Saturday, November 20, 2010

Pericardial anomalies




Pericardial deficiency


Pericardial deficiency is rare.
It is more common in males.
It is usually left-sided, but may be right-sided, diaphragmatic or total.

Etiology:
  • Deficient formation of the pleuro-pericardial membrane, or
  • Deficient formation of the septum transversum.


Pathology:

  • Deficiency of the whole left pericardium, where the heart & left lung are in a common cavity.
  • Partial deficiency of the left pericardium, where there is a foramen between the pericardium & the left pleura.
  • Defects of the diaphragmatic pericardium.
  • Partial deficiency of the right pericardium.
  • Total absence of the pericardium, or rudimentary pericardium.


Associated anomalies:

  • Cardiac anomalies: ASD, PDA, TOF, TR, bicuspid aortic valve.
  • Pulmonary anomalies: Bronchogenic cysts, aberrant pulmonary lobe, pleural defects.
  • Peritoneal defects.
  • Renal anomalies.
  • Cantrell’s pentalogy:
    • Defects of the diaphragmatic pericardium.
    • Defects of the anterior diaphragm.
    • Defects of the lower sternum.
    • Defects of the abdominal wall.
    • Associated with congenital heart disease.


Clinical picture:


Most patients are asymptomatic. 
Non-specific chest pain may occur.
The apex is shifted laterally in cases with complete deficiency of the left pericardium, and may reach the mid-axillary line.


Electrocardiogram:

With complete deficiency of the left pericardium, right axis deviation and clockwise rotation are present.

Chest x-ray:

  • In complete deficiency of the left pericardium, the heart is shifted laterally, and the aortic knuckle & pulmonary conus are prominent.
  • In partial absence of the left pericardium, there is prominence of pulmonary conus and/or the LA appendage.

Echocardiography:
  • The defect may be identified.
  • RVH.
  • Paradoxical septal motion.
  • Lateral extension of LA appendage.


Management:

  • Total deficiency of the left pericardium needs no treatment.
  • Partial defects of the left pericardium need closure with a pleural patch as they may cause herniation & strangulation of the LA appendage or the ventricle.
  • Partial defects of the right pericardium should also be closed with a pleural patch, as they can cause SVC obstruction.



Pericardial cysts and diverticula


Definition:


A cyst does not communicate, while a diverticulum communicates, with the pericardial cavity.


Types of cysts:

  • Coelomic mesodermal cysts.
  • Lymphangiomatous cysts.
  • Bronchial cysts.
  • Teratogenic cysts.


Significance:


Cysts & diverticula are usually asymptomatic.
They usually appear in the chest x-ray as a mass in the right cardio-phrenic angle.
They should be differentiated from tumors & Morgagni hernia.

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