© 2014, Wiley Periodicals, Inc. DOI: 10.1111/echo.12686
Echocardiography
Recurrent Right Ventricular Takotsubo Cardiomyopathy in a Patient with Recurrent Aspiration Aditya Chandorkar, M.D.,* J. Nicol as Codolosa, M.D.,† Michael L. Lippmann, M.D.,‡ Gregg S. Pressman, M.D., F.A.C.C.,† and Joanna P. Sta Cruz, M.D.‡ *Department of Internal Medicine, Einstein Medical Center, Philadelphia, Pennsylvania; †Einstein Center for Heart and Vascular Health, Einstein Medical Center and Jefferson Medical College, Philadelphia, Pennsylvania; and ‡Division of Pulmonary and Critical Care Medicine, Einstein Medical Center, Philadelphia, Pennsylvania
Takotsubo cardiomyopathy is described as transient hypokinesis of the apical and mid-segments of the left ventricle with hypercontractile basal segments triggered by emotional or physical stress. Variants with basal hypokinesis and apical hyperkinesis have been described, as well as simultaneous involvement of the right ventricle (RV). Proposed mechanisms include myocardial “stunning” due to excessive catecholamine release. The echocardiographic presentation of Takotsubo cardiomyopathy may be related to differences in regional sympathetic innervation and catecholamine receptor density in the myocardium in the setting of high levels of circulating catecholamines. We describe the first case of isolated, recurrent RV Takotsubo cardiomyopathy reported in the literature. (Echocardiography 2014;31:E240–E242) Key words: Takotsubo cardiomyopathy, right ventricle
Takotsubo cardiomyopathy is described as transient hypokinesis of the apical and mid-segments of the left ventricle (LV) with hypercontractile basal segments, usually triggered by emotional stress. Variants with basal hypokinesis and apical hyperkinesis, known as “reverse Takotsubo” have also been reported. Involvement of the right ventricle (RV) has been described, but in association with simultaneous involvement of the LV. Three cases of isolated RV involvement have been reported in the medical literature. This is the first described case of recurrent RV Takotsubo cardiomyopathy. Case Presentation: A 28-year-old male patient presented with hematemesis. He had a history of Type I diabetes mellitus, with multiple previous admissions for ketoacidosis. He had a family history of diabetes. He denied use of alcohol or drugs, but reported a 5 pack-year smoking history. The patient also reported a history of gastroesophageal reflux disease and erosive esophagitis. On admission, physical examination revealed a thin, ill appearing patient. Blood pressure was Address for correspondence and reprint requests: J. Nicolas Codolosa, M.D., Einstein Center for Heart and Vascular Health, Einstein Medical Center and Jefferson Medical College, Philadelphia, Philadelphia. Fax: 215-456-3533; E-mail: [email protected]
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118/78 mmHg with a heart rate of 114/min. The patient had a respiratory rate of 18/min and was maintaining adequate oxygen saturation on room air. Admitting laboratory data showed metabolic acidosis with an anion gap of 32. The patient received treatment for diabetic ketoacidosis. Despite initial improvement, he developed respiratory distress and became hypoxic. A chest radiograph showed bilateral patchy, ground glass opacities. The patient developed hypotension requiring use of vasopressors, including norepinephrine (10 l/min), vasopressin (0.04 units/ min), and phenylephrine (100 l/min). He was intubated and mechanically ventilated due to persistent hypoxia with the clinical picture resembling adult respiratory distress syndrome, possibly secondary to aspiration. He developed worsening hypoxia, and had 4 distinct episodes of pulseless electrical activity (with progressive and refractory hypotension) requiring resuscitation. Echocardiography the next day revealed RV systolic abnormalities consisting of apical hypokinesis with basal hypercontractility (Fig. 1, movie clip S1). The LV showed preserved ejection fraction (>55%) with no wall-motion abnormalities. Electrocardiography showed no abnormalities. Troponins remained within normal limits. A repeat echocardiogram 5 days later showed complete resolution of the RV wall-motion abnormalities (Fig. 2, movie clip S2). A defibrillator was
Isolated Recurrent Right Ventricular Takotsubo Cardiomyopathy
right heart catheterization with normal filling pressures and preserved cardiac output. The patient refused further procedures and signed off against medical advice. Three days after leaving the hospital, the patient was found unresponsive and was brought to the hospital. He was resuscitated for 30 minutes prior to hospital arrival, and resuscitative efforts in-hospital were unsuccessful.
Figure 1. Initial echocardiogram from the subcostal window showing hypercontractility of the basal right ventricle with hypokinesis of the mid and apical segments.
placed for secondary prevention of sudden cardiac death. Esophageal manometry studies showed evidence of achalasia. A repeat echocardiogram 2 months after the event showed a RV with normal size and systolic function, without evidence of RV wall-motion abnormalities. The patient was readmitted 5 months after the initial admission with hematemesis. He was found to be in ketoacidosis and developed aspiration pneumonitis. He became hypoxic, was intubated, and mechanically ventilated. After intubation, the patient had an episode of pulseless electrical activity and was resuscitated. The patient did not require vasopressors. An echocardiogram showed recurrence of RV wall-motion abnormalities – with apical hypokinesis and basal hyperkinesis. These anomalies resolved on a repeat echocardiographic study, 48 hours later. The patient underwent a coronary angiogram which showed no coronary artery disease and a
Figure 2. Subsequent echocardiogram from the subcostal window performed 5 days after the initial one shows complete resolution of the regional right ventricular wall-motion abnormalities.
Discussion: Takotsubo cardiomyopathy, or stress-induced cardiomyopathy is a recently described, acute, reversible form of cardiomyopathy characterized predominantly by left ventricular contractile abnormalities.1 Takotsubo cardiomyopathy is classically described in elderly women who show transient hypokinesis of the apical and mid-segments of the LV with hypercontractile basal segments, triggered by emotional stress.2 Variations of the classical presentation have been infrequently described—including patients with an inverted Takotsubo pattern.3 RV involvement in Takotsubo has been infrequently reported, arguably because associated LV findings are frequent and impressive. Case studies report RV involvement in Takotsubo cardiomyopathy in ~25–28% of patients.4–6 RV involvement is seen to be associated with worse LV function. An original study showed association with poorer outcomes7—a greater incidence of hospitalization and complications; which wasn’t confirmed in later studies. However, most patients still show recovery of LV systolic function to original values. There have only been three case reports so far about isolated RV involvement without concurrent LV involvement.7–9 Our case is only the fourth reported case of isolated RV involvement in Takotsubo cardiomyopathy. Our case is made unique by the fact the patient was the first case in literature of recurrent, isolated RV Takotsubo cardiomyopathy. Our patient had a significant departure from the standard demographic, being a younger male with episodes of Takotsubo cardiomyopathy during physiologic stress, and ultimately having a poor outcome. Takotsubo cardiomyopathy is a heterogeneous disorder, with multiple factors involved in its pathogenesis. Most theories involve increased sympathetic stimulation with cardiac effects of catecholamines. Theories now favor a functional, rather than structural, basis to Takotsubo cardiomyopathy; with dynamic changes in adrenoreceptor distribution explaining the differential LV contractile pattern.10 Other theories include a transient stress-related LV outflow tract obstruction; or an acute thrombosis involving LAD in
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apical region with rapid resolution. Isolated RV involvement in our case goes against a fixed, structural theory; which would also fail to explain departures from the normal—like inverted LV Takotsubo cardiomyopathy. The lack of a precise and thorough understanding of this newly described entity warrants further investigation in future studies.
6. 7. 8. 9.
References 1. Sharkey SW, Windenburg DC, Lesser JR, et al: Natural history and expansive clinical profile of stress (tako-tsubo) cardiomyopathy. J Am Coll Cardiol 2010;55:333–341. 2. Sharkey SW, Lesser JR, Zenovich AG, et al: Acute and reversible cardiomyopathy provoked by stress in women from the United States. Circulation 2005;111:472–479. 3. Van de Walle SO, Gevaert SA, Gheeraert PJ, et al: Transient stress-induced cardiomyopathy with an “inverted takotsubo” contractile pattern. Mayo Clin Proc 2006;81: 1499–1502. 4. Haghi D, Athanasiadis A, Papavassiliu T, et al: Right ventricular involvement in Takotsubo cardiomyopathy. Eur Heart J 2006;27:2433–2439. 5. Elesber AA, Prasad A, Bybee KA, et al: Transient cardiac apical ballooning syndrome: Prevalence and clinical
E242
10.
implications of right ventricular involvement. J Am Coll Cardiol 2006;47:1082–1083. Rodrigues AC, Guimaraes L, Lira E, et al: Right ventricular abnormalities in Takotsubo cardiomyopathy. Echocardiography 2013;30:1015–1021. Mrdovic I, Kostic J, Perunicic J, et al: Right ventricular takotsubo cardiomyopathy. J Am Coll Cardiol 2010;55:1751. Stahli BE, Ruschitzka F, Enseleit F: Isolated right ventricular ballooning syndrome: A new variant of transient cardiomyopathy. Eur Heart J 2011;32:1821. Burgdorf C, Hunold P, Radke PW, et al: Isolated right ventricular stress-induced (“Tako-Tsubo”) cardiomyopathy. Clin Res Cardiol 2011;100:617–619. Lyon AR, Rees PS, Prasad S, et al: Stress (Takotsubo) cardiomyopathy – a novel pathophysiological hypothesis to explain catecholamine-induced acute myocardial stunning. Nat Clin Pract Cardiovasc Med 2008;5:22–29.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. For Figure 1. Movie clip S2. For Figure 2.
Echocardiography
Recurrent Right Ventricular Takotsubo Cardiomyopathy in a Patient with Recurrent Aspiration Aditya Chandorkar, M.D.,* J. Nicol as Codolosa, M.D.,† Michael L. Lippmann, M.D.,‡ Gregg S. Pressman, M.D., F.A.C.C.,† and Joanna P. Sta Cruz, M.D.‡ *Department of Internal Medicine, Einstein Medical Center, Philadelphia, Pennsylvania; †Einstein Center for Heart and Vascular Health, Einstein Medical Center and Jefferson Medical College, Philadelphia, Pennsylvania; and ‡Division of Pulmonary and Critical Care Medicine, Einstein Medical Center, Philadelphia, Pennsylvania
Takotsubo cardiomyopathy is described as transient hypokinesis of the apical and mid-segments of the left ventricle with hypercontractile basal segments triggered by emotional or physical stress. Variants with basal hypokinesis and apical hyperkinesis have been described, as well as simultaneous involvement of the right ventricle (RV). Proposed mechanisms include myocardial “stunning” due to excessive catecholamine release. The echocardiographic presentation of Takotsubo cardiomyopathy may be related to differences in regional sympathetic innervation and catecholamine receptor density in the myocardium in the setting of high levels of circulating catecholamines. We describe the first case of isolated, recurrent RV Takotsubo cardiomyopathy reported in the literature. (Echocardiography 2014;31:E240–E242) Key words: Takotsubo cardiomyopathy, right ventricle
Takotsubo cardiomyopathy is described as transient hypokinesis of the apical and mid-segments of the left ventricle (LV) with hypercontractile basal segments, usually triggered by emotional stress. Variants with basal hypokinesis and apical hyperkinesis, known as “reverse Takotsubo” have also been reported. Involvement of the right ventricle (RV) has been described, but in association with simultaneous involvement of the LV. Three cases of isolated RV involvement have been reported in the medical literature. This is the first described case of recurrent RV Takotsubo cardiomyopathy. Case Presentation: A 28-year-old male patient presented with hematemesis. He had a history of Type I diabetes mellitus, with multiple previous admissions for ketoacidosis. He had a family history of diabetes. He denied use of alcohol or drugs, but reported a 5 pack-year smoking history. The patient also reported a history of gastroesophageal reflux disease and erosive esophagitis. On admission, physical examination revealed a thin, ill appearing patient. Blood pressure was Address for correspondence and reprint requests: J. Nicolas Codolosa, M.D., Einstein Center for Heart and Vascular Health, Einstein Medical Center and Jefferson Medical College, Philadelphia, Philadelphia. Fax: 215-456-3533; E-mail: [email protected]
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118/78 mmHg with a heart rate of 114/min. The patient had a respiratory rate of 18/min and was maintaining adequate oxygen saturation on room air. Admitting laboratory data showed metabolic acidosis with an anion gap of 32. The patient received treatment for diabetic ketoacidosis. Despite initial improvement, he developed respiratory distress and became hypoxic. A chest radiograph showed bilateral patchy, ground glass opacities. The patient developed hypotension requiring use of vasopressors, including norepinephrine (10 l/min), vasopressin (0.04 units/ min), and phenylephrine (100 l/min). He was intubated and mechanically ventilated due to persistent hypoxia with the clinical picture resembling adult respiratory distress syndrome, possibly secondary to aspiration. He developed worsening hypoxia, and had 4 distinct episodes of pulseless electrical activity (with progressive and refractory hypotension) requiring resuscitation. Echocardiography the next day revealed RV systolic abnormalities consisting of apical hypokinesis with basal hypercontractility (Fig. 1, movie clip S1). The LV showed preserved ejection fraction (>55%) with no wall-motion abnormalities. Electrocardiography showed no abnormalities. Troponins remained within normal limits. A repeat echocardiogram 5 days later showed complete resolution of the RV wall-motion abnormalities (Fig. 2, movie clip S2). A defibrillator was
Isolated Recurrent Right Ventricular Takotsubo Cardiomyopathy
right heart catheterization with normal filling pressures and preserved cardiac output. The patient refused further procedures and signed off against medical advice. Three days after leaving the hospital, the patient was found unresponsive and was brought to the hospital. He was resuscitated for 30 minutes prior to hospital arrival, and resuscitative efforts in-hospital were unsuccessful.
Figure 1. Initial echocardiogram from the subcostal window showing hypercontractility of the basal right ventricle with hypokinesis of the mid and apical segments.
placed for secondary prevention of sudden cardiac death. Esophageal manometry studies showed evidence of achalasia. A repeat echocardiogram 2 months after the event showed a RV with normal size and systolic function, without evidence of RV wall-motion abnormalities. The patient was readmitted 5 months after the initial admission with hematemesis. He was found to be in ketoacidosis and developed aspiration pneumonitis. He became hypoxic, was intubated, and mechanically ventilated. After intubation, the patient had an episode of pulseless electrical activity and was resuscitated. The patient did not require vasopressors. An echocardiogram showed recurrence of RV wall-motion abnormalities – with apical hypokinesis and basal hyperkinesis. These anomalies resolved on a repeat echocardiographic study, 48 hours later. The patient underwent a coronary angiogram which showed no coronary artery disease and a
Figure 2. Subsequent echocardiogram from the subcostal window performed 5 days after the initial one shows complete resolution of the regional right ventricular wall-motion abnormalities.
Discussion: Takotsubo cardiomyopathy, or stress-induced cardiomyopathy is a recently described, acute, reversible form of cardiomyopathy characterized predominantly by left ventricular contractile abnormalities.1 Takotsubo cardiomyopathy is classically described in elderly women who show transient hypokinesis of the apical and mid-segments of the LV with hypercontractile basal segments, triggered by emotional stress.2 Variations of the classical presentation have been infrequently described—including patients with an inverted Takotsubo pattern.3 RV involvement in Takotsubo has been infrequently reported, arguably because associated LV findings are frequent and impressive. Case studies report RV involvement in Takotsubo cardiomyopathy in ~25–28% of patients.4–6 RV involvement is seen to be associated with worse LV function. An original study showed association with poorer outcomes7—a greater incidence of hospitalization and complications; which wasn’t confirmed in later studies. However, most patients still show recovery of LV systolic function to original values. There have only been three case reports so far about isolated RV involvement without concurrent LV involvement.7–9 Our case is only the fourth reported case of isolated RV involvement in Takotsubo cardiomyopathy. Our case is made unique by the fact the patient was the first case in literature of recurrent, isolated RV Takotsubo cardiomyopathy. Our patient had a significant departure from the standard demographic, being a younger male with episodes of Takotsubo cardiomyopathy during physiologic stress, and ultimately having a poor outcome. Takotsubo cardiomyopathy is a heterogeneous disorder, with multiple factors involved in its pathogenesis. Most theories involve increased sympathetic stimulation with cardiac effects of catecholamines. Theories now favor a functional, rather than structural, basis to Takotsubo cardiomyopathy; with dynamic changes in adrenoreceptor distribution explaining the differential LV contractile pattern.10 Other theories include a transient stress-related LV outflow tract obstruction; or an acute thrombosis involving LAD in
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apical region with rapid resolution. Isolated RV involvement in our case goes against a fixed, structural theory; which would also fail to explain departures from the normal—like inverted LV Takotsubo cardiomyopathy. The lack of a precise and thorough understanding of this newly described entity warrants further investigation in future studies.
6. 7. 8. 9.
References 1. Sharkey SW, Windenburg DC, Lesser JR, et al: Natural history and expansive clinical profile of stress (tako-tsubo) cardiomyopathy. J Am Coll Cardiol 2010;55:333–341. 2. Sharkey SW, Lesser JR, Zenovich AG, et al: Acute and reversible cardiomyopathy provoked by stress in women from the United States. Circulation 2005;111:472–479. 3. Van de Walle SO, Gevaert SA, Gheeraert PJ, et al: Transient stress-induced cardiomyopathy with an “inverted takotsubo” contractile pattern. Mayo Clin Proc 2006;81: 1499–1502. 4. Haghi D, Athanasiadis A, Papavassiliu T, et al: Right ventricular involvement in Takotsubo cardiomyopathy. Eur Heart J 2006;27:2433–2439. 5. Elesber AA, Prasad A, Bybee KA, et al: Transient cardiac apical ballooning syndrome: Prevalence and clinical
E242
10.
implications of right ventricular involvement. J Am Coll Cardiol 2006;47:1082–1083. Rodrigues AC, Guimaraes L, Lira E, et al: Right ventricular abnormalities in Takotsubo cardiomyopathy. Echocardiography 2013;30:1015–1021. Mrdovic I, Kostic J, Perunicic J, et al: Right ventricular takotsubo cardiomyopathy. J Am Coll Cardiol 2010;55:1751. Stahli BE, Ruschitzka F, Enseleit F: Isolated right ventricular ballooning syndrome: A new variant of transient cardiomyopathy. Eur Heart J 2011;32:1821. Burgdorf C, Hunold P, Radke PW, et al: Isolated right ventricular stress-induced (“Tako-Tsubo”) cardiomyopathy. Clin Res Cardiol 2011;100:617–619. Lyon AR, Rees PS, Prasad S, et al: Stress (Takotsubo) cardiomyopathy – a novel pathophysiological hypothesis to explain catecholamine-induced acute myocardial stunning. Nat Clin Pract Cardiovasc Med 2008;5:22–29.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. For Figure 1. Movie clip S2. For Figure 2.
