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1.
JACC Case Rep ; 4(4): 226-229, 2022 Feb 16.
Article in English | MEDLINE | ID: mdl-35199021

ABSTRACT

Aortic dissection is very rare in pediatric patients, and associated risk factors include congenital heart disease, previous cardiac surgery, and vasculopathies. Acute postoperative aortic dissection in pediatric patients can be life-threatening. We performed a novel hybrid transcarotid covered stent exclusion of a postoperative ascending aortic dissection in an infant. (Level of Difficulty: Advanced.).

2.
Ann Thorac Surg ; 114(3): e169-e172, 2022 09.
Article in English | MEDLINE | ID: mdl-34968446

ABSTRACT

Despite early clinical success of transcatheter pulmonic valve replacement, there is concern for an increased risk of endocarditis requiring complex surgery to repair. We present a case of endocarditis of a Melody (Medtronic, Minneapolis, MN) valve in a 33-year-old male patient with prior neonatal repair of persistent truncus arteriosus and 2 subsequent right ventricular outflow tract to pulmonary artery conduit replacements. The infection had extended from the Melody valve through the prior ventricular septal defect patch to the truncal valve and highlights the risk of endocarditis, particularly with the Melody transcatheter valve.


Subject(s)
Cardiac Surgical Procedures , Endocarditis , Heart Defects, Congenital , Heart Valve Prosthesis Implantation , Heart Valve Prosthesis , Pulmonary Valve , Adult , Cardiac Catheterization/adverse effects , Endocarditis/surgery , Heart Defects, Congenital/surgery , Heart Valve Prosthesis/adverse effects , Heart Valve Prosthesis Implantation/adverse effects , Humans , Infant, Newborn , Male , Pulmonary Valve/surgery , Treatment Outcome
3.
World J Pediatr Congenit Heart Surg ; 13(4): 510-511, 2022 07.
Article in English | MEDLINE | ID: mdl-34962179

ABSTRACT

We report the unique case of a 2-year-old male with severe heart failure requiring mechanical circulatory support with a left ventricular assist device, who developed adenovirus pneumonitis infection requiring veno-venous extracorporeal membrane oxygenation (ECMO) support. He progressed to acute respiratory failure and refractory hypoxemia despite intubation with maximum respiratory support. The patient was placed on ECMO with improvement in lung function over four days with subsequent successful decannulation. During the ECMO run, anticoagulation required escalation given the increased circuit surface area. Patient has since recovered and undergone heart transplantation.


Subject(s)
Extracorporeal Membrane Oxygenation , Heart Failure , Heart Transplantation , Heart-Assist Devices , Respiratory Distress Syndrome , Respiratory Insufficiency , Child , Child, Preschool , Heart Failure/complications , Heart Failure/therapy , Humans , Male , Respiratory Insufficiency/etiology , Respiratory Insufficiency/therapy
5.
JACC Cardiovasc Interv ; 13(12): 1484-1488, 2020 06 22.
Article in English | MEDLINE | ID: mdl-32250751

ABSTRACT

The coronavirus disease-2019 (COVID-19) pandemic has strained health care resources around the world, causing many institutions to curtail or stop elective procedures. This has resulted in an inability to care for patients with valvular and structural heart disease in a timely fashion, potentially placing these patients at increased risk for adverse cardiovascular complications, including CHF and death. The effective triage of these patients has become challenging in the current environment as clinicians have had to weigh the risk of bringing susceptible patients into the hospital environment during the COVID-19 pandemic against the risk of delaying a needed procedure. In this document, the authors suggest guidelines for how to triage patients in need of structural heart disease interventions and provide a framework for how to decide when it may be appropriate to proceed with intervention despite the ongoing pandemic. In particular, the authors address the triage of patients in need of transcatheter aortic valve replacement and percutaneous mitral valve repair. The authors also address procedural issues and considerations for the function of structural heart disease teams during the COVID-19 pandemic.


Subject(s)
Betacoronavirus , Cardiovascular Surgical Procedures , Coronavirus Infections/epidemiology , Heart Diseases/surgery , Patient Selection , Pneumonia, Viral/epidemiology , Triage , Ambulatory Surgical Procedures , COVID-19 , Coronavirus Infections/prevention & control , Coronavirus Infections/transmission , Humans , Pandemics/prevention & control , Pneumonia, Viral/prevention & control , Pneumonia, Viral/transmission , Referral and Consultation , SARS-CoV-2
6.
Catheter Cardiovasc Interv ; 96(3): 659-663, 2020 09 01.
Article in English | MEDLINE | ID: mdl-32251546

ABSTRACT

The coronavirus disease-2019 (COVID-19) pandemic has strained health care resources around the world, causing many institutions to curtail or stop elective procedures. This has resulted in an inability to care for patients with valvular and structural heart disease in a timely fashion, potentially placing these patients at increased risk for adverse cardiovascular complications, including CHF and death. The effective triage of these patients has become challenging in the current environment, as clinicians have had to weigh the risk of bringing susceptible patients into the hospital environment during the COVID-19 pandemic against the risk of delaying a needed procedure. In this document, the authors suggest guidelines for how to triage patients in need of structural heart disease interventions and provide a framework for how to decide when it may be appropriate to proceed with intervention despite the ongoing pandemic. In particular, the authors address the triage of patients in need of transcatheter aortic valve replacement and percutaneous mitral valve repair. The authors also address procedural issues and considerations for the function of structural heart disease teams during the COVID-19 pandemic.


Subject(s)
Cardiac Surgical Procedures/statistics & numerical data , Coronavirus Infections/epidemiology , Heart Diseases/surgery , Pandemics/statistics & numerical data , Pneumonia, Viral/epidemiology , Practice Guidelines as Topic , Triage/standards , COVID-19 , Cardiac Surgical Procedures/methods , Cardiology/methods , Cardiology/standards , Coronavirus Infections/prevention & control , Cross Infection/prevention & control , Female , Heart Diseases/diagnostic imaging , Humans , Male , Occupational Health/statistics & numerical data , Pandemics/prevention & control , Patient Safety , Pneumonia, Viral/prevention & control , Societies, Medical , Triage/statistics & numerical data , United States
7.
Hand Clin ; 35(1): 43-50, 2019 02.
Article in English | MEDLINE | ID: mdl-30470330

ABSTRACT

The implementation of the wide-awake local anesthetic no tourniquet (WALANT) approach to surgical procedures in Cyprus has led to significant cost savings. In the United Kingdom, the implementation of WALANT has led to shorter waiting times for hand surgical procedures, cost savings for the National Health Service, and high patient satisfaction rates. In both countries, patient education is a prerequisite for WALANT surgery. It increases the satisfaction rate among patients and enhances the patient-surgeon relationship. Patients need to know they can participate actively in a procedure, because a patient moving the hand during a procedure can improve the outcome.


Subject(s)
Ambulatory Surgical Procedures , Anesthesia, Local , Hand/surgery , Orthopedic Procedures , Anesthetics, Local/administration & dosage , Cost Savings , Cyprus , Epinephrine/administration & dosage , Health Services Accessibility , Humans , Levobupivacaine/administration & dosage , Lidocaine/administration & dosage , Patient Acceptance of Health Care , Patient Satisfaction/statistics & numerical data , Referral and Consultation , United Kingdom , Vasoconstrictor Agents/administration & dosage
8.
J Orthop Surg (Hong Kong) ; 26(3): 2309499018792744, 2018.
Article in English | MEDLINE | ID: mdl-30111240

ABSTRACT

AIM: To determine whether a mechanical, high-frequency vibration device (Tenease™) can improve pain and function for the treatment of tennis elbow (TE), compared with standard treatment. METHODS: Adults presenting to an elbow clinic with a clinical diagnosis of TE were randomized to standard treatment with physiotherapy, activity modification and analgesia or standard treatment plus Tenease therapy. Tenease therapy consisted of a 6-week period of treatment using the Tenease device with three 10-min episodes each day. The primary outcome measure was the quick Disabilities of the Arm, Shoulder and Hand score at 6 months, with scores also taken at 6 weeks. Secondary outcome measures were the Patient Rated Tennis Elbow Evaluation Score and EuroQol 5-Dimension Visual Analogue Scale at the same time points. RESULTS: Fifty-four patients were recruited into the study. Following randomization and initial dropout, 18 patients were included in the standard group and 27 in the Tenease group. Both groups reported improvements in primary outcome measure scores. The control group had a mean score of 44.3 (standard deviation (SD) = 18.8) at baseline, which dropped to 31.2 (SD = 17.2) at 6 months ( p = 0.002). The Tenease group had a mean score of 43.2 (SD = 22.7) at baseline, which dropped to 23.4 (SD = 15.0) at 6 months ( p = 0.064). Similar improvements were seen in secondary outcome measures with none reaching statistical significance. There were no statistically significant differences seen between the primary outcome scores at 6 weeks ( p = 0.9) or 6 months ( p = 0.5). No complications were noted in either group. CONCLUSIONS: Vibration therapy did not result in any statistically significant improvement in functional outcome scores compared to standard treatment for TE. It is important to note that this was a relatively small cohort and a high dropout rate was observed.


Subject(s)
Physical Therapy Modalities , Tennis Elbow/therapy , Vibration/therapeutic use , Adult , Aged , Female , Humans , Male , Middle Aged , Outcome Assessment, Health Care , Treatment Outcome
9.
Perfusion ; 33(3): 194-202, 2018 04.
Article in English | MEDLINE | ID: mdl-28985692

ABSTRACT

BACKGROUND: Cardiac surgery on Jehovah's Witnesses (JW) can be challenging, given the desire to avoid blood products. Establishment of a blood conservation program involving the pre-, intra- and post-operative stages for all patients may lead to a minimized need for blood transfusion in all patients. METHODS: Pre-operatively, all JW patients were treated with high dose erythropoietin 500 IU/kg twice a week. JW patients were compared to matching non-JW patients from the congenital cardiac database, two per JW to serve as control. Blood use, ventilation time, bypass time, pre-operative hematocrit, first in intensive care unit (ICU) and at discharge and 24 hour chest drainage were compared. Pre-operative huddle, operating room huddle and post-operative bedside handoff were done with the congenital cardiac surgeon, perfusionist, anesthesiologist and intensive care team in all patients for goal alignment. RESULTS: Five JW patients (mean weight 24.4 ± 25.0 Kg, range 6.3 - 60 Kg) were compared to 10 non-JW patients (weight 22.0 ± 22.8 Kg, range 6.2 - 67.8 Kg). There was no difference in bypass, cross-clamp, time to extubation (0.8 vs. 2.1 hours), peak inotrope score (2.0 vs. 2.3) or chest drainage. No JW patient received a blood product compared to 40% of non-JW. The pre-operative hematocrit (Hct) was statistically greater for the JW patients (46.1 ± 3.3% vs. 36.3 ± 4.7%, p<0.001) and both ICU and discharge Hct were higher for the JW (37 ± 1.8% vs 32.4 ± 8.0% and 41 ± 8.1% vs 34.8 ± 7.9%), but did not reach statistical significance. All patients had similar blood draws during the hospitalization (JW x 18 mL/admission vs non-JW 20 mL/admission). CONCLUSION: The continuous application and development of blood conservation techniques across the continuum of care allowed bloodless surgery for JW and non-JW patients alike. Blood conservation is a team sport and to make significant strides requires participation and input by all care providers.


Subject(s)
Bloodless Medical and Surgical Procedures/methods , Cardiac Surgical Procedures/methods , Erythropoietin/therapeutic use , Adolescent , Adult , Blood Transfusion , Child , Child, Preschool , Erythropoietin/administration & dosage , Hematocrit , Humans , Infant , Intensive Care Units , Jehovah's Witnesses , Length of Stay , Preoperative Care/methods , Young Adult
10.
JACC Cardiovasc Interv ; 9(23): 2429-2437, 2016 12 12.
Article in English | MEDLINE | ID: mdl-27931595

ABSTRACT

OBJECTIVES: The goal of this study was to describe early and midterm outcomes of extremely premature newborns (EPNs) who underwent transcatheter echocardiographically guided patent ductus arteriosus (PDA) closure. BACKGROUND: Surgical ligation of PDA in EPNs confers significant risk for procedural morbidity and adverse long-term outcomes. METHODS: The Amplatzer Vascular Plug II was used in all cases. Post-ligation syndrome was defined using previously published parameters. Patients were followed at pre-specified intervals, and prospectively collected data were reviewed. RESULTS: Transcatheter closure was attempted in 24 EPNs (mean procedural age 30 days [range 5 to 80 days], mean procedural weight 1,249 g [range 755 to 2,380 g]) and was successful in 88%. The 3 procedural failures were related to the development of left pulmonary artery (LPA) stenosis caused by the device, and all devices were removed uneventfully. Complications included 2 instances of device malposition, resolved with device repositioning, and 1 instance of LPA stenosis, requiring an LPA stent. There were no procedural deaths, cases of post-ligation syndrome, residual PDA, or device embolization. Survival to discharge was 96% (23 of 24), with a single late death unrelated to the procedure. After a median follow-up period of 11.1 months, all patients were alive and well, with no residual PDA or evidence of LPA or aortic coarctation. CONCLUSIONS: This newly described technique can be performed safely with a high success rate and minimal procedural morbidity in EPNs. Early and midterm follow-up is encouraging. Future efforts should be directed toward developing specific devices for this unique application.


Subject(s)
Cardiac Catheterization , Ductus Arteriosus, Patent/therapy , Infant, Extremely Premature , Cardiac Catheterization/adverse effects , Cardiac Catheterization/instrumentation , Cardiac Catheterization/mortality , Ductus Arteriosus, Patent/diagnostic imaging , Ductus Arteriosus, Patent/mortality , Ductus Arteriosus, Patent/physiopathology , Echocardiography, Doppler, Color , Echocardiography, Doppler, Pulsed , Gestational Age , Humans , Infant, Newborn , Predictive Value of Tests , Retrospective Studies , Risk Factors , Time Factors , Treatment Outcome
11.
Eur J Emerg Med ; 23(3): 190-3, 2016 Jun.
Article in English | MEDLINE | ID: mdl-25460813

ABSTRACT

BACKGROUND: Wrist injuries are common, and there is often diagnostic uncertainty following normal initial radiographs when there is ongoing clinical suspicion of a scaphoid fracture.The aims of this study were to define the problem in our hospital, and to identify current practice relating to the management of patients with clinically suspected scaphoid injury across hospitals in England. METHODS: A retrospective review was undertaken of all patients presenting to our Emergency Department with a wrist injury, over a 12-month period.A cross-sectional survey of all hospitals in NHS England was then undertaken, using a web-based questionnaire. The results of the survey were analysed in Microsoft Excel to provide descriptive data only. RESULTS: Of 2367 patients presenting to our Emergency Department with a wrist injury, 420 (17.7%) were followed up due to ongoing clinical suspicion of a scaphoid injury.From the 141 acute hospital Trusts in NHS England, survey responses were received from 116 (82% response rate).Symptomatic patients undergo repeat radiograph in 68.0% of hospitals before second line imaging. This is performed using MRI in 63.9%, computed tomography in 27.0% or isotope bone scan in 9.0%.Secondary imaging is carried out between 10 days and 4 weeks following the initial injury in 86.8% of trusts. CONCLUSION: This is still considerable variability in the way these patients are managed in England. Further work needs to be undertaken to establish the most appropriate way to manage patients with wrist injuries with ongoing clinical suspicion of scaphoid fracture.


Subject(s)
Fractures, Bone/diagnostic imaging , Scaphoid Bone/injuries , Adult , Cross-Sectional Studies , Emergency Service, Hospital/statistics & numerical data , England , Humans , Magnetic Resonance Imaging , Positron Emission Tomography Computed Tomography , Radiography , Retrospective Studies , Scaphoid Bone/diagnostic imaging , Surveys and Questionnaires , Tomography, X-Ray Computed
12.
Catheter Cardiovasc Interv ; 87(3): 403-10, 2016 Feb 15.
Article in English | MEDLINE | ID: mdl-26527499

ABSTRACT

BACKGROUND: Transcatheter pulmonary valve replacement (tPVR) is an accepted therapy for treatment of dysfunctional right ventricular outflow tract (RVOT) conduits. At present, the majority of Fallot patients who undergo transannular patch (TAP) repair are not candidates for tPVR due to the large irregular nature of their RVOT. Herein, we describe a novel approach to assessing the RVOT in this group, which may then be used to design, test, and carry out hybrid RVOT modification and transcatheter valve implantation in this population. METHODS: A retrospective analysis of TAP patients who underwent 3D modeling of the RVOT which was then used to develop individualized hybrid procedures designed to modify the RVOT, thereby rendering patients suitable for transcatheter valve implantation. RESULTS: Eight consecutive patients underwent 3D RVOT modeling followed by hybrid implantation of a transcatheter valve via a perventricular approach. A landing zone stent was placed in all and four required additional intravascular geometric remodeling of the RVOT prior to valve implant. Transcatheter valves were successfully implanted in all. There were no instances of valve malposition, embolization, or death. There was one minor procedural complication. No patient had more than trivial pulmonary regurgitation at follow-up. CONCLUSIONS: Using a hybrid approach to remodel the RVOT in TAP patients supported by preprocedural 3D-model planning allows for successful tPVR implantation in this population. A larger cohort and longer follow-up will be needed to determine the ultimate utility of this approach.


Subject(s)
Cardiac Catheterization/methods , Cardiac Surgical Procedures/adverse effects , Heart Valve Prosthesis Implantation/methods , Pulmonary Valve Insufficiency/therapy , Pulmonary Valve , Tetralogy of Fallot/surgery , Adolescent , Adult , Cardiac Catheterization/instrumentation , Child , Heart Valve Prosthesis , Heart Valve Prosthesis Implantation/instrumentation , Humans , Imaging, Three-Dimensional , Magnetic Resonance Imaging , Middle Aged , Models, Anatomic , Models, Cardiovascular , Prosthesis Design , Pulmonary Valve/diagnostic imaging , Pulmonary Valve Insufficiency/diagnostic imaging , Pulmonary Valve Insufficiency/etiology , Pulmonary Valve Insufficiency/physiopathology , Radiography, Interventional , Retrospective Studies , Stents , Tetralogy of Fallot/diagnosis , Tomography, X-Ray Computed , Treatment Outcome , Young Adult
13.
J Thorac Cardiovasc Surg ; 150(6): 1440-50, 1452.e1-8; discussion 1450-2, 2015 Dec.
Article in English | MEDLINE | ID: mdl-26254760

ABSTRACT

OBJECTIVE: Early survival advantages after Norwood with right-ventricle-(RV)-to-pulmonary-artery conduit (NW-RVPA) over Norwood-operation with a Blalock-Taussig shunt (NW-BT) are offset by concerns regarding delayed RV dysfunction. We compared trends in survival, RV dysfunction, and tricuspid valve regurgitation (TR) between NW-RVPA and NW-BT for propensity-matched neonates with critical left ventricular outflow tract obstruction (LVOTO). METHODS: In an inception cohort (2005-2014; 21 institutions), 454 neonates with critical LVOTO underwent Norwood stage 1. Propensity-score matching paired 169 NW-RVPA patients with 169 NW-BT patients. End-states were compared between NW-RVPA and NW-BT using competing-risks, multiphase, parametric, hazard analysis. Post-Norwood echocardiogram reports (n = 2993) were used to grade RV dysfunction and TR. Time-related prevalence of ≥moderate RV dysfunction and TR were characterized using nonlinear mixed-model regression, and compared between groups via multiphase, parametric models. RESULTS: Overall 6-year survival was better after NW-RVPA (70%) versus NW-BT (55%; P < .001). Additionally, transplant-free survival during this time was better after NW-RVPA (64%) versus NW-BT (53%; P = .004). Overall prevalence of ≥moderate RV dysfunction reached 11% within 3 months post-Norwood. During this time, RV dysfunction after NW-BT was 16% versus 6% after NW-RVPA (P = .02), and coincided temporally with an increased early hazard for death. For survivors, late RV dysfunction was <5% and was not different between groups (P = .36). Overall prevalence of ≥moderate TR reached 13% at 2 years post-Norwood and was increased after NW-BT (16%) versus NW-RVPA (11%; P = .003). Late TR was similar between groups. CONCLUSIONS: Among propensity-score-matched neonates with critical LVOTO, NW-RVPA offers superior 6-year survival with no greater prevalence of RV dysfunction or TR than conventional NW-BT operations.


Subject(s)
Blalock-Taussig Procedure , Heart Ventricles/surgery , Norwood Procedures , Pulmonary Artery/surgery , Ventricular Function/physiology , Blalock-Taussig Procedure/mortality , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Norwood Procedures/mortality , Tricuspid Valve Insufficiency/etiology , Ventricular Outflow Obstruction/surgery
14.
J Thorac Cardiovasc Surg ; 149(1): 230-6, 2015 Jan.
Article in English | MEDLINE | ID: mdl-24503323

ABSTRACT

BACKGROUND: Acute kidney injury (AKI) is common in infants after cardiopulmonary bypass and is associated with poor outcomes. Peritoneal dialysis improves outcomes in adults with AKI after bypass, but pediatric data are limited. This retrospective case-matched study was conducted to determine if the practice of peritoneal dialysis catheter (PDC) placement during congenital heart surgery is associated with improved clinical outcomes in infants at high risk for AKI. METHODS: Forty-two infants undergoing congenital heart surgery with planned PDC placement (PDC+) were age-matched to infants undergoing similar surgery without PDC placement (PDC-). Demographic, baseline and outcome data were compared. Our primary outcome was negative fluid balance on postoperative days 1 to 3. Secondary outcomes included time to negative fluid balance, time to extubation, frequency of electrolyte corrective medications, inotrope scores, and other clinical outcomes. RESULTS: Baseline data did not differ between groups. The PDC+ group had a higher percentage of negative fluid balance on postoperative days 1 and 2 (57% vs 33%, P = .04; 85% vs 61%, P = .01). The PDC+ group had shorter time to negative fluid balance (16 vs 32 hours, P < .0001), earlier extubation (80 vs 104 hours, P = .02), improved inotrope scores (P = .04), and fewer electrolyte imbalances requiring correction (P = .03). PDC-related complications were rare. CONCLUSIONS: PDC use is safe and associated with earlier negative fluid balance and improved clinical outcomes in infants at high risk for AKI. Routine PDC use should be considered for infants undergoing cardiopulmonary bypass. Further prospective studies are essential to prove causative effects of PDC placement in this population.


Subject(s)
Acute Kidney Injury/prevention & control , Cardiac Surgical Procedures , Cardiopulmonary Bypass , Catheters, Indwelling , Heart Defects, Congenital/surgery , Peritoneal Dialysis/instrumentation , Water-Electrolyte Imbalance/therapy , Acute Kidney Injury/diagnosis , Acute Kidney Injury/etiology , Cardiac Surgical Procedures/adverse effects , Cardiopulmonary Bypass/adverse effects , Equipment Design , Female , Heart Defects, Congenital/diagnosis , Humans , Infant, Newborn , Male , Peritoneal Dialysis/adverse effects , Retrospective Studies , Risk Factors , Time Factors , Treatment Outcome , Water-Electrolyte Balance , Water-Electrolyte Imbalance/diagnosis , Water-Electrolyte Imbalance/etiology , Water-Electrolyte Imbalance/physiopathology
15.
Injury ; 45(12): 1896-9, 2014 Dec.
Article in English | MEDLINE | ID: mdl-25441173

ABSTRACT

INTRODUCTION: Distal radial fractures are one of the most common fractures that are presented to the emergency department (ed). The management of non-emergent cases often involves closed reduction and immobilisation before referral to orthopaedic services. Surgical intervention is offered based on the criteria for instability. This can be predicted from the initial and post-manipulation radiographs. The purpose of this study was to assess the role of various predictors of instability in the requirement for surgery, based on specific evidence-based criteria. PATIENTS AND METHOD: We audited 87 consecutive distal radial fractures that had been manipulated by the ED and analysed what factors predicted instability. RESULTS: The most significant predictor of instability and hence further surgery was the failure to anatomically restore the volar cortex (VC) (p = 0.002) during the manipulation. Other significant factors were increasing age (p = 0.006) and fracture of the ulna styloid (p = 0.028). If the VC was restored or remained intact during the manipulation, only 38% required further surgery. If the VC was displaced or not restored, 65% required further surgery. CONCLUSION: The restoration or maintenance of volar cortical alignment during the manipulation of distal radial fractures offers patients the best chance of avoiding the need for further surgery. This factor should be taken into account in the decision-making process for these fractures.


Subject(s)
Fracture Fixation, Internal , Joint Instability/surgery , Palmar Plate/surgery , Radius Fractures/surgery , Aged , Female , Fracture Healing , Humans , Joint Instability/diagnostic imaging , Male , Palmar Plate/diagnostic imaging , Palmar Plate/physiopathology , Radiography , Radius Fractures/diagnostic imaging , Radius Fractures/physiopathology , Range of Motion, Articular , Recovery of Function
16.
PLoS One ; 7(8): e43515, 2012.
Article in English | MEDLINE | ID: mdl-22937058

ABSTRACT

Mutations affecting the expression of dystrophin result in progressive loss of skeletal muscle function and cardiomyopathy leading to early mortality. Interestingly, clinical studies revealed no correlation in disease severity or age of onset between cardiac and skeletal muscles, suggesting that dystrophin may play overlapping yet different roles in these two striated muscles. Since dystrophin serves as a structural and signaling scaffold, functional differences likely arise from tissue-specific protein interactions. To test this, we optimized a proteomics-based approach to purify, identify and compare the interactome of dystrophin between cardiac and skeletal muscles from as little as 50 mg of starting material. We found selective tissue-specific differences in the protein associations of cardiac and skeletal muscle full length dystrophin to syntrophins and dystrobrevins that couple dystrophin to signaling pathways. Importantly, we identified novel cardiac-specific interactions of dystrophin with proteins known to regulate cardiac contraction and to be involved in cardiac disease. Our approach overcomes a major challenge in the muscular dystrophy field of rapidly and consistently identifying bona fide dystrophin-interacting proteins in tissues. In addition, our findings support the existence of cardiac-specific functions of dystrophin and may guide studies into early triggers of cardiac disease in Duchenne and Becker muscular dystrophies.


Subject(s)
Dystrophin-Associated Proteins/metabolism , Proteomics/methods , Dystrophin/metabolism , Humans , Immunoprecipitation , Mass Spectrometry , Muscle, Skeletal/metabolism , Myocardium/metabolism , Protein Binding , Signal Transduction/genetics , Signal Transduction/physiology , Tandem Mass Spectrometry
17.
Transpl Immunol ; 27(4): 171-4, 2012 Dec.
Article in English | MEDLINE | ID: mdl-22910399

ABSTRACT

Antibody-mediated rejection (AMR) is becoming a more recognized problem in lung transplantation. We present a case of late onset AMR in a lung transplant recipient after an acute embolic stroke requiring thrombolytic therapy, who previously had a completely unremarkable course for over 3 years.


Subject(s)
Graft Rejection/etiology , Graft Rejection/immunology , Lung Transplantation/adverse effects , Lung Transplantation/immunology , Stroke/complications , Stroke/immunology , Complement C3d/metabolism , Complement C4b/metabolism , Cystic Fibrosis/surgery , Fatal Outcome , Female , Histocompatibility Testing , Humans , Isoantibodies/metabolism , Lung/immunology , Lung/pathology , Lung Transplantation/pathology , Peptide Fragments/metabolism , Time Factors , Tissue Donors , Young Adult
18.
Hip Int ; 22(3): 329-34, 2012.
Article in English | MEDLINE | ID: mdl-22740276

ABSTRACT

PURPOSE: The purpose of this study was to investigate whether patients who have undergone surgical hip dislocation with excision of the ligamentum teres exhibited symptoms of a Type I ligamentum teres rupture. METHOD: A questionnaire was designed to enquire about specific symptoms attributed to Type I injuries (complete rupture) in the literature: gross instability, incomplete reduction, inability to bear weight and mechanical symptoms. 217 consecutive patients were surveyed and 161 patients (75%) returned 168 questionnaires (75%). Mean age was 34 and median follow-up was 51 months (35 to 97). RESULTS: All patients were able to fully weight bear after surgery while 77% experienced no groin pain and 61% experienced no pain on exercise. 35% of patients experienced popping and locking in their operated hip and 24% had subjective feeling of their hip giving way. CONCLUSION: Patients who have had surgical excision of the ligamentum teres during open osteochondroplasty for cam deformities do not exhibit symptoms of a Type I ligamentum teres rupture. Our results show that minor instability symptoms may be present but symptoms present more like to Type II (partial) injuries. We propose that Type II injuries be divided into Type IIa; partial tear and Type IIb; iatrogenic rupture.


Subject(s)
Arthroscopy/adverse effects , Femoracetabular Impingement/surgery , Hip Dislocation/etiology , Hip Joint/surgery , Joint Instability/etiology , Ligaments, Articular/surgery , Adolescent , Adult , Aged , Child , Female , Hip Dislocation/physiopathology , Hip Joint/physiopathology , Humans , Joint Instability/physiopathology , Ligaments, Articular/injuries , Ligaments, Articular/physiopathology , Male , Middle Aged , Outcome Assessment, Health Care , Postoperative Complications , Rupture , Surveys and Questionnaires , Weight-Bearing , Young Adult
19.
Congenit Heart Dis ; 7(2): 111-21, 2012.
Article in English | MEDLINE | ID: mdl-22171603

ABSTRACT

INTRODUCTION: Pulmonary atresia with ventricular septal defect (VSD) continues to be associated with significant morbidity and mortality, with significant institutional variation in therapeutic strategies. This study reports a single center experience utilizing an intensive transcatheter approach to promote pulmonary vascular growth. METHODS: A retrospective analysis of 20 patients undergoing surgical and transcatheter treatment for pulmonary atresia with VSD between 2002 and 2010. RESULTS: The median age at initial surgical palliation was 6.3 months (8 days to 2.5 years). Eleven patients (group 1) underwent initial surgical palliation without VSD closure and nine patients (group 2) underwent an initial complete repair with fenestrated or complete VSD closure. Group 1 had a smaller Nakata index (54 mm2/m2 vs. 134 mm2/m2 , P = .04) and a smaller absolute native pulmonary artery diameter (2.7 mm vs. 4.5 mm, P = .01) than group 2. Intraoperative angiography was performed in 10 cases to evaluate if early transcatheter intervention was warranted. The median follow-up during the study period was 2.3 years (1.6 months to 8.3 years). Of the 16 patients who survived the initial early postoperative period, 15 patients (94%) went on to receive surgical (n = 11) and/or interventional (n = 25) catheterization procedures. There was improvement in the mean Nakata index from the initial presurgical evaluation to the most recent catheterization data (38.4 mm2/m2 vs. 169.7 mm2/m2, P ≤ .05). To date, two of 11 (18%) patients in group 1 ultimately underwent surgical VSD closure. Overall mortality was six of 20 (30%) with four deaths in group 1 and two deaths in group 2. There were no procedural deaths. CONCLUSIONS: Combining surgical unifocalization procedures with subsequent early and intensive catheter-based pulmonary artery rehabilitation may improve vascular growth, ultimately rendering many patients suitable for fenestrated VSD closure. Risk stratification, including intraoperative exit angiography, is essential to determine the need for early transcatheter interventions.


Subject(s)
Heart Septal Defects, Ventricular/mortality , Heart Septal Defects, Ventricular/surgery , Pulmonary Atresia/mortality , Pulmonary Atresia/surgery , Pulmonary Circulation/physiology , Cardiac Catheterization , Child , Child, Preschool , DiGeorge Syndrome/mortality , Female , Follow-Up Studies , Heart Septal Defects, Ventricular/rehabilitation , Humans , Infant , Male , Morbidity , Palliative Care/methods , Pulmonary Atresia/rehabilitation , Retreatment , Retrospective Studies , Risk Factors , Tetralogy of Fallot/mortality , Tetralogy of Fallot/rehabilitation , Tetralogy of Fallot/surgery , Treatment Outcome
20.
World J Pediatr Congenit Heart Surg ; 3(1): 142-6, 2012 Jan 01.
Article in English | MEDLINE | ID: mdl-23804701

ABSTRACT

Sedation during invasive procedures provides appropriate humanitarian care as well as facilitates the completion of procedures. Although generally safe and effective, adverse effects may occur especially in patients with comorbid diseases. One particularly challenging situation is the child with an anterior mediastinal mass who requires sedation during performance of a biopsy to obtain a tissue diagnosis. When there is evidence of airway compromise, it is generally accepted that the maintenance of spontaneous ventilation is necessary as complete airway obstruction may occur, if positive pressure ventilation is chosen. We present the use of a dexmedetomidine-ketamine combination for procedural sedation in a three-year-old child who presented with a large mediastinal mass and respiratory compromise. Previous reports regarding the use of dexmedetomidine and ketamine for procedural sedation are reviewed and the potential efficacy of this combination is discussed.

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