Congenital complete absence of the left pericardium: a rare cause of chest pain or pseudo-right heart overload.

BACKGROUND

Congenital defects of the pericardium are rare and poorly known cardiac malformations. Most of them are left-sided and asymptomatic and are usually incidentally diagnosed.

AIMS

To describe the clinical, electrocardiographic, and imaging features of total absence of the left pericardium.

METHODS AND RESULTS

We report the cases of 3 patients who have been seen in our institution with a diagnosis of total congenital absence of the pericardium. All of them complained of precordial pain; one of them experienced disabling symptoms that justified surgical intervention. All of them had previously been suspected to have an atrial septal defect because of the echocardiographic appearance of right ventricular volume overload. Electrocardiogram, chest x-ray, echocardiography, and magnetic resonance imaging of the heart consistently showed remarkably similar features including leftward displacement, increased mobility, and interposition of lung tissue between the heart and other intrathoracic structures.

CONCLUSION

Congenital absence of the left pericardium should be known by clinicians as a possible differential diagnosis of chest pain or pseudo-right heart overload.