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Tetralogy of Fallot (ToF) is one of the most common congenital cyanotic heart lesions and can present to a variety of health care professionals, including teams working in pediatric intensive care. Pediatric intensive care teams may care for a child with ToF pre-operatively, peri-operatively, and post-operatively. Each stage of management presents its own unique challenges. In this paper we discuss the role of pediatric intensive care in each stage of management.
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Background: Although primary definitive repair of congenital heart disease has become the preferred management approach, pulmonary artery banding (PAB) remains a valuable palliative procedure used to restrict pulmonary blood flow in certain conditions. However, when the band is to be removed, another surgical intervention is usually required. Methods: To describe percutaneous removal of pulmonary artery band, the medical records of patients who underwent this procedure were reviewed. Results: Between 2000 and 2020, 143 patients underwent PAB. Of these, we attempted balloon debanding of the pulmonary artery in four patients. At the time of the procedure, the average age of patients was 36 ± 6.24 months, and their average weight was 12.37 kg. Band removal via catheter was successful in three cases and was associated with an adequate reduction in pressure gradient across the pulmonary artery band site (average of 71.67 ± 12.58 to 23.67 ± 2.89 mm Hg). None of the patients experienced complications during or after the procedure. Follow-up data after discharge (3-10 years) provides reassuring and satisfactory results. Conclusion: Based on our findings, we suggest that percutaneous removal of the pulmonary artery band might be a safe and effective alternative to surgical debanding. However, studies with a larger sample are required for further clinical implementation of the technique.
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Aims: Early prenatal diagnosis of congenital heart disease is feasible. Conventional autopsy is the current gold standard method for post-mortem confirmation. Radiologic techniques alternative to conventional autopsy, such as post-mortem micro-computed tomography, have been proposed in case of limited diagnostic accuracy (i.e., early termination of pregnancy, samples of small dimension or of low weight). The aim of the present study was to define accuracy of micro-computed tomography for post-mortem diagnosis of congenital heart disease in gross anatomy samples. Methods and Results: Fetal heart underwent in-utero prenatal echocardiography and ex-vivo post-mortem evaluation by 9 µm resolution micro-computed tomography and conventional autopsy. For each case, 25 indices of cardiac anatomy were studied by post-mortem micro-computed tomography and conventional autopsy; these were used to compare the two post mortem techniques. Ten samples were examined (gestational age between 12 + 4 and 21 + 6 weeks of gestation). Considering comparable indices, agreement between post-mortem micro-computed tomography and conventional autopsy was of 100% and sensitivity and specificity were of 100%. In "challenging specimens," post-mortem micro-computed tomography diagnoses more indices as compared to conventional autopsy and 84% of "not-diagnostic" indices at conventional autopsy would be diagnostic at post-mortem micro-computed tomography. Conclusion: Micro-computed tomography can be a valid diagnostic alternative to conventional autopsy for post-mortem evaluation of human fetal heart. In addition, it may prove superior to conventional autopsy particularly in cases coming from early termination of pregnancy or in samples of small dimension or of low weight.