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. Copyright 2010 Wiley Periodicals, Inc.
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. Copyright 2010 Wiley Periodicals, Inc.