Venoarteriovenous Extracorporeal Life Support for Severe Takotsubo Cardiomyopathy: A Case Report

ABSTRACT

In this case report, the authors describe the use of venoarteriovenous extracorporeal life support (V-AV ECLS) for a patient presenting with severe takotsubo cardiomyopathy (TCM) secondary to a breakthrough seizure. Given a presentation of hypoxia, V-AV ECLS was instituted and weaned quickly after cardiac recovery. The patient was discharged home after complications from cardiogenic shock resolved. The incidence of mechanical support is rising in patients presenting with TCM, and cardiogenic shock and V-AV ECLS may become a viable support method for this specific patient population.

INTRODUCTION

Mechanical circulatory support (MCS) is increasingly being used for patients with takotsubo cardiomyopathy (TCM) who suffer from cardiogenic shock.[1] In this case report, we describe the use of venoarteriovenous extracorporeal life support (V-AV ECLS) for a patient with TCM and acute respiratory distress syndrome (ARDS), as well as our rationale for its use.

CASE HISTORY

The patient was a 43-year-old woman with a medical history of epilepsy secondary to childhood meningitis, recurrent syncope requiring an automatic implantable cardioverter-defibrillator, gastroesophageal reflux, and anxiety who presented to the emergency department (ED) of an outside hospital after a breakthrough seizure due to medication noncompliance. She was initially hemodynamically stable in the ED; however, she became hypoxic after a second witnessed seizure. Upon intubation, she became progressively more hypotensive and tachycardic, requiring high doses of epinephrine (0.2 mcg/kg/min), norepinephrine (0.3 mcg/kg/min), and vasopressin (0.06 unit/min). A chest X-ray at the time showed bilateral patchy infiltrates that raised concern for ARDS. An electrocardiogram before the seizure showed T-wave inversion in leads 3 and AVF. She was transferred to our ED due to hemodynamic instability requiring a higher level of care. Point-of-care ultrasound (POCUS) demonstrated severely reduced left ventricular function, for which the ECLS team was activated. On arrival, the authors noted severe ARDS and apparent Society for Cardiovascular Angiography and Interventions (SCAI) Stage D/E shock.[2] Given these clinical findings, the patient was cannulated for V-AV ECLS.

The circuit components included a Maquet Cardiohelp extracorporeal membrane oxygenation (ECMO) system, a 25F drainage cannula in the left common femoral vein, a 17F right common femoral artery return cannula that was Y-connected to a 17F right internal jugular vein return cannula, and finally a 6F wire reinforced right superficial femoral artery distal perfusion cannula [Figure 1]. The patient was cannulated uneventfully, and ECLS commenced three hours after her first hypotensive episode, with immediate reduction in vasopressor requirements and improvement of hypoxia. Distal limb perfusion was confirmed with a bedside angiogram in the ED.

Figure 1.

Schematic diagram of V-AV cannulation strategy. Modified with permission from (1) using Creative Commons License. V-AV = Venoarteriovenous

The cardiology team was consulted for coronary catheterization, which showed severe apical and midventricular akinesis and no significant coronary arterial disease, consistent with TCM. The calculated left ventricular ejection fraction (LVEF) was 3%; notably, the left ventricular end-diastolic pressure was normal, and the patient had increased pulsatility (see Supplementary Digital Content for ventriculogram video). Considering these findings, mechanical left ventricular chamber venting was not instituted, and inodilator therapy with milrinone was initiated instead to promote left ventricular emptying.

Two days after ECLS initiation, a repeat echocardiogram showed a rapid recovery of the left ventricle to an LVEF of 49% and a normally functioning right ventricle without valvular disease. The patient was extubated on postcannulation day (PCD) 2, decannulated from venoarterial ECMO, and reconfigured to venovenous ECMO on PCD 4 after rapid left ventricular recovery to LVEF 70% on repeat echocardiography and a successful weaning trial. She was decannulated from venovenous ECMO on PCD 5. During her hospitalization, the patient suffered from complications related to cardiogenic shock and ECLS treatment. These complications included acute kidney injury requiring renal replacement therapy for 5 days after cannulation and deep venous thrombosis of the venous cannulation site despite protocolized heparin anticoagulation. For the deep venous thrombosis, she was treated for 3 months of oral anticoagulation with apixaban after clearance from the neurology team. She underwent a computed tomography scan of her head after decannulation due to some word-finding difficulty, and tiny cerebral infarctions in a watershed distribution were found. After comprehensive workup, it was determined that the most likely origin of these infarcts was from ECLS-related cerebral thromboembolism. The patient was discharged home from the hospital on PCD 16 neurologically intact except for some self-reported minor memory issues, which she described as “forgetting where items were in the home.”

DISCUSSION

The reported utilization of MCS for TCM with cardiogenic shock is rising. Advanced therapies such as venoarterial ECMO and percutaneous left ventricular assist devices, such as Impella, have continued to supplant intra-arterial balloon counterpulsation since 2012.[3] Overall, there is a propensity to use MCS in more severe cases of TCM with a potential mortality benefit.[4] This is observed in the use of mechanical support for typically more severe SCAI stage cardiogenic shock cases of TCM and in patients with advanced ages compared to patients in which MCS was not used.[4] To the authors’ knowledge, this is the first described case utilizing V-AV ECLS for TCM. Our rationale for using this ECMO configuration was primarily driven by the patient’s ongoing severe hypoxia in the setting of severe left ventricular dysfunction on the POCUS examination. We surmised that the left ventricular end diastolic pressure was increased, leading to poor forward flow through the pulmonary system and resultant pulmonary edema. Given this, we reasoned that providing oxygenated blood to the normally functioning right ventricle would provide prophylaxis against differential hypoxemia that was highly likely to develop with left ventricular recovery. Differential hypoxemia (also colloquially described as “north-south syndrome” and/or “Harlequin syndrome”) is a well-described clinical phenomenon of peripheral V-AV ECLS administration in which the recovering heart receives deoxygenated blood from still-diseased lungs and pumps this hypoxic mixture into the aorta, resulting in systemic hypoxemia.[5] In this case, the rapid improvement of left ventricular function shown by widened pulse pressure (>20 mmHg) almost immediately after cannulation may have necessitated the addition of a venous return cannula. The addition of this venous return cannula is of significantly higher risk when compared to introducing one during the initial cannulation procedure. With regards to the use of MCS for this patient, her left ventricular recovery time of less than 5 days was much more rapid than that of patients in the RETAKO database, with an average of 14 days cited.[4] In other meta-analyses of MCS in TCM, recovery times were similar to this patient.[3] We attribute this patient’s rapid recovery to the early initiation of appropriate MCS and the younger age profile of our patient.

CONCLUSION

Our approach suggests that V-AV ECLS is an appropriate treatment modality for TCM with cardiogenic shock and hypoxia and may be considered for similar cases in the future.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.