Feasibility of perfusion cardiovascular magnetic resonance in paediatric patients

AIMS: As coronary artery disease may also occur during childhood in some specific conditions, we sought to assess the feasibility and accuracy of perfusion cardiovascular magnetic resonance (CMR) in paediatric patients. METHODS AND RESULTS: First-pass perfusion CMR studies were performed under pharmacological stress with adenosine and by using a hybrid echo-planar pulse sequence with slice-selective saturation recovery preparation. Fifty-six perfusion CMR examinations were performed in 47 patients. The median age was 12 years (1 month-18 years), and weight 42.8 kg (2.6-82 kg). General anaesthesia was required in 18 patients. Mean examination time was 67 +/- 19 min. Diagnostic image quality was obtained in 54/56 examinations. In 23 cases the acquisition parameters were adapted to patient's size. Perfusion CMR was abnormal in 16 examinations. The perfusion defects affected the territory of the left anterior descending coronary artery in 11, of the right coronary artery in 3, and of the circumflex coronary artery in 2 cases. Compared to coronary angiography, perfusion CMR showed a sensitivity of 87% (CI 52-97%) and a specificity of 95% (CI 79-99%). CONCLUSION: In children, perfusion CMR is feasible and accurate. In very young children (less than 1 year old), diagnostic image quality may be limited

The fate of children with microdeletion 22q11.2 syndrome and congenital heart defect: clinical course and cardiac outcome

BACKGROUND: This study aimed to evaluate the cardiac outcome for children with microdeletion 22q11.2 and congenital heart defect (CHD). METHODS: A total of 49 consecutive children with 22q11.2 and CHD were retrospectively identified. The CHD consisted of tetralogy of Fallot and variances (n = 22), interrupted aortic arch (n = 10), ventricular septal defect (n = 8), truncus arteriosus (n = 6), and double aortic arch (n = 1). Extracardiac anomalies were present in 46 of 47 children. RESULTS: The median follow-up time was 8.5 years (range, 3 months to 23.5 years). Cardiac surgical repair was performed for 35 children, whereas 5 had palliative surgery, and 9 never underwent cardiac surgery. The median age at repair was 7.5 months (range, 2 days to 5 years). The mean hospital stay was 35 days (range, 7-204 days), and the intensive care unit stay was 15 days (range, 3-194 days). Significant postoperative complications occurred for 26 children (74%), and surgery for extracardiac malformations was required for 21 patients (43%). The overall mortality rate was 22% (11/49), with 1-year survival for 86% and 5-year survival for 80% of the patients. A total of 27 cardiac reinterventions were performed for 16 patients (46%) including 15 reoperations and 12 interventional catheterizations. Residual cardiac findings were present in 25 patients (71%) at the end of the follow-up period. CONCLUSIONS: Children with microdeletion 22q11.2 and CHD are at high risk for mortality and morbidity, as determined by both the severity of the cardiac lesions and the extracardiac anomalies associated with the microdeletion

Kartagener's syndrome: clinical presentation and cardiosurgical aspects

BACKGROUND: Long-term observations in patients with Kartagener's syndrome (situs inversus, bronchiectasis, and sinusitis) are rare. The role of additional cardiac malformations and their surgical repair is not well known. METHODS: Nine patients (5 female and 4 male) with Kartagener's syndrome were identified and followed. Four patients had associated cardiac anomalies; 4 underwent total surgical repair at the ages of 4 (2 patients), 7, and 34 years. RESULTS: The postoperative period was uneventful, and these 4 patients are doing well 7 months and 2, 9, and 19 years after repair. The other patients are being treated with conservative therapy and are in relatively good condition. CONCLUSIONS: This disease can be temporarily benign when treated with antibiotics and physiotherapy. Associated cardiac anomalies seem to be quite common, and such patients need careful cardiologic follow-up. Surgical intervention can be safely performed in patients suffering from Kartagener's syndrome associated with a congenital cardiac malformation and produces good long-term results. Bilateral lung transplantation seems to be the therapy of choice in patients with respiratory insufficiency but without concomitant cardiac anomalies