Efficacy of alcohol septal ablation in mildly symptomatic or severely symptomatic patients with hypertrophic obstructive cardiomyopathy / 中华心血管病杂志

Objective: To compare the prognosis of mildly or severely symptomatic patients with obstructive hypertrophic cardiomyopathy (OHCM) who underwent alcohol septal ablation (ASA). Methods: This retrospective study cohort consisted of patients with OHCM who received ASA treatment in Beijing Anzhen Hospital, Capital Medical University from March 2001 to August 2021. These patients were divided into mildly and severely symptomatic groups according to the severity of clinical symptoms. Long-term follow-up was conducted, and the following data were collected: duration of follow-up, postoperatire treatment, New York Heart Association (NYHA) classification, arrhythmia events and pacemaker implantation, echocardiographic parameters, and cause of death. Overall survival and survival free from OHCM-related death were observed, and the improvement of clinical symptoms and resting left ventricular outflow tract gradient (LVOTG) and the incidence of new-onset atrial fibrillation were evaluated. The Kaplan-Meier method and log-rank test were used to determine and compare the cumulative survival rates of the different groups. Cox regression analysis models were used to determine predictors of clinical events. Results: A total of 189 OHCM patients were included in this study, including 68 in the mildly symptomatic group and 121 in the severely symptomatic group. The median follow-up of the study was 6.0 (2.7, 10.6) years. There was no statistical difference in overall survival between the mildly symptomatic group (5-year and 10-year overall survival were 97.0% and 94.4%, respectively) and the severely symptomatic group (5-year and 10-year overall survival were 94.2% and 83.9%, respectively, P=0.405); there was also no statistical difference in survival free from OHCM-related death between the mildly symptomatic group (5-year and 10-year survival free from HCM-related death were 97.0% and 94.4%, respectively) and the severely symptomatic group (5-year and 10-year survival free from HCM-related death were 95.2% and 92.6%, respectively, P=0.846). In the mildly symptomatic group, NYHA classification was improved after ASA (P<0.001), among which 37 patients (54.4%) were in NYHA class Ⅰ, and the resting left ventricular outflow tract gradient (LVOTG) decreased from 67.6 (42.7, 90.1) mmHg (1 mmHg=0.133 kPa) to 24.4 (11.7, 35.6) mmHg (P<0.001). In severely symptomatic group, NYHA classification was also improved post ASA (P<0.001), among which 96 patients (79.3%) improved by at least one NYHA classification, and the resting LVOTG decreased from 69.6 (38.4, 96.1) mmHg to 19.0 (10.6, 39.8) mmHg (P<0.001). The incidence of new-onset atrial fibrillation was similar between the mildly and severely symptomatic groups (10.2% vs. 13.3%, P=0.565). Cox multivariate regression analysis showed that age was an independent predictor of all-cause mortality in OHCM patients post ASA (HR=1.068, 95%CI 1.002-1.139, P=0.042). Conclusions: Among patients with OHCM treated with ASA, overall survival and survival free from HCM-related death were similar between mildly symptomatic group and severely symptomatic group. ASA therapy can effectively relieve resting LVOTG and improve clinical symptoms in mildly or severely symptomatic patients with OHCM. Age was an independent predictor of all-cause mortality in OHCM patients post ASA.

Minimally invasive right infra-axillary thoracotomy for transaortic modified Morrow procedure: a series of 60 cases / 中华外科杂志

Objective: To examine the short-term curative effect with minimally invasive right infra-axillary thoracotomy for transaortic modified Morrow procedure. Methods: The clinical data of 60 patients who underwent video-assisted thoracoscopic transaortic modified Morrow procedure from August 2021 to August 2022 at Department of Cardiovascular Surgery, Zhejiang Provincial People's Hospital were retrospectively analyzed. There were 31 males and 29 females, with the age (M (IQR)) of 54.0(22.3) years (range: 15 to 71 years). The echocardiography confirmed the diagnosis of moderate mitral regurgitation in 30 patients, and severe mitral regurgitation in 13 patients. Systolic anterior motion (SAM) was present preoperatively in 54 patients. All 60 patients underwent transaortic modified Morrow procedure through a right infra-axillary thoracotomy using femorofemoral cardiopulmonary bypass. Surgical procedures mainly included transverse aortic incision, exposure of left ventricular outflow tract (LVOT), septal myectomy, and correction of the abnormal mitral valve and subvalvular structures. Results: All 60 patients underwent the programmatic procedures successfully without conversion to full sternotomy. The cardiopulmonary bypass time was (142.0±32.1) minutes (range: 89 to 240 minutes), while the cross-clamp time was (95.0±23.5) minutes (range: 50 to 162 minutes). The patients had a postoperative peak LVOT gradient of 7.0 (5.0) mmHg (range: 0 to 38 mmHg) (1 mmHg=0.133 kPa). A total of 57 patients were extubated on the operating table. The drainage volume in the first 24 h was (175.9±57.0) ml (range: 60 to 327 ml). The length of intensive care unit stay was 21.0 (5.8)h (range: 8 to 120 h) and postoperative hospital stay was 8 (5) days (range: 5 to 19 days). The postoperative septal thickness was 11 (2) mm (range: 8 to 14 mm). All patients had no iatrogenic ventricular septal perforation or postoperative residual SAM. The patients were followed up for 4 (9) months (range: 1 to 15 months), and none of them needed cardiac surgery again due to valve dysfunction or increased peak LVOT gradient during follow-up. Conclusion: Using a video-assisted thoracoscopic transaortic modified Morrow procedure through a right infra-axillary minithoracotomy can provide good visualization of the LVOT and hypertrophic ventricular septum, ensure optimal exposure of the mitral valve in the presence of complex mitral subvalvular structures, so that allows satisfactory short-term surgical results.

Surgical treatment for obstructive hypertrophic cardiomyopathy: a five-year single-center experience of 421 cases / 中华外科杂志

Objectives: To examine the short-term and mid-term effects of surgical treatment of obstructive hypertrophic cardiomyopathy (HCM) in one center. Methods: The perioperative data and short-term follow-up outcomes of 421 patients with obstructive HCM who received surgical treatment at Department of Cardiac Surgery, Zhongshan Hospital, Fudan University from January 2017 to December 2021 were analyzed retrospectively. There were 207 males and 214 females, aged (56.5±11.7) years (range: 19 to 78 years). Preoperative New York Heart Association (NYHA) classification included 45 cases of class Ⅱ, 328 cases in class Ⅲ, and 48 cases in class Ⅳ. Fifty-eight patients were diagnosed with latent obstructive HCM and 257 patients had moderate or more mitral regurgitation with 56 patients suffering from intrinsic mitral valve diseases. All procedures were completed by a multidisciplinary team, including professional echocardiologists involving in preoperative planning for proper mitral valve management strategies and intraoperative monitoring. A total of 338 patients underwent septal myectomy alone, and 59 patients underwent mitral valve surgery along with myectomy. A single transaortic approach was used in 355 patients, and a right atrial-atrial septal/atrial sulcus approach was used in 51 other patients. Long-handled minimally invasive surgical instruments were used for the procedures. Student t test, Wilcoxon rank sum test, χ2 test or Fisher exact test were used to compare the data before and after surgery. Results: The aortic cross-clamping time of septal myectomy alone was (34.3±8.5) minutes (range: 21 to 94 minutes). Eighteen patients had intraoperative adverse events and underwent immediate reoperation, including residual obstruction (10 patients), left ventricular free wall rupture (4 patients), ventricular septal perforation (3 patients), and aortic valve perforation (1 patient). Four patients died during hospitalization, and 11 patients developed complete atrioventricular block requiring permanent pacemaker implantation. After discharge, 384 (92.1%) patients received a follow-up visit with a median duration of 9 months. All follow-up patients survived with significantly improved NYHA classifications: 216 patients in class Ⅰ and 168 patients in class Ⅱ (χ2=662.73, P<0.01 as compared to baseline). At 6 months after surgery, follow-up echocardiography showed that the thickness of the ventricular septum ((13.6±2.5) mm vs. (18.2±3.0) mm, t=23.51, P<0.01) and the peak left ventricular outflow tract gradient ((12.0±6.3) mmHg vs. (93.4±19.8) mmHg, 1 mmHg=0.133 kPa, t=78.29, P<0.01) were both significantly lower than baseline values. Conclusion: The construction of the surgical team (including echocardiography experts), proper mitral valve management strategies, identification and management of sub-mitral-valve abnormalities, and application of long-handled minimally invasive surgical instruments are important for the successful implementation of septal myectomy with satisfactory short-and medium-term outcomes.

Current status and thought of transcatheter mitral edge-to-edge repair in the treatment of hypertrophy cardiomyopathy / 中华外科杂志

Septal reduction therapies, which include septal myectomy and alcohol septal ablation and so on, are the current treatment strategies for patients with obstructive hypertrophic cardiomyopathy and drug-refractory symptoms. With the deepening of theoretical understanding and the rapid development of interventional therapies, some researchers have tried to perform transcatheter mitral valve edge-to-edge repair to treat high-risk patients with hypertrophic cardiomyopathy, including obstructive and non-obstructive. The reported results are relatively satisfactory, but many urgent problems need to be solved, such as the lack of data on animal experiments and large cohort studies, and the unknown medium- and long-term outcomes. However, transcatheter mitral valve edge-to-edge repair brings new ideas for the diagnosis and treatment of patients with hypertrophic cardiomyopathy. On one hand, it can be used as a monotherapy, on the other hand, it can be combined with novel molecular targeted drug therapy or emerging minimally invasive surgical procedures targeting hypertrophic ventricular septum, which deserves our further attention and exploratory research.

Surgery for obstructive hypertrophic cardiomyopathy: challenge and future / 中华外科杂志

After more than 60 years of development, with the deepening of the pathophysiological understanding of obstructive hypertrophic cardiomyopathy, the extent and resection thickness of myectomy have increased significantly. Myectomy combined with the correction of anomalies of the mitral valve apparatus has become the standard treatment of obstructive hypertrophic cardiomyopathy. Only a few centers worldwide can routinely perform it due to the difficulty. Because of the advances of new drugs and interventional therapy, the development of surgical treatment faces many challenges. At the same time, generations of cardiovascular surgeons are constantly trying to promote septal myectomy, including developing devices and the surgical field, as well as improving surgical planning by advanced technology. At present, the superior long-term efficacy of septal myectomy has been confirmed. It is necessary to work together to promote the treatment of hypertrophic obstructive cardiomyopathy, so as to guard people's health.

Ectasia coronaria y miocardiopatía hipertrófica: una entidad infrecuente / Coronary artery ectasia and hypertrophic cardiomyopathy: case report

Resumen Se presenta el caso de un paciente de 54 años que consulta por angina de esfuerzo de 2 años de evolución en quien se identifica una dilatación ectásica del árbol coronario con lesiones ateroscleróticas críticas y miocardiopatía hipertrófica septal obstructiva. Una revisión bibliográfica revela que es una asociación infrecuente de la cual solo existen reportes de casos aislados.

Abstract We present the case of a 54-year-old patient who presented with a history of 2 years with angina. Invasive studies revealed critical coronary artery stenosis coexisting with obstructive hypertrophic miopathy. This is a rare association with only isolated case reports.

Long-term outcome of patients with hypertrophic obstructive cardiomyopathy post percutaneous transluminal septal ablation / 中华心血管病杂志

Objective: To evaluate the long-term outcome of patients with hypertrophic obstructive cardiomyopathy(HOCM) after percutaneous transluminal septal ablation(PTSMA). Methods: HOCM patients who underwent PTSMA and surgical myectomy at the Chest Hospital of Shanghai Jiao Tong University from April 2001 to February 2019 were included in this retrospective analysis. Patients were divided into PTSMA group and surgical myectomy group. In addition, patients undergoing PTSMA were further divided into HOCM-PTSMA non-survivor group and HOCM-PTSMA survivor group. The general clinical information, procedural/surgical information and complications during hospitalization were compared between groups. Multivariate Cox regression model was used to analyze the independent risk factors for all-cause death in HOCM patients after PTSMA. Results: A total of 104 patients with HOCM who underwent PTSMA were enrolled. Mean age of the patients was (54±15) years old, including 41 females (38.7%). The follow-up time was 37.5(14.3, 76.8) months. At the last follow-up, 12 patients died (HOCM-PTSMA non-survivor group) and 92 were alive(HOCM-PTSMA survivor group). The proportion of patients with NYHA function class Ⅲ/Ⅳ was higher(P=0.036), and the posterior wall of the left ventricle was thicker(P=0.006) in the HOCM-PTSMA non-survivor group than in the HOCM-PTSMA survivor group. The immediate success rate of PTSMA in this cohort was 66%(70/104). The amount of absolute alcohol during the operation in the HOCM-PTSMA non-survivor group was (2.9±0.8) ml, which tended to be higher as compared to that in the HOCM-PTSMA survivor group((2.4±1.0)ml, P=0.056). Kaplan-Meier survival curve analysis showed that patients with HOCM who underwent PTSMA had an all-cause mortality-free survival rate of 90.1%, 78.3%, and 56.9% at 5, 10 and 15 years, and a HOCM-free survival rate of 91.3%, 79.4% and 57.7% at 5, 10 and 15 years, respectively. Multivariate Cox regression analysis showed that age≥ 65 years was an independent risk factor for all-cause death after PTSMA in patients with HOCM (HR=2.697, 95%CI 1.292-18.977, P=0.020). There were 32 patients in the surgical myectomy group. The proportion of patients with NYHA function class Ⅲ/Ⅳ was higher than that in the PTSMA group(P<0.001), while age, gender, and major comorbidities(atrial fibrillation, coronary heart disease, hypertension, and diabetes) as well as the left atrium dimension were all similar between the two groups(all P>0.05). Patients in the surgical myectomy group were followed up for 38.0(17.6, 64.2)months, and no deaths occurred during the follow-up period. Kaplan-Meier survival curve analysis showed that there were no statistically significant differences in all-cause-free and HOCM-free survival rates between patients in PTSMA group and surgical myectomy group(P=0.089 and 0.110, respectively). Conclusion: PTSMA is safe and effective for the treatment of patients with HOCM, and the long-term survival rate of patients after PTSMA is similar as patients undergoing classical surgical myectomy surgery.

Retrospective Analysis of Risk Factors for Related Complications of Chemical Ablation on Hypertrophic Obstructive Cardiomyopathy / Análise Retrospectiva de Fatores de Risco para Complicações Relacionadas com Ablação Química na Cardiomiopatia Hipertrófica Obstrutiva

Abstract Background: The analysis of risk factors for predicting related complications has not been reported to date. Objective: This study aims to investigate the risk factors of related complications of percutaneous transluminal septal myocardial ablation (PTSMA) for hypertrophic obstructive cardiomyopathy (HOCM) retrospectively. Method: Clinical data, and one-year follow-up results of patients with HOCM, who underwent PTSMA between January 2000 and July 2013 in the Department of Cardiology, Liaoning Province People's Hospital, Liaoning Province, China, were retrospectively analyzed to determine risk factors for operative complications with multiple logistic regression analysis. All p values are two-sided, with values of p < 0.05 being considered statistically significant. Results: Among 319 patients with HOCM, PTSMA was performed in 224 patients (120 males and 104 females, mean age was 48.20 ± 14.34 years old). The incidence of PTSMA procedure-related complications was 36.23% (66/224), which included three cardiac deaths, two cardiac shocks, one ST-segment elevated myocardial infarction, two ventricular fibrillations, 20 third-degree atrioventricular (AV) blocks (four patients were implanted with a permanent pacemaker (PPM)), 32 complete right bundle branch blocks, two complete left bundle branch blocks, and four puncture-related complications. After multivariate logistic regression analysis, it was found that age, gender, coronary artery diseases, diabetes, heart rate, cardiac function on admission, the number of septal ablations, and the volume of alcohol were not independent risk factors correlated to the whole complications, except for hypertension (OR: 4.856; 95% CI: 1.732-13.609). Early experience appears to be associated with the occurrence of complications. Conclusion: Hypertension was an independent risk factor for PTSMA procedure-related complications. It might be much safer and more efficient if PTSMA procedures are restricted to experienced centers, according to the analysis results for the learning curve.

Resumo Fundamento: A análise dos fatores de risco para previsão de complicações relacionadas não foi relatada até o momento. Objetivo: Este estudo tem como objetivo investigar retrospectivamente os fatores de risco de complicações relacionadas da ablação miocárdica septal transluminal percutânea (PTSMA) para cardiomiopatia hipertrófica obstrutiva (CMHO). Método: Dados clínicos e resultados de acompanhamento de um ano de pacientes com CMHO submetidos a PTSMA entre janeiro de 2000 e julho de 2013 no Departamento de Cardiologia do Hospital Popular da Província de Liaoning, província de Liaoning, China, foram analisados retrospectivamente para determinar fatores de risco para complicações operatórias com análise de regressão logística múltipla. Todos os valores de p são bilaterais, com valores de p < 0,05 sendo considerados estatisticamente significativos. Resultados: Entre os 319 pacientes com CMHO, a PTSMA foi realizada em 224 pacientes (120 homens e 104 mulheres, com idade média de 48,20 ± 14,34 anos). A incidência de complicações relacionadas ao procedimento PTSMA foi de 36,23% (66/224), incluindo três mortes cardíacas, dois choques cardíacos, um infarto do miocárdio com supradesnivelamento de ST, duas fibrilações ventriculares e 20 bloqueios atrioventriculares (AV) de terceiro grau (quatro pacientes receberam implantes de marca-passo permanente), 32 bloqueios completos de ramo direito, dois bloqueios completos de ramo esquerdo e quatro complicações relacionadas com a punção. Após análise de regressão logística multivariada, verificou-se que idade, sexo, doenças coronarianas, diabetes, frequência cardíaca, função cardíaca à admissão, número de ablações septais e volume de álcool não foram fatores de risco independentes correlacionados com as complicações totais, exceto para hipertensão (OR: 4,856; IC 95%: 1,732-13,609). A experiência principiante parece estar associada à ocorrência de complicações. Conclusão: A hipertensão arterial foi um fator de risco independente para complicações relacionadas com o procedimento de PTSMA. Pode ser muito mais seguro e eficiente se os procedimentos de PTSMA forem restritos a centros experientes, de acordo com os resultados da análise para a curva de aprendizado.

Hypertrophic cardiomyopathy part II - Anesthetic and surgical considerations.

Hypertrophic cardiomyopathy (HCM) poses many unique challenges regarding the conduct of anesthesia and surgery. Adequate preload, control of sympathetic stimulation, heart rate, and increased afterload are required to decrease the left ventricular outfl ow tract obstruction. Comprehensive intraoperative transesophageal echocardiography (TEE) examination confi rms the diagnosis, elucidates the pathophysiology, and identifi es the various anomalies of mitral valve apparatus and allows assessment of the adequacy of surgery. In this review, we focus on the preoperative assessment, conduct of anesthesia and comprehensive TEE examination of patients presenting for surgery with HCM. The various surgical options are extended myectomy and resection, plication and release.

Resultados a corto y mediano plazo del tratamiento de la obstrucción al tracto de salida del ventrículo izquierdo mediante miectomía videoasistida / Video assisted myectomy for relief of left ventricular tract obstruction in patients with Obstructive Hypertrophic Cardiomyopathy

Objetivo: Describir nuestra experiencia y evaluar los resultados a corto y mediano plazo de la miecto-mía videoasistida en el tratamiento de la obstrucción del tracto de salida del ventrículo izquierdo (OTSVI) en pacientes con miocardiopatía hipertrófica. Materiales y métodos: 52 pacientes con edad media de 56,2 (rango 12 - 83) y Euroscore de riesgo de 4,1 +/- 1,92 con diagnóstico de OTSVI fueron intervenidos de manera consecutiva en un mismo centro mediante miectomía videoasistida. Se realizó seguimiento clínico y ecocardiográfico con controles al mes y al año del postoperatorio. Resultados: Entre las patologías asociadas encontramos 11 pacientes con valvulopatía aórtica, 2 con valvulopatía mitral, 3 con cardiopatía isquémica, 1 con aneurisma de aorta ascendente y 1 con foramen oval permeable resueltos en el mismo acto quirúrgico. La mortalidad hospitalaria global fue de 5,8 por ciento (3 pacientes). En el momento de la intervención, 8 (15,4 por ciento) se encontraban en clase funcional II de la NYHA, 42 (80,8 por ciento) en clase III y 2 (3,8 por ciento) en clase IV. El gradiente máximo subaórtico disminuyó de 80,7 mmHg +/- 29,43 en el preoperatorio a 19,0 mmHg +/- 15,57 (p<0,001) en el postoperatorio inmediato, manteniéndose en 14,6 mmHg +/- 8,88 al mes (p<0,001 en relación al preoperatorio) y al año en 13,9 mmHg +/- 7,69 (p<0,001 en relación al preoperatorio). Además, se registró una disminución del grosor del tabique interventricular en diástole de 19,4 mm +/- 3,78 en el preoperatorio a 12,9 mm +/- 2,35 (p<0,001) en el postoperatorio. Todos los pacientes se encontraban en clase funcional I-II al final del seguimiento. Conclusión: Los resultados demuestran que la miectomía videoasistida es un tratamiento seguro para la OTSVI con el que se obtienen resultados favorables a corto y mediano plazo, tanto en parámetros clínicos, como ecocardiográficos.

Aim: To report a clinical experience and to evaluate early and mid term results of video assisted myec-tomy for relief of left ventricular tract obstruction (LVOTO) in patients with Obstructive Hypertrophic Cardiomyopathy. Methods: 52 patients with Obstructive Car-diomyopathy and a mean age 56.2 years (12 - 83) carrying a Euro score risk of 4.1 +/- (SD 1.92), were consecutively operated on in a single center. Relief of LVOTO was performed with video assisted myec-tomy. Clinical and echocardiographic follow up to 1 year postoperatively was carried out. Results: Apart from the Obstructive Cardiomyo-pathy, 11 patients had aortic valve disease, 2 mitral valve disease, 3 ischemic heart disease, 1 an ascending aortic aneurysm and 1 a patent foramen ovale. All these lesions were surgically repaired in the same surgical act. In hospital mortality was 5.8 percent (3 patients). Pre-operatively 15.4 percent of patients were in NYHA Class II, 80.8 per cent in Class III and 3.8 percent in Class IV. After surgery peak sub aortic gradient decreased from 80.7+/-29.43mmHg to 19.0 +/- 15.57 (p<0.001). Corresponding values were 14.6 +/- 8.88 at 1month and 13.9 +/- 7.69 at 1 year post operatively. Interven-tricular septal thickness in diastole decreased from 19.4 +/- 3.78 mm to 12.9 +/- 2.35 mm after surgery (p<0.001). All patients were Class I or II at the end of follow up. Conclusion: Video assisted myectomy is safe and effective for relief of LVOTO in patients with hypertrophic cardiomyopathy. Good results are maintained one year after surgery.

Noonan syndrome: An anesthesiologist's perspective.

Noonan syndrome (NS) is one of the most common non chromosomal syndrome presenting to the cardiac anesthesiologist for the management of various cardiac lesions, predominantly pulmonary stenosis (PS) (80%) and hypertrophic obstructive cardiomyopathy (HOCM) (30%). The presence of HOCM in NS makes these children susceptible to acute congestive heart failure due to hemodynamic fluctuations, thus necessitating optimization of drug and fluid therapy, careful conduct of anesthesia and providing adequate analgesia in the perioperative period. We describe a case of four year old boy with NS who presented to us for the management of PS and HOCM. In our case, transesophageal echocardiography (TEE) played a major role in confirmation of the preoperative findings, detection of any new anomalies missed during the preoperative evaluation, intraoperative monitoring and assessment of the adequacy of repair in the immediate postoperative period. TEE provided invaluable help in taking critical surgical decisions, resulting in a favorable outcome.

Avaliação intraoperatória e planejamento cirúrgico na cardiomiopatia hipertrófica / Intraoperative evaluation and surgical planning in the hypertrophic cardiomyopathy / Evaluación intraoperatoria y planeamiento quirúrgico en la cardiomiopatía hipertrófica

Paciente de 45 anos do sexo masculino, com diagnóstico de cardiomiopatia hipertrófica septal assimétrica, tendo sido submetido a implante de cardiodesfibrilador no passado. Apesar do tratamento clínico otimizado, evoluiu com progressiva deterioração clínica suscitando tratamento invasivo. Entretanto, não havia gradiente importante na via de saída do ventrículo esquerdo (VSVE) ao ecocardiograma de repouso e o teste provocativo com dobutamina foi inconclusivo por não atingir a frequência cardíaca preconizada. A avaliação intraoperatória com ecocardiograma transesofágico em uso de isoproterenol foi fundamental no diagnóstico de obstrução da VSVE. A miectomia septal foi realizada com sucesso e o paciente apresentou boa evolução pós-operatória.

A male, 45 year old patient, with diagnosis of septum hypertrophic cardiomyopathy, having undergone a cardiofibrilator implant in the past. Despite the optimized clinical treatment, he evolved to a progressive clinical deterioration that led to invasive treatment. However, there was not an important gradient in the exit pathway of the left ventricle (EPLV) at the echocardiogram in rest and the challenging test with dobutamine was non conclusive because it did not reach the preconized cardiac frequency. The intraoperative evaluation with a transesophageal echocardiogram using isoproterenol was fundamental for the diagnosis of the EPLV obstruction. The septum myectomy was performed successfully and the patient presented good post-operative evolution.

Paciente de 45 años del sexo masculino, con diagnóstico de cardiomiopatía hipertrófica septal asimétrica, habiendo sido sometido a implante de cardiodesfibrilador en el pasado. A pesar del tratamiento clínico optimizado, evolucionó con progresivo deterioro clínico suscitando tratamiento invasivo. Entre tanto, no había gradiente importante en la vía de salida del ventrículo izquierdo (VSVI) al ecocardiograma de reposo y el test provocativo con dobutamina fue inconclusivo por no alcanzar la frecuencia cardíaca preconizada. La evaluación intraoperatoria con ecocardiograma transesofágico en uso de isoproterenol fue fundamental en el diagnóstico de obstrucción de la VSVI. La miectomía septal fue realizada con éxito y el paciente presentó buena evolución postoperatoria.

Resultados a longo prazo da miectomia septal no tratamento da cardiomiopatia hipertrófica / Long term results of septal myectomy in the treatment of obstructive hypertrophic cardiomyopathy

OBJETIVO: Avaliação clínica e ecocardiográfica tardia da miectomia septal cirúrgica de pacientes com cardiomiopatia hipertrófica obstrutiva (CMHO). MÉTODOS: Foram analisados, retrospectivamente, 34 pacientes adultos (média de 55,7±15,2 anos) portadores de CMHO operados consecutivamente na instituição entre 1988 e 2008. Apenas quatro (11,8%) pacientes tinham conhecimento de história familiar para CMHO. Nove (26,5%) pacientes apresentavam insuficiência cardíaca (NYHA) classe funcional IV. Trinta (88,2%) pacientes apresentavam CMHO isolada e, em quatro (11,8%), a CMHO estava associada à insuficiência coronária. A técnica cirúrgica utilizada em todos os casos foi a miectomia septal transaórtica. RESULTADOS: Em 26 (76,5%) pacientes, a insuficiência mitral decorrente do movimento anterior sistólico regrediu após a miectomia. Em oito (23,5%) pacientes, houve necessidade de abordagem da valva mitral. Houve um (2,9%) óbito hospitalar. Dois (5,9%) pacientes necessitaram de marcapasso definitivo no pós-operatório. Em média, o gradiente de pico pré-operatório na via de saída do ventrículo esquerdo, que era de 84,9±29,0 mmHg, diminuiu para 27,8±12,9 mmHg no pós-operatório inicial e caiu para 19,2±11,2 mmHg no pós-operatório tardio (49,0±33,0 meses). A classe funcional (NYHA) que, em média, era de 3,1±0,8 passou para 1,4±0,5 no pós-operatório. Com seguimento médio de 9,6±8,4 anos, a sobrevida foi de 87,9% e a sobrevida livre de eventos cardiovasculares foi de 77,7%. CONCLUSÃO: A miectomia septal cirúrgica pode ser realizada de modo seguro, com excelente sobrevida, melhora dos sintomas e alívio da obstrução na via de saída do ventrículo esquerdo em pacientes com CMHO. Os benefícios iniciais se mantiveram a longo prazo.

OBJECTIVE: This study analyzed the clinical and echocardiographic late outcomes of surgical septal myectomy in patients with obstructive hypertrophic cardiomyopathy (OHCM). METHODS: We examined, retrospectively, 34 consecutive adult patients (age 55.7±15.2 years) with OHCM operated on in our institution from 1988 to 2008. Only four (11.8%) patients had family history of OHCM. Nine (26.5%) patients were in New York Heart Association (NYHA) funcional class IV. Thirty (88.2%) patients had solely OHCM, and four (11.8%) had OHCM associated with coronary insufficiency. The surgical technique used in all patients was septal myectomy performed through an aortotomy. RESULTS: In 26 (76.5%) patients the mitral insufficiency due to systolic anterior motion, decreased after the myectomy. Eight (23.5%) patients had mitral valve procedures. There was one hospitalar death (2.9%). Two (5.9%) patients required permanent pacemaker for complete heart block after the myectomy. The mean peak preoperative left ventricular outflow tract (LVOT) obstruction gradient was 84.9±29.0 mmHg, and decreased to 27.8±12.9 mmHg in the early postoperative and it was 19.2±11.2 mmHg in the late postoperative period (49.0±33.0 months). The NYHA functional class improved from 3.1±0.8 to 1.4±0.5 in the postoperative period. Survival free from death was 87.9% and survival free from cardiovascular events was 77.7% with mean follow-up 9.6±8.4 years. CONCLUSION: Surgical septal myectomy can be performed safely, with excellent survival, improvement from symptoms and relief for LVOT obstruction in patients with OHCM. The early benefits were remained at long term.

Evaluación de resultados quirúrgicos de la miectomía como tratamiento de la miocardiopatía hipertrófica obstructiva: experiencia de un centro en Uruguay / Evaluation of surgical results of myectomy in the treatment of hypertrophic obstructive cardiomyopathy: experience of a single center in Uruguay

Introducción: la miectomía quirúrgica del septum interventricular es el gold standard en el tratamiento de la miocardiopatía hipertrófica (MH) que requiere cirugía para alivio sintomático. No se encontró publicada ninguna serie de pacientes tratados mediante miectomía en Uruguay, lo que motivó analizar una serie de nuestro centro, valorando variables para su comparación con series de referencia. Material y método: se incluyeron todos los pacientes con miectomía entre enero de 2001 y enero de 2008. Se consignaron variables demográficas, ecocardiográficas y sobrevida a los 18 y 72 meses. Resultados: se incluyeron 23 pacientes, 14 mujeres. Edad promedio 59 años. Siete presentaban familiar de primer grado con MH. Seis presentaban lesiones coronarias significativas. El promedio del gradiente intraventricular máximo fue 96,32 mmHg, SIVD 21,4 mm y FEVI 66%. 17 tenían SAM, y de ellos, 15 presentaban insuficiencia mitral severa. En el preoperatorio, 14 presentaban CF NYHA III y nueve CF IV; 20 recibieron algún procedimiento asociado. La mortalidad operatoria fue de 8,7% (dos pacientes). El gradiente máximo postoperatorio promedio fue 12 mmHg, SIVD 16,65 mm y FEVI 64%. A los 18 meses, 20 estaban vivos y en CF I. La sobrevida actuarial a seis años fue 76,7%. Conclusiones: en 20 de los 23 pacientes se asoció otro procedimiento, mayormente sustitución valvular mitral. La miectomía redujo significativamente los gradientes intraventriculares, el espesor del SIVD y los síntomas en todos los pacientes (p < 0,0001). La mortalidad operatoria fue de 8,7% (dos pacientes) y la sobrevida actuarial a seis años de 76,7%. Estos resultados se equiparan a series de referencia.

Introduction: surgical septum myectomy remains the gold standard for the treatment of obstructive hypertrophic cardiomyopathy requiring surgery forsymptomatic relief. Wasn’t found in the literature, any series of patients underwent myectomy in Uruguay.Our objetive was the analysis of our center series, assessing variables for comparison with reference series.Materials and methods: were included all patients with myectomy from january 2001 to 2008. Demographicsand ultrasonographics variables, and survival at 18 and 72 months were consignated. Results: Were included 23 patients, 14 women. Mean age 59 years. Seven with a first grade relativeaffected of hypertrophic cardiomyopathy (HM). Six with coronary lessions. The mean maximum intraventricular gradient was 96,32 mmHg, dyastolic interventricular septum wide 21,4 mm, EF 66%. 17with systolic anterior motion, 15 with severe mitral insufficience. Before surgery, 14 shows NYHA functionalclass (FC) III, and 9 FCIV. 20 with one additional surgical procedure. Operative mortality was 8,7% (two patients). The mean maximum intraventriculargradient after surgery was 12 mmHg, dyastolic interventricular septum wide 16,65mmand EF 64%. At 18 months, survival was 20 patients, all in FC I. Actuarial survival at 6 years was 76,7%. Conclussions: 20 of 23 patients with an additional procedure, mainly mitral valve substitution. Myectomyimproved intraventricular gradients, DIVS wide and symptoms in all patients (p<0,0001). Operative mortality was 8,7% (two patients), and actuarialsurvival at 6 years was 76,7%. This results are comparable with reference series.

Ecocardiografia transesofágica intraoperatória na cardiomiopatia hipertrófica septal / Ecocardiografía transesofágica intraoperatoria en miocardiopatía hipertrófica septal / Intraoperative transesophageal echocardiography in septal hypertrophic cardiomyopathy

Relatamos caso de paciente portador de cardiomiopatia hipertrófica septal submetido a correção cirúrgica em que o emprego da ecocardiografia transesofágica intraoperatória proporcionou o planejamento da abordagem cirúrgica e o reconhecimento imediato do resultado operatório.

We report the case of a patient with septal hypertrophic cardiomyopathy undergoing surgical correction in which the use of intraoperative transesophageal echocardiography permitted the planning of the surgical approach and an immediate knowledge of the surgical outcome.

Relatamos caso de paciente portador de cardiomiopatia hipertrófica septal submetido a correção cirúrgica em que o emprego da ecocardiografia transesofágica intraoperatória proporcionou o planejamento da abordagem cirúrgica e o reconhecimento imediato do resultado operatório.