Annals of Cardiac Anaesthesia Annals of Cardiac Anaesthesia Annals of Cardiac Anaesthesia
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Table of Contents
Year : 2017  |  Volume : 20  |  Issue : 4  |  Page : 485
Hypertrophic obstructive cardiomyopathy, yamaguchi syndrome and kounis syndrome: Clinical challenges

Department of Cardiology, University of Patras Medical School, Rion, Patras, Achaia, Greece

Click here for correspondence address and email

Date of Web Publication9-Oct-2017

How to cite this article:
Kounis NG, Koniari I, Soufras G, Patsouras N, Hahalis G. Hypertrophic obstructive cardiomyopathy, yamaguchi syndrome and kounis syndrome: Clinical challenges. Ann Card Anaesth 2017;20:485

How to cite this URL:
Kounis NG, Koniari I, Soufras G, Patsouras N, Hahalis G. Hypertrophic obstructive cardiomyopathy, yamaguchi syndrome and kounis syndrome: Clinical challenges. Ann Card Anaesth [serial online] 2017 [cited 2022 Jan 20];20:485. Available from:

The Editor,

In the interesting report concerning the case of an apical variant of hypertrophic cardiomyopathy also called as Yamaguchi syndrome published in Annals of Cardiac Anesthesia,[1] the authors stated that anesthetic management of these patients is similar to case of hypertrophic obstructive cardiomyopathy. Therefore, alleviating sympathetic stimulation such as avoiding tachycardia, maintaining normal sinus rhythm and euvolemia, and avoiding any increase in cardiac contractility is helpful. However, serious challenges concerning anaphylaxis, labor, anesthesia, and surgery should be always considered in anesthesia and surgery.

Anaphylaxis creates increase heart rate, increase myocardial contractility, decrease preload, and decrease afterload due fluid extravasation that exacerbates left ventricular outflow obstruction. Hypertrophic cardiomyopathy is correlated genetically with atopic diathesis, whereas involved genes predispose to eosinophilic esophagitis manifesting with chest pain as a primary symptom.[2] Patients with obstructive cardiomyopathy may demonstrate hypersensitivity myocarditis toward the administered drugs, a life-threatening but also a potentially treatable complication.[3]

Anaphylaxis can induce coronary spasm, the so-called type I of Kounis hypersensitivity-associated acute coronary syndrome that aggravates preexisting myocardial ischemia. Hypertrophic cardiomyopathy coexistence with coronary spasm plays a significant role in myocardial ischemia induction, a commonly encountered feature of the disease. Especially, coronary vasospasm was induced in 10 out of 36 (28%) hypertrophic cardiomyopathy patients who had undergone challenging with acetylcholine provocation test.[4]

In anesthesia, β-adrenergic agents, dopamine, dobutamine, epinephrine, and isoproterenol, contribute to obstruction worsening due to their positive inotropic and chronotropic effects, while α1-adrenergic agonist, phenylephrine, reduces obstruction through systemic vascular resistance reduction.

Major blood loss during labor could further aggravate obstruction by preload decrease and reflex tachycardia provocation. While oxytocin reduces the systemic vascular resistance (SVR) and increases the heart rate, ergometrine increases the SVR and appears to have beneficial impact on these patients.[5]

Therefore, anesthetists, surgeons, and physicians in general should be aware that patients with hypertrophic cardiomyopathy pose unique challenges. Adequate preload, control of sympathetic stimulation, control heart rate, and increased afterload are of paramount importance.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Sindwani G, Sahu S, Singh UP, Khanna R. Anesthetic management of a patient with Yamaguchi syndrome for laparoscopic radical nephroureterectomy. Ann Card Anaesth 2017;20:119-20.  Back to cited text no. 1
[PUBMED]  [Full text]  
Davis BP, Epstein T, Kottyan L, Amin P, Martin LJ, Maddox A, et al. Association of eosinophilic esophagitis and hypertrophic cardiomyopathy. J Allergy Clin Immunol 2016;137:934-6.e5.  Back to cited text no. 2
Frustaci A, Verardo R, Sale P, Toscano F, Critelli G, Russo MA, et al. Hypersensitivity myocarditis induced by beta-blockers: An unexpected cause of abrupt deterioration in hypertrophic cardiomyopathy. Intensive Care Med 2007;33:1848-9.  Back to cited text no. 3
Gajewski M, Hillel Z. Anesthesia management of patients with hypertrophic obstructive cardiomyopathy. Prog Cardiovasc Dis 2012;54:503-11.  Back to cited text no. 4
Stergiopoulos K, Shiang E, Bench T. Pregnancy in patients with pre-existing cardiomyopathies. J Am Coll Cardiol 2011;58:337-50.  Back to cited text no. 5

Correspondence Address:
Nicholas G Kounis
Queen Olgas Square, 7 Aratou Street, Patras 26221, Achaia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/aca.ACA_94_17

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