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Outcome 50 years after surgical repair of pulmonary valve stenosis: a longitudinal cohort study

Por: Ünlütürk · S. · Kauling · R. M. · Cuypers · J. A. A. E. · van den Bosch · A. E. · Hirsch · A. · Pelosi · C. · Bowen · D. J. · Bogers · A. J. J. C. · Helbing · W. A. · Kardys · I. · Roos-Hesselink · J. W.
Objectives

Data on long-term outcomes after surgical repair of pulmonary valve stenosis are limited. This study evaluated survival, clinical outcomes and quality of life (QoL) after surgery during childhood.

Methods

Single centre, longitudinal cohort study evaluating consecutive patients with pulmonary valve stenosis who underwent surgical repair between 1968–1980 and were evaluated every decade since 1990.

Results

Of the original cohort of 89 operated patients, 11 died (12%), including 2 who died within 30 days postsurgery (2%), and 7 (8%) were lost to follow-up. Survival at 50 years follow-up was 87%, which was not significantly different from the GDP. Of the remaining 71 survivors, 32 refrained earlier from participating in this cohort study, leaving 39 eligible, of whom 34 (87%) participated again (50% male, median age 48 years) with a median follow-up of 45 (range 40–52) years. Event-free survival was 50%, with supraventricular tachycardia (14%) and reintervention (13%) being the most frequent events, although less frequently in the last 10 years. At last follow-up, biventricular function was preserved in most patients. Reduced right and left ventricular ejection fraction (EF) was found in 33% and 13%, respectively. Exercise capacity and maximum rate of oxygen consumption were mildly impaired in 14% and 32% of patients. Patients who underwent an infundibulectomy during initial surgery were significantly more likely to undergo reintervention (HR=8.32, p=0.003). Patient-reported QoL scores remained stable over time and consistently exceeded those of the age-matched GDP.

Conclusion

Fifty-year survival after surgery for pulmonary valve stenosis was excellent and comparable to the GDP. Most patients maintained preserved ventricular function, functional capacity and excellent QoL. Routine lifelong follow-up may not be necessary for all patients, but should be considered for those who underwent an infundibulectomy or have residual lesions.

Individualised stimulation parameters in deep brain stimulation for Parkinsons disease based on disease phenotype and brain connectivity: protocol for a randomised feasibility study in a tertiary care centre (iDBS trial)

Por: de Ronde · E. M. · Birnie · E. · Rijpma · A. · Arnts · H. · Bartels · R. H. M. A. · Esselink · R. · Vinke · R. S.
Background

Bilateral deep brain stimulation (DBS) of the subthalamic nucleus (STN) is a well-accepted treatment for advanced Parkinson’s disease (PD). Currently, programming of the DBS is done in a trial-and-error manner and it can take up to 12 months to reach optimal stimulation parameters. Technological advances in electrode design and implantable pulse generator capabilities lead to an almost infinite number of stimulation options. To explore the potential benefit of all these technological advances, a conventional trial-and-error approach is no longer sufficient. Consequently, there is a clear need for a more computational approach to programming DBS systems. This pilot study is a prospective trial to prove the feasibility of programming bilateral STN-DBS for PD in a computational fashion based on patient anatomy, electrode position and brain connectivity. In this study, we aim to assess the safety, practical feasibility and technical feasibility of a computational approach for programming newly implanted STN-DBS patients with PD. This computational approach will be based on a patient-specific DBS setting regarding sweet spots and structural connectivity of the STN. The results of this pilot study will be used to develop a computational approach for DBS programming to use in a future randomised clinical trial.

Methods and analysis

The iDBS trial will be a prospective randomised feasibility study carried out at the Radboud university medical center. A total of 24 patients with PD eligible for bilateral STN-DBS surgery implanted with Boston Scientific Cartesia leads will be included. Patients will be randomised to receive either (1) computational DBS programming (n=12) or (2) conventional DBS programming based on monopolar review (n=12). The primary endpoints are safety (occurrence of stimulation-induced side effects, duration of induced side effects (temporary or permanent), severity of the stimulation-induced side effects) and technical feasibility (time from surgery to DBS initiation, time from surgery to reaching optimal DBS stimulation settings) of the computational workflow.

Ethics and dissemination

Ethical approval for this study has been granted by the Medical Ethical Committee region Arnhem-Nijmegen, the Netherlands (2024–17453). This study will be conducted in accordance with the Declaration of Helsinki and all applicable European and Dutch law. All participants will have to provide written informed consent. Results of the study will be submitted for publication in peer-reviewed journals and conferences.

Trial registration number

The study is registered in the OMON-registry (NL87334.091.24, NL-OMON57446).

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