherapy-Induced Cardiotoxicity

Cardiovascular Toxicities of Radiation Therapy

Electrophysiologic Complications in Cancer Patients

Vascular Toxicity in Patients with Cancer: Is There a Recipe to Clarify Treatment?

Future Directions in Cardio-Oncology

CASE REPORTS See More

A Rare Case of Pancreatitis-Induced Thrombosis of the Aorta and Superior Mesenteric Artery

Anomalous Origin of the Right Coronary Artery from the Left Main Coronary Artery in the Setting of Critical Bicuspid Aortic Valve Stenosis

Simultaneous Transfemoral Mitral and Tricuspid Valve in Ring Implantation: First Case Report with Edwards Sapien 3 Valve

Uneventful Follow-Up 2 Years after Endovascular Treatment of a High Flow Iatrogenic Aortocaval Fistula Causing Pulmonary Hypertension and Right Heart Failure

MUSEUM OF HMH MULTIMODALITY IMAGING CENTER See More

Do Not Pass Flow: Microvascular Obstruction on Cardiac Magnetic Resonance After Reinfarction Following Primary Percutaneous Coronary Intervention

CLINICAL PERSPECTIVES See More

EXCERPTA

Cardio-Oncology, Then and Now: An Interview with Barry Trachtenberg

POINTS TO REMEMBER

Onconephrology: An Evolving Field

POINTS TO REMEMBER

Herbal Nephropathy

EXCERPTA

Rolling the Dice on Red Yeast Rice

EDITORIALS

Letter to the Editor in Response to “Cardiac Autonomic Neuropathy in Diabetes Mellitus”

Vol 15, Issue 3 (2019)

Article Full Text

MUSEUM OF HMH MULTIMODALITY IMAGING CENTER

Snoopy’s Heart: A Case of Complete Congenital Absence of the Pericardium

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Article Citation:

Vosko I, Zhan Y, Nabi F, Zoghbi WA. Snoopy’s Heart: A Case of Complete Congenital Absence of the Pericardium. Methodist DeBakey Cardiovasc J. 2019;15(3):226-7.



Keywords
congenital absence of the pericardium , adult congenital heart disease , ACHD , echocardiography , computed tomography

A 52-year-old male presented to our facility with dyspnea and dizziness. His past medical history was significant for smoking and hyperlipidemia. An electrocardiogram (ECG) showed a normal sinus rhythm, a rightward axis, low voltage, and poor R-wave progression (Figure 1 A). Transthoracic echocardiography revealed an abnormal atrial-ventricular axis; however, it was difficult to image the heart from the usual parasternal long axis because the apex was shifted laterally and posteriorly and the atria was elongated (Figure 1 B). In addition, the chest x-ray displayed levoposition of the heart with an obscured right heart border, an elongated and flattened left heart border (“Snoopy sign”),1 and a radiolucency between the main pulmonary artery and aorta (Figure 1 C).

Consequently, the patient underwent computed tomography (CT) to determine if there was a congenital absence of the pericardium (CAP). The CT confirmed the leftward displacement of the heart and the absence of the pericardial layer (Figure 1 D). An interposition of lung tissue between the main pulmonary artery and the aorta—a characteristic sign for CAP—could be seen in the axial view (Figure 1 E).2

Congenital absence of the pericardium is a very rare disease that can be categorized as a complete or partial absence of the pericardium, with the latter having a higher risk for complications.3 Often found incidentally, CAP has a nonspecific clinical presentation, with possible symptoms being chest pain, dizziness, and shortness of breath. In our case, the diagnosis was suspected because of the unexplained shifted heart position in the chest.

Although CT and magnetic resonance imaging are the gold standard for diagnosing CAP,4 certain findings on an ECG, echocardiography, and chest x-ray can also indicate its presence.2 Nevertheless, the rarity of CAP can make it difficult to detect; therefore, clinicians should familiarize themselves with the different findings that can lead to a prompt diagnosis.

Figure 1. Congenital absence of the pericardium. (A) Electrocardiogram demonstrating rightward axis, low voltage, and poor R-wave progression due to leftward shift of the heart and precordial transition zone. (B) Transthoracic echocardiography, approached apical four-chamber view. Note the lateral position of the apex and the elongated right atrium (teardrop appearance). (C) Posterioranterior chest radiograph showing leftward displacement of cardiac silhouette, causing the right heart borders to be obscured, and an elongated heart border. There is a radiolucent space between the aorta and main pulmonary artery demonstrating interposed lung tissue (arrow). (D) Computed tomography (CT) showing the laterally displaced left ventricular apex. (E) CT showing the interposition of lung tissue between the main pulmonary artery and the ascending aorta (arrow).
References
  1. Panagiotopoulos N, Barnard J, Wells F. The Snoopy sign. Eur J Cardiothorac Surg. 2014 Mar;45(3):586.
  2. Lopez D, Asher CR. Congenital Absence of the Pericardium. Prog Cardiovasc Dis. 2017 Jan – Feb;59(4):398-406.
  3. Shah AB, Kronzon I. Congenital defects of the pericardium: a review. Eur Heart J Cardiovasc Imaging. 2015 Aug;16(8):821-7.
  4. Kim MJ, Kim HK, Jung JH, et al. Echocardiographic diagnosis of total or left congenital pericardial absence with positional change. 2017 Aug;103(15):1203-1209.

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