A 16-year-old male with congenital absence of the left pericardium and a secundum-type atrial septal defect was successfully treated with surgical repair of the ASD.
Case Report (n=1)
No
Congenital absence of the pericardium can be associated with congenital heart disease and should be considered when chest X-ray shows leftward displacement of the cardiac silhouette without tracheal deviation.
Absence of the pericardium (CAP) is a rare condition that can be either congenital or acquired. The acquired form typically occurs following pericardiectomy to treat constrictive or recurrent pericarditis. The congenital variant is extremely rare, with an incidence of less than 1 in 10,000. CAP can be further classified based on the location of the defect and whether the absence is partial or complete. Complete left-sided defects are the most common, accounting for approximately 70% of all pericardial defects. The patient is a 16-year-old Black African male who presented with worsening chest pain, palpitations, and difficulty of breathing after strenuous activity. He has also experienced occasional dizziness and poor appetite since the age of 14. One month prior to the current admission, he was diagnosed with congenital heart disease. On physical examination, the patient was alert and clinically stable, with a pulse of 81 beats per minute, blood pressure of 118/62 mmHg, temperature of 36.2 °C, and oxygen saturation of 91% on room air. Physical examination revealed a palpable radial pulse, with audible S1 and a fixed, wide split S2 at the left lower sternal border. The rest of the examination was unremarkable. Upon admission, basic laboratory investigations, were normal. Chest X-ray showed a leftward and posterior shift of the cardiac silhouette, with a straight left heart border and effacement of the right heart border. Echocardiography revealed a dilated right atrium and right ventricle, as well as 38 mm upper secundum-type atrial septal defect (ASD) with left-to-right shunting. The interventricular septum was intact, and left ventricular systolic function was normal. Intraoperatively, there was absence of the left lateral and anteroinferior parts of the pericardium. Congenital absence of the pericardium can be associated with congenital heart disease. Clinicians should consider the possibility of CAP in patients with chest radiographic findings of leftward displacement of the cardiac silhouette without tracheal deviation and a flattened, elongated left ventricular border.
Gizaw et al. (2026) conducted a case report in Congenital absence of the left pericardium with congenital heart disease (n=1). Surgical repair of atrial septal defect was evaluated. A 16-year-old male with congenital absence of the left pericardium and a secundum-type atrial septal defect was successfully treated with surgical repair of the ASD.