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Archives of cardiovascular diseases
Volume 109, n° 3
pages 223-225 (mars 2016)
Doi : 10.1016/j.acvd.2015.11.005
Received : 20 November 2015 ;  accepted : 21 November 2015
Midodrine hydrochloride and unexpected improvement in hypertrophic cardiomyopathy symptoms
Amélioration inattendue des symptômes de la cardiomyopathie hypertrophique sous midodrine
 

Stephane Lafitte , Jérôme Peyrou, Amelie Reynaud, Marina Dijos, Claire Cornolle, Raymond Roudaut, Cecile Touche, Philippe Ritter, Patricia Réant
 Université de Bordeaux, CHU de Bordeaux, CIC 0005, 33076 Bordeaux cedex, France 

Corresponding author. Hôpital cardiologique Haut-Lévêque, avenue de Magellan, 33600 Pessac, France.

Keywords : Hypertrophic cardiomyopathy, Medical treatment, Midodrine, Dyspnea

Mots clés : Cardiomyopathie hypertrophique, Traitement médical, Midodrine, Dyspnée


Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy (1 per 200 with an inherited cardiomyopathy) [1]. Half of all patients with HCM present with symptoms such as dyspnea on exertion, chest pain or dizziness, irrespective of the degree of left ventricular hypertrophy or obstruction [1]. These functional limitations can seriously affect the patient's quality of life, both professional and private. The primary medical treatments proposed are beta-blockers or calcium antagonists, which have varying levels of efficacy and substantial potential side-effects [2]. We report the first series, of six patients, exhibiting impressive improvement following administration of midodrine hydrochloride.

The first patient, aged 45 years with a history of obstructive HCM, was admitted in April 2015 for orthostatic hypotension, confirmed by a positive tilt-table test. He also complained of significant discomfort when walking (New York Heart Association [NYHA] class II). Treatment did not prevent several lipothymic and syncopal episodes (Table 1). Although the echocardiography work-up revealed no left ventricular outflow tract obstruction (LVOTO) at rest, the maximal gradient during exercise was recorded at 50mmHg, reaching 100mmHg in the early recovery phase. Considering the patient's orthostatic hypotension, midodrine hydrochloride treatment was proposed. This drug produces a direct and selective sympathomimetic effect on peripheral alpha-adrenergic receptors, resulting in vasoconstriction of first the veins and then the arteries. After 1 month of treatment, consultation records reported no recurrence of faintness, less shortness of breath during exercise (NYHA class I), and no LVOTO.

The second patient, a 67-year-old woman presenting with familial HCM, was admitted for disabling chest pain during exercise (Table 1). She also exhibited an NYHA class III dyspnea (walking distance of 10m). A leg-raise test performed during echocardiography revealed an immediate reduction in the exercise obstruction, from 145mmHg to 80mmHg. Considering the improvement achieved by administering midodrine hydrochloride to the first patient, the same treatment was applied. Within 48hours, the patient became asymptomatic. Echocardiographic follow-up examination revealed a significantly reduced maximum LVOTO gradient, falling to 50mmHg at rest and to 95mmHg during exercise.

Consecutively, four other patients were proposed for testing this medication, all of which resulted in similar results, along with excellent tolerance (Table 1).

We believe that this vasoactive treatment improved left ventricular filling in these patients, as well as decreasing LVOTO by increasing left ventricular volume, thus demonstrating the clinical pertinence of venous return and preload conditions [3]. A prospective ongoing double-blind study versus placebo has been designed to confirm these preliminary results. If they prove positive, this approach could significantly improve the quality of life for thousands of patients with HCM.

Disclosure of interest

The authors declare that they have no competing interest.

References

Semsarian C., Ingles J., Maron M.S., Maron B.J. New perspectives on the prevalence of hypertrophic cardiomyopathy J Am Coll Cardiol 2015 ;  65 : 1249-1254 [cross-ref]
Maron B.J., Rowin E.J., Casey S.A., Link M.S., Lesser J.R., Chan R.H., and al. Hypertrophic cardiomyopathy in adulthood associated with low cardiovascular mortality with contemporary management strategies J Am Coll Cardiol 2015 ;  65 : 1915-1928 [cross-ref]
Lafitte S., Reant P., Touche C., Pillois X., Dijos M., Arsac F., and al. Paradoxical response to exercise in asymptomatic hypertrophic cardiomyopathy: a new description of outflow tract obstruction dynamics J Am Coll Cardiol 2013 ;  62 : 842-850 [cross-ref]



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