Total absence of the pericardium associated with hypogammaglobulinemia and bronchiectasis in a girl.

We report the case of an 8-years-old girl with recurrent pulmonary infections and wheezing since infancy, in whom asthma and immunoglobulin-G deficiency were diagnosed at the age of 7 months. Since then, the patient was treated for asthma without any satisfactory control of the disease. Cardiomegaly was finally diagnosed radiologically that led to cardiac assessment. Echocardiography suggested left sided partial anomalous pulmonary venous return that was not confirmed at angio-computed tomography scan and cardiac magnetic resonance imaging. Instead, total absence of the pericardium with relative left lung hypoplasia and left-sided bronchiectasis was diagnosed. Immune defect was confirmed. Adequate treatment by immunoglobulin supplementation and observance of the recommended care of bronchiectasis allowed favorable evolution. This case of an unusual association between an exceptional pericardial malformation and immune deficiency causing lower respiratory tract infections complicated by leftsided bronchiectasis highlights the absolute necessity to explore further any child with insufficient asthma control.

Cardiac papillary fibroelastoma of a bicuspid aortic valve in an adolescent: A case report.

Cardiac papillary fibroelastomas (CPFE) are exceptional primary benign cardiac tumours affecting the heart valves. We report here the case of a 15-year-old boy in whom echocardiography performed for non-specific chest pain during follow-up for bicuspid aortic valve showed as accidental finding the presence of a round mobile mass without stalk attached on the inferior side of the aortic valve. The mass did not cause any outflow tract obstruction or aortic insufficiency. Electrocardiogram-gated cardiac computed tomography and magnetic resonance imaging allowed to suspect CPFE. Although the patient was asymptomatic, open cardiac surgery with elective surgical resection of the tumour was performed to avoid systematic emboli. Histology confirmed the diagnosis of CPFE. This is an exceptional case of acquired CPFE in a young patient with bicuspid aortic valve. Due to the risk of systemic embolization, aortic or coronary ostium obstruction, elective excision of such lesions is recommended.

Exercise-Triggered Chest Pain as an Isolated Symptom of Myocarditis in Children.

In childhood, chest pain occurring at exercise is a common complaint. A cardiac etiology for it is exceptionally found, explaining that most children do not undergo systematic cardiological investigation. However, chest pain at exercise may manifest as the unique symptom of a viral myocarditis. Recognizing this form of myocardial injury, however, might help to avoid clinical deterioration by providing adequate care. In this paper, we report on two children presenting with the unique clinical symptom of chest pain related to physical activity and in whom laboratory and cardiac investigations suggested transient myocardial damage related to myocarditis.