Congenital Absence of the Pericardium

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Tex Heart Inst J. 2005;32(2):241-3.
Complete left-sided absence of the pericardium in association with ruptured type A aortic dissection complicated by severe left hemothorax.
Nisanoglu V, Erdil N, Battaloglu B.
Department of Cardiovascular Surgery, University of Inonu, Turgut Ozal Medical Center, 44315 Malatya, Turkey.

We report an unusual clinical presentation of an acute type A aortic dissection as a left hemothorax in a patient with a congenital pericardial defect. Although the pericardial defect was diagnosed preoperatively, we could not exclude the possibility of a ruptured descending aorta until we discovered the site of the rupture during operation. The presence of a pericardial defect would at first appear to be a fatal disadvantage in such a situation as this, due to massive bleeding into the pleural space; but we believe that in our patient spontaneous drainage of blood into the pleural cavity prevented severe cardiac tamponade. The only reason for his deteriorating hemodynamic status was hypovolemia, which was corrected with volume replacement.

Birth Defects Res A Clin Mol Teratol. 2005 Sep;73(9):624-7.
Bilateral congenital diaphragmatic hernia with absent pleura and pericardium.
St Peter SD, Shah SR, Little DC, Calkins CM, Sharp RJ, Ostlie DJ.
Department of Pediatric Surgery, The Children's Mercy Hospital, Kansas City, Missouri.

BACKGROUND: Bilateral congenital diaphragmatic hernia is a rare form of diaphragmatic hernia. Independently, pericardial defects are an extremely rare phenomenon. In the case presented, we provide the first complete description of an infant with bilateral congenital diaphragmatic hernia with complete agenesis of the pericardium and inferior parietal pleura. CASE: A male infant was born at 38 weeks of gestation with a prenatal diagnosis of left-sided congenital diaphragmatic hernia. After 1 week of aggress ive management, the patient was taken to the operating room for repair. Intraoperatively, the patient was found to have absence of the diaphragm bilaterally, no pleura inferiorly, and no pericardium. A biological mesh was used to construct a diaphragm. At 6 months of age, the patient is growing normally, requiring only supplemental oxygen without pressure support. CONCLUSIONS: Embryologically, this anomaly represents complete lack of development of the pleurocardial folds, pleuroperitoneal folds, and tran sverse septum, which is previously unreported. Birth Defects Research (Part A), 2005. (c) 2005 Wiley-Liss, Inc.

Pediatr Surg Int. 2005 Jul;21(7):557-559. Epub 2005 Apr 26
Congenital absence of the pericardium associated with congenital diaphragmatic hernia and hepatic hemangioendothelioma: case report and review of the literature.
Tebruegge MO, Rennie JM, Haugen SE.
Department of Child Health, King's College Hospital, Denmark Hill, London, SE5 9RS, UK,

An unusual case of a neonate with absent pericardium, left-sided diaphragmatic hernia, and hepatic hemangioendothelioma is described. The posterolateral diaphragmatic hernia was successfully repaired, and agenesis of the pericardium did not interfere with cardiac function postoperatively. The hepatic tumour was a coincidental radiological finding, while liver function and the biochemical profile were normal. We also present a review of the literature on congenital absence of the pericardium and conclude t hat all cases should be screened for cardiac and hepatic anomalies.

Int J Cardiol. 2005 Jan;98(1):21-5.
Congenital absence of the pericardium: case presentation and review of literature.
Abbas AE, Appleton CP, Liu PT, Sweeney JP.
Division of Cardiovascular Diseases, Mayo Clinic, Scottsdale, AZ 85259, USA.

Congenital absence of the pericardium is an uncommon finding that may or may not be symptomatic. Asymptomatic patients are discovered incidentally during cardiac surgery for an unrelated condition or postmortem. However, symptomatic patients may experienc e non-exertional paroxysmal stabbing chest pain. It may occur with other cardiac or extracardiac abnormalities and a variety of imaging modalities may identify the condition. Complete cases are more rare than partial effects. However, complications are mo re common with partial absence due to strangulation of the heart into the defect thus requiring surgical intervention.

J Heart Valve Dis. 2004 Sep;13(5):790-1.
Isolated congenital tricuspid insufficiency associated with right-sided congenital partial absence of the pericardium.
Chatzis AC, Giannopoulos NM, Sarris GE.
Department of Pediatric and Congenital Cardiac Surgery, Onassis Cardiac Surgery Center, Athens, Greece.

A unique case of successfully treated congenital tricuspid insufficiency (CTI) associated with partial absence of the right pericardium is described, and the clinical implications are discussed.

Eur J Cardiothorac Surg. 2004 Oct;26(4):848-9.
Comment in: Eur J Cardiothorac Surg. 2005 Apr;27(4):728-9; author reply 729.
Tricuspid valve repair in a case with congenital absence of left thoracic pericardium.
Goetz WA, Liebold A, Vogt F, Birnbaum DE.
Department of Cardiothoracic Surgery, University Hospital, 93042 Regensburg, Germany.

We present a case of severe tricuspid valve insufficiency because of disruption of the anterior tricuspid leaflet with congenital absence of left thoracic pericardium. Findings suggest that tricuspid valve disruption was a result of distorted right ventri cular geometry because of luxation of the heart into left thoracic cavity. Tricuspid valve could be repaired by reinsertion of anterior tricuspid leaflet and De-Vega annuloplasty. Normal hemodynamic was obtained and weaning from cardio pulmonary bypass wa s possible by lifting the heart in orthotopic position using increased positive end expiratory pressure. Postoperative course was uneventful.

Tex Heart Inst J. 2004;31(1):87-9.
Congenital absence of anterior pericardium at left ventricular assist device implantation.
Messner GN, Prendergast P, Wadia Y, Chu T, Gregoric ID, Flamm SD, Frazier OH.
Department of Cardiopulmonary Transplantation, Texas Heart Institute, St. Luke's Episcopal Hospital, Houston, Texas 77030, USA.

We report a case of congenital absence of the anterior pericardium in a 41-year-old man who was undergoing implantation of a left ventricular assist device for treatment of congestive heart failure.

Rev Port Cardiol. 2003 Jun;22(6):801-10.
Erratum in: Rev Port Cardiol. 2003 Oct;22(10):1272.
Congenital absence of left pericardium.
Victor AR, Osorio P, Matos P, de Oliveira LM, Carrageta M.
Servico de Cardiologia, Hospital Garcia de Orta, Almada.

The authors present a clinical case of right chamber dilatation in a 34-year-old patient with no symptoms, investigation of which led to a diagnosis of congenital absence of left pericardium, established by magnetic resonance imaging (MRI). They also pres ent a review on absence of the pericardium, and discuss associated congenital abnormalities, the clinical presentations and findings in diagnostic tests, with particular emphasis on MRI.

Am J Perinatol. 2003 Apr;20(3):121-3.
Congenital partial pericardial defect in a neonate: a case report.
Puvabanditsin S, Garrow E, Biswas A, Davydov A, Gomez M, Liangthanasarn P.
Department of Pediatrics, Jersey City Medical Center, New Jersey 07304, USA.

This report describes a neonate with congenital partial absence of the pericardium associated with hypoplastic left heart syndrome. The patient presented with severe heart failure caused by a hypoplastic left ventricle. The infant died at 15 hours of age because of intractable congestive heart failure. Necropsy revealed, in addition to a hypoplastic left ventricle, a transverse biventricular constriction suggestive of partial absence of the pericardium. In addition to cardiovascular malformations, a left hydronephrosis and a bicornuate uterus was found. We report a rare clinical entity and review the literature.

Int J Cardiovasc Imaging. 2002 Feb;18(1):67-73.
Congenital absence of pericardium revisited.
Faridah Y, Julsrud PR.
Department of Radiology, Mayo Clinic, Rochester, MN 55905, USA.

Although much have been published regarding congenital absence of pericardium, it is essential that this anomaly, like an old friend, be revisited from time to time. Review of this anomaly with emphasis on its embryological process is discussed. Furthermo re, with the advances in magnetic resonance imaging, absence of pericardium can now be diagnosed with ease and the radiological findings of this condition are reviewed as well.

J Sports Med Phys Fitness. 2002 Mar;42(1):89-91.
Partial absence of pericardium in an endurance athlete. A case report.
Enad JG.
Department of Orthopaedic Surgery, Naval Medical Center, Portsmouth, Virginia 23708, USA.

A twenty-nine-year-old competitive endurance runner was noted to have a congenital absence of the pericardium on a routine screening exam. No recommendations for activity restrictions have been previously described for this condition. After maximal exerci se performance on a cardiac stress test without symptoms of cardiac compromise and consultation with cardiologists and cardiothoracic surgeons, the patient was returned to full, unrestricted competitive running. Once the diagnosis of absent pericardium is confirmed by imaging modalities, cardiac stress testing may be employed to determine a recommended activity level for the patient.

Singapore Med J. 2001 Feb;42(2):78-9.
Diastolic pressure alternans: a new sign in congenital absence of the pericardium.
Shah RP.
Department of Cardiology, Penang General Hospital, Malaysia.

Congenital absence of the pericardium is a rare condition,which is frequently missed due its subtle presentation. It may be misdiagnosed as another condition causing right heart dilatation such as an intracardiac shunt.We report the finding of diastolic p ressure alternans during cardiac catheterization in this single case report of a patient with congenital total absence of the pericardium.The occurrence of this phenomenon is hypothesized to be due to excessive cardiac hypermobility and paradoxical septal movement.We propose that this finding may be a useful clue to the diagnosis

Jpn Circ J. 2001 Jan;65(1):56-9.
Congenital giant aneurysm of the left atrial appendage mimicking pericardial absence case report.
Kunishima T, Musha H, Yamamoto T, Aoyagi H, Kongoji K, Imai M, Ozawa A, So T, Nagashima J, Murayama M.
Department of Cardiology, Yokohama Seibu Hospital, St Marianna University, Japan.

A 25-year-old man was found to have an abnormal cardiac contour on a chest radiograph, and was referred. Transesophageal echocardiography suggested herniation of the left atrial appendage (LAA) through a gap in the pericardium, and magnetic resonance imag ing indicated congenital partial absence of the pericardium. Cardiac dysfunction was caused by compression from the enlarged left atrium and thrombi were thought to be present in the appendage, so surgery was performed. The intraoperative diagnosis was co ngenital LAA aneurysm. Although distinguishing between congenital LAA aneurysm and congenital absence of the pericardium is reported to be possible with magnetic resonance imaging, we were unable to so in this case.

Ann Thorac Surg. 2000 Apr;69(4):1209-15.
Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.
Gatzoulis MA, Munk MD, Merchant N, Van Arsdell GS, McCrindle BW, Webb GD.
Department of Medicine, Toronto Hospital, Ontario, Canada.

BACKGROUND: Congenital absence of the pericardium (CAP) is a rare clinical entity. METHODS: We identified from the two hospital databases all patients with isolated CAP, reviewed their data, and invited them for prospective clinical evaluation with electr ocardiography, chest x-ray findings (CXR), echocardiography, and magnetic resonance imaging (MRI). RESULTS: Ten patients (3 males, 7 females) presented at a median age of 21 years (range, 2-53 years) with paroxysmal stabbing chest pain, largely nonexertio nal (9), and heart murmur with an abnormal CXR (1). Three patients had partial and 7 had complete CAP (all 7 had marked lateral displacement of the cardiac apex). CXR combined with MRI were key to establishing the diagnosis; a "tongue" of lung tissue inte rposing between the main pulmonary artery and aorta was the most consistent diagnostic feature. Four patients underwent pericardioplasty, 3 for debilitating symptoms and 1 for left atrial appendage herniation, followed by improvement or resolution of symp toms. At a mean of 10.5 years from presentation all patients were alive. No complications were seen in the nonsurgical group. CONCLUSIONS: Isolated CAP has a common presentation pattern with periodic stabbing chest pain mimicking coronary artery disease. CXR and MRI are required for definitive diagnosis. Symptomatic patients with the complete form may benefit from pericardioplasty.

Heart transplant in a patient with complete absence of the pericardium.
Gregori F Jr, Cordeiro CO, Croti UA, Gregori TE, Moure O.
Universidade Estadual de Londrina, Londrina, PR, Brazil.

We report the case of a heart transplant in which the recipient patient had a total congenital absence of the pericardium. Associated with this, we found a major disproportion between the size of the recipient's mediastinal cavity and the size of the dono r's heart. To prevent twisting of the great arteries, we placed the graft on the left diaphragm muscle and beneath the left lung, which resulted in an uneventful early and late postoperative course.

Thorac Cardiovasc Surg. 1999 Dec;47(6):401-4.
A congenital defect of the pericardium.
Skalski J, Wites M, Haponiuk I, Przybylski R, Grzybowski A, Zembala M, Religa Z.
Department of Cardiac Surgery in Zabrze, Silesian School of Medicine in Katowice, Zabrze, Poland.

Ten patients with congenital defects of the pericardium were treated in Departments of Cardiac Surgery, Silesian School of Medicine in Zabrze and Katowice between 1989 and 1998. There were eight children and two adults, eight males and two females. In eac h case the pericardial defect was discovered intraoperatively during surgery for congenital heart defect. There were no cases with clinical symptoms that could be clearly related to the defect of the pericardial sac. In the case of a child with a complete absence of the left pericardial wall the heart was significantly rotated contrary to the defect. The final outcome of the congenital heart defect surgery was satisfactory in each case. An abbreviated historical review of the diagnosis and treatment of th e pericardial defects is presented with special attention placed on therapeutic management. Surgical correction of pericardial defects is concluded to be justified in patients with clinical symptoms. In most cases pericardial defects are discovered intrao peratively, but when they are large the said defects do not require any treatment.

Vestn Rentgenol Radiol. 1998 Jul-Aug;(4):15-8.
The X-ray diagnosis of congenital absence of the pericardium
Ivanitskii AV, Zhandarova IN.
A. N. Bakulev Research Center of Cardiovascular Surgery, Russian Academyof Medical Sciences, Moscow.

The X-ray signs of such a rare abnormality as the congenital absence of the pericardium are presented. The results of examinations and follow-up of 4 patients with this abnormality are analyzed. The patients underwent X-ray, echocardiographic studies, car diac catheterization and angiography. The diagnosis of the absence of pericardium was confirmed at surgery and in one case at autopsy.

Clin Imaging. 1998 Nov-Dec;22(6):404-7.
Congenital absence of the pericardium confirmed by spontaneous pneumothorax.
Pickhardt PJ.
Mallinckrodt Institute of Radiology, Washington Univ. School of Medicine, St. Louis, MO 63110, USA.

This article describes a case of complete absence of the left pericardium which was diagnosed on the chest radiograph and subsequently confirmed on computed tomography and thoracoscopy. Diagnostic confidence was bolstered by the presence of pneumopericard ium which communicated via the defect with a spontaneous left pneumothorax. The clinical and imaging findings of pericardial absence are reviewed.

Heart. 1997 Mar;77(3):247-51.
Cross sectional echocardiographic demonstration of the mechanisms of abnormal interventricular septal motion in congenital total absence of the left pericardium.
Oki T, Tabata T, Yamada H, Manabe K, Fukuda K, Abe M, Iuchi A, Fukuda N, Ito S.
Tokushima University School of Medicine, Japan.

OBJECTIVE: To investigate the influence of the absence of the pericardium on the left ventricular wall, particularly on interventricular septal motion, using M mode and cross sectional short axis echocardiography in patients with congenital total absence of the left pericardium. METHODS: 21 patients with, congenital total absence of the left pericardium were divided into three groups according to the interventricular septal motion; systolic type (n = 6) with paradoxical motion during systole, diastolic ty pe (n = 11) with abnormal posterior motion during mid to late diastole, and mixed type (n = 4) with paradoxical motion during systole and abnormal posterior motion during diastole. RESULTS: On cross sectional short axis echocardiograms of the left ventric le, in the diastolic type the degree of angular displacement of the papillary muscles during end diastole to end systole showed excessive anticlockwise rotation about the long axis of the left ventricle without marked anteroposterior displacement. In the systolic type, there was shift of the left ventricle towards the anteromedial portion in systole and towards the posterolateral portion in diastole without significant rotation. There was a significantly positive correlation between the degree of angular displacement and the amplitude of diastolic interventricular septal motion during mid to late diastole in all patients. CONCLUSIONS: There was abnormal interventricular septal motion during systole and diastole in patients with total absence of the left p ericardium. Abnormal systolic motion was induced by anteroposterior displacement of the left ventricle, and abnormal diastolic motion by left ventricular rotation about the long axis of the heart during the cardiac cycle. Analysis using cross sectional ec hocardiography was useful for elucidating the mechanisms of abnormal interventricular septal motion.

G Ital Cardiol. 1996 Oct;26(10):1187-93.
A paradigmatic case of partial agenesis of the pericardium
Scapellato F, Imparato A, Brunetto A, Temporelli PL, Giannuzzi P.
Divisione di Cardiologia, Fondazione Salvatore Maugeri, IRCCS, Istituto Di Riabilitazione di Veruno.

Complete or partial absence of the pericardium is an uncommon congenital abnormality. Since its first description, several cases have been reported. Although the clinical and instrumental features of this defect are well described, at present it is often overlooked. We report a case of a 19-year old boy referred to our Echocardiography laboratory because of a suspected interatrial septal defect. For this reason it has been recommended to undergo cardiac catheterization. The patient, completely asymptomati c, had a negative physical examination. The echocardiogram excluded the presence of an interatrial septal defect, but some abnormalities, regarding overall right-sided heart, were found (paradoxical interventricular septal movement, apparent right-sided h eart enlargement, with unusual bulging of the apex of the right ventricle, an excessive basculant heart movement). In the absence of any other heart disease, we thought that these abnormalities reflected a congenital absence of the pericardium. Indeed, th e chest X-ray showed some features, considered patognomonic for congenital absence of the pericardium (levoposition of the heart, lung interposition between the diaphragm and the base of the heart and between the aorta and pulmonary artery). Nuclear magne tic resonance study showed the absence of the pericardium in the posterior and posterolateral wall, confirming our suspicion. Furthermore, the posterior wall of the heart leaned to herniate through the pericardial defect. Since the partial absence of the pericardium can lead to severe complications, surgery was recommended. The correct diagnosis of this disease is very important because of its prognostic implications; this case represents a further contribution to the understanding of this defect.

Congenital absence of the left pericardium with extreme intrathoracic displacement of the heart. Case report and review of the literature.
Gersbach P, Kaufmann U, Althaus U.
Department of Thoracic and Cardiovascular Surgery, University of Bern, Inselspital, Switzerland.

Large pericardial defects are exceptional and difficult to diagnose. A 62-year-old man underwent elective coronary surgery which was seriously complicated by the unexpected intrapleural position of the heart due to the absence of a large amount of pericar dium on the left. It appeared retrospectively that the patient had presented typical signs of the malformation and these are described in detail.

Herniation of the left atrial appendage due to partial congenital absence of the left pericardium.
Vanderheyden M, De Sutter J, Nellens P, D'Haenens P, Andries E.
Cardiovascular Center, O.L.V. Hospital Aalst, Belgium.

Partial congenital absence of the left pericardium is a rare abnormality which may provoke serious complications. We report the case of a young adult, suffering from chest pain due to incarceration of atrial tissue. Based on this case report the clinical, pathophysiological and diagnostic features of this condition are described

Pulmonary and systemic venous flow patterns assessed by transesophageal Doppler echocardiography in congenital absence of the pericardium.
Fukuda N, Oki T, Iuchi A, Tabata T, Manabe K, Kageji Y, Sasaki M, Yamada H, Ito S.
Second Department of Internal Medicine, School of Medicine, University of Tokushima, Japan.

In conclusion, alterations in venous return are more marked in the right side of the heart than in the left side of the heart in patients with complete absence of the left pericardium.

Echocardiographic diagnosis of congenital absence of the pericardium in a patient with VATER association defects.
Lu C, Ridker PM.
Division of Cardiology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts 02115.

The VATER association denotes a nonrandom pattern of congenital malformations which typically include vertebral defects, anal atresia, tracheo-esophageal fistula, and radial and renal dysplasia. Vascular anomalies including ventricular septal defect and s ingle umbilical artery have also been described, although the frequency of cardiovascular anomalies in this congenital association is unknown. In this report, we describe a 26-year-old patient with several VATER association defects who, in addition, was f ound to have congenital absence of the left pericardium and an aberrant right subclavian artery.

Agenesis of the left pericardium: report of a case
Ferrante A, Pedi C, Cusumano S, Malfitano D.
Servizio di Cardiologia, Ospedale Gravina, Caltagirone.

We report a case of left pericardial agenesia in a 44 year old woman. Clinical picture was not specific on clinical field: atypical chest pain, asthenia, syncope, cystolic murmur 1/6 Levine on the second left interspace were found. ECG showed clockwise de viation of axis in the precordial leads and incomplete right bundle branch block. Chest X-ray showed marked levocardia and dilatation of pulmonary trunk. Levocardia increased with the patient on the left side. Echocardiography (possible only with patient recumbent on his right side) was normal. Magnetic resonance imaging showed absence of the left pericardium. We analyse the diagnostic problems and the prognostic doubts of this rare congenital anomaly.

Congenital pulmonary venolobar syndrome revisited.
Woodring JH, Howard TA, Kanga JF.
Department of Diagnostic Radiology, University of Kentucky Medical Center, Lexington 40536-0084.

The term "congenital pulmonary venolobar syndrome" (CPVS) encompasses a number of congenital abnormalities of the thorax that often occur in combination. Major components of CPVS include hypogenetic lung, partial anomalous pulmonary venous return (this an d the former are two of the most constantly occurring components), absence of a pulmonary artery, pulmonary sequestration, systemic arterialization of the lung, absence of the inferior vena cava, and accessory diaphragm. Minor components of CPVS include t racheal trifurcation, eventration and partial absence of the diaphragm, phrenic cyst, horseshoe lung, esophageal and gastric lung, anomalous superior vena cava, and absence of the left pericardium. Most patients with CPVS have no symptoms and require no t herapy; however, surgical intervention is often necessary in infants with severe symptoms. The authors review the imaging findings in 29 patients with CPVS and review the literature concerning the diagnosis and management of this complex syndrome in order to improve the understanding of CPVS among radiologists and clinicians.

Congenital absence of the pericardium: displacement of the heart associated with tricuspid insufficiency.
van Son JA, Danielson GK, Callahan JA.
Division of Thoracic and Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota 55905.

This report describes a patient with congenital absence of the left pericardium with displacement of the heart into the left pleural space and associated tricuspid insufficiency. Excision of the ruptured chordae, plication of the flail anterior leaflet, a nd tricuspid annuloplasty rendered a competent tricuspid valve.

Comment in: Orv Hetil. 1993 Dec 19;134(51):2847.
Congenital absence of the left pericardium. Case report and review of the literature
Kornyei V, Kiss A, Kamaras J.
Kaposi Mor Megyei Korhaz Kaposvar Gyermekosztaly, Orszagos Kardiologiai Intezet Gyermekosztaly, Budapest.

Congenital absence of the pericardium, whether partial or total, is a rare abnormality. If suspicion of such a malformation arises it should be classified because of possible herniation of the atrium or ventricle. The authors report the case of an asympto matic young male patient in whom the diagnosis was suggested radiographically, electrocardiographically (total electrical alternans has been noticed) and confirmed by cross-sectional echocardiography, computed tomography and magnetic resonance imaging. On ly in exceptional cases should it be necessary to perform an exploratory thoracotomy. In patients with complete pericardial defects no specific therapy is indicated, however, in patients with partial defects surgical treatment is indicated. Congenital per icardial defects are not completely benign, as has been suggested before.

Congenital partial and complete absence of the pericardium.
Van Son JA, Danielson GK, Schaff HV, Mullany CJ, Julsrud PR, Breen JF.
Division of Thoracic and Cardiovascular Surgery, Mayo Clinic Rochester, Minnesota 55905.

Between 1952 and 1991, 15 Mayo patients were found to have partial or complete absence of the pericardium at the time of a cardiovascular surgical procedure. One patient with complete absence of the left pericardium had symptoms possibly related to the pe ricardial abnormality. This 42-year-old man had severe insufficiency of the tricuspid valve attributable to chordal rupture of the anterior leaflet, possibly precipitated by complete displacement of the heart into the left pleural space. Excision of the r uptured chordae and plication of the anterior flail leaflet rendered a competent tricuspid valve. In two patients, a small defect in the pericardium was repaired. Three patients who underwent operation for complex congenital heart disease died: two early postoperatively and one late after a reoperation. In the other 12 patients, no early or late postoperative complications were encountered. Although rare and usually asymptomatic, complete and partial deficiency of the pericardium may lead to serious compl ications such as cardiac valvular insufficiency or incarceration of cardiac tissue.

Function and importance of the pericardium Dubiel JP.
I Kliniki Kardiologii Akademii Medycznej im. M. Kopernika, Krakowie.

The pericardium plays an important role in the normal cardiac work. Its major functions include: maintenance of adequate cardiac position, separation from the surrounding tissues of the mediastinum, protection against ventricular dilatation, maintenance o f low transmural pressure, facilitation of ventricular interdependence and atrial filling. Congenital absence of the pericardium should be differentiated first of all from atrial septal defect ostium secundum type, idiopathic dilatation of the pulmonary a rtery and tumours of the left mediastinum.

J Am Soc Echocardiogr. 1995 Jan-Feb;8(1):87-92.
Congenital absence of the pericardium: echocardiography as a diagnostic tool.
Connolly HM, Click RL, Schattenberg TT, Seward JB, Tajik AJ.
Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA.

Between 1982 and 1992, 10 patients who underwent echocardiography at the Mayo Clinic were found to have congenital absence of the pericardium. Clinical, electrocardiographic, chest roentgenographic, echocardiographic, computed tomographic, and magnetic re sonance imaging features were reviewed in this patient group. The characteristic features of this entity are reviewed. The echocardiographic features in order of frequency are (1) unusual echocardiographic windows, seen in all 10 patients, (2) cardiac hyp ermobility in nine patients, (3) abnormal ventricular septal motion in eight patients, and (4) abnormal swinging motion of the heart in seven patients.

Clin Cardiol. 1992 Nov;15(11):859-61.
Congenital absence of pericardium as unusual cause of T wave abnormalities in a young athlete.
Di Pasquale G, Ruffini M, Piolanti S, Gambari PI, Roversi R, Pinelli G.
Department of Cardiology, Bellaria Hospital, Bologna, Italy.

The congenital absence of pericardium is a rare condition which may determine electrocardiographic ST-T abnormalities. The case of a 16-year-old athlete who presented T-wave abnormalities at standard electrocardiogram performed during a physical checkup i s reported. Ventricular repolarization tended to normalize during exercise and in right lateral decubitus. Chest x-ray and echocardiography showed typical signs of complete absence of the left pericardium. The definite diagnosis was made by computer tomog raphy (CT) scan. The importance of a complete investigation of athletes with T-wave abnormalities is emphasized. Diagnostic and therapeutic features of the congenital absence of pericardium are discussed.

Chest. 1982 May;81(5):610-3.
M-mode and two-dimensional echocardiography in congenital absence of the pericardium.
Nicolosi GL, Borgioni L, Alberti E, Burelli C, Maffesanti M, Marino P, Slavich G, Zanuttini D.
The M-mode and two-dimensional echocardiographic features of congenital absence of the pericardium are described in two cases that had been confirmed by clinical and radiological data. The M-mode echocardiogram showed right ventricular dilatation and abno rmal systolic motion of the interventricular septum. Echo contrast studies with peripheral injection of saline solution revealed normal persistence of microbubbles in the right side of the heart. Two-dimensional short axis parasternal views showed some di latation of the right ventricle, with anterior displacement of the left ventricular cavity in systole, which appeared to be wider than the posterior motion of the interventricular septum towards the posterior wall. The resulting positive motion of the int erventricular septum toward the transducer could account for the abnormal pattern seen in the M-mode echocardiogram. Congenital absence of the left pericardium has particular echocardiographic features not diagnostic for the anomaly. However, the M-mode a nd two-dimensional echocardiographic studies, used in conjunction with the echocontrast techniques, seem to help in the differential diagnosis and for excluding associated anomalies.

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