Browse
Submit Data
Databases
API
Help

Dataset Information

25 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Implantation of a left ventricular assist device to provide long-term support for end-stage Duchenne muscular dystrophy-associated cardiomyopathy.

ABSTRACT: A young man with Duchenne muscular dystrophy presented to the UT Southwestern Neuromuscular Cardiomyopathy Clinic with advanced heart failure. Despite maximal medical therapy, his cardiac function continued to decline requiring initiation of inotrope therapy. Given the patient's clinical deterioration, a left ventricular assist device (LVAD) was implanted as destination therapy after undergoing a multidisciplinary assessment. The patient tolerated the surgical implantation of the LVAD without any significant complications, and he has had a relatively unremarkable course 38 months post-LVAD implantation. A critical factor contributing to the long-term success of this patient was the decision to select an LVAD that would not disrupt the diaphragm and thus preserve the respiratory muscle strength. This case demonstrates that permanent mechanical LVADs should be considered for appropriately selected Duchenne muscular dystrophy patients with medically refractory end-stage cardiomyopathy.

SUBMITTER: Stoller D

PROVIDER: S-EPMC5542741 | biostudies-other | 2017 Aug

REPOSITORIES: biostudies-other

ACCESS DATA

Json Xml

Similar Datasets

Left ventricular noncompaction cardiomyopathy in Duchenne muscular dystrophy carriers.

Project description:Duchenne and Becker muscular dystrophies are X-linked hereditary myopathies secondary to a dystrophinopathy resulting in progressive cardiomyopathy and heart failure. The most commonly associated cardiac involvements in these patients are dilated cardiomyopathy and conduction abnormalities; however, recent studies have shown a high prevalence of left ventricular noncompaction cardiomyopathy in patients with Duchenne muscular dystrophy. Furthermore, there is increasing awareness of cardiomyopathy in female heterozygous dystrophinopathy carriers. We report a case series of two dystrophinopathy carriers with the dilated form of left ventricular noncompaction cardiomyopathy, a newly identified association. <Learning objective: Dystrophinopathy carriers can manifest cardiac disease in the form of cardiomyopathy. We present a novel finding of carriers who manifest their cardiomyopathy in the form of left ventricular noncompaction, dilated phenotype. This has been described previously in patients with Duchenne muscular dystrophy.>.

| S-EPMC6279986 | biostudies-literature

Left ventricular dysfunction in duchenne muscular dystrophy and genotype.

Project description:Prognosis in patients with Duchenne muscular dystrophy (DMD) is guarded, and most deaths are due to cardiac or respiratory causes. It is unclear if some DMD gene mutations might be predictive of either mild or severe cardiac dysfunction. We studied 75 patients with DMD followed at our institution. Cardiac function, as assessed by yearly echocardiography, showed marked variability in left ventricular (LV) function. Some patients in their 3rd decade had no or minimal dysfunction, whereas others in their 2nd decade had very severe dysfunction. Therefore, 4 severity groups were defined ranging from no or mild LV dysfunction to severe LV dysfunction using patient age at first abnormal echocardiographic finding and degree of LV dysfunction. Genetic data were collected for all patients. Most patients had mutations from exon 1 to 20 to exon 41 to 55. The distribution of the 4 severity groups of LV dysfunction did not significantly differ between these 2 mutation groups. An analysis based on the number of exons involved (<5 vs ?5 exons) also found no significant difference in cardiac severity. When patients having identical mutations were compared with their cardiac course, concordance was often not evident. Steroid therapy had no apparent protection for the development of cardiomyopathy. In conclusion, 75 patients with DMD showed marked variability in the severity of LV dysfunction. Neither the age of onset nor the severity of cardiomyopathy correlated with any of the mutation groups.

| S-EPMC4768789 | biostudies-literature

Predictors of right ventricular failure after left ventricular assist device implantation.

Project description:Number of left ventricular assist device (LVAD) implantations increases every year, particularly LVADs for destination therapy (DT). Right ventricular failure (RVF) has been recognized as a serious complication of LVAD implantation. Reported incidence of RVF after LVAD ranges from 6% to 44%, varying mostly due to differences in RVF definition, different types of LVADs, and differences in patient populations included in studies. RVF complicating LVAD implantation is associated with worse postoperative mortality and morbidity including worse end-organ function, longer hospital length of stay, and lower success of bridge to transplant (BTT) therapy. Importance of RVF and its predictors in a setting of LVAD implantation has been recognized early, as evidenced by abundant number of attempts to identify independent risk factors and develop RVF predictor scores with a common purpose to improve patient selection and outcomes by recognizing potential need for biventricular assist device (BiVAD) at the time of LVAD implantation. The aim of this article is to review and summarize current body of knowledge on risk factors and prediction scores of RVF after LVAD implantation. Despite abundance of studies and proposed risk scores for RVF following LVAD, certain common limitations make their implementation and clinical usefulness questionable. Regardless, value of these studies lies in providing information on potential key predictors for RVF that can be taken into account in clinical decision making. Further investigation of current predictors and existing scores as well as new studies involving larger patient populations and more sophisticated statistical prediction models are necessary. Additionally, a short description of our empirical institutional approach to management of RVF following LVAD implantation is provided.

| S-EPMC4295071 | biostudies-literature

Longitudinal Trajectories of Hemodynamics Following Left Ventricular Assist Device Implantation.

Project description:BACKGROUND:Continuous-flow left ventricular assist devices (LVADs) improve the hemodynamics of patients with advanced heart failure. However, the longitudinal trajectories of hemodynamics in patients after LVAD implantation remain unknown. The aim of this study was to investigate the trends of hemodynamic parameters following LVAD implantation. METHODS AND RESULTS:We retrospectively reviewed patients who underwent LVAD implantation between April 2014 and August 2018. We collected hemodynamic parameters from right heart catheterizations. Of 199 consecutive patients, we enrolled 150 patients who had both pre- and postimplant right heart catheterizations. They had 3 (2, 4) postimplant right heart catheterizations during a follow-up of 2.3 (1.3, 3.1) years. The mean age was 57 ± 13 years, and 102 patients (68%) were male. Following LVAD implantation, pulmonary arterial pressure and pulmonary capillary wedge pressure decreased, and cardiac index increased significantly, then remained unchanged throughout follow-up. Right atrial pressure decreased initially and then gradually increased to preimplant values. The pulmonary artery pulsatility index decreased initially and returned to preimplant values, then progressively decreased over longer follow-up. Subgroup analysis showed significant differences in the trajectories of the pulmonary artery pulsatility index based on gender. CONCLUSIONS:Despite improvement in left-side filling pressures and cardiac index following LVAD implantation, right atrial pressure increased and the pulmonary artery pulsatility index decreased over time, suggesting progressive right ventricular dysfunction.

| S-EPMC7259826 | biostudies-literature

Aortic Root Thrombus Directly After Left Ventricular Assist Device Implantation.

Project description:A 70-year-old female heart failure patient could not be weaned from temporary left ventricular mechanical support with Impella CP (Abiomed Inc, Danvers, MA) after myocardial infarction; therefore, she underwent left ventricular assist device implantation (HeartMate 3; Abbott, Chicago, IL). After uneventful surgery, the patient had an early postoperative thrombus in the aortic root, and surgical thrombectomy on extracorporeal circulation was performed on the seventh postoperative day. The patient recovered well and presented in good condition with no neurologic symptoms at the 6-month follow-up visit. Surgical excision of aortic root thrombus is a feasible option even for frail patients with a left ventricular assist device.

| S-EPMC8636229 | biostudies-literature

Left ventricular assist device implantation and clinical outcomes in the Netherlands.

Project description:BackgroundLeft ventricular assist device (LVAD) therapy is an established treatment for advanced heart failure with reduced ejection fraction. We evaluated the characteristics and clinical outcomes of patients implanted with an LVAD in the Netherlands.MethodsPatients implanted with an LVAD in the Netherlands between 2016 and 2020 were included in the analysis. Baseline characteristics entered into this registry, as well as clinical outcomes (death on device, heart transplantation) and major adverse events (device dysfunction, major bleeding, major infection and cerebrovascular event), were evaluated.ResultsA total of 430 patients were implanted with an LVAD; mean age was 55 ± 13 years and 27% were female. The initial device strategy was bridge to transplant (BTT) in 50%, destination therapy (DT) in 29% and bridge to decision (BTD) in the remaining 21%. After a follow-up of 17 months, 97 (23%) patients had died during active LVAD support. Survival was 83% at 1 year, 76% at 2 years and 54% at 5 years. Patients implanted with an LVAD as a BTT had better outcomes compared with DT at all time points (1 year 86% vs 72%, 2 years 83% vs 59% and 5 years 58% vs 33%). Major adverse events were frequently observed, most often major infection, major bleeding and cerebrovascular events (0.84, 0.33 and 0.09 per patient-year at risk, respectively) and were similar across device strategies. Patients supported with HeartMate 3 had a lower incidence of major adverse events.ConclusionsLong-term survival on durable LVAD support in the Netherlands is over 50% after 5 years. Major adverse events, especially infection and bleeding, are still frequently observed, but decreasing with the contemporary use of HeartMate 3 LVAD.

| S-EPMC10140239 | biostudies-literature

QRS complex fragmentation and survival following left ventricular assist device implantation.

Project description:In patients with heart disease, the presence of a fragmented QRS complex (fQRS) on the surface electrocardiogram (ECG) is associated with an increased risk of mortality. We sought to evaluate the prevalence and location of fQRS before and after left ventricular assist device (LVAD) implantation and any associated risk of mortality.Twelve-lead surface ECGs before (pre-LVAD, n = 98) and after (early [< 7 days], n = 96, and late [≥ 30 days], n = 85, post-LVAD) LVAD implantation were evaluated for fQRS. Mortality data were gathered via review of medical records. The prevalence of fQRS increased significantly following LVAD implantation on early post-LVAD ECGs (31% to 47%, p < 0.01). Patients with fQRS in the anterior territory (precordial leads V1 to V5) on late post-LVAD ECGs had decreased survival or survival to cardiac transplantation over a 30 month follow-up period compared with patients who did not exhibit anterior fQRS (30% and 59%, respectively, p < 0.01).The prevalence of fragmented QRS on 12-lead ECG increases significantly in the anterior territory following LVAD implantation and is associated with decreased survival.

| S-EPMC5497182 | biostudies-other

Left Atrial Appendage Inversion Presenting as Acute Right Ventricular Failure after Left Ventricular Assist Device Implantation.

Project description: Not available

| S-EPMC7451932 | biostudies-literature

Left Ventricular Assist Device With a Left Atrial Inflow Cannula for Hypertrophic Cardiomyopathy.

Project description:Patients with restrictive or hypertrophic cardiomyopathy (HCM) are often ineligible for a left ventricular assist device (LVAD) due to the risk of suction events with a small left ventricular cavity size and left ventricular inflow cannula. We describe an alternative LVAD configuration using a left atrial inflow cannula as a bridge to transplantation in an adult with HCM. (Level of Difficulty: Advanced.).

| S-EPMC8299761 | biostudies-literature

Left Ventricular Assist Device Support for Fabry Cardiomyopathy: A Case Series.

Project description:Patients with restrictive cardiomyopathy due to Fabry disease are often deemed ineligible for left ventricular assist device (LVAD) support due to the risk of suction events with a small LV cavity. We describe the first case series of LVAD support for Fabry disease. LVAD therapy can improve survival, quality of life, and provide clinical stability to start enzyme replacement therapy. Precautions at the time of surgery include rapid treatment of fever to avoid Fabry crises, involvement of a multidisciplinary team, and early initiation of rehabilitation. We describe LVAD support for both bridging and destination therapy.

| S-EPMC7893197 | biostudies-literature

OmicsDI is part of the ELIXIR infrastructure

OmicsDI is an Elixir interoperability service. Learn more ›

Tweets

OmicsDI Databases

PRIDE
PeptideAtlas
MassIVE
JPOST Repository
Physiome Model Repository

EGA
EVA
ENA
LINCS
PAXDB
Cell Collective

MetaboLights
Metabolomics Workbench
MetabolomeExpress
GNPS
BioModels
FAIRDOMHub

ArrayExpress
dbGaP
ExpressionAtlas
GEO
NODE

Information

Databases
Help
API
Contact us
Code on GitHub
Terms of use
Submit Data