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Pulmonary embolism is an uncommon but potentially fatal disease in children, especially with congenital heart disease where pulmonary perfusion depends on caval flow. Anticoagulation is the mainstay of therapy for children with pulmonary embolism. However, thrombolytic therapy can also be considered. The outcome of pediatric pulmonary embolism is uncertain and needs further investigation. We suggest increased awareness and a high index of suspicion in the presence of suggestive clinical signs in high-risk patients in order to initiate prompt diagnostic imaging and treatment.

Fat is the primary energy source for heart muscle. In the heart, carnitine is essential for normal fatty acid beta-oxidation and even partial deficiency could lead to organ dysfunction. Dilated cardiomyopathy is one of the cardinal manifestations of carnitine deficiency in children. We report the case of a female infant referred to our hospital at the age of 15 months with a 2-week history of dyspnea. She presented signs and symptoms of congestive heart failure. Severe cardiomegaly was seen on chest radiographs, while the echocardiogram showed a dilated and hypokinetic left ventricle with depressed systolic function, decreased fractional shortening and ejection fraction, and left atrial enlargement. Laboratory investigations showed carnitine deficiency. She needed intravenous inotropic support in association with conventional heart failure therapy. Treatment with L-carnitine was started as soon as the samples for laboratory testing were taken. Cardiac function clearly and rapidly improved while on treatment.

Background: Percutaneous aortic valvuloplasty is an effective means of treatment for congenital aortic valve stenosis. The aim of this study was to evaluate the immediate results of valvuloplasty, to analyze the medium to long-term outcome and to assess its efficacy in preventing or postponing a new percutaneous valvuloplasty or aortic valve surgery., Methods: We retrospectively analyzed the reports of 37 patients aged > 1 month (mean age 6.3 years) who underwent aortic valvuloplasty for severe aortic stenosis. Associated congenital cardiac defects were present in 16% of the patients. The average time of follow-up was 5.07 years. Particular attention was focused on occurrence and progression of aortic regurgitation., Results: Hemodynamic gradient after aortic valvuloplasty decreased from 58.5 to 22.5 mmHg, with an average decrease of 61.5%. On echography, the maximum gradient decreased from 93.0 to 40.5 mmHg, with an average decrease of 56.5%; mean gradient decreased from 52.0 to 20.5 mmHg with an average decrease of 60.6%. At last follow-up the average maximum and mean gradient on echo were 50.0 and 27.0 mmHg. A reintervention was needed in 21.6% of cases: a second valvuloplasty in 8.1% and aortic surgery in 13.5%. The mortality rate was 2.7%. Survival after 14 years was 97.2%; freedom from aortic valve surgery was 85.5%, from a second valvuloplasty was 89.5%, and from any type of procedure was 76.1%., Conclusions: Percutaneous aortic valvuloplasty is a safe and effective treatment for congenital aortic stenosis in patients aged > 1 month. Aortic regurgitation is the main concern in the follow-up. Nonetheless, 14 years after valvuloplasty, over 75% of patients are free from any type of aortic valve reintervention.