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Comparison of Outcomes in Patients With Nonobstructive, Labile-Obstructive, and Chronically Obstructive Hypertrophic
Iraklis Pozios1, Celia Corona-Villalobos2, Lars L Sorensen1
1Johns Hopkins HCM Center of Excellence, Baltimore, Maryland.
Insights
Patients with nonobstructive hypertrophic cardiomyopathy (HC) face higher risks of ventricular arrhythmias than previously thought. This study reveals nonobstructive HC is linked to more fibrosis and ischemia, predicting sudden cardiac events.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Electrophysiology
Background:
- Nonobstructive hypertrophic cardiomyopathy (HC) is often considered low-risk.
- Traditionally, nonobstructive and labile-obstructive HC have been grouped, potentially masking distinct risk profiles.
Purpose of the Study:
- To compare cardiovascular outcomes between nonobstructive, labile-obstructive, and obstructive HC subgroups.
- To determine if nonobstructive hemodynamics predict adverse cardiovascular events.
Main Methods:
- Compared cardiovascular outcomes in 293 HC patients (nonobstructive, labile-obstructive, obstructive) using exercise echocardiography and MRI.
- Assessed fibrosis with late gadolinium enhancement (LGE) and ischemia with positron emission tomography (PET) in a subgroup.
- Followed patients for a mean of 3.3 years, recording major adverse events.
Main Results:
- Nonobstructive HC patients showed significantly higher LGE (≥20% myocardial mass) and regional PET perfusion abnormalities.
- Nonobstructive HC was an independent predictor of ventricular tachycardia/ventricular fibrillation (VT/VF).
- Appropriate defibrillator discharges were more frequent in nonobstructive patients (18%) compared to labile-obstructive (0%).
Conclusions:
- Nonobstructive hemodynamics in HC are associated with greater myocardial fibrosis and ischemia.
- Nonobstructive HC is an independent predictor of VT/VF, challenging the low-risk classification.
- Risk stratification for HC should differentiate between nonobstructive and labile-obstructive phenotypes.
Abstract:
Patients with nonobstructive hypertrophic cardiomyopathy (HC) are considered low risk, generally not requiring aggressive intervention. However, nonobstructive and labile-obstructive HC have been traditionally classified together, and it is unknown if these 2 subgroups have distinct risk profiles. We compared cardiovascular outcomes in 293 patients HC (96 nonobstructive, 114 labile-obstructive, and 83 obstructive) referred for exercise echocardiography and magnetic resonance imaging and followed for 3.3 ± 3.6 years. A subgroup (34 nonobstructive, 28 labile-obstructive, 21 obstructive) underwent positron emission tomography. The mean number of sudden cardiac death risk factors was similar among groups (nonobstructive: 1.4 vs labile-obstructive: 1.2 vs obstructive: 1.4 risk factors, p = 0.2). Prevalence of late gadolinium enhancement (LGE) was similar across groups but more non-obstructive patients had late gadolinium enhancement ≥20% of myocardial mass (23 [30%] vs 19 [18%] labile-obstructive and 8 [11%] obstructive, p = 0.01]. Fewer labile-obstructive patients had regional positron emission tomography perfusion abnormalities (12 [46%] vs nonobstructive 30 [81%] and obstructive 17 [85%], p = 0.003]. During follow-up, 60 events were recorded (36 ventricular tachycardia/ventricular fibrillation, including 30 defibrillator discharges, 12 heart failure worsening, and 2 deaths). Nonobstructive patients were at greater risk of VT/VF at follow-up, compared to labile obstructive (hazed ratio 0.18, 95% confidence interval 0.04 to 0.84, p = 0.03) and the risk persisted after adjusting for age, gender, syncope, family history of sudden cardiac death, abnormal blood pressure response, and septum ≥3 cm (p = 0.04). Appropriate defibrillator discharges were more frequent in nonobstructive (8 [18%]) compared to labile-obstructive (0 [0%], p = 0.02) patients. In conclusion, nonobstructive hemodynamics is associated with more pronounced fibrosis and ischemia than labile-obstructive and is an independent predictor of VT/VF in HC.
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