Hypertrophic cardiomyopathy echocardiography
Hypertrophic Cardiomyopathy Microchapters
Hypertrophic cardiomyopathy echocardiography On the Web
Echocardiography is the imaging modality of choice in the diagnosis of hypertrophic cardiomyopathy. Classically there is a small left ventricular cavity with hypertrophy out of proportion to any underlying condition that would cause LVH. The hypertrophy is often asymmetric.
Echo with doppler is the primary procedure used to diagnose hypertrophic cardiomyopathy. There is a prolonged isovolumic relaxation time, reduced peak E velocity, prolonged deceleration time, increased peak A velocity, and decreased E/A ratio as compared to normal controls.
Proper examination should evaluate :
- Left ventricular asymmetric hypertrophy
- Parasternal long axis shows relationship of the septal hypertrophy and the outflow tract
- Left ventricular diastolic dysfunction
- LV inflow across the mitral valve
- LA inflow in the pulmonary vein
- Myocardial Doppler tissue velocity
- Isovolumetric relaxation time
- Dynamic outflow tract obstruction
- SAM (systolic anterior motion) of the mitral leaflet
- Mid-systolic closure of the aortic valve
- Late peaking, high velocity flow in the outflow tract
- Variability of obstruction with maneuvers (exercise, amyl nitrate inhalation, and post-PVC beats)
- Doppler Techniques
- Use continuous wave doppler to measure the systolic flow velocity in the LV outflow tract and mid-cavity (both at rest and during maneuvers such as the Valsalva maneuver or during dobutamine administration.
Because of the turbulent, high-velocity jet in the left ventricular outflow tract (LVOT), the anterior mitral leaflet moves anteriorly in systole, exacerbating the outflow tract obstruction, and promoting mitral regurgitation. The following images show classic systolic anterior motion (SAM) of the mitral valve leaflets:
On parasternal long-axis view
On parasternal short-axis view
Frequency of Hypertrophy
While LVH is usually present, not all carriers with HCM demonstrate left ventricular hypertrophy. Left ventricular hypertrophy may be absent in childhood. It may then appear following the rapid growth of adolescence and may first appear at age 17 to 18.LVH may be time dependent and may appear late, particularly in patients with a mutation in the myosin-binding protein C gene. Patients generally present later in life and in general, have a better prognosis than beta myosin heavy chain or cardiac troponin T mutations. Up to 60% of patients at age 50 years have no evidence of LVH. LVH may appear later in life in these patients. Because of this, a normal EKG and a normal ECHO at maturity does not exclude the presence of an HCM mutation .
Diagnostic Features of Hypertrophy
- Left ventricular hypertrophy (LVH) most often in an asymmetric distribution,
- Thickening of the left ventricular wall in the absence of cavitary dilation and the presence of hyperdynamic activity (in fact there may be systolic cavity obliteration or near obliteration)
- Absence of any other cause of left ventricular hypertrophy such as aortic stenosis or hypertension
Left Ventricular Wall Thickness
- The traditional definition requires a wall thickness > 15 mm.
- Borderline wall thicknesses of 13-14 mm must be distinguished from extreme variants of athletes heart
- There are some genetic variants that yield a normal wall thickness .
- Among children < 13 years of age, LVH is often absent.
2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy (DO NOT EDIT)
Echocardiography (DO NOT EDIT)
|"1. A TTE is recommended in the initial evaluation of all patients with suspected HOCM. (Level of Evidence: B) "|
|"2. A TTE is recommended as a component of the screening algorithm for family members of patients with HOCM unless the family member is genotype negative in a family with known definitive mutations. (Level of Evidence: B) "|
|"3. Periodic (12 to 18 months) TTE screening is recommended for children of patients with HOCM, starting by age 12 years or earlier if a growth spurt or signs of puberty are evident and/or when there are plans for engaging in intense competitive sports or there is a family history of sudden cardiac death. (Level of Evidence: C) "|
|"4. Repeat TTE is recommended for the evaluation of patients with HOCM with a change in clinical status or new cardiovascular event. (Level of Evidence: B) "|
|"5. A transesophageal echocardiogram (TEE) is recommended for the intra-operative guidance of surgical myectomy. (Level of Evidence: B) "|
|"6. TTE or TEE with intracoronary contrast injection of the candidate’s septal perforator(s) is recommended for the intra-procedural guidance of alcohol septal ablation. (Level of Evidence: B) "|
|"7. TTE should be used to evaluate the effects of surgical myectomy or alcohol septal ablation for obstructive HOCM. (Level of Evidence: C) "|
|Class III (No Benefit)|
|"1. TTE studies should not be performed more frequently than every 12 months in patients with HOCM when it is unlikely that any changes have occurred that would have an impact on clinical decision making. (Level of Evidence: C) "|
|"2. Routine TEE and/or contrast echocardiography is not recommended when TTE images are diagnostic of HOCM and/or there is no suspicion of fixed obstruction or intrinsic mitral valve pathology. (Level of Evidence: C) "|
|"1. TTE studies performed every 1 to 2 years can be useful in the serial evaluation of symptomatically stable patients with HOCM to assess the degree of myocardial hypertrophy, dynamic obstruction, and myocardial function. (Level of Evidence: C) "|
|"2. Exercise TTE can be useful in the detection and quantification of dynamic LVOT obstruction in the absence of resting outflow tract obstruction in patients with HOCM. (Level of Evidence: B) "|
|"3. TEE can be useful if TTE is inconclusive for clinical decision making about medical therapy and in situations such as planning for myectomy, exclusion of sub-aortic membrane or mitral regurgitation secondary to structural abnormalities of the mitral valve apparatus, or in assessment for the feasibility of alcohol septal ablation. (Level of Evidence: C) "|
|"4. TTE combined with the injection of an intravenous contrast agent is reasonable if the diagnosis of apical HOCM or apical infarction or severity of hypertrophy is in doubt, particularly when other imaging modalities such as CMR are not readily available, not diagnostic, or are contraindicated. (Level of Evidence: C)"|
|"5. Serial TTE studies are reasonable for clinically unaffected patients who have a first-degree relative with HOCM when genetic status is unknown. Such follow-up may be considered every 12 to 18 months for children or adolescents from high-risk families and every 5 years for adult family members. (Level of Evidence: C)"|
Detection of Concomitant Coronary Disease 
|Class III (No Benefit)|
|"1. Routine SPECT MPI or stress echocardiography is not indicated for detection of silent CAD-related ischemia in patients with HOCM who are asymptomatic. (Level of Evidence: C) "|
- ↑ Otto, Catherine. Textbook of Clinical Echocardiography. 3rd Edition, 2004
- ↑ Hagege AA, Dubourg O, Desnos M et al. Familial hypertrophic cardiomyopathy. Cardiac ultrasonic abnormalities in genetically affected subjects without echocardiographic evidence of left ventricular hypertrophy. Eur Heart J 1998;19:490–9.
- ↑ Maron BJ, Spirito P, Wesley Y, Arce J. Development and progression of left ventricular hypertrophy in children with hypertrophic cardiomyopathy. N Engl J Med 1986;315:610–4.
- ↑ Spirito P, Maron BJ. Absence of progression of left ventricular hypertrophy in adult patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 1987;9:1013–7.
- ↑ Erdmann J, Raible J, Maki-Abadi J et al. Spectrum of clinical phenotypes and gene variants in cardiac myosin-binding protein C mutation carriers with hypertrophic cardiomyopathy. J Am Coll Cardiol 2001;38:322–30.
- ↑ Niimura H, Bachinski LL, Sangwatanaroj S et al. Mutations in the gene for cardiac myosin-binding protein C and late-onset familial hypertrophic cardiomyopathy. N Engl J Med 1998;338:1248–57.
- ↑ Maron BJ, Niimura H, Casey SA et al. Development of left ventricular hypertrophy in adults in hypertrophic cardiomyopathy caused by cardiac myosin-binding protein C gene mutations. J Am Coll Cardiol 2001;38:315–21.
- ↑ Charron P, Dubourg O, Desnos M et al. Diagnostic value of electrocardiography and echocardiography for familial hypertrophic cardiomyopathy in a genotyped adult population. Circulation 1997; 96:214–9.
- ↑ Charron P, Dubourg O, Desnos M et al. Diagnostic value of electrocardiography and echocardiography for familial hypertrophic cardiomyopathy in genotyped children. Eur Heart J 1998;19:1377–82.
- ↑ Panza JA, Maron BJ. Relation of electrocardiographic abnormalities to evolving left ventricular hypertrophy in hypertrophic cardiomyopathy during childhood. Am J Cardiol 1989;63:1258–65.
- ↑ 11.0 11.1 11.2 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 (December 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 on 2011-12-19.
- ↑ Maron BJ (March 2002). "Hypertrophic cardiomyopathy: a systematic review". JAMA : the Journal of the American Medical Association 287 (10): 1308–20. PMID 11886323. Retrieved on 2011-12-22.
- ↑ 13.0 13.1 Klues HG, Schiffers A, Maron BJ (December 1995). "Phenotypic spectrum and patterns of left ventricular hypertrophy in hypertrophic cardiomyopathy: morphologic observations and significance as assessed by two-dimensional echocardiography in 600 patients". Journal of the American College of Cardiology 26 (7): 1699–708. doi:10.1016/0735-1097(95)00390-8. PMID 7594106. Retrieved on 2011-12-22.
- ↑ Wigle ED, Sasson Z, Henderson MA, Ruddy TD, Fulop J, Rakowski H, Williams WG (1985). "Hypertrophic cardiomyopathy. The importance of the site and the extent of hypertrophy. A review". Progress in Cardiovascular Diseases 28 (1): 1–83. PMID 3160067. Retrieved on 2011-12-22.
- ↑ 15.0 15.1 Wigle ED, Rakowski H, Kimball BP, Williams WG (October 1995). "Hypertrophic cardiomyopathy. Clinical spectrum and treatment". Circulation 92 (7): 1680–92. PMID 7671349. Retrieved on 2011-12-22.
- ↑ Adabag AS, Kuskowski MA, Maron BJ (December 2006). "Determinants for clinical diagnosis of hypertrophic cardiomyopathy". The American Journal of Cardiology 98 (11): 1507–11. doi:10.1016/j.amjcard.2006.07.029. PMID 17126660. Retrieved on 2011-12-22.
- ↑ 17.0 17.1 Afonso LC, Bernal J, Bax JJ, Abraham TP (November 2008). "Echocardiography in hypertrophic cardiomyopathy: the role of conventional and emerging technologies". JACC. Cardiovascular Imaging 1 (6): 787–800. doi:10.1016/j.jcmg.2008.09.002. PMID 19356516. Retrieved on 2011-12-22.
- ↑ Fifer MA, Vlahakes GJ (January 2008). "Management of symptoms in hypertrophic cardiomyopathy". Circulation 117 (3): 429–39. doi:10.1161/CIRCULATIONAHA.107.694158. PMID 18212300. Retrieved on 2011-12-22.
- ↑ Soor GS, Luk A, Ahn E, Abraham JR, Woo A, Ralph-Edwards A, Butany J (March 2009). "Hypertrophic cardiomyopathy: current understanding and treatment objectives". Journal of Clinical Pathology 62 (3): 226–35. doi:10.1136/jcp.2008.061655. PMID 18930982. Retrieved on 2011-12-22.
- ↑ 20.0 20.1 Bos JM, Towbin JA, Ackerman MJ (July 2009). "Diagnostic, prognostic, and therapeutic implications of genetic testing for hypertrophic cardiomyopathy". Journal of the American College of Cardiology 54 (3): 201–11. doi:10.1016/j.jacc.2009.02.075. PMID 19589432. Retrieved on 2011-12-22.
- ↑ 21.0 21.1 21.2 Maron BJ, Seidman JG, Seidman CE (December 2004). "Proposal for contemporary screening strategies in families with hypertrophic cardiomyopathy". Journal of the American College of Cardiology 44 (11): 2125–32. doi:10.1016/j.jacc.2004.08.052. PMID 15582308. Retrieved on 2011-12-22.
- ↑ Binder J, Ommen SR, Gersh BJ, Van Driest SL, Tajik AJ, Nishimura RA, Ackerman MJ (April 2006). "Echocardiography-guided genetic testing in hypertrophic cardiomyopathy: septal morphological features predict the presence of myofilament mutations". Mayo Clinic Proceedings. Mayo Clinic 81 (4): 459–67. PMID 16610565. Retrieved on 2011-12-22.
- ↑ 23.0 23.1 Hershberger RE, Cowan J, Morales A, Siegfried JD (May 2009). "Progress with genetic cardiomyopathies: screening, counseling, and testing in dilated, hypertrophic, and arrhythmogenic right ventricular dysplasia/cardiomyopathy". Circulation. Heart Failure 2 (3): 253–61. doi:10.1161/CIRCHEARTFAILURE.108.817346. PMID 19808347. Retrieved on 2011-12-22.
- ↑ 24.0 24.1 Schwartz ML, Cox GF, Lin AE, Korson MS, Perez-Atayde A, Lacro RV, Lipshultz SE (October 1996). "Clinical approach to genetic cardiomyopathy in children". Circulation 94 (8): 2021–38. PMID 8873681. Retrieved on 2011-12-22.
- ↑ Harris KM, Spirito P, Maron MS, Zenovich AG, Formisano F, Lesser JR, Mackey-Bojack S, Manning WJ, Udelson JE, Maron BJ (July 2006). "Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy". Circulation 114 (3): 216–25. doi:10.1161/CIRCULATIONAHA.105.583500. PMID 16831987. Retrieved on 2011-12-22.
- ↑ 26.0 26.1 Maron MS, Olivotto I, Betocchi S, Casey SA, Lesser JR, Losi MA, Cecchi F, Maron BJ (January 2003). "Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy". The New England Journal of Medicine 348 (4): 295–303. doi:10.1056/NEJMoa021332. PMID 12540642. Retrieved on 2011-12-22.
- ↑ Maron BJ, Olivotto I, Spirito P, Casey SA, Bellone P, Gohman TE, Graham KJ, Burton DA, Cecchi F (August 2000). "Epidemiology of hypertrophic cardiomyopathy-related death: revisited in a large non-referral-based patient population". Circulation 102 (8): 858–64. PMID 10952953. Retrieved on 2011-12-22.
- ↑ Dimitrow PP, Dubiel JS (January 2005). "Echocardiographic risk factors predisposing to sudden cardiac death in hypertrophic cardiomyopathy". Heart (British Cardiac Society) 91 (1): 93–4. doi:10.1136/hrt.2003.030353. PMID 15604346. Retrieved on 2011-12-22.
- ↑ 29.0 29.1 Efthimiadis GK, Parcharidou DG, Giannakoulas G, Pagourelias ED, Charalampidis P, Savvopoulos G, Ziakas A, Karvounis H, Styliadis IH, Parcharidis GE (September 2009). "Left ventricular outflow tract obstruction as a risk factor for sudden cardiac death in hypertrophic cardiomyopathy". The American Journal of Cardiology 104 (5): 695–9. doi:10.1016/j.amjcard.2009.04.039. PMID 19699347. Retrieved on 2011-12-22.
- ↑ Ommen SR, Shah PM, Tajik AJ (October 2008). "Left ventricular outflow tract obstruction in hypertrophic cardiomyopathy: past, present and future". Heart (British Cardiac Society) 94 (10): 1276–81. doi:10.1136/hrt.2008.154435. PMID 18653577. Retrieved on 2011-12-22.
- ↑ 31.0 31.1 Sorajja P, Nishimura RA, Gersh BJ, Dearani JA, Hodge DO, Wiste HJ, Ommen SR (July 2009). "Outcome of mildly symptomatic or asymptomatic obstructive hypertrophic cardiomyopathy: a long-term follow-up study". Journal of the American College of Cardiology 54 (3): 234–41. doi:10.1016/j.jacc.2009.01.079. PMID 19589436. Retrieved on 2011-12-22.
- ↑ 32.0 32.1 Grigg LE, Wigle ED, Williams WG, Daniel LB, Rakowski H (July 1992). "Transesophageal Doppler echocardiography in obstructive hypertrophic cardiomyopathy: clarification of pathophysiology and importance in intraoperative decision making". Journal of the American College of Cardiology 20 (1): 42–52. PMID 1607537. Retrieved on 2011-12-22.
- ↑ 33.0 33.1 Marwick TH, Stewart WJ, Lever HM, Lytle BW, Rosenkranz ER, Duffy CI, Salcedo EE (November 1992). "Benefits of intraoperative echocardiography in the surgical management of hypertrophic cardiomyopathy". Journal of the American College of Cardiology 20 (5): 1066–72. PMID 1401604. Retrieved on 2011-12-22.
- ↑ 34.0 34.1 Yu EH, Omran AS, Wigle ED, Williams WG, Siu SC, Rakowski H (December 2000). "Mitral regurgitation in hypertrophic obstructive cardiomyopathy: relationship to obstruction and relief with myectomy". Journal of the American College of Cardiology 36 (7): 2219–25. PMID 11127464. Retrieved on 2011-12-22.
- ↑ 35.0 35.1 Sorajja P, Valeti U, Nishimura RA, Ommen SR, Rihal CS, Gersh BJ, Hodge DO, Schaff HV, Holmes DR (July 2008). "Outcome of alcohol septal ablation for obstructive hypertrophic cardiomyopathy". Circulation 118 (2): 131–9. doi:10.1161/CIRCULATIONAHA.107.738740. PMID 18591440. Retrieved on 2011-12-22.
- ↑ Faber L, Seggewiss H, Welge D, Fassbender D, Schmidt HK, Gleichmann U, Horstkotte D (October 2004). "Echo-guided percutaneous septal ablation for symptomatic hypertrophic obstructive cardiomyopathy: 7 years of experience". European Journal of Echocardiography : the Journal of the Working Group on Echocardiography of the European Society of Cardiology 5 (5): 347–55. doi:10.1016/j.euje.2004.01.001. PMID 15341870. Retrieved on 2011-12-22.
- ↑ Monakier D, Woo A, Puri T, Schwartz L, Ross J, Jamorski M, Yang H, Liu Z, Vannan M, Wigle ED, Rakowski H (December 2004). "Usefulness of myocardial contrast echocardiographic quantification of risk area for predicting postprocedural complications in patients undergoing septal ethanol ablation for obstructive hypertrophic cardiomyopathy". The American Journal of Cardiology 94 (12): 1515–22. doi:10.1016/j.amjcard.2004.08.030. PMID 15589007. Retrieved on 2011-12-22.
- ↑ Nagueh SF, Lakkis NM, He ZX, Middleton KJ, Killip D, Zoghbi WA, Quiñones MA, Roberts R, Verani MS, Kleiman NS, Spencer WH (July 1998). "Role of myocardial contrast echocardiography during nonsurgical septal reduction therapy for hypertrophic obstructive cardiomyopathy". Journal of the American College of Cardiology 32 (1): 225–9. PMID 9669274. Retrieved on 2011-12-22.
- ↑ Ommen SR, Maron BJ, Olivotto I, Maron MS, Cecchi F, Betocchi S, Gersh BJ, Ackerman MJ, McCully RB, Dearani JA, Schaff HV, Danielson GK, Tajik AJ, Nishimura RA (August 2005). "Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy". Journal of the American College of Cardiology 46 (3): 470–6. doi:10.1016/j.jacc.2005.02.090. PMID 16053960. Retrieved on 2011-12-22.
- ↑ Carasso S, Woo A, Yang H, Schwartz L, Vannan MA, Jamorski M, Linghorne M, Wigle ED, Rakowski H (May 2008). "Myocardial mechanics explains the time course of benefit for septal ethanol ablation for hypertrophic cardiomyopathy". Journal of the American Society of Echocardiography : Official Publication of the American Society of Echocardiography 21 (5): 493–9. doi:10.1016/j.echo.2007.08.020. PMID 17961980. Retrieved on 2011-12-22.
- ↑ Fernandes VL, Nielsen C, Nagueh SF, Herrin AE, Slifka C, Franklin J, Spencer WH (October 2008). "Follow-up of alcohol septal ablation for symptomatic hypertrophic obstructive cardiomyopathy the Baylor and Medical University of South Carolina experience 1996 to 2007". JACC. Cardiovascular Interventions 1 (5): 561–70. doi:10.1016/j.jcin.2008.07.005. PMID 19463359. Retrieved on 2011-12-22.
- ↑ Jassal DS, Neilan TG, Fifer MA, Palacios IF, Lowry PA, Vlahakes GJ, Picard MH, Yoerger DM (August 2006). "Sustained improvement in left ventricular diastolic function after alcohol septal ablation for hypertrophic obstructive cardiomyopathy". European Heart Journal 27 (15): 1805–10. doi:10.1093/eurheartj/ehl106. PMID 16774986. Retrieved on 2011-12-22.
- ↑ Woo A, Williams WG, Choi R, Wigle ED, Rozenblyum E, Fedwick K, Siu S, Ralph-Edwards A, Rakowski H (April 2005). "Clinical and echocardiographic determinants of long-term survival after surgical myectomy in obstructive hypertrophic cardiomyopathy". Circulation 111 (16): 2033–41. doi:10.1161/01.CIR.0000162460.36735.71. PMID 15824202. Retrieved on 2011-12-22.
- ↑ Yoerger DM, Picard MH, Palacios IF, Vlahakes GJ, Lowry PA, Fifer MA (May 2006). "Time course of pressure gradient response after first alcohol septal ablation for obstructive hypertrophic cardiomyopathy". The American Journal of Cardiology 97 (10): 1511–4. doi:10.1016/j.amjcard.2005.12.040. PMID 16679095. Retrieved on 2011-12-22.
- ↑ Maron MS, Olivotto I, Zenovich AG, Link MS, Pandian NG, Kuvin JT, Nistri S, Cecchi F, Udelson JE, Maron BJ (November 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 on 2011-12-22.
- ↑ Sherrid MV, Barac I, McKenna WJ, Elliott PM, Dickie S, Chojnowska L, Casey S, Maron BJ (April 2005). "Multicenter study of the efficacy and safety of disopyramide in obstructive hypertrophic cardiomyopathy". Journal of the American College of Cardiology 45 (8): 1251–8. doi:10.1016/j.jacc.2005.01.012. PMID 15837258. Retrieved on 2011-12-22.
There is no pharmaceutical or device industry support for this site and we need your viewer supported Donations | Editorial Board | Governance | Licensing | Disclaimers | Avoid Plagiarism | Policies