Acute Cardiac Care, December 2014; 16(4): 115–117 Copyright © 2014 Informa UK, Ltd ISSN 1748-2941 print/ISSN 1748-295X online DOI: 10.3109/17482941.2014.944538
ORIGINAL ARTICLE
Recurrent takotsubo syndrome in a patient with myotonic dystrophy 1 Josef Finsterer1, Claudia Stöllberger2, Dita Demirtas3, Martin Gencik4, Irene Ohnutek5 & Anette Hornykewycz6 Rudolfstiftung, Vienna, Austria, 22nd Medical Department with Cardiology and Intensive Care Medicine Krankenanstalt Rudolfstiftung, Vienna, Austria, 3Central Laboratory, Krankenanstalt Rudolfstiftung, Vienna, Austria, 4Human Genetic Laboratory, Private office, Vienna, Austria, 5Department of Internal Medicine, Otto-Wagner Spital, Vienna, Austria, 6Abteilung für Anästhesie, Intensiv- und Schmerzmedizin, Wilhelminenspital, Vienna, Austria Acute Card Care Downloaded from informahealthcare.com by Selcuk Universitesi on 01/26/15 For personal use only.
1Krankenanstalt
pain, ECG abnormalities imitating myocardial infarction, normal coronary angiography, typical akinesia or hypokinesia of the apex and the left midventricular myocardium, severely reduced systolic function and complete regression of ECG and echocardiographic abnormalities within some weeks after onset (1). Recurrence of TTS is extremely rare but has been occasionally reported (2–7). TTS and its recurrence have not been reported in myotonic dystrophy-1 (MD1).
Objectives: Stress-induced cardiomyopathy (takotsubosyndrome, TTS) and its recurrence have not been described in myotonic dystrophy-1. Case report: The patient was a 47-year-old female who was suspected to suffer from myotonic dystrophy-1 at 20 years of age, upon the typical clinical presentation and the electrophysiological findings. During weaning from general anesthesia for resectioning of a pelvic tumour she developed ventricular fibrillation, but was successfully resuscitated. During coronary angiography two days later she experienced recurrent QT-prolongation, torsades de pointes, and ventricular fibrillation, but was successfully resuscitated again each time. Echocardiography and electrocardiography were indicative of TTS, which was confirmed by normal findings on echocardiography and electrocardiography two months later. Ten months after the first TTS she developed dyspnea, leg edema, and anginal chest pain. Recurrence of TTS was diagnosed upon a typical electrocardiography and echocardiography findings. Shortly after onset of the second TTS, she unexpectedly died from sepsis. Conclusions: TTS may also occur in patients with myotonic dystrophy-1 induced by stress from surgery, respiratory insufficiency, or infection. In patients with myotonic dystrophy-1, takotsubo-syndrome may recur and may represent a previously unreported feature of cardiac involvement in myotonic dystrophy-1.
Case report The patient was a 47 years-old, Caucasian female, height 157 cm, weight 55 kg, who was suspected to suffer from myotonic dystrophy-1 at 20 years of age. Already her deceased mother and sister were suspected to have had MD1 without genetic confirmation. At onset the proband presented with ptosis and later developed clinical myotonia and weakness of the distal upper and lower limbs. In March 2012 she was able use her hands, to walk independently and to take care of herself. In August 2012 clinical neurologic examination revealed a myopathic face, mild bilateral ptosis, right-sided weakness of the tongue and weak anteflexion (M3), quadruparesis (M3) with left-sided predominance on the upper limbs and proximal predominance on the lower limbs, general wasting, clinical myotonic reaction on the upper limbs, and generally reduced tendon reflexes. The neurological diagnosis was established genetically post-mortem in a stored ethylene-diamine-tetra-acetate blood sample. Work-up of the sample by DNA isolation, PCR, and Southern blot after EcoR1 and BglII digestion revealed a heterocygous CTG-repeat expansion of 1200–1900 repeats in the non-translating 3ʹ-end of the DMPK gene on chromosome 19q13.3. The remaining history was noteworthy for resection of a macro-follicular kolloidal struma in 2006, and deep venous thrombosis with consecutive bilateral pulmonary embolism after a fall in February 2012, initially treated with heparin and followed by dabigatran (150 mg bid) for secondary
Keywords: Apical ballooning, stress cardiomyopathy, neuromuscular
disorder, cardiac involvement, cardiopulmonary resuscitation, trinucleotide expansion
Introduction Takotsubo syndrome (TTS, apical ballooning, stress cardiomyopathy, transient left ventricular dysfunction, broken heart syndrome) is a rare, unclassified cardiomyopathy induced by stress (1). TTS is characterized by anginal chest
Correspondence: J. Finsterer, Postfach 20, 1180 Vienna, Austria. Fax: ⫹ 43 1 4781711. E-mail: fifi[email protected] (Received 3 March 2014; accepted 10 July 2014)
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prophylaxis. Diagnostic work-up in February 2012 additionally revealed bilateral hydronephrosis being caused by a gross gynecological tumor compressing the ureters and the inferior caval vein. Echocardiography showed an expanded pulmonary trunk exclusively. In March 2012 Wertheim surgery was performed to remove a leiomyoma of the uterus. During surgery, thrombosis of the iliac veins was recognized, requiring ligature of the left iliac vein and thrombectomy of the right iliac vein. During weaning from general anesthesia two days after surgery she experienced four cardiac arrests due to ventricular fibrillation but was successfully resuscitated each time. Transthoracic echocardiography revealed reduced systolic function (ejection fraction: 20%), and hypokinesia of the apex and the anterior wall. Coronary angiography was normal. Periprocedurally, she experienced QT-prolongation (up to 690 ms) and recurrent torsades de pointes, which resolved upon defibrillation each tine. Transthoracic echocardiography revealed highly reduced systolic function and dyskinesia of the apex, septum, anterior wall, and partially also of the posterior wall. On subsequent transesophageal echocardiography, TTS was suspected (Figure 1). Amiodarone and levofloxcine were discontinued because of their QT-prolonging effect and esmolol initiated. Under this regimen, cardiac dysfunction progressively resolved within five weeks. Seventeen days after surgery she underwent tracheostomy because weaning from the respirator was assessed as improbable. Follow-up echocardiography in May 2012 was normal. In May 2012 a Porth-a-Cath system was implanted because prolonged intravenous antibiotic therapy was necessary. During rehabilitation she received a percutaneous endoscopic gastrostomy (PEG), because of poor feeding, which was removed in August 2012. In August 2012 the tracheostomy was successfully closed. In January 2013 she developed dyspnea, leg edema, and anginal chest pain. ECG showed sinustachycardia, left bundle-branch block, QT-prolongation (580 respectively 480 ms), and ST-elevation. Echocardiography revealed severely reduced systolic function, severe hypokinesia of the
Figure 2. Transthoracic echocardiographic apical 4-chamber end diastolic view from January 2013 showing typical ballooning of the left ventricle due to hypokinesia/akinesia of the apical and midventricular myocardium during the second TTS of the described patient.
apex and the mid-ventricular segments, and pulmonary hypertension. ProBNP was 3871 ng/l (n, 0–153 ng/l) (Figure 2). Because of respiratory insufficiency she was transferred to the intensive care unit. She received noninvasive positive pressure ventilation and levosimendan. Pulmonary infection was treated with meropenem. Since blood cultures were positive for candida albicans, the Porth-a-Cath system was removed and fluconazole administered. Additionally, dabigatran (150 mg bid) was replaced by rivaroxaban because dabigatran is not approved for pulmonary embolism. To find out if an implantable cardioverter defibrillator was indicated, she was transferred to a cardiology department. One day later she experienced fever, developed tachycardia, systolic dysfunction, and was transferred to the intensive care unit where she died from sepsis and respiratory insufficiency the same day. Autopsy revealed purulent pericarditis and bilateral purulent pneumonia. Cultures of the pus were negative.
Discussion
Figure 1. Transesophageal echocardiography four-chamber view in March 2012 showing hypercontractility of the left ventricular basal region and ballooning of the apical parts.
The presented patient is interesting for TTS in MD1, for recurrence of TTS 10 months after the first TTS, for surgery as the trigger of the first TTS and muscular respiratory insufficiency, or infection as the triggers of the second TTS. Cardiac involvement (CI) in MD1 is a frequent finding (8). The most common manifestation of CI in MD1 is AVblock 1 (9). With progression of the disease, however, severity of CI increases as well, resulting in the occurrence of more severe arrhythmias, including atrial fibrillation, bundlebranch block, QT-prolongation, ventricular arrhythmias, or sudden cardiac death (SCD) (10). Additionally, some patients develop cardiomyopathy, manifesting as hypertrophic cardiomyopathy (11), dilative cardiomyopathy (12), or left ventricular hypertrabeculation/noncompaction (13). CI in MD1 Acute Cardiac Care
Acute Card Care Downloaded from informahealthcare.com by Selcuk Universitesi on 01/26/15 For personal use only.
Recurrent taktosubo syndrome in myotonic dystrophy-1 is managed according to general guidelines and is a strong prognostic factor. Since a number of MD1 patients experiences SCD from severe rhythm abnormalities, these patients require regular 24 h ECG recordings, or implantation of a loop recorder to improve their outcome (14). CI may increase in severity between the generations because of anticipation. TTS is a stress-triggered unclassified cardiomyopathy characterized by reversible apical or mid-segmental left ventricular dysfunction due to enhanced central or focal sympathetic stimulation, triggered by emotional or physical stress (15,16). TTS mimics acute myocardial infarction presenting with precordial pain and transient ST-elevation, followed by negative T-waves and normalization of the ECG within several weeks (15). Any form of physical or psychological stress may trigger TTS. Possible triggers of the first TTS in the presented patient include stress from general anesthesia or stress from surgery (17). Medication which potentially triggered ventricular fibrillation leading to TTS includes amiodarone and levofloxacine (18). Possible triggers of the second TTS include sepsis or muscular respiratory failure, or the combination of both (19). The presented patient also experienced muscular respiratory insufficiency from her muscle disease in addition to heart failure from CI in MD1. Arguments for such a scenario are that weaning from the respirator after surgery for the pelvic tumor was markedly prolonged, that the tracheostomy could be removed only weeks after application, and that coughing was severely impaired shortly before her decease. Whether dabigatran given during 10 months was involved in the pathogenesis of TTS or the infection lastly leading to death, remains speculative. No reports about TTS in a patient under dabigatran have been published. There are indications, however, that dabigatran blocks the agglutination of Staphylococcus aureus with fibrin (20) and that the prevalence of wound healing problems increases under thrombin antagonists (21). Infections may be further promoted by an immune deficiency frequently found in MD1 patients, as expressed by low serum IgG levels, which correlate with the CTG-repeat expansion size (22). This case shows that TTS may also occur in patients with MD1 and may be triggered by stress from surgery, respiratory insufficiency due to involvement of the respiratory muscles in MD1, or infection. In patients with MD1, TTS may recur and may represent a previously unreported feature of CI in MD1. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References 1. Neil CJ, Nguyen TH, Sverdlov AL, Chirkov YY, Chong CR, Stansborough J, et al. Can we make sense of takotsubo cardiomyopathy? An update on pathogenesis, diagnosis and natural history. Expert Rev Cardiovasc Ther. 2012;10:215–21. 2. Bridgman PG, Chan CW, Elliott JM. A case of recurrent earthquake stress cardiomyopathy with a differing wall motion abnormality. Echocardiography 2012;29:E26–7.
© 2014 Informa UK, Ltd.
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3. Gach O, Lempereur M, Pierard LA, Lancellotti P. Recurrent stress cardiomyopathy with variable pattern of left ventricle contraction abnormality. J Am Coll Cardiol. 2012;60:e5. 4. Kaushik M, Alla VM, Madan R, Arouni AJ, Mohiuddin SM. Recurrent stress cardiomyopathy with variable regional involvement: insights into etiopathogenetic mechanisms. Circulation 2011;124:e556–7. 5. Kubiak G, Jacheć W, Kawecki D, Traczewska M, Irlik G, Nowalany-Kozielska E. Tako-tsubo cardiomyopathy as a recurrent disease with doubtful prognosis of recovery and heterogenic symptoms. Cardiol J. 2012;19:521–3. 6. Tait J, Cleveland D, Ashton T. Unusual reversed wall motion pattern in a patient with recurrent takotsubo cardiomyopathy. Can J Cardiol. 2013;29:1015.e13-4. 7. Zlotnick DM, Helisch A. Recurrent stress cardiomyopathy induced by Sudafed PE. Ann Intern Med. 2012;156:171–2. 8. Petri H, Vissing J, Witting N, Bundgaard H, Køber L. Cardiac manifestations of myotonic dystrophy type 1. Int J Cardiol. 2012;160:82–8. 9. Nazarian S, Wagner KR, Caffo BS, Tomaselli GF. Clinical predictors of conduction disease progression in type I myotonic muscular dystrophy. Pacing Clin Electrophysiol. 2011;34:171–6. 10. Facenda-Lorenzo M, Hernández-Afonso J, Rodríguez-Esteban M, de León-Hernández JC, Grillo-Pérez JJ. Cardiac manifestations in myotonic dystrophy type 1 patients followed using a standard protocol in a specialized unit. Rev Esp Cardiol. 2013;66:193–7. 11. Nakada T, Yonesaka S. ‘Hypertrophic cardiomyopathy’ in a girl with adult-type myotonic dystrophy. Pediatr Int. 1999;41:586–8. 12. Gunkel O, Reichenbach H, Thamm B, Wetzel U, Bratanow S, Kirchhof M, et al. Late diagnosis of Curschmann-Steinert myotonic dystrophy in a female patient with dilated cardiomyopathy and in her son. Z Kardiol. 2000;89:599–605. 13. Finsterer J, Stölberger C, Kopsa W. Noncompaction in myotonic dystrophy type 1 on cardiac MRI. Cardiology 2005;103:167–8. 14. Finsterer J, Stöllberger C, Avanzini M, Prager E, Weidinger F. Asymptomatic, nonsustained ventricular tachycardia in myotonic dystrophy type 1 detected with a loop recorder. Eur Neurol. 2008;60:109–11. 15. Porto I, Della Bona R, Leo A, Proietti R, Pieroni M, Caltagirone C, et al. Stress cardiomyopathy (tako-tsubo) triggered by nervous system diseases: A systematic review of the reported cases. Int J Cardiol 2013;167:2441–8. 16. Tsuchihashi K, Ueshima K, Uchida T, Oh-mura N, Kimura K, Owa M, et al. Transient left ventricular apical ballooning without coronary artery stenosis: a novel heart syndrome mimicking acute myocardial infarction. Angina pectoris – myocardial Infarction Investigations in Japan. J Am Coll Cardiol. 2001;38:11–8. 17. Bortnik M, Verdoia M, Schaffer A, Occhetta E, Marino P. Ventricular fibrillation as primary presentation of takotsubo cardiomyopathy after complicated cesarean delivery. World J Cardiol. 2012;4:214–7. 18. Amariles P. A comprehensive literature search: Drugs as possible triggers of Takotsubo cardiomyopathy. Curr Clin Pharmacol. 2011;6:1–11. 19. Osuorji I, Williams C, Hessney J, Patel T, Hsi D. Acute stress cardiomyopathy following treatment of status asthmaticus. South Med J. 2009;102:301–3. 20. McAdow M, Kim HK, Dedent AC, Hendrickx AP, Schneewind O, Missiakas DM. Preventing Staphylococcus aureus sepsis through the inhibition of its agglutination in blood. PLoS Pathog. 2011;7:e1002307. doi:10.1371/journal.ppat.1002307. 21. Gill SK, Theodorides A, Smith N, Maguire E, Whitehouse SL, Rigby MC, et al. Wound problems following hip arthroplasty before and after the introduction of a direct thrombin inhibitor for thromboprophylaxis. Hip Int. 2011;21:678–83. 22. Kaminsky P, Lesesve JF, Jonveaux P, Pruna L. IgG deficiency and expansion of CTG repeats in myotonic dystrophy. Clin Neurol Neurosurg. 2011;113:464–8.
ORIGINAL ARTICLE
Recurrent takotsubo syndrome in a patient with myotonic dystrophy 1 Josef Finsterer1, Claudia Stöllberger2, Dita Demirtas3, Martin Gencik4, Irene Ohnutek5 & Anette Hornykewycz6 Rudolfstiftung, Vienna, Austria, 22nd Medical Department with Cardiology and Intensive Care Medicine Krankenanstalt Rudolfstiftung, Vienna, Austria, 3Central Laboratory, Krankenanstalt Rudolfstiftung, Vienna, Austria, 4Human Genetic Laboratory, Private office, Vienna, Austria, 5Department of Internal Medicine, Otto-Wagner Spital, Vienna, Austria, 6Abteilung für Anästhesie, Intensiv- und Schmerzmedizin, Wilhelminenspital, Vienna, Austria Acute Card Care Downloaded from informahealthcare.com by Selcuk Universitesi on 01/26/15 For personal use only.
1Krankenanstalt
pain, ECG abnormalities imitating myocardial infarction, normal coronary angiography, typical akinesia or hypokinesia of the apex and the left midventricular myocardium, severely reduced systolic function and complete regression of ECG and echocardiographic abnormalities within some weeks after onset (1). Recurrence of TTS is extremely rare but has been occasionally reported (2–7). TTS and its recurrence have not been reported in myotonic dystrophy-1 (MD1).
Objectives: Stress-induced cardiomyopathy (takotsubosyndrome, TTS) and its recurrence have not been described in myotonic dystrophy-1. Case report: The patient was a 47-year-old female who was suspected to suffer from myotonic dystrophy-1 at 20 years of age, upon the typical clinical presentation and the electrophysiological findings. During weaning from general anesthesia for resectioning of a pelvic tumour she developed ventricular fibrillation, but was successfully resuscitated. During coronary angiography two days later she experienced recurrent QT-prolongation, torsades de pointes, and ventricular fibrillation, but was successfully resuscitated again each time. Echocardiography and electrocardiography were indicative of TTS, which was confirmed by normal findings on echocardiography and electrocardiography two months later. Ten months after the first TTS she developed dyspnea, leg edema, and anginal chest pain. Recurrence of TTS was diagnosed upon a typical electrocardiography and echocardiography findings. Shortly after onset of the second TTS, she unexpectedly died from sepsis. Conclusions: TTS may also occur in patients with myotonic dystrophy-1 induced by stress from surgery, respiratory insufficiency, or infection. In patients with myotonic dystrophy-1, takotsubo-syndrome may recur and may represent a previously unreported feature of cardiac involvement in myotonic dystrophy-1.
Case report The patient was a 47 years-old, Caucasian female, height 157 cm, weight 55 kg, who was suspected to suffer from myotonic dystrophy-1 at 20 years of age. Already her deceased mother and sister were suspected to have had MD1 without genetic confirmation. At onset the proband presented with ptosis and later developed clinical myotonia and weakness of the distal upper and lower limbs. In March 2012 she was able use her hands, to walk independently and to take care of herself. In August 2012 clinical neurologic examination revealed a myopathic face, mild bilateral ptosis, right-sided weakness of the tongue and weak anteflexion (M3), quadruparesis (M3) with left-sided predominance on the upper limbs and proximal predominance on the lower limbs, general wasting, clinical myotonic reaction on the upper limbs, and generally reduced tendon reflexes. The neurological diagnosis was established genetically post-mortem in a stored ethylene-diamine-tetra-acetate blood sample. Work-up of the sample by DNA isolation, PCR, and Southern blot after EcoR1 and BglII digestion revealed a heterocygous CTG-repeat expansion of 1200–1900 repeats in the non-translating 3ʹ-end of the DMPK gene on chromosome 19q13.3. The remaining history was noteworthy for resection of a macro-follicular kolloidal struma in 2006, and deep venous thrombosis with consecutive bilateral pulmonary embolism after a fall in February 2012, initially treated with heparin and followed by dabigatran (150 mg bid) for secondary
Keywords: Apical ballooning, stress cardiomyopathy, neuromuscular
disorder, cardiac involvement, cardiopulmonary resuscitation, trinucleotide expansion
Introduction Takotsubo syndrome (TTS, apical ballooning, stress cardiomyopathy, transient left ventricular dysfunction, broken heart syndrome) is a rare, unclassified cardiomyopathy induced by stress (1). TTS is characterized by anginal chest
Correspondence: J. Finsterer, Postfach 20, 1180 Vienna, Austria. Fax: ⫹ 43 1 4781711. E-mail: fifi[email protected] (Received 3 March 2014; accepted 10 July 2014)
115
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prophylaxis. Diagnostic work-up in February 2012 additionally revealed bilateral hydronephrosis being caused by a gross gynecological tumor compressing the ureters and the inferior caval vein. Echocardiography showed an expanded pulmonary trunk exclusively. In March 2012 Wertheim surgery was performed to remove a leiomyoma of the uterus. During surgery, thrombosis of the iliac veins was recognized, requiring ligature of the left iliac vein and thrombectomy of the right iliac vein. During weaning from general anesthesia two days after surgery she experienced four cardiac arrests due to ventricular fibrillation but was successfully resuscitated each time. Transthoracic echocardiography revealed reduced systolic function (ejection fraction: 20%), and hypokinesia of the apex and the anterior wall. Coronary angiography was normal. Periprocedurally, she experienced QT-prolongation (up to 690 ms) and recurrent torsades de pointes, which resolved upon defibrillation each tine. Transthoracic echocardiography revealed highly reduced systolic function and dyskinesia of the apex, septum, anterior wall, and partially also of the posterior wall. On subsequent transesophageal echocardiography, TTS was suspected (Figure 1). Amiodarone and levofloxcine were discontinued because of their QT-prolonging effect and esmolol initiated. Under this regimen, cardiac dysfunction progressively resolved within five weeks. Seventeen days after surgery she underwent tracheostomy because weaning from the respirator was assessed as improbable. Follow-up echocardiography in May 2012 was normal. In May 2012 a Porth-a-Cath system was implanted because prolonged intravenous antibiotic therapy was necessary. During rehabilitation she received a percutaneous endoscopic gastrostomy (PEG), because of poor feeding, which was removed in August 2012. In August 2012 the tracheostomy was successfully closed. In January 2013 she developed dyspnea, leg edema, and anginal chest pain. ECG showed sinustachycardia, left bundle-branch block, QT-prolongation (580 respectively 480 ms), and ST-elevation. Echocardiography revealed severely reduced systolic function, severe hypokinesia of the
Figure 2. Transthoracic echocardiographic apical 4-chamber end diastolic view from January 2013 showing typical ballooning of the left ventricle due to hypokinesia/akinesia of the apical and midventricular myocardium during the second TTS of the described patient.
apex and the mid-ventricular segments, and pulmonary hypertension. ProBNP was 3871 ng/l (n, 0–153 ng/l) (Figure 2). Because of respiratory insufficiency she was transferred to the intensive care unit. She received noninvasive positive pressure ventilation and levosimendan. Pulmonary infection was treated with meropenem. Since blood cultures were positive for candida albicans, the Porth-a-Cath system was removed and fluconazole administered. Additionally, dabigatran (150 mg bid) was replaced by rivaroxaban because dabigatran is not approved for pulmonary embolism. To find out if an implantable cardioverter defibrillator was indicated, she was transferred to a cardiology department. One day later she experienced fever, developed tachycardia, systolic dysfunction, and was transferred to the intensive care unit where she died from sepsis and respiratory insufficiency the same day. Autopsy revealed purulent pericarditis and bilateral purulent pneumonia. Cultures of the pus were negative.
Discussion
Figure 1. Transesophageal echocardiography four-chamber view in March 2012 showing hypercontractility of the left ventricular basal region and ballooning of the apical parts.
The presented patient is interesting for TTS in MD1, for recurrence of TTS 10 months after the first TTS, for surgery as the trigger of the first TTS and muscular respiratory insufficiency, or infection as the triggers of the second TTS. Cardiac involvement (CI) in MD1 is a frequent finding (8). The most common manifestation of CI in MD1 is AVblock 1 (9). With progression of the disease, however, severity of CI increases as well, resulting in the occurrence of more severe arrhythmias, including atrial fibrillation, bundlebranch block, QT-prolongation, ventricular arrhythmias, or sudden cardiac death (SCD) (10). Additionally, some patients develop cardiomyopathy, manifesting as hypertrophic cardiomyopathy (11), dilative cardiomyopathy (12), or left ventricular hypertrabeculation/noncompaction (13). CI in MD1 Acute Cardiac Care
Acute Card Care Downloaded from informahealthcare.com by Selcuk Universitesi on 01/26/15 For personal use only.
Recurrent taktosubo syndrome in myotonic dystrophy-1 is managed according to general guidelines and is a strong prognostic factor. Since a number of MD1 patients experiences SCD from severe rhythm abnormalities, these patients require regular 24 h ECG recordings, or implantation of a loop recorder to improve their outcome (14). CI may increase in severity between the generations because of anticipation. TTS is a stress-triggered unclassified cardiomyopathy characterized by reversible apical or mid-segmental left ventricular dysfunction due to enhanced central or focal sympathetic stimulation, triggered by emotional or physical stress (15,16). TTS mimics acute myocardial infarction presenting with precordial pain and transient ST-elevation, followed by negative T-waves and normalization of the ECG within several weeks (15). Any form of physical or psychological stress may trigger TTS. Possible triggers of the first TTS in the presented patient include stress from general anesthesia or stress from surgery (17). Medication which potentially triggered ventricular fibrillation leading to TTS includes amiodarone and levofloxacine (18). Possible triggers of the second TTS include sepsis or muscular respiratory failure, or the combination of both (19). The presented patient also experienced muscular respiratory insufficiency from her muscle disease in addition to heart failure from CI in MD1. Arguments for such a scenario are that weaning from the respirator after surgery for the pelvic tumor was markedly prolonged, that the tracheostomy could be removed only weeks after application, and that coughing was severely impaired shortly before her decease. Whether dabigatran given during 10 months was involved in the pathogenesis of TTS or the infection lastly leading to death, remains speculative. No reports about TTS in a patient under dabigatran have been published. There are indications, however, that dabigatran blocks the agglutination of Staphylococcus aureus with fibrin (20) and that the prevalence of wound healing problems increases under thrombin antagonists (21). Infections may be further promoted by an immune deficiency frequently found in MD1 patients, as expressed by low serum IgG levels, which correlate with the CTG-repeat expansion size (22). This case shows that TTS may also occur in patients with MD1 and may be triggered by stress from surgery, respiratory insufficiency due to involvement of the respiratory muscles in MD1, or infection. In patients with MD1, TTS may recur and may represent a previously unreported feature of CI in MD1. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References 1. Neil CJ, Nguyen TH, Sverdlov AL, Chirkov YY, Chong CR, Stansborough J, et al. Can we make sense of takotsubo cardiomyopathy? An update on pathogenesis, diagnosis and natural history. Expert Rev Cardiovasc Ther. 2012;10:215–21. 2. Bridgman PG, Chan CW, Elliott JM. A case of recurrent earthquake stress cardiomyopathy with a differing wall motion abnormality. Echocardiography 2012;29:E26–7.
© 2014 Informa UK, Ltd.
117
3. Gach O, Lempereur M, Pierard LA, Lancellotti P. Recurrent stress cardiomyopathy with variable pattern of left ventricle contraction abnormality. J Am Coll Cardiol. 2012;60:e5. 4. Kaushik M, Alla VM, Madan R, Arouni AJ, Mohiuddin SM. Recurrent stress cardiomyopathy with variable regional involvement: insights into etiopathogenetic mechanisms. Circulation 2011;124:e556–7. 5. Kubiak G, Jacheć W, Kawecki D, Traczewska M, Irlik G, Nowalany-Kozielska E. Tako-tsubo cardiomyopathy as a recurrent disease with doubtful prognosis of recovery and heterogenic symptoms. Cardiol J. 2012;19:521–3. 6. Tait J, Cleveland D, Ashton T. Unusual reversed wall motion pattern in a patient with recurrent takotsubo cardiomyopathy. Can J Cardiol. 2013;29:1015.e13-4. 7. Zlotnick DM, Helisch A. Recurrent stress cardiomyopathy induced by Sudafed PE. Ann Intern Med. 2012;156:171–2. 8. Petri H, Vissing J, Witting N, Bundgaard H, Køber L. Cardiac manifestations of myotonic dystrophy type 1. Int J Cardiol. 2012;160:82–8. 9. Nazarian S, Wagner KR, Caffo BS, Tomaselli GF. Clinical predictors of conduction disease progression in type I myotonic muscular dystrophy. Pacing Clin Electrophysiol. 2011;34:171–6. 10. Facenda-Lorenzo M, Hernández-Afonso J, Rodríguez-Esteban M, de León-Hernández JC, Grillo-Pérez JJ. Cardiac manifestations in myotonic dystrophy type 1 patients followed using a standard protocol in a specialized unit. Rev Esp Cardiol. 2013;66:193–7. 11. Nakada T, Yonesaka S. ‘Hypertrophic cardiomyopathy’ in a girl with adult-type myotonic dystrophy. Pediatr Int. 1999;41:586–8. 12. Gunkel O, Reichenbach H, Thamm B, Wetzel U, Bratanow S, Kirchhof M, et al. Late diagnosis of Curschmann-Steinert myotonic dystrophy in a female patient with dilated cardiomyopathy and in her son. Z Kardiol. 2000;89:599–605. 13. Finsterer J, Stölberger C, Kopsa W. Noncompaction in myotonic dystrophy type 1 on cardiac MRI. Cardiology 2005;103:167–8. 14. Finsterer J, Stöllberger C, Avanzini M, Prager E, Weidinger F. Asymptomatic, nonsustained ventricular tachycardia in myotonic dystrophy type 1 detected with a loop recorder. Eur Neurol. 2008;60:109–11. 15. Porto I, Della Bona R, Leo A, Proietti R, Pieroni M, Caltagirone C, et al. Stress cardiomyopathy (tako-tsubo) triggered by nervous system diseases: A systematic review of the reported cases. Int J Cardiol 2013;167:2441–8. 16. Tsuchihashi K, Ueshima K, Uchida T, Oh-mura N, Kimura K, Owa M, et al. Transient left ventricular apical ballooning without coronary artery stenosis: a novel heart syndrome mimicking acute myocardial infarction. Angina pectoris – myocardial Infarction Investigations in Japan. J Am Coll Cardiol. 2001;38:11–8. 17. Bortnik M, Verdoia M, Schaffer A, Occhetta E, Marino P. Ventricular fibrillation as primary presentation of takotsubo cardiomyopathy after complicated cesarean delivery. World J Cardiol. 2012;4:214–7. 18. Amariles P. A comprehensive literature search: Drugs as possible triggers of Takotsubo cardiomyopathy. Curr Clin Pharmacol. 2011;6:1–11. 19. Osuorji I, Williams C, Hessney J, Patel T, Hsi D. Acute stress cardiomyopathy following treatment of status asthmaticus. South Med J. 2009;102:301–3. 20. McAdow M, Kim HK, Dedent AC, Hendrickx AP, Schneewind O, Missiakas DM. Preventing Staphylococcus aureus sepsis through the inhibition of its agglutination in blood. PLoS Pathog. 2011;7:e1002307. doi:10.1371/journal.ppat.1002307. 21. Gill SK, Theodorides A, Smith N, Maguire E, Whitehouse SL, Rigby MC, et al. Wound problems following hip arthroplasty before and after the introduction of a direct thrombin inhibitor for thromboprophylaxis. Hip Int. 2011;21:678–83. 22. Kaminsky P, Lesesve JF, Jonveaux P, Pruna L. IgG deficiency and expansion of CTG repeats in myotonic dystrophy. Clin Neurol Neurosurg. 2011;113:464–8.
