Modified Hybrid Procedure in Hypoplastic Left Heart Syndrome: Initial Experience of a Center in Northeastern Brazil.
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ABSTRACT:
SUBMITTER: Faria RM
PROVIDER: S-EPMC5570387 | biostudies-other | 2017 May-Jun
REPOSITORIES: biostudies-other
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Project description:BackgroundLeft atrial appendage (LAA) closure has been an alternative to oral anticoagulation (OAC) for stroke prevention in patients with non-valvular atrial fibrillation (NVAF).ObjectivesTo report the first results of an initial multicenter experience in Brazil and to investigate the feasibility, safety, and efficacy of LAA closure with the new LAmbre device.MethodsWe collected procedural and follow-up data of 51 consecutive patients with non-valvular atrial fibrillation, restrictions for long-term OAC and suitable anatomy that underwent LAA closure with the LAmbre device in 18 centers in Brazil. Procedural indications were significant bleeding under OAC (47.1%), stroke or persistent LAA thrombus despite OAC (27.5%), bleeding plus stroke (17.6%), other clinical contraindications for OAC (5.9%), and patient's choice due to sports practice (1.9%).ResultsTwenty-five men (49%) and 26 women (51%), with a mean age of 76±7.7 years, mean CHA2DS2-VASc score of 4.6± 1.7 and mean HAS-BLED score of 3.4± 1.1 were studied. Procedural success rate was 100%. Procedure-related immediate complications were pericardial effusion in two patients, and immediate device embolization in one case. No large residual shunts (> 5 mm) were observed, and small shunts (<5mm) were detected in four patients by color Doppler at the end of the procedure. After a mean follow-up of 18 ± 12 months, there were no deaths, strokes nor any other major complications.ConclusionLAA occlusion with the LAmbre device was safe and effective in this small case series. Despite these encouraging initial results, the small number of cases warrants further studies with longer-term follow-up.
| S-EPMC9352115 | biostudies-literature
Project description:Catheter ablation of ventricular tachycardia (VT) can be technically challenging due to difficulty with catheter positioning in the left ventricle (LV) and achieving stable contact. The Hansen Sensei Robotic system (HRS) has been used in atrial fibrillation but its utility in VT is unclear.The purpose of this study was to test the technical feasibility of robotic catheter ablation of LV ventricular tachycardia (VT) using the HRS.Twenty-three patients underwent LV VT mapping and ablation with the HRS via a transseptal, transmitral valve approach. Nineteen patients underwent substrate mapping and ablation (18 had ischemic cardiomyopathy, 1 had an apical variant of hypertrophic cardiomyopathy). Four patients had focal VT requiring LV VT mapping and ablation. Procedural endpoints included substrate modification by endocardial scar border ablation and elimination of late potentials, or elimination of inducible focal VT.Mapping and ablation were entirely robotic without requiring manual catheter manipulation in all patients and reaching all LV regions with stable contact. Fluoroscopy time of the LV procedure was 22.2 ± 11.2 minutes. Radiofrequency time was 33 ± 21 minutes. Total procedural times were 231 ± 76 minutes. Complications included a left groin hematoma (opposite to the HRS sheath), 1 pericardial effusion without tamponade that was drained successfully, and transient right ventricular failure in a patient with previous left ventricular assist device. At 13.4 ± 6.7 months of follow-up (range 1-19 months), recurrence of VT occurred in 3 of 23 patients.Our initial experience suggests that the HRS allows successful mapping and ablation of LV VT.
| S-EPMC3298677 | biostudies-literature
Project description:BACKGROUND:Most studies have not demonstrated improved survival after prenatal diagnosis of critical congenital heart disease, including hypoplastic left heart syndrome (HLHS). However, the effect of delivery near a cardiac surgical center (CSC), the recommended action after prenatal diagnosis, on HLHS mortality has been poorly investigated. METHODS AND RESULTS:Using Texas Birth Defects Registry data, 1999 through 2007, which monitored >3.4 million births, we investigated the association between distance (calculated driving time) from birth center to CSC and neonatal mortality in 463 infants with HLHS. Infants with extracardiac birth defects or genetic disorders were excluded. The associations between prenatal diagnosis, CSC HLHS volume, and mortality were also examined. Neonatal mortality in infants born <10 minutes from a CSC was 21.0%, 10 to 90 minutes 25.2%, and >90 minutes 39.6% (P for trend <0.001). Prenatal diagnosis alone was not associated with improved survival (P=0.14). In multivariable analysis, birth >90 minutes from a CSC remained associated with increased mortality (odds ratio, 2.03; 95% confidence interval, 1.19-3.45), compared with <10 minutes. In subanalysis, birth >90 minutes from a CSC was associated with higher pretransport mortality (odds ratio, 6.69; 95% confidence interval, 2.52-17.74) and birth 10 to 90 minutes with higher presurgical mortality (odds ratio, 4.45; 95% confidence interval, 1.17-17.00). Higher surgical mortality was associated with lower CSC HLHS volume (odds ratio per 10 patients, 0.88; 95% confidence interval, 0.84-0.91). CONCLUSIONS:Infants with HLHS born far from a CSC have increased neonatal mortality, and most of this mortality is presurgical. Efforts to improve prenatal diagnosis of HLHS and subsequent delivery near a large volume CSC may significantly improve neonatal HLHS survival.
| S-EPMC7302400 | biostudies-literature
Project description:IntroductionWhipple procedure is one of the major surgeries performed in tertiary care centers. Once considered a high mortality procedure is now being practicing with mortality declining to less than 5%. This study describes our five-year experience of the Whipple procedure in terms of preoperative, operative, and postoperative parameters of patients undergoing surgery in a local tertiary care setting.Material and methodsThis was a non-randomized interventional study that was conducted at the Surgical Department of Services Hospital Lahore from January 2014 to December 2018. A total of 57 Whipple procedures were performed during this period. Demographic data, presenting symptoms, physical signs, past medical history, preoperative stenting details, intra-operative duration of surgery, postoperative course and complications, pathology, and causes of postoperative death were collected on a pre-designed questionnaire. Data were entered and analyzed by using SPSS 22 (IBM Corp., Armonk, USA).ResultsOut of 57 patients, 19 were females and 38 were males. The mean age of patients was 53±05 years. The most common presenting symptom was jaundice 39 (68.4%), followed by abdominal pain 32 (56.1%). The mean size of the tumor on CT-scan was 2.8±1.4 cm, the mean operation time was 315±38.3 min, mean blood loss during surgery was 500±130 ml, and mean hospital stay was 10±6 days. The major postoperative complication was the pancreatic fistula (12%). Twenty-one out of 39 patients presented with jaundice had undergone preoperative biliary stenting by endoscopic retrograde biliary stenting. The most common histological diagnosis was adenocarcinoma of pancreas 19 (33.3%). Out of 57 patients, nine (15.8%) patients expired in the first 30 days and the most frequent cause of mortality was septic shock.ConclusionIn this study, the most common presentation of patients undergoing Whipple procedure was obstructive jaundice, the most frequent operative complication was pancreatic fistula, and the most prevalent histopathology was carcinoma of the pancreas. Perioperative parameters such as mean operative time, mean blood loss during surgery, and mean length of hospital stay were comparable with other studies. However, mortality in this study was slightly higher. It can be concluded that with meticulous surgical technique, securing hemostasis strictly and standard critical care postoperatively can decrease morbidity and mortality after the Whipple procedure.
| S-EPMC7733773 | biostudies-literature
Project description:Retinoblastoma is the most common intraocular malignancy of childhood. In most cases, parents are the first to notice leukocoria and other symptoms before undergoing a prolonged period of stress before diagnosis. The purpose of this study was to determine prediagnostic intervals of patients with retinoblastoma at an oncology tertiary center (Instituto Nacional de Cancer) in Rio de Janeiro, Brazil, and relate them to stage at diagnosis, eye salvage, and survival.Parents or caregivers of children with retinoblastoma registered between January 2006 and September 2013 were interviewed using a semistructured individually applied questionnaire, concerning their trajectory before registration.Out of 76 patients, 39 (51%) were girls, 52 (68%) had unilateral retinoblastoma, and 24 (32%) had bilateral retinoblastoma, totaling 100 affected eyes. The most common stage of diagnosis was the intraocular group, with 63 (83%) patients; nine (12%) were extraocular, and four (5%) had metastatic disease. During the follow-up time of 37 ± 24.5 months, 10 (13%) patients died and 70 (70%) eyes were enucleated. Mean family interval was 1.6 ± 2.6 months, mean medical interval was 5.0 ± 6.2 months, mean referral interval was 0.2 ± 1.4 months, and mean overall interval was 7.1 ± 6.9 months. In univariate analysis, age at diagnosis, maternal education, medical interval, and overall interval were significantly related to advanced stage at diagnosis and survival. In multivariate analysis, maternal education and medical interval were significantly related to advanced stage at diagnosis and survival. No variables affected eye salvage.Medical interval was responsible for 70% of the overall interval; therefore, programs or campaigns targeting retinoblastoma early diagnosis should focus emphasize in medical awareness.
| S-EPMC5560453 | biostudies-other
Project description:The hybrid palliation for hypoplastic left heart syndrome has emerged as an alternative approach to the Norwood procedure. The development of patent ductus arteriosus (PDA) in-stent stenosis can cause retrograde aortic arch stenosis (RAAS), leading to significant morbidity. This study aimed to identify potential mechanisms of PDA in-stent stenosis contributing to RAAS.Tissues from stented PDAs were collected from 17 patients undergoing comprehensive stage II repair between 2009 and 2014. Patients requiring RAAS intervention based on cardiology-surgery consensus were defined as RAAS(+) (n = 10), whereas patients without any RAAS intervention were defined as RAAS(-) (n = 7). Tissues were examined by quantitative polymerase chain reaction analysis for vascular smooth muscle cell (VSMC) differentiation and proliferation markers.Patient characteristics were hypoplastic left heart syndrome with aortic atresia in 6 and with aortic stenosis in 3; unbalanced atrioventricular canal in 3; double-inlet left ventricle/transposition of the great arteries in 3; and double-outlet right ventricle in 2. VSMC differentiation markers (?-actin, SM22, and calponin) and signaling pathways for VSMC modulation (transforming growth factor-?1, Notch, and platelet derived growth factor-BB) were significantly higher in the RAAS(+) than in RAAS(-) patients. The proliferation marker Ki67 was increased in RAAS(+) patients. Cell cycle markers were comparable in both groups.Increased VSMC differentiation and proliferation markers suggest a mechanism for inward neointima formation of the PDA in RAAS. The apparent lack of change in cell cycle markers is contrary to coronary artery in-stent stenosis, suggesting further targets should be examined. Combined primary in vitro PDA cell culture and proteomics can be strong tools to elucidate targets to reduce PDA in-stent stenosis for RAAS in the future.
| S-EPMC4567479 | biostudies-literature
Project description:BackgroundTransvenous Lead Extraction (TLE) is a standard treatment for some late Cardiac Implantable Electronics Device (CIED) complications. The outcome of transvenous lead extraction procedure in Thailand is not robust.MethodsA Single-center retrospective cohort of TLE procedures performed at Ramathibodi hospital between January 2008 and December 2020 was studied.ResultsThere were 157 leads from 105 patients who underwent lead removal procedure during the specified period. Data analysis was performed from 79 TLE patients due to incomplete data and lead explant procedure of the excluded subjects. Mean patients' age was 57.7 ± 18.7 years, with 70.9% male. There were 82 pacemaker leads, 35 ICD leads, and 5 CS leads (mean number of leads were 1.54 ± 0.66 per patient), with mean implanted duration of 87.8 ± 68.2 months. Main indication for TLE was infection-related, which accounted for 67.1% of the cases. Overall clinical success rate was 97.5%. Mean operative time was 163.8 ± 69.5 min. Major complications occurred in 4 patients (5.1%) with one in-hospital mortality from severe sepsis.ConclusionTLE using laser sheath and rotating mechanical sheath for transvenous lead extraction is effective and safe, even outside high-volume center.
| S-EPMC9091722 | biostudies-literature
Project description:Congenital heart disease (CHD) affects up to 1% of live births. Although a genetic etiology is indicated by an increased recurrence risk, sporadic occurrence suggests that CHD genetics is complex. Here, we show that hypoplastic left heart syndrome (HLHS), a severe CHD, is multigenic and genetically heterogeneous. Using mouse forward genetics, we report what is, to our knowledge, the first isolation of HLHS mutant mice and identification of genes causing HLHS. Mutations from seven HLHS mouse lines showed multigenic enrichment in ten human chromosome regions linked to HLHS. Mutations in Sap130 and Pcdha9, genes not previously associated with CHD, were validated by CRISPR-Cas9 genome editing in mice as being digenic causes of HLHS. We also identified one subject with HLHS with SAP130 and PCDHA13 mutations. Mouse and zebrafish modeling showed that Sap130 mediates left ventricular hypoplasia, whereas Pcdha9 increases penetrance of aortic valve abnormalities, both signature HLHS defects. These findings show that HLHS can arise genetically in a combinatorial fashion, thus providing a new paradigm for the complex genetics of CHD.
| S-EPMC5737968 | biostudies-literature
Project description:ObjectiveMicro-RNAs (miRNAs) are important regulators of gene expression through interaction with the 3'UTR of target messenger RNAs (mRNAs). The role of miRNAs has been extensively studied in adult human and nonhuman animal models of heart disease. Hypoplastic left heart syndrome (HLHS) is the most common form of severe congenital heart disease and is an important cause of morbidity and mortality in infants and children. The objective of this work was to analyze the miRNA profile in HLHS patients.Methods and resultsmiRNA profile was determined in the right ventricle with the use of miRNA array, and expression was validated with the use of reverse-transcription polymerase chain reaction (RT-PCR). Based on bioinformatics analysis, targets were selected and their expression analyzed with the use of RT-PCR.We found that the miRNA profile of HLHS is novel, with few similarities between pediatric and adult idiopathic dilated cardiomyopathy. Moreover, our analysis identified putative targets for these miRNAs that are known to be important for cardiac development and disease, and that miRNAs and their putative targets are antithetically regulated. We also found that miRNA expression changes with stage of surgery, suggesting that volume unloading of the ventricle has important consequences for gene expression.ConclusionsOur data suggest a unique miRNA profile for HLHS that may be associated with defects in cardiac development and disease.
| S-EPMC4276459 | biostudies-literature
Project description:In patients with longstanding persistent atrial fibrillation (AF), outcomes from catheter ablation remain suboptimal. The convergent procedure combines minimally invasive surgical ablation with subsequent catheter ablation, and may contribute towards maintenance of sinus rhythm in this patient group. We performed the convergent procedure on 43 patients with longstanding persistent AF from 2013-2018. Patients underwent clinical review at 3, 6, and 12 months and thereafter as necessitated by their symptoms. Our dataset describes patients' baseline characteristics and rhythm control protocols, as well as outcomes including arrhythmia recurrence, the need for antiarrhythmic drugs, requirement for repeat rhythm control procedures, and complications. These data provide a real world insight into the risks and benefits of the convergent procedure in patients with longstanding persistent AF.
| S-EPMC7118295 | biostudies-literature