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Congenital total absence of pericardium in a patient with left lung lower lobe bronchiectasis/ Sol akciger alt lob bronsektazisi olan bir hastada konjenital total perikart yoklugu.


Congenital absence of the pericardium is a rare condition, which is usually encountered incidentally in surgical procedures or autopsies (1,2). We reported a patient with total agenesis of the pericardium. The anomaly was missed despite of the abnormal chest X-ray and computed tomography findings.

Case Report

A 32-year-old man was referred to our clinic for surgical treatment of left lower lobe bronchiectasis. His major complaint was productive cough with sputum and frequent respiratory tract infection. He was also complaining about atypical chest pain, which had no relation with exercise or posture.

On physical examination, his apical impulse was displaced to the left and pulmonary sounds were decreased in the left lower zone with some bronchial rales. Examination of the other systems was unremarkable. The preoperative laboratory parameters were in normal range. The preoperative electrocardiogram (ECG) showed incomplete right bundle branch block and nonspecific T-wave changes (Fig. 1).

In chest X-ray, the heart was displaced to the left with the prominence of the pulmonary artery.

The bronchiectatic lung tissue of the left lower lobe superposing on the heart shadow was seen. The contour of the left hemi-diaphragma was disappeared because of the position of the heart and the left lung (Fig. 2). Thoracic computed tomography showed left lower lobe bronchi ectasis and compensatory hypertrophic right lung with cardiac displacement to the left (Fig. 3). Additionally, a part of lung tissue inserted between the ascending aorta and the main pulmonary artery was seen (Fig. 4). The echocardiography performed preoperatively had been reported to have right ventricular enlargement and mild pulmonary insufficiency.


The patient underwent a left posterolateral thoracotomy for left lower lobectomy of the lung. The total agenesis of the pericardium was seen during operation (Fig. 5,6). Heart and the other structures seemed to be normal except the macroscopic findings of the left lower lobe bronchiectasis. The visceral pleura of the affected lung was seemed to be intact. Left lower lobectomy was performed without any complication. The patient was discharged from the hospital in the 5th postoperative day.







Congenital absence of the pericardium is usually overlooked as in our case. It is may be due to the rarity of the anomaly or to the concomitant situations. Approximately 30% of patients with pericardial agenesis have additional anomalies such as atrial septal defect, bicuspid aortic valve, patent ductus arteriosus, tetralogy of Fallot, pulmonary sequestration, bronchogenic cyst and congenital diaphragmatic hernia (2, 3). Bronchiectasis as in our case with pericardial agenesis has been also reported (4). Pericardial agenesis has no specific finding and it can also be asymptomatic. Chest pain, palpations, dyspnea or dizziness can be detected (5). Our patient had an atypical chest pain but the whole attention was given to the symptoms of the bronchiectasis. The chest pain was thought to be pleuretic due to the frequent pulmonary infections. Displacement of the apical impulse into the mid axillary line as in our patient (1,5,6) and recurrent pulmonary infections which was also seen in the bronchiectatic patient was described with the pericardial absence (2).

Incomplete right bundle branch block and ST-T wave abnormalities in precordial leads as in our patient can be seen as the ECG changes (1,2, 7). Echocardiography provides valuable information which may support the diagnosis (6). But the expectancy of this the anomaly is the most important point otherwise it can easily be missed during performing the echocardiography. Unusual echocardiographic window, abnormal ventricular septal motion, enlarged right ventricle during systole can accompany to pericardial agenesis as echocardiographic findings (2, 6) and these findings have reported in our patient at the echocardiography performed postoperatively (Video). Additionally, the angulation between the interatrial and interventricular septum was remarkable (Fig. 7).

Chest X-ray usually shows a marked displacement of the cardiac silhouette to the left without tracheal deviation (1, 3, 5, 7, 9). We think that it is an important clue in pericardial agenesia especially when any other explanations could not be made about the displacement of the heart in chest X-ray. In left sided bronchiectatic patients similar but not that much movement of the heart can be seen because of the volume loss and compensatory enlargement of the contralateral lung. CT and MRI are capable to determine the extent of the defect, herniation of structures and additional anomalies (8). Computed tomography can show prominence of the main pulmonary artery and the interposition of the lung tissue between the aorta and the main pulmonary artery as in our patient (5, 8).

Usually complete defects as in our case or small defects which herniation of the heart is not possible do not need any intervention (9,10). In partial defects if herniation is probable pericardiectomy or pericardioplasty must be considered (1). At the other hand, resection of the bronchiectatic tissue is much more appropriate with this congenital anomaly, because the anomaly is thought to be harmful by exposing the heart directly to pulmonary infections (5).


Although pericardial agenesis is a very rare anomaly, it must be remembered in the patients with nonspecific chest complaints, especially when abnormal radiographic and cardiac studies accompany the situation.


doi: 10.5152/akd.2011.014


(1.) Abbas AE, Appleton CP, Liu PT, Sweeney JR Congenital absence of the pericardium: case presentation and review of literature. Int J Cardiol 2005; 98:21-5.

(2.) Alizad A, Sweard JB. Echocardiographic features of genetic diseases: Part 8. Organ system. J Am Soc Echocardiogr 2000; 13: 796-800.

(3.) Eyileten Z, Ankbuka M, Yazicioglu L, Ozyurda U. Left pericardial agenesis in a patient with sinus venosus type atrial septal defect. Anadolu Kardiyol Derg 2007; 7: 205-6.

(4.) Arinaga M, Tanaka K, Miura T, Chujo M, Hadama T, Uchida Y. A case of congenital partial pericardial defect and anomaly of phrenic nerve with cystic bronchiectasis. Jpn J Thorac Cardiovasc Surg 1998; 46: 446-9.

(5.) Vesely T, Julsrud PR. Congenital absence of the pericardium and its relationship to the ligamentum arteriosum. Surg Radiol Anat 1989; 11:171-4.

(6.) Centola M, Longo M, De Marco E Cremonesi G, Marconi M, Danzi GB. Does echocardiography play a role in the clinical diagnosis of congenital absence of pericardium? A case presentation and systematic review. J Cardiovasc Med 2009; 10: 687-5.

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

(8.) Alexandros H, Yousef S. Pericardial agenesis: a rare cause of chest pain. Postgrad Med J 2010; 86:380-2.

(9.) Kandemir O, Yildiz U, Can B, Kale A, Tezcaner T, Zorlutuna Y. Congenital complete absence of the left pericardium in a patient with ascending aortic aneurysm. Anadolu Kardiyol Derg 2006; 6:112-1.

(10.) Drury NE, De Silva RJ, Hall RM, Large SR. Congenital defects of the pericardium. Ann Thorac Surg. 2007; 83: 1552-3.

Muammer Cumhur Sivrikoz, Erhan Durceylan, Hacer Boztepe, Alparslan Birdane *

From Departments of Thoracic Surgery and * Cardiology, Faculty of Medicine, Eskisehir Osmangazi University, Eskisehir, Turkey

Address for Correspondence/Yazisma Adresi: Dr. Erhan Durceylan Department of Thoracic Surgery, Faculty of Medicine, Eskisehir Osmangazi University, Eskisehir, Turkey Phone: +90 222 239 29 79 Fax:+90 222 239 37 72 E-mail:

Cevrimici Yayin Tarihi/Available Online Date: 11.01.2011
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Title Annotation:Case Reports/Olgu Sunumlari
Author:Sivrikoz, Muammer Cumhur; Durceylan, Erhan; Boztepe, Hacer; Birdane, Alparslan
Publication:The Anatolian Journal of Cardiology (Anadolu Kardiyoloji Dergisi)
Date:Feb 1, 2011
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