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1.
JACC Case Rep ; 29(13): 102385, 2024 Jul 03.
Artigo em Inglês | MEDLINE | ID: mdl-38912320

RESUMO

The incidence of coronary artery aneurysm is between 1.4% and 4.9% based on autopsy or angiographic series. Mycotic coronary arteries aneurysms are very rare and represent less than 3% of all coronary aneurysms. We report the case of a patient who presented with multiple coronary mycotic aneurysms.

2.
CJC Pediatr Congenit Heart Dis ; 3(2): 67-73, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38774683

RESUMO

Low- and middle-incomes countries (LMICs) have limited resources for the diagnosis and treatment of congenital heart diseases such as tetralogy of Fallot. This is in part due to lack of infrastructures, financial means, and expertise. As a result, patients undergo surgery much later than in high-income countries. This delay in treatment results in right ventricular dysfunction, cardiac arrhythmias, and poor psychomotor development-complications that are all related to chronic hypoxia. There are limited data and a few small studies of patients treated for tetralogy of Fallot in LMICs, and, therefore, the aim of this review is to analyse and summarize the surgical outcomes of this LMIC population.


Dans les pays à revenu faible ou intermédiaire (PRFI), les ressources sont limitées pour diagnostiquer et prendre en charge les cardiopathies congénitales comme la tétralogie de Fallot. Cette situation est attribuable en partie au manque d'infrastructures, de moyens financiers et d'expertise. Les patients subissent donc une correction chirurgicale beaucoup plus tard que dans les pays à revenu élevé. Les délais de traitement peuvent entraîner une dysfonction ventriculaire droite, une arythmie cardiaque et des problèmes de développement psychomoteur : des complications toutes liées à l'hypoxie chronique. Il existe des données limitées et quelques études de faible envergure sur des patients traités pour une tétralogie de Fallot dans les PRFI. L'objectif du présent article de synthèse est donc d'analyser et de résumer les issues des interventions chirurgicales dans les PRFI pour cette population.

6.
Clin Invest Med ; 45(4): E1-10, 2022 12 31.
Artigo em Inglês | MEDLINE | ID: mdl-36586100

RESUMO

PURPOSE: Clinician-investigators have an important role in the development and implantation of new therapies and treatment modalities; however, there have been several reports highlighting a pending shortage in the clinician-investigators' workforce. In Canada, the Royal College has promoted the development of clinician-investigators programs (CIP) to facilitate the training of these individuals. There is currently a paucity of data regarding the outcomes of such programs. This study aims to identify the strengths and areas of improvement of the Montreal University CIP.  Methods: An internet-based 51-question survey was distributed to all the alumni from the University of Montreal CIP. Participation was voluntary and no incentives were provided. The response rate was 64%.  Results: Among respondents, 50% (n=16) had completed their clinical residency and all CIP requirements. The majority of these individuals (63%) had become independent investigators and had secured provincial and national funding. Satisfaction of the respondents was high regarding the overall program (85%), the research skills developed during the CIP (84%) and the financial support obtained during the program (72%). The satisfaction rate regarding career planning was lower (63%).  Conclusion: This survey demonstrates that, while indicators are favorable, some areas still require improvement. Several steps to improve the CIP have been identified; notably, the transition from the CIP to early independent career has been identified as critical in the development of clinician-investigators and steps have been taken to improve this progression.


Assuntos
Pesquisa Biomédica , Internato e Residência , Humanos , Pesquisa Biomédica/educação , Canadá , Inquéritos e Questionários , Pesquisadores/educação , Avaliação de Programas e Projetos de Saúde
7.
Eur J Cardiothorac Surg ; 62(3)2022 08 03.
Artigo em Inglês | MEDLINE | ID: mdl-35916717

RESUMO

OBJECTIVES: Root reimplantation has been the favoured approach for patients with heritable aortic disorder requiring valve-sparring root replacement. In the past few years, root remodelling with annuloplasty has emerged as an alternative to root reimplantation in the general population. The aim of this study was to examine the late outcomes of patients with heritable aortic disorder undergoing valve-sparring root replacement and compare different techniques. METHODS: Using the AVIATOR registry (Aortic Valve Insufficiency and ascending aorta Aneurysm InternATiOnal Registry), data were collected from 5 North American and European centres. Patients were divided into 4 groups according to the technique of valve-sparing used (root reimplantation, root remodelling with ring annuloplasty, root remodelling with suture annuloplasty and root remodelling alone). The primary endpoints were freedom from aortic regurgitation (AR) ≥2 and freedom from reintervention on the aortic valve. Secondary endpoints were survival and changes in annular dimensions over time. RESULTS: A total of 237 patients were included in the study (reimplantation = 100, remodelling + ring annuloplasty = 76, remodelling + suture annuloplasty = 34, remodelling alone = 27). The majority of patients had Marfan syndrome (83%). Preoperative AR ≥2 was present in 41% of the patients. Operative mortality was 0.4% (n = 1). No differences were found between techniques in terms of postoperative AR ≥2 (P = 0.58), reintervention (P = 0.52) and survival (P = 0.59). Changes in aortic annulus dimension were significantly different at 10 years (P < 0.05), a difference that started to emerge 4 years after surgery. CONCLUSIONS: Overall, valve-sparring root replacement is a safe and durable procedure in patients with heritable aortic disorder. Nevertheless, root remodelling alone is associated with late annular dilatation. The addition of an annuloplasty, however, results in similar freedom from AR, reintervention, survival and changes in annulus size compared to reimplantation.


Assuntos
Insuficiência da Valva Aórtica , Anuloplastia da Valva Cardíaca , Pilotos , Aorta/cirurgia , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/cirurgia , Anuloplastia da Valva Cardíaca/métodos , Humanos , Sistema de Registros , Reoperação , Resultado do Tratamento
9.
Physiol Rep ; 10(8): e15251, 2022 04.
Artigo em Inglês | MEDLINE | ID: mdl-35439345

RESUMO

Male sex and hypertension represent risk factors in the progression of an aortic aneurysm. The present study examined the morphological/cellular phenotype of the ascending aorta (AA) of male and female patients diagnosed with a bicuspid aortic valve (BAV) to test the hypothesis that hypertension-induced remodeling of male BAV patients preferentially recapitulated the expression of a panel of proteins favoring aneurysm formation. The diameter of the AA of hypertensive male (35 ± 6 mm) and female (39 ± 5 mm) BAV patients was comparable to normotensive patients reflecting an early phase of vessel expansion. Morphological/structural remodeling of the medial region of the AA of male normotensive and hypertensive BAV patients were comparable. Protein levels of non-muscle myosin IIB, the cell cycle inhibitor p27kip1, tumor suppressor p53 and matrix metalloproteinase-2 and -9 were significantly upregulated in the AA of male hypertensive BAV patients. In female hypertensive BAV patients, collagen content was significantly increased whereas elastin content and medial width of the AA were similar to normotensive BAV patients. In the AA of female hypertensive BAV patients, matrix metalloproteinase-9 and p27kip1 protein levels were unchanged whereas p53 and matrix metalloproteinase-2 protein expression was significantly reduced. Nestin protein levels were diminished in the AA of male and female hypertensive BAV patients. Thus, sexual dimorphic remodeling of the AA was prevalent in hypertensive BAV patients. Moreover, during the early phase of vessel expansion, the AA of male hypertensive BAV patients was preferentially associated with the upregulation of a panel of proteins linked to progressive dilatation and potential aneurysm formation.


Assuntos
Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Hipertensão , Aorta/fisiologia , Valva Aórtica/metabolismo , Feminino , Doenças das Valvas Cardíacas/complicações , Humanos , Hipertensão/metabolismo , Masculino , Metaloproteinase 2 da Matriz/genética , Metaloproteinase 2 da Matriz/metabolismo , Fenótipo , Proteína Supressora de Tumor p53/genética
10.
J Thorac Cardiovasc Surg ; 164(3): 835-844.e5, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-33158565

RESUMO

BACKGROUND: The Ross procedure offers several advantages in nonelderly adults; however, the optimal age cutoff remains undetermined. The aim of this study was to compare the safety and mid-term outcomes after the Ross procedure in adult patients age ≤50 years and those age >50 years. METHODS: Between 2011 and 2019, 497 consecutive patients (mean age, 47 ± 12 years; 73% male) underwent a Ross procedure in 5 Canadian centers and were followed prospectively. Of these patients, 232 (47%) were age >50 years (mean, 57 ± 4 years) and 265 (53%) were age ≤50 years (mean, 38 ± 10 years). Early and mid-term outcomes were compared between the 2 groups. RESULTS: Patients age >50 years had more comorbidities: diabetes (14% vs 4%; P < .01), chronic obstructive pulmonary disease (8% vs 2%; P < .01), and coronary artery disease (17% vs 3%; P < .01). In contrast, patients age ≤50 years had more redo surgeries (24% vs 8%; P < .01), pure aortic regurgitation (21% vs 6%; P < .01) and unicuspid valves (42% vs 9%; P < .01). In-hospital mortality was similar in the 2 groups (0.4% vs 0.4%; P = .99). There were no between-group differences in perioperative complications. The cumulative incidence of reintervention was similar at 6 years (>50 years: 0.7 ± 0.7%; ≤50 years: 4 ± 2%; P = .12). Survival at 6 years was 98 ± 2% in patient age >50 years versus 96 ± 2% in those age ≤50 years (P = .43), similar to the age- and sex-matched general population. CONCLUSIONS: The Ross procedure is a safe operation in patients age >50 years and provides excellent hemodynamics, stable valve function, and restored survival at mid-term follow-up. In expert centers, it should be considered as an alternative in selected patients age >50 years.


Assuntos
Valva Aórtica , Implante de Prótese de Valva Cardíaca , Adulto , Fatores Etários , Valva Aórtica/cirurgia , Canadá/epidemiologia , Feminino , Seguimentos , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/métodos , Hemodinâmica , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
11.
J Thorac Cardiovasc Surg ; 163(4): 1296-1305.e3, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32888704

RESUMO

OBJECTIVES: Pulmonary homograft dysfunction is a limitation after the Ross procedure. Decellularized pulmonary homografts can potentially mitigate this complication. The aim of this study was to examine the incidence, predictors, progression, and morphology of pulmonary homograft dysfunction using data from the Canadian Ross Registry. METHODS: From 2011 to 2019, 466 consecutive patients (mean age: 47 ± 12 years, 73% male) underwent a Ross procedure using a decellularized cryopreserved pulmonary homograft (SynerGraft SG; CryoKife, Kennesaw, Ga). Pulmonary homograft dysfunction was defined as any of the following: peak pulmonary gradient ≥30 mm Hg, pulmonary regurgitation >2, or pulmonary homograft reintervention. Patients meeting ≥1 of these criteria (n = 30) were compared with the rest of the cohort (n = 436). Median follow-up is 2.2 years (maximum = 8.5 years) and 99% complete (1176 patient-years). RESULTS: The cumulative incidence of pulmonary homograft dysfunction was 11 ± 2% at 6 years. Pulmonary homograft stenosis was the most frequent presentation (n = 28 patients, 93%). Morphologically, stenosis occurred most often along the conduit (59%). Overall, 4 patients required homograft reintervention. At 6 years, the cumulative incidence of homograft reintervention was 3 ± 1%. The instantaneous risk was greatest in the first year after surgery (3.5%/year) and decreased to <1%/year thereafter. Patient age <45 years was the only independent risk factor associated with pulmonary homograft dysfunction (hazard ratio, 3.1, 95% confidence interval, 1.1-8.6, P = .03). CONCLUSIONS: The use of decellularized cryopreserved pulmonary homografts results in a low incidence of dysfunction and reintervention after the Ross procedure. The risk is greater in the first postoperative year. Younger age is the only independent risk factor for pulmonary homograft dysfunction.


Assuntos
Aloenxertos , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/métodos , Valva Pulmonar/cirurgia , Adulto , Fatores Etários , Criopreservação , Feminino , Sobrevivência de Enxerto , Humanos , Masculino , Pessoa de Meia-Idade , Estenose da Valva Pulmonar/epidemiologia , Sistema de Registros , Reoperação/estatística & dados numéricos , Fatores de Risco
12.
Ann Thorac Surg ; 113(6): e429-e431, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-34536376

RESUMO

A 68-year-old woman with a right aortic arch and an aberrant left subclavian artery underwent urgent operation for type A aortic dissection. Surgical management included total arch repair with the frozen elephant trunk technique and left subclavian artery reimplantation. Her postoperative course was complicated by severe renal failure, delirium, and pneumonia. She underwent reoperation 3 weeks later for an expanding proximal aortic anastomosis pseudoaneurysm. Right aortic arch is a rare vascular anomaly requiring thoughtful preoperative planning for adequate myocardial, cerebral, and spinal protection. The frozen elephant trunk technique facilitates management of extensive dissection, relieves malperfusion, and may alleviate the need for subsequent operations.


Assuntos
Aneurisma da Aorta Torácica , Implante de Prótese Vascular , Idoso , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Aneurisma da Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/cirurgia , Prótese Vascular , Implante de Prótese Vascular/métodos , Feminino , Humanos , Stents , Resultado do Tratamento
13.
Semin Thorac Cardiovasc Surg ; 34(2): 386-392, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-34089828

RESUMO

To examine the perioperative outcomes following aortic arch repair using frozen elephant trunk (FET) vs conventional elephant trunk (ET) techniques. Between 2002 and 2018, 390 patients underwent aortic repair with elephant trunk reconstruction at 9 centers: 172 patients received a FET (mean age: 65+/-13 years, 30% female, 37% aortic dissection) and 218 patients received an ET (mean age: 63+/-13 years, 37% female, 43% aortic dissection). Outcomes of interest included in-hospital mortality; stroke; and spinal cord injury (SCI). In-hospital mortality rate was 11% (n = 43) overall, 9% (n = 15) for FET and 13% (n = 28) for ET. Post-operative stroke occurred in 13% (n = 49) overall, 13% (n = 22) for FET and 12% (n = 27) for ET. The rate of post-operative SCI was 3% (n = 13) overall, 5.0% (n = 9) for FET and 2.0% (n = 4) for ET. When compared to ET, the propensity score analysis confirmed FET to be associated with lower mortality (adjusted risk difference -7.0% (95% CI -13.0 to -1.0), P = 0.02). There was no significant difference in the propensity score-adjusted risk difference for stroke between FET and ET (-0.7%, 95% CI -7.4% to 6.1%, P = 0.85), nor for SCI (3.3%, 95% CI -0.4% to 7.0%, P = 0.085) On multivariable analysis, FET was associated with lower odds of mortality (OR 0.44, 95% CI 0.21-0.95, P = 0.04), and had similar odds of stroke (OR 0.83, 95% CI 0.41-1.70, P = 0.62) and SCI (OR 2.83, 95% CI 0.83-9.60, P = 0.1). FET repair is associated with lower in-hospital mortality as compared to conventional ET, and results in similar risk of stroke and spinal cord injury. Further investigation is warranted.


Assuntos
Aneurisma da Aorta Torácica , Dissecção Aórtica , Implante de Prótese Vascular , Traumatismos da Medula Espinal , Acidente Vascular Cerebral , Idoso , Dissecção Aórtica/diagnóstico por imagem , Dissecção Aórtica/cirurgia , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Aneurisma da Aorta Torácica/cirurgia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Traumatismos da Medula Espinal/diagnóstico , Traumatismos da Medula Espinal/etiologia , Acidente Vascular Cerebral/etiologia , Resultado do Tratamento
14.
Ann Cardiothorac Surg ; 10(4): 476-484, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34422559

RESUMO

BACKGROUND: Repair failure remains one of the most important complications of aortic valve reconstruction. Young patients might benefit from a Ross procedure in such a scenario, provided it can be performed safely and with adequate durability. The aim of this study was to assess the safety and clinical outcomes of a Ross operation following a failed repair. METHODS: Between 1996 and 2019, 80 patients (male, 76%; mean age, 31±13 years) underwent a Ross procedure after a median of 6.6 (1.7-15.9) years following an initial aortic valve repair. The previous valve repair was performed for unicuspid (53%), bicuspid (39%), tricuspid (7%), and quadricuspid morphology (1%). Median follow-up after the Ross operation was 2.8 (0.964-13.25) years, mean 5±5 years (92% complete). RESULTS: Median cardiopulmonary bypass and cross-clamp times were 144 [106-154] minutes and 98 [79-113] minutes, respectively. Thirty-two patients (40%) required a concomitant procedure, most commonly, an ascending aortic replacement (n=23). There were no peri-operative deaths, myocardial infarctions, or neurological complications. There was one late death from a non-cardiac cause. At 10 years, overall survival was 99%±1%, similar to that of an age- and gender-matched population. Nine patients required re-intervention after their Ross procedure (five on the autograft and four on the pulmonary conduit). The autograft re-interventions were valve-sparing procedures in all patients. The cumulative incidence of re-intervention on the autograft at 8 years was 5.1%±3.1%. CONCLUSIONS: The stepwise strategy of an initial valve repair followed by Ross operation represents a safe and valid option for failed aortic valve repair. It is associated with low peri-operative morbidity. Mid-term survival is excellent, similar to that of a matched general population. The probability of re-intervention after the Ross procedure appears similar to that of a primary Ross operation, deeming it a warranted consideration in cases of failed aortic valve repair.

17.
Eur J Cardiothorac Surg ; 60(3): 623-630, 2021 09 11.
Artigo em Inglês | MEDLINE | ID: mdl-33769490

RESUMO

OBJECTIVES: The aim of this study was to examine the effect of the addition of an aortic root replacement or sinus repair on mortality and morbidity during aortic arch repair. METHODS: A total of 2472 patients underwent proximal or total aortic arch repair with hypothermic circulatory arrest between 2002 and 2018 at 12 centres. Multivariable logistic regressions (MV) and propensity score (PS) with inverse probability of treatment weighting (IPTW) analyses were performed. RESULTS: A total of 1099 (44.5%) patients had additional aortic root replacement (n = 934) or sinus repair (n = 165). Those with aortic root interventions were younger (61 ± 13 vs 64 ± 13 years, P < 0.001) and had less females (23% vs 35%, P < 0.001), less dissection (31% vs 36%, P = 0.004), less urgent cases (35% vs 39%, P = 0.047), more connective tissue disease (7% vs 3%, P < 0.001) and less total arch replacements (14% vs 22%, P < 0.001). On adjusted analyses, the addition of aortic root procedure was associated with increased mortality [MV: odds ratio (OR) 1.41, 95% confidence interval (CI) 1.03-1.92; PS-IPTW: risk increased by 3.7%, 95% CI 1.2-6.3%, P = 0.004]. Reoperation for bleeding was also increased with the addition of aortic root intervention (MV: OR 1.48, 95% 1.10-1.99; PS-IPTW: risk increased by 3.2%, 95% CI 0.8-5.6%, P = 0.009). The risks of stroke and dialysis-dependent renal failure were similar. When looking only at non-elective cases, the increased risk of mortality was more pronounced (MV: OR 1.60, 95% CI 1.11-2.32, P = 0.013; PS-IPTW: risk increased by 6.8%, 95 CI 1.7-11.8%, P = 0.008, and a number need to harm of 15 patients to cause 1 additional death). CONCLUSIONS: The addition of aortic root replacement or sinus repair during proximal or total aortic arch repair seems to increase postoperative mortality only in non-elective cases.


Assuntos
Aneurisma da Aorta Torácica , Implante de Prótese Vascular , Aorta Torácica/cirurgia , Aneurisma da Aorta Torácica/cirurgia , Valva Aórtica/cirurgia , Implante de Prótese Vascular/efeitos adversos , Canadá , Feminino , Humanos , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
18.
J Cardiothorac Surg ; 16(1): 44, 2021 Mar 23.
Artigo em Inglês | MEDLINE | ID: mdl-33757537

RESUMO

BACKGROUND: Chest tubes are routinely used after cardiac surgery to evacuate shed mediastinal blood. Incomplete chest drainage due to chest tube clogging can lead to retained blood after cardiac surgery. This can include cardiac tamponade, hemothorax, bloody effusions and postoperative atrial fibrillation (POAF). Prior published non randomized studies have demonstrated that active tube clearance (ATC) of chest tubes can reduce retained blood complications prompting the ERAS Cardiac Society guidelines to recommend this modality. OBJECTIVE: A randomized prospective trial to evaluate whether an ATC protocol aimed at improving chest tube patency without breaking the sterile field could efficiently reduce complications related to retained blood after cardiac surgery. METHODS: This was a pragmatic, single-blinded, parallel randomized control trial held from November 2015 to June 2017 including a 30-day post index surgery follow-up. The setting was two academic centers affiliated with the Université de Montréal School of Medicine; the Montreal Heart Institute and the Hôpital du Sacré-Coeur de Montréal. Adult patients admitted for non-emergent coronary bypass grafting and/or valvular heart surgery through median sternotomy, in sinus rhythm for a minimum of 30 days prior to the surgical intervention were eligible for inclusion. In the active tube clearance group (ATC), a 28F PleuraFlow device was positioned within the mediastinum. In the standard drainage group, a conventional chest tube (Teleflex Inc.) was used. Other chest tubes were left at the discretion of the operating surgeon. RESULTS: A total of 520 adult patients undergoing cardiac surgery were randomized to receive either ATC (n = 257) or standard drainage (n = 263). ATC was associated with a 72% reduction in re-exploration for bleeding (5.7% vs 1.6%, p = .01) and an 89% reduction in complete chest tube occlusion (2% vs 19%, p = .01). There was an 18% reduction in POAF between the ATC and control group that was not statistically significant (31% vs 38%, p = .08). CONCLUSIONS AND RELEVANCE: In this RCT, the implementation of active clearance of chest tubes reduced re-exploration and chest tube clogging in patients after cardiac surgery further supporting recommendations to consider this modality postoperatively. TRIAL REGISTRATION: Clinical Trials NCT02808897 . Retrospectively registered 22 June 2016.


Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Tubos Torácicos , Drenagem/métodos , Cuidados Pós-Operatórios/métodos , Complicações Pós-Operatórias/terapia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Método Simples-Cego
19.
Future Cardiol ; 17(7): 1171-1181, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-33544641

RESUMO

The frozen elephant trunk technique has revolutionized aortic arch repair to enable more extensive arch and descending thoracic aortic treatment in a single setting. We review the current evidence supporting the use of the Thoraflex Hybrid (Terumo Aortic, FL, USA) device and discuss advantages, pitfalls and future design considerations.


Lay abstract Disease of the aorta, the biggest blood vessel in the body, is challenging. In recent years, new technologies such as the frozen elephant trunk (FET) have improved the treatment of patients with these complicated diseases. The FET is a hybrid device, made of medical fabrics and a covered metal stent (a tube inserted into the blood vessel to keep it open). In this paper, we provide an in-depth review of a FET device known as Thoraflex Hybrid (Terumo Aortic, FL, USA). We discuss its advantages and pitfalls as well as future areas of research.


Assuntos
Aneurisma da Aorta Torácica , Implante de Prótese Vascular , Procedimentos Endovasculares , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Aneurisma da Aorta Torácica/cirurgia , Prótese Vascular , Humanos , Estudos Retrospectivos , Resultado do Tratamento
20.
J Card Surg ; 36(4): 1282-1288, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33547670

RESUMO

BACKGROUND: Hypertrophic obstructive cardiomyopathy (HOCM) is one of the most common genetic cardiac diseases and encompasses an array of clinical presentations. Little is known about the impact of genetic background on outcomes after septal myectomy (SM). The aim of this study was to evaluate the effect of specific genetic mutations on midterm outcomes in adults undergoing SM for HOCM. METHODS: From 2003 to 2020, a total of 59 patients (male = 66%, mean age = 52 ± 13) underwent SM after a preoperative genetic test. Patients were divided into two groups according to their test result (positive or negative). Preoperative echocardiograms were examined to identify phenotypical characteristics of each mutation. RESULTS: A total of thirty-one patients (53%) had a positive genetic test. MYBPC3 was the most common mutation (15/31 patients). Four different phenotypes were identified on preoperative echocardiograms. Overall, Type 1 phenotype was the most common (37% of the cohort). Type 3 was found exclusively in patients with a positive genetic test. Following SM, none of the patients required a redo myectomy or septal ablation. At 10 years, the survival was 97 ± 3% and 100% in patients with a positive and negative genetic test (p = .33), respectively. CONCLUSION: Although our results suggest that the multiple gene mutations present with different characteristics and phenotypes, midterm results of SM appear to be good regardless of genetic mutation presence.


Assuntos
Cardiomiopatia Hipertrófica , Ablação por Cateter , Testes Genéticos , Adulto , Idoso , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/cirurgia , Septos Cardíacos/diagnóstico por imagem , Septos Cardíacos/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
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