Methodist Journal

IN THIS ISSUE

Nutritional Supplements and the Heart

Vol 15, Issue 3 (2019)


FEATURED GUEST EDITOR

ISSUE INTRO

Dietary Supplements: Facts and Fallacies

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RECOGNITIONS

Drs. Raizner and Cooke Take the Lead in Special Issue on Supplements

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REVIEW ARTICLES See More

Recent Clinical Trials Shed New Light on the Cardiovascular Benefits of Omega-3 Fatty Acids

Supplemental Vitamins and Minerals for Cardiovascular Disease Prevention and Treatment

Coenzyme Q10

Red Yeast Rice for Hypercholesterolemia

Inorganic Nitrate Supplementation for Cardiovascular Health

Vitamin D and Calcium Supplements: Helpful, Harmful, or Neutral for Cardiovascular Risk?

Cardiovascular Risk of Proton Pump Inhibitors

Advanced Cardiac Imaging for Complex Adult Congenital Heart Diseases

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

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

CLINICAL PERSPECTIVES See More

POINTS TO REMEMBER

Herbal Nephropathy

EXCERPTA

Rolling the Dice on Red Yeast Rice

POINTS TO REMEMBER

The Kidney in Congenital Cyanotic Heart Disease

EXCERPTA

Talking Statins with Antonio Gotto

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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