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1.
JACC Clin Electrophysiol ; 8(3): 281-294, 2022 03.
Article in English | MEDLINE | ID: mdl-35331422

ABSTRACT

OBJECTIVES: This study sought to report our single-center experience with left cardiac sympathetic denervation (LCSD) for long QT syndrome (LQTS) since 1973. BACKGROUND: LCSD is still underutilized because clinicians are often uncertain whether to use it versus an implantable cardioverter-defibrillator (ICD). METHODS: We performed LCSD in 125 patients with LQTS (58% women, mean QT interval corrected for frequency [QTc] 527 ± 60 ms, 90% on beta blockers) with a follow-up of 12.9 ± 10.3 years. They were retrospectively divided into 4 groups according to the clinical/genetic status: very high risk (n = 18, symptomatic in the first year of life or with highly malignant genetics), with aborted cardiac arrest (ACA) (n = 31), with syncope and/or ICD shocks on beta blockers (n = 45), in primary prevention (n = 31). RESULTS: After LCSD, 17% in the very high risk group remained asymptomatic, compared with 52%, 47%, and 97% in the other 3 groups (P < 0.0001), with an overall 86% decrease in the mean yearly cardiac event rate (P < 0.0001). Among 45 patients with only syncope/ICD shocks before LCSD, none had ACA/sudden death as first symptom after LCSD and a 6-month post-LCSD QTc <500 ms predicted excellent outcome. Patients with a QTc ≥500 ms have a 50% chance of shortening it by an average of 60 ms. LCSD results are not affected by common genotypes. CONCLUSIONS: We provide definitive evidence for the long-term efficacy of LCSD in LQTS. The degree of antiarrhythmic protection is influenced by patient's specificity and amount of QTc shortening. This novel approach to the analysis of the outcome allows cardiologists to rationally decide and tailor their management strategies to the individual features of their patients.


Subject(s)
Long QT Syndrome , Adrenergic beta-Antagonists/therapeutic use , Female , Humans , Long QT Syndrome/diagnosis , Long QT Syndrome/surgery , Male , Retrospective Studies , Sympathectomy/adverse effects , Sympathectomy/methods , Syncope/etiology , Treatment Outcome
3.
Circulation ; 131(25): 2185-93, 2015 Jun 23.
Article in English | MEDLINE | ID: mdl-26019152

ABSTRACT

BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. ß-Βlockers are the mainstay of therapy; when they fail, implantable cardioverter-defibrillators (ICDs) are used but often cause multiple shocks. Preliminary results with flecainide appear encouraging. We proposed left cardiac sympathetic denervation (LCSD) as useful additional therapy, but evidence remains anecdotal. METHODS AND RESULTS: We report 63 patients with CPVT who underwent LCSD as secondary (n=54) or primary (n=9) prevention. The median post-LCSD follow-up was 37 months. The 9 asymptomatic patients remained free of major cardiac events. Of the 54 patients with prior major cardiac events either on (n=38) or off (n=16) optimal medical therapy, 13 (24%) had at least 1 recurrence: 0 patients had an aborted cardiac arrest, 2 patients had syncope only, 10 patients had ≥1 appropriate ICD discharges, and 1 patient died suddenly. The 1- and 2-year cumulative event-free survival rates were 87% and 81%. The percentage of patients with major cardiac events despite optimal medical therapy (n=38) was reduced from 100% to 32% (P<0.001) after LCSD, and among 29 patients with a presurgical ICD, the rate of shocks dropped by 93% from 3.6 to 0.6 shocks per person per year (P<0.001). Patients with an incomplete LCSD (n=7) were more likely to experience major cardiac events after LCSD (71% versus 17%; P<0.01) than those with a complete LCSD. CONCLUSIONS: LCSD is an effective antifibrillatory intervention for patients with CPVT. Whenever syncope occurs despite optimal medical therapy, LCSD could be considered the next step rather than an ICD and could complement ICDs in patients with recurrent shocks.


Subject(s)
Disease Management , Sympathectomy , Tachycardia, Ventricular/diagnosis , Tachycardia, Ventricular/surgery , Adolescent , Child , Female , Follow-Up Studies , Humans , Male , Sympathectomy/methods , Tachycardia, Ventricular/physiopathology , Treatment Outcome
4.
Stem Cells Dev ; 22(20): 2806-12, 2013 Oct 15.
Article in English | MEDLINE | ID: mdl-23750591

ABSTRACT

Critical limb ischemia (CLI) is burdened by a 40% major amputation rate, and a 5-year life expectancy <50%. We report the first in-human injection of lethally γ-irradiated non-human leukocyte antigen (HLA)-matched cord blood (CB)-derived mononuclear cells in a no-option CLI patient, to induce therapeutic neo-angiogenesis, with evidence of successful outcome supported by clinical findings (ulcer healing and pain relief), instrumental assessment (transcutaneous O2 pressure, ankle/brachial index, and contrast-enhanced ultrasonography), and histological demonstration of muscular tissue repair and capillary network expansion. If our approach will be confirmed, the huge number of CB units currently discarded might be redirected toward regenerative medicine purposes, leading to cutting-edge solutions for important unmet clinical needs, such as ischemic diseases, which remain the main cause of disability and mortality in western countries.


Subject(s)
Fetal Blood/cytology , Foot Ulcer/therapy , Foot/pathology , Graft Survival/immunology , Ischemia/therapy , Leukocytes, Mononuclear/cytology , Aged , Compassionate Use Trials , Foot/blood supply , Foot Ulcer/diagnostic imaging , Foot Ulcer/immunology , Foot Ulcer/pathology , Gamma Rays , HLA Antigens/immunology , Humans , Ischemia/diagnostic imaging , Ischemia/immunology , Ischemia/pathology , Leukocytes, Mononuclear/radiation effects , Leukocytes, Mononuclear/transplantation , Male , Neovascularization, Physiologic , Recovery of Function , Transplantation, Homologous , Treatment Outcome , Ultrasonography
5.
Ann Vasc Surg ; 27(6): 802.e13-5, 2013 Aug.
Article in English | MEDLINE | ID: mdl-23688942

ABSTRACT

Bronchial artery aneurysm (BAA) is a rare entity, detected in <1% of all patients who undergo selective bronchial arteriography. BAAs are potentially life-threatening when untreated. We describe the first BAA case, in the English language, treated only by thoracic aorta endografting, with an uneventful postoperative course with exclusion and thrombosis of the BAA. The endovascular stent graft provides a safe, reliable tool complementing the armamentarium of surgical and percutaneous techniques in the treatment of patients with BAA.


Subject(s)
Blood Vessel Prosthesis Implantation/methods , Catheterization, Peripheral/methods , Embolization, Therapeutic/methods , Endovascular Procedures/methods , Stents , Aneurysm/diagnostic imaging , Aneurysm/therapy , Angiography , Bronchial Arteries , Humans , Male , Middle Aged , Tomography, X-Ray Computed
9.
Vascular ; 21(1): 43-5, 2013 Feb.
Article in English | MEDLINE | ID: mdl-22871544

ABSTRACT

Ehlers-Danlos syndrome type 4, the vascular type, is a rare, life-threatening inherited disorder of the connective tissue. Affected patients are at risk of arterial, bowel and uterine rupture during pregnancy. Generally, this syndrome remains undiagnosed until a sudden, acute presentation with organ rupture, and results in premature death, even if the patients survive the first and second major complications. An early diagnosis with genetic assays can help to plan the best treatment, which is often challenging due to the frailty of the arterial tissue. We report on a 28-year-old lady who presented with spontaneous rupture of a pseudoaneurysm of the posterior tibial artery.


Subject(s)
Aneurysm, False/etiology , Aneurysm, Ruptured/etiology , Ehlers-Danlos Syndrome/complications , Tibial Arteries , Adult , Aneurysm, False/diagnosis , Aneurysm, False/surgery , Aneurysm, Ruptured/diagnosis , Aneurysm, Ruptured/surgery , Compartment Syndromes/etiology , Ehlers-Danlos Syndrome/diagnosis , Female , Hematoma/etiology , Humans , Rupture, Spontaneous , Tibial Arteries/diagnostic imaging , Tibial Arteries/surgery , Tomography, X-Ray Computed , Treatment Outcome
11.
Spine (Phila Pa 1976) ; 37(26): E1665-71, 2012 Dec 15.
Article in English | MEDLINE | ID: mdl-22990367

ABSTRACT

STUDY DESIGN: Case report and review of literature. OBJECTIVE: To highlight the specific features of a rare, life-threatening, clinical picture. SUMMARY OF BACKGROUND DATA: Vertebral erosion (VE) is rarely associated with contained rupture of an abdominal aortic aneurysm. The involvement of radicular nerves can mimic a discus hernia syndrome; eventually vertebral erosion induces isolated lower back pain. These features often lead to a delayed or wrong diagnosis of a life-threatening condition. Forty-two complete similar case reports have been published in the English literature since 1962. The most prevalent symptoms are low back pain and neurological signs due to compression of radicular nerves. METHODS: A 73-year-old man presented to Vascular Surgery department complaining of continuous pain in the lumbar region during the previous 6 months. The duplex examination revealed a huge infrarenal aortic aneurysm with an undefined posterior wall. Spiral CT and MR scan confirmed the aneurysm and a scalloping of the second and third lumbar vertebral bodies. RESULTS: A double-team intervention, vascular and orthopedic, consisted in aneurysm graft replacement; vertebral bodies excision and anterior and posterior spinal stabilization. Postoperatively the patient experienced reversible respiratory and renal failure and was discharged home in good health after 30 days. CONCLUSION: The presence of aortic abdominal aneurysm is always to be considered in the evaluation of an elderly patient complaining lower back pain or lower limb neuropathy of recent onset, especially in the presence of a degenerative process of the spine.


Subject(s)
Aortic Aneurysm, Abdominal/complications , Aortic Rupture/complications , Low Back Pain/etiology , Lumbar Vertebrae/surgery , Spinal Diseases/etiology , Aged , Aortic Aneurysm, Abdominal/diagnostic imaging , Aortic Aneurysm, Abdominal/surgery , Aortic Rupture/diagnostic imaging , Aortic Rupture/surgery , Humans , Low Back Pain/diagnostic imaging , Low Back Pain/surgery , Lumbar Vertebrae/diagnostic imaging , Male , Radiography , Spinal Diseases/diagnostic imaging , Spinal Diseases/surgery , Treatment Outcome
17.
J Endovasc Ther ; 18(3): 397-406, 2011 Jun.
Article in English | MEDLINE | ID: mdl-21679082

ABSTRACT

PURPOSE: To quantitatively evaluate the impact of carotid stent cell design on vessel scaffolding by using patient-specific finite element analysis of carotid artery stenting (CAS). METHODS: The study was organized in 2 parts: (1) validation of a patient-specific finite element analysis of CAS and (2) evaluation of vessel scaffolding. Micro-computed tomography (CT) images of an open-cell stent deployed in a patient-specific silicone mock artery were compared with the corresponding finite element analysis results. This simulation was repeated for the closed-cell counterpart. In the second part, the stent strut distribution, as reflected by the inter-strut angles, was evaluated for both cell types in different vessel cross sections as a measure of scaffolding. RESULTS: The results of the patient-specific finite element analysis of CAS matched well with experimental stent deployment both qualitatively and quantitatively, demonstrating the reliability of the numerical approach. The measured inter-strut angles suggested that the closed-cell design provided superior vessel scaffolding compared to the open-cell counterpart. However, the full strut interconnection of the closed-cell design reduced the stent's ability to accommodate to the irregular eccentric profile of the vessel cross section, leading to a gap between the stent surface and the vessel wall. CONCLUSION: Even though this study was limited to a single stent design and one vascular anatomy, the study confirmed the capability of dedicated computer simulations to predict differences in scaffolding by open- and closed-cell carotid artery stents. These simulations have the potential to be used in the design of novel carotid stents or for procedure planning.


Subject(s)
Angioplasty/instrumentation , Carotid Arteries/anatomy & histology , Computer Simulation , Finite Element Analysis , Models, Cardiovascular , Numerical Analysis, Computer-Assisted , Stents , Aged, 80 and over , Carotid Arteries/diagnostic imaging , Computer-Aided Design , Humans , Male , Models, Anatomic , Prosthesis Design , Reproducibility of Results , Silicones , X-Ray Microtomography
18.
Heart ; 97(4): 321-6, 2011 Feb.
Article in English | MEDLINE | ID: mdl-21212136

ABSTRACT

OBJECTIVE: To evaluate the prevalence and phenotype of smooth muscle alpha-actin (ACTA2) mutations in non-syndromic thoracic aortic aneurysms and dissections (TAAD). DESIGN: Observational study of ACTA2 mutations in TAAD. SETTING: Centre for Inherited Cardiovascular Diseases. PATIENTS: A consecutive series of 100 patients with TAAD. Exclusion criteria included genetically confirmed Marfan syndrome, Loeys-Dietz type 2, familial bicuspid aortic valve and Ehlers-Danlos type IV syndromes. INTERVENTIONS: Multidisciplinary clinical and imaging evaluation, genetic counselling and testing of ACTA2, and family screening. MAIN OUTCOME MEASURES: Prevalence of ACTA2 mutations and corresponding phenotypes. RESULTS: TAAD was familial in 43 cases and sporadic in 57 cases. Five mutations in the familial TAAD group (12%) were identified that were absent in controls. The known p.Arg149Cys and the novel p.Asp82Glu, p.Glu243Lys and p.Val45Leu mutations affected evolutionarily conserved residues. The IVS4+1G>A mutation was novel. Of 14 affected relatives, 13 were carriers of the mutation identified in the corresponding proband while one deceased relative had no genetic test. Type A dissection was the first manifestation of aortic aneurysm in four probands and occurred unexpectedly in five relatives. The aortic aneurysm was age dependent and absent in mutated children. Of nine patients who had acute dissection, five died following surgery. At dissection, the size of the aortic aneurysm ranged from 40 mm to 95 mm. Extravascular, ocular, skeletal, nervous and pulmonary traits were variably associated with TAAD, with iris flocculi being most common. CONCLUSIONS: Timely diagnosis of TAAD in the probands, genetic counselling and family screening identify predisposed relatives and prevent catastrophic aortic dissections.


Subject(s)
Actins/genetics , Aortic Aneurysm, Thoracic/genetics , Aortic Dissection/genetics , Mutation/genetics , Adolescent , Adult , Aged , Aortic Valve/abnormalities , Child , Female , Genetic Markers , Humans , Loeys-Dietz Syndrome/genetics , Male , Marfan Syndrome/genetics , Middle Aged , Pedigree , Phenotype , Risk Factors
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