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426 Congenital complete and partial absence of the left pericardium 1 3

Kongenitale komplete und partielle Absenz des Perikardiums

Zusammenfassung Das kongenitale Fehlen des Peri-kards ist eine seltene Fehlbildung. Wir zeigen diese Dia-gnose anhand der Kasuistiken zweier junger Männer. Bei der posteroanterioren Röntgendarstellung der Brust wurde eine Verschiebung der linken Herzgrenze in den linken Hemitorax festgestellt. Ungewöhnliche akusti-sche Fenster und abnormale Herz- und septale Bewe-gungen während der Echokardiographie wiesen auf die Diagnose der kongenitalen Absenz des Perikardiums hin. Die Magnetresonanzuntersuchungen bestätigten endgültig die Diagnose.

Schlüsselwörter: Perikard fehlend, Magnetresonanz-untersuchung, Herzfehler angeboren

Summary Congenital absence of pericardium is a rare malformation. We report 2 young patients with a diag-nosis of congenital absence of the pericardium. The pos-teroanterior view of the chest X-ray showed displace-ment of the left cardiac border into the left hemithorax. Unusual acoustical windows and abnormal cardiac and septal motion during echocardiography suggested the diagnosis of congenital absence of pericardium. Mag-netic resonance imaging definitive confirmed diagnosis of congenital absence of pericardium.

Keywords: Absence of pericardium, Congenital, Mag-netic resonance imaging, Congenital heart defects

Introduction

The congenital absence of pericardium is a rare malfor-mation. Most pericardial defects are partial and occur on the left side. Infrequently, defects also occur on the right side or at the diaphragmatic surface. Complete absence of the pericardium is exceedingly rare [1]. The clinical presentation is variable. Most often it is clinically silent, but may become manifest, particularly in partial absence. Herniation, incarceration or torsion of cardio-vascular structures (atria, atrial appendages, ventricles, great vessels) can impare cardiac filling and output [2]. Some patients may have paroxysmal chest pain.

Case 1

The patient is a 17-year-old female, with a history of hav-ing fainting episodes and non-frequent, short-lasting chest pain. She was referred to cardiology clinic for heart murmur. On auscultation, there was a short, 1/6 systolic murmur with the maximum in the 3rd intercostal space on the left. There was a negative T wave in all left pre-cordial leads. The posteroanterior view of the chest x-ray showed displacement of the left cardiac border to the left. Echocardiography showed unusual acoustical windows and abnormal cardiac and septal motion. We presumed a congenital absence of the left pericardium. Magnetic res-onance imaging (MRI) confirmed congenital complete absence of the left pericardium. Left-sided absence of the pericardium allows interposition of lung tissue between the aorta and the main segment of the pulmonary artery (Fig. 1).

Wien Med Wochenschr (2013) 163:426–428DOI 10.1007/s10354-013-0178-4

Congenital complete and partial absence of the left pericardiumAlexander Jurko, Milan Minarik, Viera Cisarikova, Hubert Polacek, Ingrid Schusterova

A. Jurko Jr., MD, PhD ()Pediatric Cardiology Clinic, Kollárova 13, 036 01 Martin, Slovakiae-mail: ajurko@zmail.sk

M. Minarik, MD, PhDDepartment of Anesthesia and Intensive Care, Zilina, Slovakia

V. Cisarikova, MD · H. Polacek, MDDepartment of Radiology, Comenius University, Jessenius School of Medicine, Martin, Slovakia

I. Schusterova, MDDepartment of Pediatrics, Children’s Faculty Hospital, Kosice, Slovakia

Received: 4 February 2011 / Accepted: 11 March 2011 / Published online: 5 February 2013© Springer-Verlag Wien 2013

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Congenital complete and partial absence of the left pericardium 4271 3

Case 2

The patient is a 20-year-old female, who was referred to the cardiologist after birth with suspected ASD, but echocardiography did not confirm the diagnosis. Clini-cally she has been without any problems. Later on, she was referred to the cardiology clinic again because of 2/6 systolic murmur with maximum in the 3rd intecos-tal space on the left. Her ECG was without abormalities. Chest x-ray showed marked displacement of the cardiac silhouette into the left hemithorax. Echocardiography confirmed characteristic motions of the interventricular septum and left ventricular posterior wall and displace-ment of acoustical window laterally. Congenital absence of the left pericardium was suspected. The definitive diagnosis of partial congenital absence of the left peri-cardium was confirmed by MRI (Fig. 2).

Discussion

The congenital absence of the pericardium is a rare mal-formation in which the pericardium is incomplete, in part or in total [3]. It results from faulty partitioning of the pleuropericardic cavity during the 5th week of develop-ment [4]. Congenital absence of the pericardium is a rare entity estimated to occur in one in 10,000–14,000 people [5]. The left side of the pericardium is most often absent, whereas absence of the right pericardium is less com-mon [6]. Absence of the inferior pericardium is associ-ated with diaphragmatic defects [3]. Complete absence of the pericardium is extremely rare [1, 7]. A family with absence of the left pericardium has been described [4]. Pericardial defects are rare in childhood and their out-

come is usually benign, but some cases of sudden death have even been reported in the literature [8]. The case of a 3-month-old girl with partial pericardial defect with atrial herniation has been described [9].

Absence of the pericardium is most often clinically asymptomatic, but may become manifest, particularly in partial absence [3]. Herniation, incarceration or torsion of cardiovascular structures (atria, atrial appendages, ventricles, great vessels) can impare cardiac filling and output [2, 9]. Rupture of the tricuspid valve chordae has been reported [10]. Some patients may have paroxysmal chest pain and clinical picture may suggest myocardial infarction [5]. Congenital absence of the pericardium should be known by clinicians as a possible differential diagnosis of chest pain or a pseudo-right heart overload [7]. Findings on physical examination and electrocardio-gram may be absent or non-specific. T wave abnormali-ties at standard electrocardiogram performed during a physical checkup have been reported [11]. The pos-teroanterior view of the chest x-ray may show displace-ment of the left cardiac border into the left hemithorax. Echocardiography may accurately identify abnormali-ties in myocardial wall motion and in cardiac silhouette. Unusual acoustical windows and abnormal cardiac and septal motion may strongly suggest the diagnosis [12]. MRI is the definitive modality for delineating the precise anatomy and diagnosis and can differentiate partial from complete absence of the left pericardium [13]. Left-sided absence of the pericardium allows interposition of lung tissue between the aorta and the main segment of the pulmonary artery.

Conflict of interest The authors declare that there is no conflict of interest.

Fig. 2 MRI—congenital absence of the left pericaridum (hol-low arrow)

Fig. 1 MRI—interposition of lung tissue (arrow) between the aorta and main segment of the pulmonary artery

428 Congenital complete and partial absence of the left pericardium

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