Balloon-Expandable Pulmonary Valves for Patched or Native Right Ventricular Outflow Tracts.

The implantation of percutaneous balloon expandable valves in native or patched right ventricular outflow tracts (nRVOT) is a challenging technique due to the diversity of anatomies and shapes, the large sizes, and the distensibility of the nRVOT, for which specific techniques have been developed. We present a single center experience with balloon expandable percutaneous pulmonary valves in nRVOT, describing the techniques used, complications observed, and a short-mid term follow-up.. This is a single center descriptive study of patients who underwent a percutaneous pulmonary valve implantation in a nRVOT with a balloon expandable pulmonary valve in our center between September 2012 and June 2022.. We implanted successfully 45 valves in 46 patients (20 Sapien and 25 Melody). Tetralogy of Fallot or pulmonary atresia with VSD were the main congenital heart disease (n = 32). All were pre-stented, 18 in a one step procedure. We used a Dryseal sheath in 13/21 Sapien. In 6 patients we used the anchoring technique, 5 with a very large nRVOT and one pyramidal nRVOT. In the 3.5 year follow-up 7 patients developed endocarditis and 3 required a valve redilation, no fractures were observed. PPVI of native RVOT with balloon expandable valves is feasible in a number of selected anatomies, including large or pyramidal nRVOT, using specific techniques, (presenting, LPA anchoring).

Timing of Pulmonary Valve Replacement: How Much Can the Right Ventricle Dilate Before it Looses Its Remodeling Potential?

Congenital heart disease patients that develop secondary pulmonary regurgitation require a pulmonary valve replacement (PVR) in their follow-up. The indications for PVR in asymptomatic patients are debated. Most guidelines consider a RV end-diastolic volume (RVEDV) over 150 ml/m(2) as an indication for PVR. We analyzed clinical, echocardiographic and MRI variables of patients that underwent a surgical PVR between September 2006 and February 2013. The included patients were asymptomatic, without pulmonary stenosis and with both pre- and post-surgery MRI. Thirty-five patients (74.3 % males) were included. Mean age at PVR was 25.8 years (SD = 7.18), and weight was 64.5 Kg (SD = 12.03). The main diagnosis was tetralogy of Fallot (n = 28), pulmonary atresia (n = 2), primary pulmonary regurgitation (n = 2) and pulmonary regurgitation after percutaneous treatment (n = 2). The maximal RVEDV pre-PVR was 267 ml/m(2), and right ventricular end-systolic volume (RVESV) was 183 ml/m(2). RV size and function were established by MRI: Pre-PVR Post-PVR p RVEDV (ml/m(2)) 162 (SD = 39.1) 94 (SD = 23.6) <0.001 RVESV (ml/m(2)) 87 (SD = 28.9) 44 (SD = 15.7) <0.001 RVEF 44.8 % (SD = 8.17) 52 % (SD = 9.9) <0.001 Patients with a RVEDV under 170 ml/m(2) combined with a RVESV under 90 ml/m(2) had a favorable RV remodeling, defined as RVEDV under 110 ml/m(2) (sensitivity 87.5 %), RVESV under 55 ml/m(2) (sensitivity 100 %) and RVEF over 50 % (sensitivity 100 %). When deciding the optimal PVR timing in asymptomatic patients, both RVEDV and RVESV should be considered. Our results suggest that higher volumes than used in the clinical practice can achieve a good remodeling. Therefore, PVR could be performed later in the follow-up reducing the number of cardiac interventions.

[Complex regional pain syndrome and myoclonus: an uncommon combination]. / Distrofia simpático-refleja y mioclonías: una asociación infrecuente.

Complex regional pain syndrome (CRPS) is quite uncommon in paediatric patients. There is no identified organic aetiology. CRPS has a very significant psychosomatic component; therefore it is necessary to take a multidisciplinary approach to its treatment, which should include psychiatric assessment. CRPS is very difficult to diagnose, and can take months to control its symptoms. The association with myoclonus is rare, and since myoclonus is the clinical manifestation of a vast spectrum of different neurological disorders, some of which are degenerative, it is important to make a diagnosis as quickly as possible. We present the case of an adolescent male with an atypical presentation of CRPS. CRPS was located in the thorax, which is very unusual and was associated with myoclonus and dystonias. This made the diagnosis harder and widened the aetiological spectrum.