The Case

30yo F previously healthy G1P1, postpartum day 3, presented for sharp, retrosternal chest pain with associated shortness of breath and bitemporal headache. No prior cardiopulmonary or other significant medical history. Pregnancy complicated by possible chorio, delivered at term by SVD.

Presented hypertensive to 150s systolic, bradycardic to the 50s. Well-appearing, playing with her baby and laughing, no distress, euvolemic-appearing, benign abdomen. Exam unremarkable.

Initial workup in the ED including EKG, troponin, CT PE negative. Labs not consistent with pre-eclampsia.

Point-of-care ultrasound revealed the following:

Parasternal views showing dilated LV with reduced squeeze and anterior mitral leaflet mobility (increased EPSS).

On apical 4 chamber view you can see that the LV apex has preserved function whereas the base isn’t moving so well.

The patient was admitted and serial troponins peaked at 3.9, BNP 3500. Comprehensive echo (clips below) was consistent with POCUS findings (basal hypokinesis with preserved apical function). Had clean LHC, and the patient was ultimately diagnosed with reverse (basal-type) takotsubo cardiomyopathy. Managed supportively and was discharged a few days later.

Postpartum Cardiomyopathy

Brief background

New onset heart failure (usually dilated cardiomyopathy) in the peripartum period (last month of pregnancy up to 5 months post-partum), most commonly shortly after delivery
Risk factors:
- Advanced maternal age
- High parity/gravidity
- Multiple gestation pregnancy
- Hypertension
- Pre-eclampsia
- African descent
- Low socioeconomic status
Etiology not entirely clear, multiple hypotheses for pathophysiology
Diagnosis: clinical & echo findings (see below)
- Signs/symptoms mimic those of heart failure and late pregnancy
Management: usually supportive
- Treat HF accordingly, optimize volume status, may require anticoagulation as it’s associated with increased thromboembolic risk
- Mechanical support +/- cardiac transplantation in severe cases
Complications:
- Progression to severe +/- chronic heart failure
- Cardiogenic shock
- Arrhythmias
- Thromboembolic events
Prognosis:
- Estimated 50% fully recover (usually by 6 months), ~25% develop chronic heart failure
- Mortality up to ~9%, higher worldwide [1-3]
- High risk of recurrence in future pregnancies, especially if persistent LVEF [4].

Common ultrasound findings of PPM [1-3].

Left ventricular dilation/systolic dysfunction (EF <45%)
Often develop RV and bi-atrial enlargement
May have mitral valve regurgitation, dilated mitral annulus with papillary muscle displacement

Takotsubo Cardiomyopathy

Transient, reversible dilated cardiomyopathy, usually stress-induced (physical or emotional)
More common in post-menopausal females
Pathophys: exact mechanism not entirely clear but sympathetic stimulation plays a role
Risk factors thought to include hormonal or genetic factors, underlying neurologic/psychiatric disorders
Clinically mimics ACS, including elevated cardiac enzymes and EKG changes
Diagnosis:
- No clearly established diagnostic criteria; combination of history, clinical findings including echocardiography supports/suggests the diagnosis. Coronary angiography to rule out obstructive coronary artery disease [5-7].
- Proposed diagnostic criteria & likelihood: table 1 [7] & table 2 [8-9], respectively

Classic takotsubo: apical ballooning/hypokinesis with relatively preserved basal function [5-7].
Reverse (basal type) takotsubo (our patient)
- LV dilation with basal hypokinesis, preserved apical function or hyperkinesis (as seen above/below)
- Extremely rare, more common in younger patients & more associated with neurologic disease [6-9, 10]
- Very few known cases in the peri-partum setting [9]

Complications thought to be similar to those of ACS, especially in the acute phase
- Risk for LVOT especially in classic Takotsubo
Treatment: generally supportive, follow ACS guidelines until ACS is ruled out, inotropic support as needed
Prognosis:
- Overall good — vast majority have complete resolution of LV dysfunction in days to weeks
- Overall mortality 0-8%, higher rates in the acute phase [5, 8, 11]
- Risk of recurrence is low (1.8% per year) [12]
**Prognosis and complication rate similar between typical and atypical types of takotsubo [10, 11]

Bottom line: Have high suspicion in any peripartum patient presenting with signs/sx of heart failure and low threshold for POCUS-ing!

References

1. Elkayam U, Akhter MW, Singh H, et al. Pregnancy-associated cardiomyopathy: Clinical characteristics and a comparison between early and late presentation. Circulation. 2005;111(16):2050-2055.

2. Arany Z, Elkayam U. Peripartum Cardiomyopathy. Circulation. 2016;133(14):1397-1409.

3. Honigberg MC, Givertz MM. Peripartum cardiomyopathy. BMJ. January 2019:k5287.

4. Elkayam U. Risk of subsequent pregnancy in women with a history of peripartum cardiomyopathy. J Am Coll Cardiol 2014;64:1629-36.

5. Bybee KA, Kara T, Prasad A, et al. Systematic Review: Transient Left Ventricular Apical Ballooning: A Syndrome That Mimics ST-Segment Elevation Myocardial Infarction. Annals. 2004; 141(11): 858-865.

6. Ghadri JR, Wittstein IS, Prasad A, et al. International Expert Consensus Document on Takotsubo Syndrome (Part I): Clinical Characteristics, Diagnostic Criteria, and Pathophysiology. Eur Heart J. 2018;39(22):2032-2046.

7. Awad HH, McNeal AR, Goyal H. Reverse Takotsubo cardiomyopathy: a comprehensive review. Ann Transl Med. 2018;6(23):460-460.

8. Ghadri JR, Wittstein IS, Prasad A, et al. International Expert Consensus Document on Takotsubo Syndrome (Part II): Diagnostic Workup, Outcome, and Management. Eur Heart J. 2018;39(22):2047-2062.

9. Kilian L, Haaf P, Pfister O, Vischer AS, Lapaire O, Burkard T. Reverse Takotsubo syndrome, a case report of a rare cause for postpartum heart failure. Cardiogenetics. 2018;8(1):13-16.

10. Ghadri JR, Cammann VL, Napp LC, et al. Differences in the clinical profile and outcomes of typical and atypical takotsubo syndrome: data from the International Takotsubo Registry. JAMA Cardiol 2016;1: 335–340.

11. Elesber AA, Prasad A, Lennon RJ, Wright RS, Lerman A, Rihal CS. Four-Year Recurrence Rate and Prognosis of the Apical Ballooning Syndrome. J Am Coll Cardiol. 2007;50(5):448-452.

12. Templin C, Ghadri JR, Diekmann J, et al. Clinical features and outcomes of takotsubo (stress) cardiomyopathy. N Engl J Med 2015;373:929–938.

POST BY: DR. MATTHEW MULLINS, PGY1

FACULTY EDITING BY: DR. LAUREN MCCAFFERTY