Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease, with a
prevalence of 1 case in 500 individuals.[1] The disease is very heterogeneous regarding its phenotype, being the
main cause of sudden death in athletes who they die in competitions.[1],[2] Fortunately, most patients are asymptomatic or have few
symptoms and will have a life expectancy very close to the individuals without the
disease.[2] However, some
patients will develop symptoms such as angina, dyspnea, palpitations, syncope and even
sudden death, usually caused by ventricular arrhythmia. Approximately 2/3 of patients
with HCM have a significant left ventricular outflow tract (LVOT) gradient at rest or
during drug or physical exertion provocation tests.[3] The presence of a significant gradient, mainly at rest,
characterizes obstructive hypertrophic cardiomyopathy (OHCM) and the presence of the
gradient is related to greater symptom intensity and a higher risk of death.[1]-[3]The standard treatment of symptomatic patients comprise the use of drugs such as
beta-blockers and/or calcium channel blockers, which decrease the gradient and improve
angina, diastolic function and increase tolerance to physical exertion.[1]-[3] Between 5 and 10% of patients with OHCM are refractory
to pharmacological treatment and should be considered for invasive treatment: surgical
myomectomy (SM) or septal ablation (SA) (alcoholization) with the aim of reducing septal
muscle mass and relieve LVOT obstruction.[4],[5] Since
its introduction in 1995 by Sigwart et al.,[5] SA has become an alternative to surgical treatment (which was
considered the gold standard treatment for patients with OHCM and refractory to clinical
treatment). After the introduction of SA, because it was found to be attractive to the
patient and to the physician, a rapid and progressive increase in the number of
performed procedures was observed, especially in the European countries, which quickly
surpassed the number of surgeries performed annually worldwide and with results in the
short and medium term that were similar to the results obtained with surgical procedures
in centers of excellence, according to data from patient cohorts, registries and
meta-analyses,[6],[7]
since there are no randomized trials comparing the two forms of intervention. But
despite the significant increase in the number of SA performed and after two decades of
experience, some controversy remains about the choice of invasive procedure (SA or
SM?).[4],[8]-[11]As it has more than 4 decades of experience, consistent results in the longer term and it
is more effective in reducing the gradient (eliminates the gradient in >90% of the
cases), the European guideline recommends surgery (septal myomectomy) performed in
specialized centers (mortality rate <2.0% and rate of complications <5%) as the
procedure of choice (degree of recommendation Ia and evidence level B) and SA as an
alternative, with degree of recommendation IIa and evidence level C for
selected patients, with contraindication to surgery or at high surgical risk or even in
cases of myomectomy failure.[9]It is worth noting that the determinant factor for having good results with both
procedures is the experience of centers, which must be measured by more than 50
procedures performed per year and more than 20 procedures performed by the operator
(surgeon or interventional cardiologist), seeking to attain mortality rates <2% and
complication rates <5%.[10],[11]In this issue, Li et al.[12] report the
experience of a single center in China with SA for treatment of symptomatic OHCM. The
author shows the results of the procedure in 224 patients, performed according to the
preference of the patient and/or the attending physician, over a period of 13 years and
after the 1-year follow-up, they retrospectively analyzed the risk factors for
complications related to the procedure (in-hospital phase). The rate of complications
related to the procedure was 36.23%, including 4 deaths, 3 cardiogenic shocks, 6
episodes of ventricular fibrillation, 1 myocardial infarction, 20 advanced AV blocks and
4 permanent pacemaker implants, plus 28 minor complications. At the multivariate
analysis, only arterial hypertension stood out as a strong complication predictor. The
rate of severe complications reported by the author is very high when compared to those
of large series, in specialized centers and with a high volume of procedures.[4],[6]-[8],[13]In the study by Li et al.,[12] it becomes
clear that one of the factors associated with high complication rates may have been the
relatively low number of procedures per year, the inclusion of older patients with
comorbidities, and the inclusion of 46 hypertensivepatients, who usually have a
sigmoid, less thick interventricular septum; moreover, the hypertrophy may be secondary
to hypertension and not necessarily observed in patients with OHCM, in addition to
worsening diastolic function and being accompanied by comorbidities such as diabetes,
coronary disease and atrial fibrillation.In the large series that evaluated complications, factors related to the experience of
the center and the operator and also to patient selection for SA always stand out as
predictors of low rates of complications, especially age, comorbidities, preexisting
bundle branch blocks, as well as anatomical and functional factors as determinants of
complications and also of the success rate of the procedure.[13]When selecting the patients for invasive treatment, one must ascertain the actual
refractoriness of the clinical treatment (present in 5% of the patients in our center),
evaluate the presence and impact of comorbidities, perform a careful assessment of the
gradient, especially the resting gradient, since we do not know the actual influence of
the inotropic stimulus on the genesis of the symptoms and the risk of death. The resting
gradient should be >30 mmHg or ideally >50 mmHg; the basal septum thickness >15
mm or ideally >18 mm; one should determine that the gradient is in the outflow tract
and not the mid-ventricular portion (10-15% of cases), the presence of the anterior
systolic movement of the mitral leaflet, degree and mechanism of mitral regurgitation,
anatomy of the papillary muscle and, mainly, the anatomy of the dominant septal artery,
collateral dependence, source of collateral, risk of remote infarction, and, finally,
technical factors with appropriate material, balloon test to verify whether there is a
gradient reduction, amount of alcohol to be injected and procedure monitoring with
contrast echocardiogram to prevent large infarctions.[1],[3],[4],[13]When choosing the type of invasive procedure, SM or SA, in addition to careful patient
selection, one has to consider very thoroughly the fact that even symptomatic patients
have an annual risk of death <3%. Thus, the availability of specialized centers and
operators with experience in both procedures is mandatory, as both invasive procedures
have only been shown to date to have an impact and reduce symptoms and improve quality
of life, and none has shown to be capable of reducing the risk of sudden death, which is
a major concern, especially in younger patients.[8],[13]
"We must always remember that the most important thing is to "treat the patient, not
just the gradient."
Authors: Barry J Maron; Steve R Ommen; Christopher Semsarian; Paolo Spirito; Iacopo Olivotto; Martin S Maron Journal: J Am Coll Cardiol Date: 2014-07-08 Impact factor: 24.094
Authors: Pieter A Vriesendorp; Max Liebregts; Robbert C Steggerda; Arend F L Schinkel; Rik Willems; Folkert J Ten Cate; Johan van Cleemput; Jurriën M Ten Berg; Michelle Michels Journal: JACC Heart Fail Date: 2014-10-22 Impact factor: 12.035
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Authors: Josef Veselka; Lothar Faber; Max Liebregts; Robert Cooper; Jaroslav Januska; Jan Krejci; Thomas Bartel; Maciej Dabrowski; Peter Riis Hansen; Vibeke Marie Almaas; Hubert Seggewiss; Dieter Horstkotte; Radka Adlova; Henning Bundgaard; Jurriën Ten Berg; Rodney Hilton Stables; Morten Kvistholm Jensen Journal: J Am Heart Assoc Date: 2017-05-16 Impact factor: 5.501