Annuloaortic Ectasia in a Patient With Congenital Absence of the Left Pericardium

doi:10.1016/j.athoracsur.2013.04.106

The Annals of Thoracic Surgery

Volume 96, Issue 6, December 2013, Pages 2243-2245

https://doi.org/10.1016/j.athoracsur.2013.04.106 Get rights and content

We report a patient with congenital absence of the left pericardium with development of progressive annuloaortic ectasia and aortic insufficiency during a 12-year period. The patient was treated with a Bentall procedure. Pathologic examination of the aorta revealed cystic medial necrosis. The surgical management and a possible association between congenital absence of pericardium and Marfan syndrome are discussed.

Section snippets

Comment

CAP is uncommon. It is usually diagnosed as an incidental finding in patients being evaluated for chest pain or other cardiac symptoms [1]. Coronary artery bypass grafting and ascending aorta replacement for type A dissection have been reported in these patients 2, 3, 5, 6. Cardiac operations in these patients can be challenging because of the abnormal location of the cardiac structures. Particular attention should be paid to the location of the left phrenic nerve, which is usually located

References (9)

W. Nasser et al.
Congenital absence of the left pericardium
Circulation
(1966)
M. Gatzoulis et al.
Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management
Ann Thorac Surg
(2000)
N. Muthialu et al.
Phrenic nerve at greater risk: congenital absence of pericardium exposing the nerve
Ann Thorac Surg
(2012)
S. Raman et al.
Congenital absence of the pericardium
Circulation
(2001)

There are more references available in the full text version of this article.

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Citation Excerpt :
Thirty to 50% of patients with congenital absence of pericardium also have congenital abnormalities like atrial septal defect, patent ductus arteriosus, or tetralogy of Fallot. Patients with aortic connective tissue diseases and Marfan syndrome have also been found to have pericardial anomalies [5,6]. Congenital absence of the pericardium is usually benign; however, it can be mistaken for other pathologic illnesses using standard imaging and screening techniques.
Congenital absence of pericardium is an exceedingly rare condition with a prevalence of 0.002%-0.004%. Due to its rarity and absence of association with any specific clinical examination finding, the diagnosis may be challenging. Due to the absence of symptoms and clinical awareness, pericardial agenesis is commonly misdiagnosed. It is important to consider this as a differential diagnosis of exertional chest pains. We report the case of a 9-year-old boy who attended our institution for non-specific but frequent symptoms and was diagnosed with congenital complete absence of the pericardium. The rarity of the condition can hinder timely diagnosis, as a physician or radiologist may never encounter a single case in their lifetime.
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Albeit described since 1948, cardiac herniations still represent an under-recognized clinical entity in the general population, due to their difficult diagnostic detection and to a limited knowledge in clinical practice. This paper aims to provide an updated overview focusing on definition, clinical presentation and diagnostic work-up of cardiac herniations, as well as on further findings concerning prognoses and available therapeutic options.
This narrative review was performed by following the standard methods of the Cochrane Collaboration and the PRISMA statement. A Pubmed search was conducted using the following keywords (in Title and/or Abstract): (“cardiac” OR “heart”) AND (“herniation” OR “hernia”). All available high quality resources containing information on epidemiology, etiopathogenesis, clinical findings, diagnostic tools and therapeutic strategies, were included in our search.
Causative agents, location and the degree of haemodynamic impairment were found to play a pivotal role in defining the patient's natural history and prognostic outcomes, and in detecting potential associated clinical conditions which may significantly impact on first line decision-making strategy.
Cardiac herniations remain a rare and life-threatening pathological injury, whose knowledge is still very limited in clinical practice. Clinicians should be aware of such a clinical entity and of its wide spectrum of clinical manifestations, particularly in high-risk subsets of patients, in order to provide the most appropriate decision-making strategy and timely therapeutic approach.
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Case reportAnnuloaortic Ectasia in a Patient With Congenital Absence of the Left Pericardium

Section snippets

Comment

Congenital absence of the left pericardium

Circulation

Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management

Ann Thorac Surg

Phrenic nerve at greater risk: congenital absence of pericardium exposing the nerve

Ann Thorac Surg

Congenital absence of the pericardium

Circulation

Case report
Annuloaortic Ectasia in a Patient With Congenital Absence of the Left Pericardium