Skip to main content

Advertisement

Log in

Is There a Role for Alcohol Septal Ablation in Young Patients with Medically Refractory Hypertrophic Obstructive Cardiomyopathy?

  • Research
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

Surgical myectomy is recommended for symptomatic hypertrophic obstructive cardiomyopathy (HOCM) after optimal pharmacological therapy. Percutaneous transluminal septal myocardial ablation (PTSMA) is reserved for high-risk adults. Symptomatic patients below 25 years underwent either surgery or PTSMA after heart-team discussion and informed consent. Echocardiography assessed gradients in surgical group. PTSMA group underwent invasive transseptal hemodynamic assessment, selective coronary angiography and super-selective cannulation of septal perforators using microcatheters. Contrast echocardiography through the microcatheter identified the myocardial target for PTSMA. Hemodynamic and electrocardiographic monitoring guided alcohol injection. Both groups were continued on beta-blockers. Symptoms, echocardiographic gradients and Brain natriuretic peptide (NTproBNP) measurements were assessed on follow-up. Twelve patients aged 5–23 years (11–98 kg) formed the study group. Indications for PTSMA in 8 patients included abnormal mitral valve anatomy warranting replacement (n = 3), Jehovah’s witness (n = 2), severe neurodevelopmental and growth retardation (n = 1) and refusal of surgery (n = 2). PTSMA targeted first perforator (n = 5), second perforator (n = 2) and anomalous septal artery from left main trunk (n = 1). Outflow gradient reduced from 92.5 ± 19.7 to 33.1 ± 13.5 mmHg. At a median follow-up of 38 months (range 3–120 weeks), the peak instantaneous echocardiographic gradient was 32 ± 16.5 mmHg. Gradient reduced in four surgical patients from 86.5 ± 16.3 mmHg to 42 ± 14.7 mm Hg. All patients were in NYHA class I/II on follow-up. The mean NTproBNP in PTSMA group reduced from 6084 ± 3628 pg/ml to 3081 ± 2019 pg/ml; it was 1396 and 1795 pg/ml in surgery. PTSMA may be considered in medically refractory high-risk young patients. It relieves symptoms and reduces gradient. Though surgery is preferred in young patients, PTSMA may have a role in selected patients.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

References

  1. Elliott PM, Anastasakis A, Borger MA, Borggrefe M, Cecchi F, Charron P et al (2014) ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the task force for the diagnosis and management of hypertrophic cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J 35:2733–2779

    Article  PubMed  Google Scholar 

  2. Colan SD, Lipshultz SE, Lowe AM, Sleeper LA, Messere J, Cox GF et al (2007) Epidemiology and cause-specific outcome of hypertrophic cardiomyopathy in children: findings from the pediatric cardiomyopathy registry. Circulation 115:773–781

    Article  PubMed  Google Scholar 

  3. Arghami A, Dearani JA, Said SM, O’Leary PW, Schaff HV (2017) Hypertrophic cardiomyopathy in children. Ann Cardiothorac Surg 6:376–385

    Article  PubMed  PubMed Central  Google Scholar 

  4. Lipshultz SE, Orav EJ, Wilkinson JD, Towbin JA, Messere JE, Lowe AM et al (2013) Risk stratification at diagnosis for children with hypertrophic cardiomyopathy: an analysis of data from the Pediatric Cardiomyopathy Registry. Lancet 382:1889–1897

    Article  PubMed  PubMed Central  Google Scholar 

  5. Maron BJ, Spirito P, Wesley Y, Arce J (1986) Development and progression of left ventricular hypertrophy in children with hypertrophic cardiomyopathy. N Engl J Med 315:610–614

    Article  CAS  PubMed  Google Scholar 

  6. Maron BJ, Roberts WC, Edwards JE, McAllister HA Jr, Foley DD, Epstein SE (1978) Sudden death in patients with hypertrophic cardiomyopathy: characterization of 26 patients with functional limitation. Am J Cardiol 41:803–810

    Article  CAS  PubMed  Google Scholar 

  7. Dearani JA, Ommen SR, Gersh BJ, Schaff HV, Danielson GK (2007) Surgery insight: Septal myectomy for obstructive hypertrophic cardiomyopathy-the Mayo Clinic experience. Nat Clin Pract Cardiovasc Med 4:503–512

    Article  PubMed  Google Scholar 

  8. Kunkala MR, Schaff HV, Nishimura RA et al (2013) Transapical approach to myectomy for midventricular obstruction in hypertrophic cardiomyopathy. Ann Thorac Surg 96:564–570

    Article  PubMed  Google Scholar 

  9. Iacovoni A, Spirito P, Simon C et al (2012) A contemporary European experience with surgical septal myectomy in hypertrophic cardiomyopathy. Eur Heart J 33:2080–7

    Article  PubMed  PubMed Central  Google Scholar 

  10. Kim LK, Swaminathan RV, Looser P et al (2016) Hospital volume outcomes after septal myectomy and alcohol septal ablation for treatment of obstructive hypertrophic cardiomyopathy: US nationwide inpatient database, 2003–2011. JAMA Cardiol 1:324–332

    Article  PubMed  Google Scholar 

  11. Kwon DH, Kapadia SR, Tuzcu EM et al (2008) Long-term outcomes in high-risk symptomatic patients with hypertrophic cardiomyopathy undergoing alcohol septal ablation. JACC Cardiovasc Interv 1:432–438

    Article  PubMed  Google Scholar 

  12. Fernandes VL, Nielsen C, Nagueh SF et al (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 Cardiovasc Interv 1:561–570

    Article  PubMed  Google Scholar 

  13. Veselka J, Faber L, Jensen MK, Cooper R, Januska J, Krejci J et al (2018) Effect of institutional experience on outcomes of alcohol septal ablation for hypertrophic obstructive cardiomyopathy. Can J Cardiol 34:16–22

    Article  PubMed  Google Scholar 

  14. Gersh BJ, Maron BJ, Bonow RO et al (2011) ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: executive summary: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. J Thorac Cardiovasc Surg 142:1303–1338

    Article  PubMed  Google Scholar 

  15. Holmes DR Jr, Valeti US, Nishimura RA (2005) Alcohol septal ablation for hypertrophic cardiomyopathy: indications and technique. Catheter Cardiovasc Interv 66:375–389

    Article  PubMed  Google Scholar 

  16. Veselka J, Zemanek D, Tomasov P et al (2009) Alcohol septal ablation for obstructive hypertrophic cardiomyopathy: ultra-low dose of alcohol (1 ml) is still effective. Heart Vessels 24:27–31

    Article  PubMed  Google Scholar 

  17. Leonardi RA, Kransdorf EP, Simel DL et al (2010) Meta-analyses of septal reduction therapies for obstructive hypertrophic cardiomyopathy: comparative rates of overall mortality and sudden cardiac death after treatment. Circ Cardiovasc Interv 3:97–104

    Article  PubMed  Google Scholar 

  18. Alam M, Dokainish H, Lakkis NM (2009) Hypertrophic obstructive cardiomyopathy-alcohol septal ablation vs. myectomy: a meta-analysis. Eur Heart J 30:1080–1087

    Article  PubMed  Google Scholar 

  19. Olivotto I, Ommen SR, Maron MS et al (2007) Surgical myectomy versus alcohol septal ablation for obstructive hypertrophic cardiomyopathy: will there ever be a randomized trial? J Am Coll Cardiol 50:831–834

    Article  PubMed  Google Scholar 

  20. Nishimura RA, Ommen SR (2010) Septal reduction therapy for obstructive hypertrophic cardiomyopathy and sudden death: what statistics cannot tell you. Circ Cardiovasc Interv 3:91–93

    Article  PubMed  Google Scholar 

  21. Seggewiss H, Rigopoulos A (2003) Management of hypertrophic cardiomyopathy in children. Paediatr Drugs 5:663–672

    Article  PubMed  Google Scholar 

  22. Lawin D, Lawrenz T, Radke K, Stellbrink C (2022) Safety and efficacy of alcohol septal ablation in adolescents and young adults with hypertrophic obstructive cardiomyopathy. Clin Res Cardiol 111:207–217

    Article  CAS  PubMed  Google Scholar 

  23. El Masry H, Breall JA (2008) Alcohol septal ablation for hypertrophic obstructive cardiomyopathy. Curr Cardiol Rev 4:193–197

    Article  PubMed  PubMed Central  Google Scholar 

  24. Marino B, Digilio MC, Toscano A et al (1999) Congenital heart disease in children with Noonan syndrome: an expanded cardiac spectrum with high prevalence of atrioventricular canal. J Pediatr 135:703–706

    Article  CAS  PubMed  Google Scholar 

  25. Gelb BD, Roberts AE, Tartaglia M (2015) Cardiomyopathies in Noonan syndrome and the other RASopathies. Prog Pediatr Cardiol 39:13–19

    Article  PubMed  PubMed Central  Google Scholar 

  26. Fifer MA (2018) Septal reduction therapy for hypertrophic obstructive cardiomyopathy. J Am Coll Cardiol 72:3095–3097

    Article  PubMed  Google Scholar 

  27. Liebregts M, Faber L, Jensen MK et al (2017) Outcomes of alcohol septal ablation in younger patients with obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol Intv 10:1134–1143

    Article  Google Scholar 

  28. Cano MN, Fortunato de Cano SJ, Sousa JEMR (2010) Alcohol septal ablation after myomectomy failure—solutions for unusual cases. Catheter Cardiovasc Interv 76:719–723

    Article  PubMed  Google Scholar 

  29. Kimmelsteil C (2010) Alcohol septal ablation in young children: addressing large gradients in little people. Catheter Cardiovasc Interv 76:724–725

    Article  Google Scholar 

  30. Emmel M, Sreeram N, de Giovanni JV et al (2005) Radiofrequency catheter septal ablation for hypertrophic obstructive cardiomyopathy in childhood. Z Kardiol 94:699–703

    Article  CAS  PubMed  Google Scholar 

  31. Teo EP, Teoh JG, Hung J (2015) Mitral valve and papillary muscle abnormalities in hypertrophic obstructive cardiomyopathy. Curr Opin Cardiol 30:475–482

    Article  PubMed  Google Scholar 

  32. Carvalho JL, Schaff HV, Morris CS et al (2022) Anomalous papillary muscles-Implications in the surgical treatment of hypertrophic obstructive cardiomyopathy. J Thorac Cardiovasc Surg 163:83-89.e1

    Article  Google Scholar 

  33. Marinakis S, Van der Linden P, Tortora R et al (2016) Outcomes from cardiac surgery in Jehovah’s witness patients: experience over twenty-one years. J Cardiothorac Surg 11:67

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Acknowledgements

Dr Thejaswi Ram MD DNB, Ms Monica Rajendran BS for assistance in acquiring patient data.

Author information

Authors and Affiliations

Authors

Contributions

KS and GJ wrote the main manuscript, prepared figures 1–, prepared tables 1, 2 and reviewed the manuscript.

Corresponding author

Correspondence to Kothandam Sivakumar.

Ethics declarations

Conflict of interest

The authors have no relevant financial or non-financial interests to disclose.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary Information

Below is the link to the electronic supplementary material.

Supplementary file1 (MOV 4846 KB)

Supplementary file2 (MOV 8329 KB)

Supplementary file3 (MOV 4186 KB)

Supplementary file4 (MOV 5301 KB)

Supplementary file5 (MOV 1611 KB)

Supplementary file6 (MOV 3887 KB)

Supplementary file7 (MOV 3342 KB)

Supplementary file8 (MOV 2520 KB)

Supplementary file9 (MOV 665 KB)

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Sivakumar, K., Jain, G. Is There a Role for Alcohol Septal Ablation in Young Patients with Medically Refractory Hypertrophic Obstructive Cardiomyopathy?. Pediatr Cardiol 45, 648–659 (2024). https://doi.org/10.1007/s00246-023-03145-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-023-03145-6

Keywords

Navigation