Postpartum takotsubo cardiomyopathy with reversible cerebral vasoconstriction syndrome: a case report

Introduction

Postpartum takotsubo cardiomyopathy, also known as stress-induced cardiomyopathy, apical ballooning syndrome, and “broken heart syndrome,” is a transient condition mimicking acute myocardial infarction [3]. Indeed, the presenting clinical symptoms, electrocardiogram (EKG) changes, and laboratory findings are identical to those of acute myocardial infarction [1]. The appellation for the disorder is derived from the Japanese term for “fish pot for trapping octopus” that the left ventricle resembles in patients with the condition. The etiology is thought to be due to stress-induced, elevated levels of catecholamines and vasoconstrictive agents. Vasoconstrictive drugs such as bromocriptine and ergonovine have also been associated with the onset of postpartum takotsubo cardiomyopathy [8]. The condition is transient and complete resolution is the usual clinical course. However, it is estimated that maternal mortality is approximately 1% due to intractable pulmonary edema from right heart failure and the recurrence risk is appropriately 1–8% [4].

Postpartum reversible cerebral vasoconstrictive syndrome includes many previously described conditions including “thunderclap headaches,” severe migraine variants, and vasoconstrictive headaches caused by vasoactive drugs such as ergotamine tartrate, bromocriptine, and methergine [5]. Like postpartum takotsubo cardiomyopathy, the etiology is thought to be caused by stress-induced, elevated levels of catecholamines and other vasoconstrictive agents [6]. The clinical presentation includes the onset of the patient’s “worst headache in her life,” nausea, vomiting, photophobia, and, rarely, seizures and focal neurological deficits. Complete recovery is expected within days to weeks, although there are reported cases of death from stroke and residual hemiparesis [7].

Presentation of the case

A 36-year-old African-American female delivered her sixth child via an uncomplicated spontaneous vaginal delivery at term with epidural anesthesia. The pregnancy was complicated by chronic hypertension; however, the patient was not on anti-hypertensives. During her prenatal and labor and delivery course, her blood pressures remained below 140/90. Following delivery, she underwent a postpartum tubal ligation via mini laparotomy and was discharged on postpartum day 1 in satisfactory condition.

Three days after discharge, the patient returned to the emergency department complaining of headache, bilateral lower extremity edema, heart palpitations, and tingling in her left arm radiating to her hand. At initial evaluation in the emergency room, her vital signs were as follows: temperature, 37.1°C; pulse, 60 beats/min; respiratory rate, 18 breaths/min; blood pressure, 145/77 mm Hg; and oxygen saturation, 97% on room air. The EKG showed sinus bradycardia with first-degree atrioventricular (AV) block (Figure 1).

Figure 1

Admitting EKG: sinus bradycardia with first-degree AV block.

Initial laboratory assessment was significant for the following: hemoglobin, 10.5 g/dL; hematocrit, 37.7%; platelets, 263×103/μL; aspartate transaminase, 36 IU/L; alanine transaminase (ALT), 42 IU/L; serum creatinine, 0.74 mg/dL; and urine protein screen, negative. The thyroid-stimulating hormone level was normal. The lower extremity venous Doppler study and chest X-ray results were negative for any underlying pathology.

Due to the initial elevated blood pressure, elevated ALT, and patient’s complaint of unrelenting headache, a presumptive clinical diagnosis of postpartum superimposed preeclampsia with reversible cerebral vasoconstriction syndrome was made and the patient was admitted to the obstetrical service for observation. The patient was started on intravenous magnesium sulfate for seizure prophylaxis.

While on the obstetrical service, an echocardiogram was performed that showed a normal ejection fraction of 65% (±15%) and trivial tricuspid regurgitation. The patient was symptomatically treated with narcotic pain medication for her headache, and compression stockings with sequential compression device were used to help alleviate the swelling in her lower extremities. She remained on magnesium sulfate for 24 h and her headache resolved. On hospital day 1 in the evening after breast pumping, the patient had sudden onset of crushing chest pain, dyspnea, and restlessness.

Repeat EKG showed a possible ectopic atrial rhythm with marked first-degree AV block, left axis deviation, non-specific intraventricular block, and elevation in the ST segment of the anterolateral leads (Figure 2). The patient was given intravenous morphine, oxygen, and aspirin. Initial cardiac enzymes were elevated: troponin I, 4.490 ng/mL; creatine kinase (CK), 486 IU/L; creatine kinase (CK-MB) index, 5.2; and CK-MB, 25.5 ng/mL. The patient was admitted to the Cardiovascular Intensive Care Unit for evaluation. Repeat cardiac enzyme studies done at 8-h intervals increased initially before trending down: troponin I, 30.9 and 18.7 ng/mL; CK, 1458 and 1135 IU/L; CK-MB index, 5.3 and 4.7; and CK-MB, 77.5 and 52.8 ng/mL.

Figure 2

Subsequent EKG after onset of chest pain: possible ectopic atrial rhythm with marked first-degree AV block, left axis deviation, non-specific intraventricular block, and elevation in the ST segment of the anterolateral leads.

Serial 12-lead EKGs were performed showing return to sinus rhythm with first-degree AV block. Repeat chest X-ray result was also negative for acute pleuropulmonary process. A computed tomography angiogram of the chest was performed with intravenous contrast showing no filling defects in the pulmonary arteries.

On hospital day 2, the patient underwent cardiac catherization that showed normal coronary arteries but elevated left ventricular end diastolic pressure (LVEDP). Repeat echocardiogram was performed showing a decrease in ejection fraction to 50% (±5%) with left ventricular akinesis in the apical, apical septal, apical lateral, apical inferior, apical anterior, and mid anteroseptal walls. Findings were consistent with stress-induced cardiomyopathy otherwise known as postpartum takotsubo cardiomyopathy.

The patient remained stable and asymptomatic during the rest of her hospitalization and was discharged home on hospital day 4 on metoprolol. Four months after discharge the patient is still under the care of her cardiologist for persistent akinesis of the left ventricular wall and decreased ejection fraction but is clinically improved and stable.

Discussion

It is probable that acute intracranial disease processes and injuries may be triggers for takotsubo cardiomyopathy. Complete recovery from reversible cerebral vasoconstriction syndrome is expected within 12 weeks, although our patient recovered more quickly than this [2]. The simultaneous occurrence of two rare conditions – postpartum takotsubo cardiomyopathy and reversible cerebral vasoconstriction syndrome – suggests a common etiology and is consistent with a stress-induced theory of causation [5, 8]. In our patient, there was a history of chronic hypertension, but the patient was not on any medications during her pregnancy and did not have pregnancy-induced hypertension. She was not taking any ergotrates in the postpartum period.

Our patient was managed with intravenous magnesium sulfate, a vasorelaxation agent, for 24 h and the usual medication protocol for suspected myocardial infarction: aspirin, narcotics, and oxygen. Partial resolution of takotsubo cardiomyopathy occurred prior to discharge. This course of therapy with similar clinical outcomes has been used with the few reported cases of postpartum takotsubo cardiomyopathy in the literature [6]. Vasoconstrictors such as ergotrate and methergine should be avoided in the postpartum period when the reversible cerebral vasoconstriction syndrome or takotsubo cardiomyopathy is suspected.

Although clinical outcomes are usually good, the clinician must beware that maternal mortality and residual neurological or cardiovascular deficits may occur. Since the recurrence risk is significant for both conditions – 1–8% for postpartum takotsubo cardiomyopathy and reported recurrence in several women with postpartum reversible cerebral vasoconstriction – patients with these conditions should be counseled appropriately [4].