The clinical presentation and prognosis of hypertrophic cardiomyopathy (HCM) are heterogeneous between nonobstructive HCM (HNCM) and obstructive HCM (HOCM). Electrocardiography (ECG) has been used as ...a screening tool for HCM. However, it is still unclear whether the features presented on ECG could be used for the initial classification of HOCM and HNCM.
We aimed to develop a pragmatic model based on common 12-lead ECG features for the initial identification of HOCM/HNCM.
Between April 1
and September 30
, 2020, 172 consecutive HCM patients from the International Cooperation Center for Hypertrophic Cardiomyopathy of Xijing Hospital were prospectively included in the training cohort. Between January 4
and February 30
, 2021, an additional 62 HCM patients were prospectively included in the temporal internal validation cohort. External validation was performed using retrospectively collected ECG data with definite classification (390 HOCM and 499 HNCM ECG samples) from January 1
, 2010 to March 31
, 2020. Multivariable backward logistic regression (LR) was used to develop the prediction model. The discrimination performance, calibration and clinical utility of the model were evaluated.
Of all 30 acquired ECG parameters, 10 variables were significantly different between HOCM and HNCM (all
< 0.05). The P wave interval and SV1 were selected to construct the model, which had a clearly useful C-statistic of 0.805 (0.697, 0.914) in the temporal validation cohort and 0.776 (0.746, 0.806) in the external validation cohort for differentiating HOCM from HNCM. The calibration plot, decision curve analysis, and clinical impact curve indicated that the model had good fitness and clinical utility.
The pragmatic model constructed by the P wave interval and SV1 had a clearly useful ability to discriminate HOCM from HNCM. The model might potentially serve as an initial classification of HCM before referring patients to dedicated centers and specialists.
Evident differences exist in the ECG presentations between HOCM and HNCM.To the best of our knowledge, this study is the first piece of evidence to quantify the difference in the ECG presentations between HOCM and HNCM.Based on routine 12-lead ECG data, a probabilistic model was generated that might assist in the initial classification of HCM patients.
Background: Patients with hypertrophic cardiomyopathy (HCM) show various physical findings, but their clinical significance has not been systematically evaluated.Methods and Results: This study ...evaluated 105 consecutive patients with HCM who had undergone phonocardiography and external pulse recording. Physical examinations included a visible jugular a-wave (Jug-a), audible 4th sound (S4), and double or sustained apex beat. The primary outcome was a composite of all-cause death and hospitalization for cardiovascular disease. A total of 104 non-HCM subjects served as controls. The prevalence of visible Jug-a in the seated or supine position, audible S4, and a sustained or double apex beat in patients with HCM were 10%, 71%, 70%, 42%, and 27%, respectively, all of which were significantly higher than in the controls (0%, 20%, 11%, 17%, and 2%; P<0.001 for all comparisons). The combination of visible Jug-a in the supine position and audible S4 yielded a specificity of 94% and sensitivity of 57%. During a follow-up period of 6.6 years, 6 patients died and 10 were hospitalized. The absence of audible S4 was a predictor of cardiovascular events (hazard ratio, 3.91; 95% confidence interval, 1.41 to 10.8; P=0.005).Conclusions: Detection of these findings has clinical importance in the diagnosis and risk stratification of HCM prior to the use of advanced imaging techniques.
We reviewed the long-term outcome of children with hypertrophic cardiomyopathy (HCM) based on the type.
We reviewed the medical records of 100 patients (male 54 female 46) with HCM at our hospital ...between 1977 and 2015. The survival and cardiac event-free survival rates were calculated by the Kaplan–Meier method.
The age at the time of the diagnosis ranged from 0 to 15 years with a median of 8 years. The number of patients with Noonan syndrome and hypertrophic obstructive cardiomyopathy (HOCM), idiopathic HCM (i-HCM), and secondary HCM (s-HCM) was 13, 13, 65, and 9 respectively. A dilated phase of HCM occurred in 24 patients. Nineteen (79 %) of the 24 patients died of heart failure, and two underwent a heart transplantation. Eight (33 %) of the 24 patients had s-HCM. The median age when a dilated phase occurred was 15 years old, and the median interval from the initial diagnosis to the dilated phase was 8 years. The median time from the diagnosis of a dilated phase to death was 1.6 years. Sudden death and implantable cardioverter defibrillator implantations occurred in 6 and 11 patients at around 15 years old, respectively. The 20-year survival rates were as follows: Noonan syndrome 84 %; HOCM 82 %; i-HCM 71 %; and s-HCM 17 %. Overall, the survival rates at 10, 20, and 30 years were 83 % (95 % confidence interval 73–89), 69 % (58–78), and 63 % (50–74), respectively. The overall cardiac event-free survival rates at 10, 20, and 30 years were 57 % (47–67), 39 % (31–50), and 32 % (21–44), respectively.
The long-term outcome in children with HCM was poor, and the outcome of s-HCM was very poor. The occurrence of a dilated phase worsened the outcome in HCM patients. Sudden death and d-HCM often occurred at around 15 years old.
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•The overall 30-year cardiac event free survival rate was 32 %.•The long-term outcome in children with hypertrophic cardiomyopathy was poor.•The median age when a dilated phase occurred was 15 years old.•Sudden death often occurred at around 15 years old.•The outcome of secondary hypertrophic cardiomyopathy was very poor.
Noninvasive left ventricular (LV) pressure-strain loop analysis is emerging as a new echocardiographic method to evaluate LV function, integrating longitudinal strain by speckle-tracking analysis and ...sphygmomanometrically measured blood pressure to estimate myocardial work. The aims of this study were (1) to describe global and segmental myocardial work in patients with hypertrophic cardiomyopathy (HCM), (2) to assess the correlation between myocardial work and other echocardiographic parameters, and (3) to evaluate the association of myocardial work with adverse outcomes.
One hundred ten patients with nonobstructive HCM (mean age, 55 ± 15 years; 66% men), with different phenotypes (apical, concentric, and septal hypertrophy), and 35 age- and sex-matched healthy control subjects were included. The following myocardial work indices were included: myocardial work index, constructive work (CW), wasted work, and cardiac efficiency. The combined end point included all-cause mortality, heart transplantation, heart failure hospitalization, aborted sudden cardiac death, and appropriate implantable cardioverter-defibrillator therapy.
Mean global CW (1,722 ± 602 vs 2,274 ± 574 mm Hg%, P < .001), global cardiac efficiency (93% 89%–95% vs 96% 96%–97%, P < .001), and global MWI (1,534 ± 551 vs 1,929 ± 473 mm Hg%) were significantly reduced, while global wasted work (104 mm Hg% 66–137 mm Hg% vs 71 mm Hg% 49–92 mm Hg%, P < .001) was increased in patients with HCM compared with control subjects. Segmental impairment in CW colocalized with maximal wall thickness (HCM phenotype), and global CW correlated with LV wall thickness (r = −0.41, P < .001), diastolic function (r = −0.27, P = .001), and QRS duration (r = −0.28, P = .001). Patients with global CW > 1,730 mm Hg% (the median value) experienced better event-free survival than those with global CW < 1,730 mm Hg% (P < .001).
Myocardial work, assessed noninvasively using echocardiography and blood pressure measurement, is reduced in patients with nonobstructive HCM; it correlates with maximum LV wall thickness and is significantly associated with a worse long-term outcome.
•Myocardial work parameters are impaired in patients with HCM.•CW is associated with adverse events in patients with HCM.•Segmental differences of CW were observed among HCM phenotypes.
Hypertrophic cardiomyopathy (HCM), a relatively common, globally distributed, and often inherited primary cardiac disease, has now transformed into a contemporary highly treatable condition with ...effective options that alter natural history along specific personalized adverse pathways at all ages. HCM patients with disease-related complications benefit from: matured risk stratification in which major markers reliably select patients for prophylactic defibrillators and prevention of arrhythmic sudden death; low risk to high benefit surgical myectomy (with percutaneous alcohol ablation a selective alternative) that reverses progressive heart failure caused by outflow obstruction; anticoagulation prophylaxis that prevents atrial fibrillation-related embolic stroke and ablation techniques that decrease the frequency of paroxysmal episodes; and occasionally, heart transplant for end-stage nonobstructive patients. Those innovations have substantially improved outcomes by significantly reducing morbidity and HCM-related mortality to 0.5%/y. Palliative pharmacological strategies with currently available negative inotropic drugs can control symptoms over the short-term in some patients, but generally do not alter long-term clinical course. Notably, a substantial proportion of HCM patients (largely those identified without outflow obstruction) experience a stable/benign course without major interventions. The expert panel has critically appraised all available data and presented management insights and recommendations with concise principles for clinical decision-making.
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