Cardiogenic shock secondary to stress-induced cardiomyopathy precipitated by severe diabetic ketoacidosis

ABSTRACT

Stress-induced cardiomyopathy is caused by neurohormonal release of catecholamines. Although diabetic ketoacidosis (DKA) is a well-known complication of diabetes, diabetes mellitus is usually protective of stress-induced cardiomyopathy. Stress-induced cardiomyopathy secondary to DKA is extremely rare; however, cardiogenic shock is a potential complication of stress-induced cardiomyopathy. We present a rare case describing diabetic ketoacidosis leading to profound acidosis causing stress-induced cardiomyopathy and cardiogenic shock that is successfully managed with venoarterial extracorporeal membrane oxygenation in adults.

CASE REPORT

The patient is a 25-year-old woman with no prior medical problems who felt weakness and lethargy for 2 days. She was found unresponsive and brought to the hospital. Initial physical exam revealed Kussmaul breathing at a rate of 32 respirations/minute, heart rate of 113 beats per minute and blood pressure of 113/68 mmHg. The patient’s extremities were cool and clammy. Initial glucose was incalculably high. She received 3 liters (L) fluid and started on insulin at 6 units per hour. Initial lab work revealed pH < 7.0 and a bicarbonate of 2 meq. Initial EKG demonstrated sinus tachycardia with borderline prolonged QTc at 501 ms. BNP elevated at 329 on admission. Initial troponin 0.27 ng/mL, peaking at 4.68 ng/mL.

Shortly after her arrival to ICU, she became hypotensive. The patient’s acidosis was managed aggressively with 8 additional liters of isotonic fluid, a sodium bicarbonate drip, a total of 550 meq of sodium bicarbonate, electrolyte repletion and maintenance on an insulin infusion. Bedside cardiac ultrasound was concerning for an ejection fraction (EF) 20–25% with regional wall motion abnormality, suspicious but typical for stress-induced cardiomyopathy. Formal transthoracic echocardiogram (TTE) on admission confirmed these findings and demonstrated basal and mid anterior wall abnormalities as well as basal and mid-septal abnormalities, consistent with the typical pattern of stress-induced cardiomyopathy (Fig. 1). A bubble study was negative for shunts. Cardiology and cardiac surgery were consulted emergently.

Figure 1.

Transthoracic Echocardiography, Hospitalization Day 1. Apical four chamber view. Ejection fraction 25%, multiple wall motion abnormalities. Basal and mid anterior wall abnormalities as well as basal and mid septal abnormalities, consistent with typical pattern of stress induced cardiomyopathy.

The patient continued to be acidotic and persistently hypotensive despite aggressive fluid resuscitation and bicarbonate repletion, ultimately requiring five different vasopressors and inotropes: including norepinephrine, epinephrine, milrinone, phenylephrine and vasopressin at maximum doses per hospital policies. Patient clinical picture was more consistent with cardiogenic shock at this point. The patient was also developing an acute kidney injury with oliguria despite aggressive fluid resuscitation. She was not a candidate for a mechanical assist device, including Impella, because of her short stature, so the decision was made to initiate venoarterial extracorporeal membrane oxygenation (VA-ECMO). TEE was performed at the request of the cardiac surgery team at time of VA-ECMO cannulation. With initial settings of pump speed 3905 revolutions/minute (rpm), blood flow 4.75 liters/min, FiO2 of 100%, Sweep O2 of 2, she was transferred to a tertiary center.

The cardiac transplant team evaluated the patient while she remained on VA-ECMO for 6 days. Right heart catheterisation was performed on hospital day 6 while the patient was maintained on milrinone, revealing central venous pressure 17, pulmonary artery pressure 35/24 (27), pulmonary capillary wedge pressure 23, cardiac output 7.4, cardiac index 4, systemic vascular resistance 832, prior to decannulation. A left heart catheterisation revealed no coronary artery disease.

Further work to evaluate potential alternative causes of cardiomyopathy included COVID testing that was negative on admission; additionally, COVID antibodies were negative. Viral titers including Epstein–Barr virus, Coxsackie virus, Human Herpes Virus, Parvovirus, Cytomegalovirus, Hepatitis panel and HIV were essentially unremarkable. Autoimmune cardiomyopathy was considered, and ANA and ANCA were negative. All four criteria for diagnosis of stress-induced cardiomyopathy were met based on the mayo clinic criteria [1]. A right ventricular endomyocardial biopsy (Fig. 2) revealed no significant inflammation, granulomas, giant cells or fibrosis. Additional staining with Congo Red excluded amyloid deposition. Additionally, iron staining did not reveal any significant hemosiderosis. The only explanation for this patient’s cardiomyopathy is stress-induced cardiomyopathy, secondary to profound acidemia in DKA. The patient’s hospital course was further complicated by the development of polyglandular failure and polyradiculoneuropathy of bilateral lower extremities, confirmed with EMG testing, ultimately consistent with toxic/inflammatory neuropathy secondary to profound shock and metabolic derangements after extensive imaging and lab studies, including MRI, US, were unrevealing for other attributable causes. A diagnosis of type I diabetes mellitus was confirmed with anti-GAD antibodies. It was felt that her polyglandular failure was strongly related to an autoimmune process related to her type-1 diabetes. The patient received five sessions of IVIG.

Figure 2.

Endomyocardial biopsy. Hematoxylin and eosin stain. No significant inflammation, granulomas or giant cells.

Before decannulation, cardiac and renal function improved. The patient was successfully decannulated after 6 days. Her cardiac and renal function eventually fully recovered. Repeat TTE on hospital day 6 demonstrated a recovery of EF to 50% with global hypokinesis (Fig. 3). She was transferred out of the cardiac ICU on hospital day 10 and was discharged to an acute rehabilitation unit after a 23-day hospital stay, maintained on daily aspirin, lisinopril and metoprolol succinate.

Figure 3.

Transthoracic echocardiography, hospitalisation day 6. Apical four-chamber view. EF 50%, global hypokinesis of the left ventricle with minor regional variations.

DISCUSSION

Stress-induced cardiomyopathy is typically transient. It has been described more frequently in recent years as a cause of cardiomyopathy, when ischemic cardiomyopathy and all other potential causes are ruled out [2, 3]. Cardiogenic shock is a potentially fatal possible complication of stress-induced cardiomyopathy if it is not recognised and managed appropriately [4]. Cardiogenic shock is described in less than 10% of cases related to stress-induced cardiomyopathy [5].

We present a rare case of stress-induced cardiomyopathy and cardiogenic shock that was successfully managed by VA-ECMO in a previous healthy 25-year-old female who was diagnosed with severe DKA.

Various endocrine conditions have been described as precipitating stress-induced cardiomyopathy [6]. Although diabetes has been described as protective against stress-induced cardiomyopathy, the patient’s profound acidosis was significant enough to overcome any protective benefit and lead to cardiogenic shock [7]. All other causes of transient cardiomyopathy were ruled out with extensive work during her admission.

The prompt recognition of cardiogenic shock is vital to ensuring favorable outcomes. In our case, cardiogenic shock was identified within the first few hours of presentation, and prompt management and initiation of VA-ECMO led to the patient’s meaningful recovery.

CONFLICT OF INTEREST STATEMENT

None declared.

FUNDING

There were no sources of funding.

ETHICAL APPROVAL

No ethical approval is required.

CONSENT

Patient consent has been obtained.

GUARANTOR

Riley Gurreri, DO.