J A C C : C A S E R E P O R T S VO L . - , N O . - , 2 0 1 9

ª 2 0 1 9 T H E A U T H O R S . P U B L I S H E D B Y E L S E V I E R O N B E H A L F O F T H E A M E R I C A N

C O L L E G E O F C A R D I O L O G Y F OU N D A T I O N . T H I S I S A N O P E N A C C E S S A R T I C L E U N D E R

T H E C C B Y - N C - N D L I C E N S E ( h t t p : / / c r e a t i v e c o mm o n s . o r g / l i c e n s e s / b y - n c - n d / 4 . 0 / ) .

CASE REPORT

CLINICAL CASE

An Exceptional Cause ofAcute Right Heart Failure

Isolated Right Ventricular Takotsubo Syndrome

Jose Carreras-Mora, MD, Albert Duran-Cambra, MD, David Vilades-Medel, MD, Marcelo Jimenez-Kockar, MD,Eduard Sole-Gonzalez, MD, Isaac Llao-Ferrando, MD, PHD, Jordi Sans-Rosello, MD, Maria Vidal-Burdeus, MD,Montserrat Vila-Perales, MD, Alessandro Sionis, MD

ABSTRACT

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This study describes a case of acute right ventricular dysfunction secondary to right ventricular isolated Takotsubo

syndrome (TTS). The importance of an adequate differential diagnosis of acute right ventricular dysfunction and

the differences in diagnosis and management of right ventricular TTS and typical left ventricular TTS are

highlighted. (Level of Difficulty: Intermediate.) (J Am Coll Cardiol Case Rep 2019;-:-–-) © 2019 The Authors.

Published by Elsevier on behalf of the American College of Cardiology Foundation. This is an open access article under

the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

HISTORY OF PRESENTATION

An 82-year-old woman presented to the emergencydepartment with nonspecific discomfort and in poorgeneral condition. Physical examination showednormal pulmonary and cardiac auscultation. The pa-tient showed signs of shock and respiratory failure:blood pressure 90/60 mm Hg, 110 beats/min, arterialoxygen saturation 80%, and lactate levels 7.3 mmol/l.An electrocardiogram (ECG) showed sinus rhythm

EARNING OBJECTIVES

To review the differential diagnosis of acuteRV dysfunction and highlight the importanceof the clinical history.To understand the differences in clinicalpresentation and evolution of RV TTS andclassic left ventricular TTS.Phosphodiesterase-3 inhibitors could beuseful in cases of TTS complicated withcardiogenic shock.

N 2666-0849

m the Cardiology Department, Hospital de la Santa Creu i Sant Pau, Barcel

relationships relevant to the contents o this paper to disclose.

nuscript received September 26, 2019; revised manuscript received Octob

with no repolarization abnormalities. Right-sidedECG showed nondeep T waves in leads V3R to V6R(Figure 1). Transthoracic echocardiography revealednormal left ventricular (LV) systolic function butdilation and severe systolic dysfunction of the rightventricle (RV) with apical dyskinesia and basalhypercontractility, also known as the reverseMcConnell sign (1).

PAST MEDICAL HISTORY

The patient had a chronic depressive affective disor-der. Four days earlier, she had suffered a Collesfracture and coccyx fracture with persistent low backpain in the following days.

DIFFERENTIAL DIAGNOSIS

Because the patient presented in respiratory failureand shock with RV dysfunction, the initial differentialdiagnosis should include pulmonary thromboembo-lism and acute myocardial infarction (AMI).

https://doi.org/10.1016/j.jaccas.2019.10.038

ona, Spain. The authors have reported that they have

er 22, 2019, accepted October 24, 2019.

FIGURE 1 Initial ECG

(A) ECG at admission showing pr

V3R to V6R. ECG ¼ electrocardio

ABBR EV I A T I ON S

AND ACRONYMS

AMI = acute myocardial

infarction

ECG = electrocardiogram

LV = left ventricle

MRI = magnetic resonance

imaging

RV = right ventricle

TTS = Takotsubo syndrome

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INVESTIGATIONS

Chest computed tomographic scan ruled outpulmonary embolism as the first suspectedetiology. High sensitivity troponin T and cre-atine kinase levels were markedly elevated,with significant dynamic change (high sensi-tivity troponin T: 469 to 556 to 368 ng/dl;creatine kinase: 354 to 516 to 202 U/l), as wasthe N-terminal pro–B-type natriuretic peptidelevel (9,116 ng/l). Coronary angiography per-

formed to rule out RV AMI showed normal coronaryarteries (Figure 2). Right ventriculography confirmedmidapical dyskinesia (Video 1).

MANAGEMENT

The patient was admitted to the coronary care unit.Oxygen therapy and administration of milrinonewere initiated, with hemodynamic improvement.A pulmonary artery catheter was implantedand revealed pulmonary artery pressures of34/14 mm Hg, central venous pressure 9 mm Hg,and cardiac index 2.5 l/min/m2. In the following 24hours, the patient showed progressive clinicalimprovement with resolution of respiratory failureand hemodynamic stability, allowing the with-drawal of inotropic support. Evolutionary ECG onday 2 showed the appearance of negative T waves

ecordial leads with no repolarization abnormalities. (B) Right-sided

gram.

in leads V1 to V4 with a slight QT-interval prolon-gation (Figure 3).

Cardiac magnetic resonance imaging (MRI) per-formed on day 2 showed a nonhypertrophic butdilated RV with dyskinetic movement of the apex andmidanterior wall segments, conditioning mild RVsystolic dysfunction (Figure 2, Video 2). Sequences toevaluate for the presence of myocardial edema couldnot be performed because the patient had low backpain due to prolonged recumbency.

On day 3, the patient was transferred to the cardi-ology ward. Control transthoracic echocardiographyand cardiac MRI (days 7 and 10 after admission)showed normalization of volumes and regional/globalRV systolic function without late gadoliniumenhancement (Figure 2, Video 3).

After pulmonary embolism, acute coronary syn-drome, and other pathologies associated with tran-sient acute RV dysfunction (e.g., acute myocarditis)were ruled out and considering the typical clinicalscenario of a post-menopausal woman with a previ-ous stressful situation and a compatible ECG evolu-tion, the diagnosis of Takotsubo syndrome (TTS) withisolated RV involvement was finally established.

DISCUSSION

TTS, also known as stress cardiomyopathy, is an acuteheart failure syndrome that usually affects post-

precordial leads (right) showing nondeep negative T waves in leads

FIGURE 2 Right Coronary Angiography, Chest CT Scan, and Cardiac MRI

(Top row) Angiography (left) showing normal right coronary artery (RCA) with no presence of lesions and chest computed tomographic (CT)

scan (right) showing no evidence of pulmonary thromboembolism and normal diameter pulmonary trunk. (Middle, bottom rows)

Four-chamber cine images by cardiac magnetic resonance imaging (MRI) in diastole (left) and systole (right) with dilated right ventricle (RV)

and dyskinesia of the RV free wall at admission and recovery of the volumes and contractility of the RV at cardiac MRI performed 10 days

after. LVEDV ¼ left ventricular end-diastolic volume; LVEF ¼ left ventricular ejection fraction; RVEDV ¼ right ventricular end-diastolic

volume; RVEF ¼ right ventricular ejection fraction.

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menopausal women with a recent physical or psy-chological stressor. Unlike AMI, TTS is characterizedby the absence of significant coronary lesions and thereversibility of cardiac dysfunction. However,because of the similar acute clinical presentations, itoften triggers a differential diagnosis with AMI. TTS isincreasingly recognized and reported, probablybecause of better knowledge of the disease and itsparticular characteristics, but the precise incidence isunknown (2,3). The apical and midregional systolicdefect is the most frequent form of LV regional wallmotion abnormality. It is classified as the typicalanatomic variant of TTS, but other atypical formshave been described. Nevertheless, the isolatedinvolvement of the RV is exceptional, and few caseshave been reported to date (4,5), so this variant hasnot yet been included as a type of TTS in expertconsensus (2,3). Unlike classic forms of TTS affectingthe LV, some specific characteristics present in thiscase that we found in common with previously

reported cases of isolated RV TTS are: 1) developmentof nondeep negative T waves in leads V1 to V4 on theevolutionary ECGs, in opposition to the deep andpredominantly anterolateral negative T wavesgenerally observed in the typical form of TTS; 2)dilation of the affected ventricle, which is notcommonly observed in the LV forms of TTS, withcharacteristic systolic impairment resembling thereverse McConnell sign (i.e., apical dyskinesia andhyperkinesia of the basal segments); and 3) frequentsevere clinical presentation, with a reasonably rapidclinical recovery in accordance with the rapidimprovement of RV systolic dysfunction seen in theimaging tests. Interestingly, this recovery seems tooccur faster than in patients with LV involvement.

In this case, some particular characteristics werenoted. First, the initial ECG showed nonpathologicnegative T waves limited to lead V1. The initial ECGcan be normal up to 14% of patients with classic LVTTS (6), and usually these patients develop deep

FIGURE 3 Evolutionary ECG

ECG performed on day 2 showing evolutionary negative T waves in leads V1 to V4 with slight QT-interval prolongation. ECG ¼ electrocardiogram.

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negative T waves over the course of several days. Thisfact, in conjunction with the low magnitude of ECGchanges reflecting RV pathology in the standard leads,could explain the normal ECG upon admission of thispatient. Second is the choice of milrinone treatmentand its successful response. We decided to administera phosphodiesterase-3 inhibitor rather than dobut-amine in order to avoid the use of catecholamines. Useof phosphodiesterase-3 inhibitors has been describedfor treatment of TTS complicated by cardiogenicshock, with successful results (7,8). Dobutamine usehas been described as a trigger of TTS (9). Nonethe-less, there is a lack of evidence on the benefit of usingmilrinone over dobutamine in clinical practice.

FOLLOW-UP

After 2 years of follow-up by a cardiologist, the pa-tient is in New York Heart Association functional classI. Ambulatory echocardiography showed normal

biventricular function with no recurrence of TTSepisodes.

CONCLUSIONS

Although very uncommon, RV TTS has to beconsidered in the differential diagnosis of acuteright heart failure. This entity has several specificfeatures that differentiate it from typical LV TTS.Previously reported cases describe a frequent se-vere clinical presentation, usually cardiogenicshock, with more rapid recovery than cases of LVTTS.

ADDRESS FOR CORRESPONDENCE: Dr. JoseCarreras-Mora, Coronary Care Unit, CardiologyDepartment. Hospital de la Santa Creu i Sant Pau,Sant Antoni Maria Claret 167, 08025 Barcelona, Spain.E-mail: jcarreras@santpau.cat.

RE F E RENCE S

1. Liu K, Carhart R. “Reverse McConnell’s sign?”: aunique right ventricular feature of Takotsubocardiomyopathy. Am J Cardiol 2013;111:1232–5.

2. Lyon AR, Bossone E, Schneider B, et al. Currentstate of knowledge on Takotsubo syndrome: aposition statement from the Taskforce on

Takotsubo Syndrome of the Heart Failure Associ-ation of the European Society of Cardiology. Eur JHeart Fail 2016;18:8–27.

3. Ghadri J-R, Wittstein IS, Prasad A, et al. Inter-national expert consensus document on Takot-subo syndrome (part I): clinical characteristics,

diagnostic criteria, and pathophysiology. Eur HeartJ 2018;39:2032–46.

4. Stähli BE, Ruschitzka F, Enseleit F. Isolatedright ventricular ballooning syndrome: a newvariant of transient cardiomyopathy. Eur Heart J2011;32:1821.

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5. Sumida H, Morihisa K, Katahira K, Sugiyama S,Kishi T, Oshima S. Isolated right ventricular stress(Takotsubo) cardiomyopathy. Intern Med 2017;56:2159–64.

6. Frangieh AH, Obeid S, Ghadri J-R, et al. ECGcriteria to differentiate between takotsubo (stress)cardiomyopathy and myocardial infarction. J AmHeart Assoc 2016;5:e003418.

7. Mrozek S, Srairi M, Marhar F, et al. Successfultreatment of inverted Takotsubo cardiomyopathy

after severe traumatic brain injury with milrinoneafter dobutamine failure. Heart Lung 2016;45:406–8.

8. Padayachee L. Levosimendan: the inotrope ofchoice in cardiogenic shock secondary to takot-subo cardiomyopathy? Heart Lung Circ 2007;16Suppl 3:S65–70.

9. Hajsadeghi S, Rahbar MH, Iranpour A, Salehi A,Asadi O, Jafarian SR. Dobutamine-induced takot-subo cardiomyopathy: a systematic review of the

literature and case report. Anatol J Cardiol 2018;19:412–6.

KEY WORDS cardiac magnetic resonance,cardiogenic shock, right ventricle, stresscardiomyopathy, Takotsubo syndrome

APPENDIX For supplemental videos,please see the online version of this paper.