Hypertrophic cardiomyopathy other diagnostic studies

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Soroush Seifirad, M.D.[2]

Overview

Left heart catheterization can be a useful diagnostic study to ascertain the severity of the dynamic outflow obstruction and its location. Among patients who have chest discomfort or an anginal equivalent, coronary angiography carries a class I recommendation to evaluate for the presence of obstructive coronary artery disease.The prognostic value of electrophysiologic testing of patients with HOCM in the absence of spontaneous, sustained ventricular tachycardia is limited, and in fact, the study itself may be dangerous. Paced electrogram fractionation in hypertrophic cardiomyopathy may helpful in determining which patients are at risk for ventricular fibrillation.

Cardiac Cathetererization

Left heart catheterization can be a useful diagnostic study to ascertain the severity of the dynamic outflow obstruction and its location.

Left Heart Catheterization

Upon cardiac catheterization, catheters can be placed in the left ventricle and the ascending aorta, to measure the pressure difference between these structures. In normal individuals, during ventricular systole, the pressure in the ascending aorta and the left ventricle will equalize, and the aortic valve is open. In individuals with aortic stenosis or with HCM with an outflow tract gradient, there will be a pressure gradient (difference) between the left ventricle and the aorta, with the left ventricular pressure higher than the aortic pressure. This gradient represents the degree of obstruction that has to be overcome in order to eject blood from the left ventricle.

The Brockenbrough–Braunwald–Morrow sign is observed in individuals with HCM with outflow tract gradient. This sign can be used to differentiate HCM from aortic stenosis. In individuals with aortic stenosis, after a premature ventricular contraction (PVC), the following ventricular contraction will be more forceful, and the pressure generated in the left ventricle will be higher. Because of the fixed obstruction that the stenotic aortic valve represents, the post-PVC ascending aortic pressure will increase as well. In individuals with HCM, however, the degree of obstruction will increase more than the force of contraction will increase in the post-PVC beat. The result of this is that the left ventricular pressure increases and the ascending aortic pressure decreases, with an increase in the LVOT gradient.

Pressure tracings demonstrating the Brockenbrough–Braunwald–Morrow sign
AO = Descending aorta; LV = Left ventricle; ECG = Electrocardiogram.
After the third QRS complex, the ventricle has more time to fill. Since there is more time to fill, the left ventricle will have more volume at the end of diastole (increased preload). Due to the Frank–Starling law of the heart, the contraction of the left ventricle (and pressure generated by the left ventricle) will be greater on the subsequent beat (beat #4 in this picture). Because of the dynamic nature of the outflow obstruction in HCM, the obstruction increases more that the left ventricular pressure increase. This causes a fall in the aortic pressure as the left ventricular pressure rises (seen as the yellow shaded area in the picture).

While the Brockenbrough–Braunwald–Morrow sign is most dramatically demonstrated using simultaneous intra-cardiac and intra-aortic catheters, it can be seen on routine physical examination as a decrease in the pulse pressure in the post-PVC beat in individuals with HCM.

Coronary Angiography

Among patients who have chest discomfort or an anginal equivalent, coronary angiography carries a class I recommendation to evaluate for the presence of obstructive coronary artery disease.

Detection of Concomitant Coronary Disease (DO NOT EDIT)[1]

Class I
"1. Coronary arteriography (invasive or computed tomographic imaging) is indicated in patients with HCM with chest discomfort who have an intermediate to high likelihood of CAD when the identification of concomitant CAD will change management strategies. (Level of Evidence: C) "

Stress Test

Stress testing can be used to assess a hypertrophic obstructive cardiomyopathy patient's functional capacity and response to therapy, their risk of sudden cardiac death, and the magnitude of dynamic outflow obstruction on stress echocardiography.

2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy A Report of the American College of Cardiology/American Heart  Association Joint Committee on Clinical Practice Guidelines[2]

Class I
1. For symptomatic patients with HCM who do not have resting or provocable outflow tract gradient ≥50 mm Hg on TTE, exercise TTE is recommended for the detection and quantification of dynamic LVOTO(Level of Evidence: B-NR )

2. In patients with nonobstructive HCM and advanced HF (NYHA functional class III to class IV despite GDMT), cardiopulmonary exercise stress testing should be performed to quantify the degree of functional limitation and aid in selection of patients for heart transplantation or mechanical circulatory support(Level of Evidence: B-NR )

Class IIa
3. In patients with HCM, exercise stress testing is reasonable to determine functional capacity and to provide prognostic information as part of initial evaluation.(Level of Evidence: B-NR )

4. For asymptomatic patients with HCM who do not have a resting or provocable outflow tract gradient ≥50 mm Hg on standard TTE, exercise TTE is reasonable for the detection and quanti-fication of dynamic LVOTO(Level of Evidence: C-LD )

Class IIb
5. In patients with obstructive HCM who are being considered for SRT and in whom functional capacity or symptom status is uncertain, exercise stress testing may be reasonable(Level of Evidence: C-EO )

6. In patients with HCM in whom functional capacity or symptom status is uncertain, exercise stress testing may be considered every 2 to 3 years(Level of Evidence: C-EO)

2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy (DO NOT EDIT)[1]

Stress Testing (DO NOT EDIT)[1]

Class IIa
"1. Treadmill exercise testing is reasonable to determine functional capacity and response to therapy in patients with HCM. (Level of Evidence: C) "
"2. Treadmill testing with monitoring of an ECG and blood pressure is reasonable for SCD risk stratification in patients with HCM.[3][4][5] (Level of Evidence: B) "
"3. In patients with HCM who do not have a resting peak instantaneous gradient of greater than or equal to 50 mm Hg, exercise echocardiography is reasonable for the detection and quantification of exercise-induced dynamic LVOT obstruction.[6][7][3][4] (Level of Evidence: B) "

Electrophysiologic Stress Test

The prognostic value of electrophysiologic testing of patients with HOCM in the absence of spontaneous, sustained ventricular tachycardia is limited, and in fact, the study itself may be dangerous. Sustained ventricular tachyarrhythmias, predominantly rapid polymorphic ventricular tachycardia, have been induced in 27 to 43 percent of patients with HOCM at electrophysiologic study, but their prognostic significance is controversial. The predictive value of asymptomatic nonsustained ventricular tachycardia is also limited. Paced electrogram fractionation in hypertrophic cardiomyopathy may helpful in determining which patients are at risk for ventricular fibrillation.

The absence of inducible, sustained monomorphic ventricular tachyarrhythmias, absence of nonsustained ventricular tachycardia on ambulatory ECG, and no history of impaired consciousness (i.e., cardiac arrest or syncope) identified a subset (22 percent) of patients with HCM with a low (<1 percent) risk for sudden cardiac death.

References

  1. 1.0 1.1 1.2 Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW (2011). "2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". Journal of the American College of Cardiology. 58 (25): e212–60. doi:10.1016/j.jacc.2011.06.011. PMID 22075469. Retrieved 2011-12-19. Unknown parameter |month= ignored (help)
  2. Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P; et al. (2020). "2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines". Circulation. 142 (25): e558–e631. doi:10.1161/CIR.0000000000000937. PMID 33215931 Check |pmid= value (help).
  3. 3.0 3.1 Sadoul N, Prasad K, Elliott PM, Bannerjee S, Frenneaux MP, McKenna WJ (1997). "Prospective prognostic assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy". Circulation. 96 (9): 2987–91. PMID 9386166. Retrieved 2011-12-22. Unknown parameter |month= ignored (help)
  4. 4.0 4.1 Olivotto I, Maron BJ, Montereggi A, Mazzuoli F, Dolara A, Cecchi F (1999). "Prognostic value of systemic blood pressure response during exercise in a community-based patient population with hypertrophic cardiomyopathy". Journal of the American College of Cardiology. 33 (7): 2044–51. PMID 10362212. Retrieved 2011-12-22. Unknown parameter |month= ignored (help)
  5. Ciampi Q, Betocchi S, Lombardi R, Manganelli F, Storto G, Losi MA, Pezzella E, Finizio F, Cuocolo A, Chiariello M (2002). "Hemodynamic determinants of exercise-induced abnormal blood pressure response in hypertrophic cardiomyopathy". Journal of the American College of Cardiology. 40 (2): 278–84. PMID 12106932. Retrieved 2011-12-22. Unknown parameter |month= ignored (help)
  6. Maron MS, Olivotto I, Zenovich AG, Link MS, Pandian NG, Kuvin JT, Nistri S, Cecchi F, Udelson JE, Maron BJ (2006). "Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction". Circulation. 114 (21): 2232–9. doi:10.1161/CIRCULATIONAHA.106.644682. PMID 17088454. Retrieved 2011-12-22. Unknown parameter |month= ignored (help)
  7. Frenneaux MP, Counihan PJ, Caforio AL, Chikamori T, McKenna WJ (1990). "Abnormal blood pressure response during exercise in hypertrophic cardiomyopathy". Circulation. 82 (6): 1995–2002. PMID 2242524. Retrieved 2011-12-22. Unknown parameter |month= ignored (help)