The n e w e ng l a n d j o u r na l of
Case Records of the Massachusetts General Hospital
From the Departments of Medicine
(N.S.S., S.S.T., D.D.Y.), Radiology (A.S.F.),
and Pathology (R.N.S.), Massachusetts
General Hospital, and the Departments
of Medicine (N.S.S., S.S.T., D.D.Y.), Radiol‑
ogy (A.S.F.), and Pathology (R.N.S.), Har‑
vard Medical School — both in Boston.
N Engl J Med 2021;384:272-82.
DOI: 10.1056/NEJMcpc2027086
Copyright © 2021 Massachusetts Medical Society.
272
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m e dic i n e
Founded by Richard C. Cabot
Eric S. Rosenberg, M.D., Editor
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Dennis C. Sgroi, M.D., Jo‑Anne O. Shepard, M.D., Associate Editors
Kathy M. Tran, M.D., Assistant Editor
Matthew B. Roberts, M.B., B.S., Case Records Editorial Fellow
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Case 2-2021: A 26-Year-Old Pregnant Woman
with Ventricular Tachycardia and Shock
Nandita S. Scott, M.D., Sunu S. Thomas, M.D., Doreen DeFaria Yeh, M.D.,
Andrew S. Fox, M.D., and R. Neal Smith, M.D.​​
Pr e sen tat ion of C a se
Dr. Sunu S. Thomas: A 26-year-old pregnant woman was transferred to this hospital for
evaluation and treatment of cardiogenic shock after a cardiac arrest resulting from
ventricular tachycardia.
The patient had had two previous uncomplicated pregnancies and was currently
pregnant at 32 weeks of gestation with monochorionic, diamniotic twins. Her preg-
nancy had been monitored for polyhydramnios by her obstetrician with the use of
serial fetal ultrasonography.
Three weeks before presentation to this hospital, one of the patient’s preschool-
age children had an ear infection and the other had a sore throat. The patient had
erythema in the eyes with greenish drainage, for which topical erythromycin and
sulfacetamide were prescribed. One week later, a nonproductive cough complicated
by a sore throat and ear pain developed. The patient also noted new edema in her
legs that was not associated with pain, redness, or warmth. However, during the
subsequent week, progressive pain in her right leg occurred, which prompted her
to seek care at an emergency department of another hospital.
On examination, the temperature was 37.0°C, the pulse 113 beats per minute,
and the blood pressure 90/50 mm Hg. Pitting edema of the legs was noted, with no
associated erythema, warmth, or tenderness on palpation of the calves. Ultrasonog-
raphy of the right leg reportedly showed no evidence of deep-vein thrombosis, and
a rapid test for streptococcal antigen was negative. While the patient was in the
emergency department, painful uterine contractions with back discomfort devel-
oped; the cervix was 2 cm dilated. She was admitted to the labor and delivery unit
for management of preterm labor. Intramuscular betamethasone and magnesium
sulfate were administered for maturation of the fetal lungs and tocolysis, respec-
tively. Continuous intravenous infusion of lactated Ringer’s solution was initiated.
Subcutaneous heparin was not administered, because delivery was thought to be
imminent. After 24 hours of magnesium sulfate therapy, the uterine contractions
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 Case Records of the Massachuset ts Gener al Hospital
remained unchanged, and no further cervical
dilatation was noted. Because of concern about
preterm labor, she remained hospitalized, with
continuous monitoring of the fetal heart rate.
Five days after admission to the labor and
delivery unit, the patient had tenderness in the
left leg, and the edema in both legs had wors-
ened. She also had episodes of severe coughing
but reported no chest pain or shortness of breath.
On examination, the temperature was 36.9°C,
the pulse 110 beats per minute, the blood pres-
sure 96/51 mm Hg, and the oxygen saturation
98% while the patient was breathing ambient
air. Auscultation of the lungs revealed expiratory
wheezes. Laboratory test results are shown in
Table 1. Doppler ultrasound examination of the
legs revealed an occlusive thrombus in the left
peroneal vein. The hematology service was con-
sulted, and an intravenous heparin infusion was
initiated. Intravenous furosemide and oral azithro-
mycin and guaifenesin were also administered.
The next morning, after the patient had eaten
breakfast, she had sudden severe, “crushing” chest
pain and became unresponsive and pulseless.
Telemetry revealed monomorphic ventricular
tachycardia at a rate of 178 beats per minute,
with a right bundle-branch block pattern with a
superior axis. Cardiopulmonary resuscitation was
initiated, and after the administration of epineph-
rine and cardioversion, the return of spontaneous
circulation was achieved within 4 minutes. Intra-
venous infusions of norepinephrine, vasopressin,
dopamine, propofol, and fentanyl were adminis-
tered. The trachea was intubated, and ventilatory
support was initiated. An emergency cesarean
section was performed, and two healthy male in-
fants were delivered, with an estimated intraop-
erative blood loss of 1600 ml. Laboratory test re-
sults are shown in Table 1.
Dr. Andrew S. Fox: Computed tomography (CT)
of the chest (Fig. 1A and 1B), performed after
the administration of intravenous contrast mate-
rial, revealed diffuse ground-glass opacities with
interlobular septal thickening, findings that were
consistent with pulmonary edema; bilateral pleu-
ral effusions and pericardial effusion; cardio-
megaly with biventricular enlargement; and dila-
tation of the main pulmonary artery, which
measured 3.8 cm in diameter. No pulmonary
embolus was identified.
Dr. Thomas: A transthoracic echocardiogram
showed biventricular dilatation and severely de-
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creased biventricular systolic function, moderate
mitral and tricuspid regurgitation, and a small
circumferential pericardial effusion. Furosemide
was administered intravenously, and the patient
was transferred to a nearby tertiary care center for
further treatment.
On arrival at the second hospital, 210 minutes
after the cardiac arrest, the pulse was 177 beats
per minute, and the mean arterial pressure was
65 mm Hg while the patient was receiving intra-
venous infusions of norepinephrine, dopamine,
and vasopressin. Physical examination was notable
for diffusely coarse breath sounds, rapid regular
heart sounds, and generalized edema. Thirty min-
utes after arrival at the second hospital, the pa-
tient underwent cannulation for femoral veno­
arterial extracorporeal membrane oxygenation
(ECMO), and an intravenous infusion of milrinone
was initiated. Ventricular tachycardia recurred;
intravenous magnesium and lidocaine were ad-
ministered, and the arrhythmia was terminated
with cardioversion. Plans were made to transfer
the patient by helicopter to this hospital, and in-
travenous vecuronium was given in preparation
for transport. During the flight, severe vaginal
bleeding developed, and 2 units of packed red
cells were transfused; however, the heparin infu-
sion was continued, given the presence of the
ECMO circuit.
On arrival at this hospital, 10 hours after the
cardiac arrest, additional information was ob-
tained from the patient’s sister and grandmother.
The patient had a history of childhood asthma,
obesity, anxiety, and depression. She had previ-
ously delivered two healthy, full-term children;
her current pregnancy was complicated by gesta-
tional diabetes mellitus and polyhydramnios of
one of the fetuses. Her medication history in-
cluded insulin, inhaled albuterol, sertraline, iron
sulfate, and folic acid. She had had no known
adverse drug reactions. She did not smoke to-
bacco, drink alcohol, or use illicit drugs. She was
married and had two healthy preschool-age chil-
dren, in addition to her two newborns. Her moth-
er had died from ovarian cancer; her sister was
healthy.
On examination, the temperature was 36.2°C,
the pulse 123 beats per minute, and the mean arte-
rial pressure 76 mm Hg without intrinsic arterial
pulsatility while the patient was receiving ECMO
support. The respiratory rate was 16 breaths per
minute and the oxygen saturation 99% while the
nejm.org January 21, 2021 273
 The n e w e ng l a n d j o u r na l of m e dic i n e

Table 1. Laboratory Data.*

1 Day before Day of Transfer, Reference Range,

Reference Range, Transfer, after Cardiac Arrest, Adults, On Arrival,
Variable First Hospital First Hospital First Hospital This Hospital† This Hospital
Hemoglobin (g/dl) 11.2–15.7 10.9 11.0 12.0–16.0 9.6
Hematocrit (%) 34.1–44.9 32.5 33.1 36.0–46.0 28.2
Platelet count (per μl) 150,000–400,000 132,000 190,000 150,000–400,000 158
White-cell count (per μl) 3980–10,040 8920 12,670 4500–11,000 19,500
Differential count (per μl)
Neutrophils 1400–7700 6333 5448 1800–7700 16,302
Lymphocytes 1100–4000 892 3928 1000–4800 1014
Eosinophils 0–700 89 127 0–900 0
Bands 0–4 714 1140 0–1100 838
Sodium (mmol/liter) 136–145 135 142 135–145 143
Potassium (mmol/liter) 3.5–5.1 3.6 3.8 3.4–5.0 4.0
Chloride (mmol/liter) 98–107 102 108 98–108 109
Carbon dioxide (mmol/liter) 21–32 20 19 23–32 21
Urea nitrogen (mg/dl) 7–18 9 8 8–25 9
Creatinine (mg/dl) 0.55–1.02 0.57 1.0 0.60–1.50 0.67
Glucose (mg/dl) 74–100 98 152 70–110 202
Calcium (mg/dl) 8.5–10.1 7.9 7.5 8.5–10.5 6.7
Total protein (g/dl) 6.4–8.2 4.9 5.0 6.0–8.3 3.6
Albumin (g/dl) 3.4–5.0 1.9 1.6 3.3–5.0 1.9
Aspartate aminotransferase 5–37 26 44 9–32 648
(U/liter)
Alanine aminotransferase 7–65 25 31 7–33 101
(U/liter)
Alkaline phosphatase (U/liter) 45–117 142 141 30–100 132
Total bilirubin (mg/dl) 0.2–1.0 0.59 0.28 0.0–1.0 0.8
Magnesium (mg/dl) 1.6–2.6 2.0 1.7–2.4 1.8
Prothrombin time (sec) 9.5–12.1 10.1 11.5–14.5 15.0
International normalized ratio 0.91–1.16 0.97 0.9–1.1 1.2
Partial-thromboplastin time 23.9–31.3 73.8 22.0–35.0 >150.0
(sec)
Central venous oxygen satura‑ 70–80 76.9
tion (%)
Phosphorus (mg/dl) 2.6–4.5 4.1
Ionized calcium (mmol/liter) 1.14–1.30 0.96
Globulin (g/dl) 1.9–4.1 1.3
Lactic acid (mmol/liter) 0.5–2.2 1.3
Troponin T (ng/ml) <0.03 28.32
Creatine kinase (U/liter) 40–150 11,012
Creatine kinase MB isoenzyme 0.0–6.9 552.0
(U/liter)
Fibrinogen (mg/dl) 150–400 259

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 Case Records of the Massachuset ts Gener al Hospital

Table 1. (Continued.)

1 Day before Day of Transfer, Reference Range,

Reference Range, Transfer, after Cardiac Arrest, Adults, On Arrival,
Variable First Hospital First Hospital First Hospital This Hospital† This Hospital
Arterial blood gases
Fraction of inspired oxygen 1.0 1.0
pH 7.35–7.45 7.19 7.35–7.45 7.31
Partial pressure of carbon 35–45 42.7 35–42 42
dioxide (mm Hg)
Partial pressure of oxygen 75–100 251.9 80–100 449
(mm Hg)

* To convert the values for urea nitrogen to millimoles per liter, multiply by 0.357. To convert the values for creatinine to micromoles per
liter, multiply by 88.4. To convert the values for glucose to millimoles per liter, multiply by 0.05551. To convert the values for calcium to
millimoles per liter, multiply by 0.250. To convert the values for bilirubin to micromoles per liter, multiply by 17.1. To convert the values for
magnesium to millimoles per liter, multiply by 0.4114. To convert the values for phosphorus to millimoles per liter, multiply by 0.3229. To
convert the values for lactic acid to milligrams per deciliter, divide by 0.1110.
† Reference values are affected by many variables, including the patient population and the laboratory methods used. The ranges used at
Massachusetts General Hospital are for adults who are not pregnant and do not have medical conditions that could affect the results. They
may therefore not be appropriate for all patients.

patient was receiving oxygen through volume
assist–control mechanical ventilation, with a de-
livered tidal volume of 290 ml and a fraction of
inspired oxygen of 1.0. The weight was 105 kg,
the height 163 cm, and the body-mass index (the
weight in kilograms divided by the square of the
height in meters) 39.5. The central venous pres-
sure was 4 mm Hg. The pupils were equal and
briskly reactive to light; once the patient was
weaned off propofol, she was able to follow com-
mands and move her arms and legs. Breath sounds
were coarse bilaterally and diminished at the bas-
es. Heart sounds were distant. The abdomen was
soft and obese; no active bleeding was noted
from the surgical incision, but large-volume vagi-
nal bleeding and clots were observed. The arms
and legs were warm and had moderate symmet-
ric pitting edema. The femoral venous and arterial
cannulas were secured in place in the right groin.
The blood level of thyrotropin was normal; other
laboratory test results are shown in Table 1. Tests
for influenza viruses, adenovirus, human meta-
pneumovirus, and parainfluenza virus were neg-
ative.
Dr. Fox: Chest radiography (Fig. 1C), performed
at the time of transfer to this hospital, revealed
interstitial opacities, a small left pleural effusion,
and a dense retrocardiac opacity on the left side.
The venous ECMO drainage cannula and a central
venous catheter were visible in the right atrium.
Dr. Thomas: An electrocardiogram (Fig. 2A)
showed normal sinus rhythm, right bundle-branch
block, borderline-low QRS voltage, and Q waves
with T-wave inversions in the anterior and lateral
leads.
Dr. Doreen DeFaria Yeh: Transthoracic echocar-
diography (Fig. 2B; and Videos 1 and 2, available Videos showing
transthoracic
with the full text of this article at NEJM.org),
echocardiography
performed while the patient was receiving ECMO are available at
support, revealed a dilated left ventricular cavity NEJM.org
with normal wall thickness and severe diffuse
left ventricular hypokinesis that was most prom-
inent in the anterior, anteroseptal, and lateral
territories, with an estimated ejection fraction of
11%. Additional notable findings included mod-
erate mitral and tricuspid regurgitation, moder-
ate right ventricular dysfunction and dilatation,
an estimated pulmonary-artery systolic pressure
of 53 mm Hg, a small pericardial effusion, and
a pleural effusion. The ECMO inflow cannula was
visualized in the inferior vena cava, and consis-
tent with systemic ECMO blood flows, the aortic
valve was observed not to open during the cardiac
cycle, which suggested very poor native left ven-
tricular contraction. There was no evidence of
intracardiac shunting.
Dr. Thomas: The dose of milrinone was in-
creased, and the dopamine infusion was discon-
tinued. An oxytocin infusion and rectal misopro-
stol were administered. Ongoing large-volume

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 The n e w e ng l a n d j o u r na l of m e dic i n e

Figure 1. Imaging Studies of the Chest. A

A contrast‑enhanced coronal CT image in the pulmo‑
nary angiographic phase (Panel A) shows bilateral
patchy ground‑glass opacities with interlobular septal
thickening compatible with pulmonary edema. A con‑
trast‑enhanced axial CT image in the pulmonary angi‑
ographic phase (Panel B) shows cardiomegaly with bi‑
ventricular enlargement and small bilateral pleural
effusions with associated relaxation atelectasis in the
lower lobes (arrows). A chest radiograph obtained at
the time of transfer to this hospital (Panel C) shows
interstitial pulmonary edema, cardiomegaly, a trace
left pleural effusion, and left basilar atelectasis. The
venous extracorporeal membrane oxygenation (ECMO)
inflow cannula and the tip of a central venous catheter
are visible in the right atrium, and an endotracheal
tube and enteric tubes are in appropriate positions.
B

vaginal bleeding was observed, and an intrauter-

ine balloon was placed to control the bleeding.
A diagnosis and management decisions were
made.

Differ en t i a l Di agnosis
Dr. Nandita S. Scott: This 26-year-old woman with
no known history of cardiovascular disease pres-
ents with monomorphic ventricular tachycardia
and biventricular cardiomyopathy. The presence
of a twin pregnancy makes her clinical care chal-
lenging. Health care professionals are increasingly C
tasked with caring for pregnant women with
cardiovascular disease, which has become one of
the leading causes of maternal complications and
death in the United States.1 The first step in con-
structing a differential diagnosis in this patient
is to determine whether ventricular tachycardia
caused the cardiac arrest and cardiomyopathy or
whether this patient had cardiomyopathy that re-
sulted in ventricular tachycardia.

Which Came First — Ventricular Tachycardia

or Cardiomyopathy?
First, I will consider the possibility that this pa-
tient’s initial problem was ventricular tachycardia
and that cardiomyopathy was a result of myocar-
dial dysfunction after cardiac arrest. New onset
or increased frequency of arrhythmias is often
noted during pregnancy for multiple reasons,
including dilatation of the cardiac chambers, a
higher resting heart rate, hormonal effects, in-
creased adrenergic responsiveness, and the hyper-
dynamic state of pregnancy. However, ventricu-
lar tachycardia is not common during pregnancy,2
especially in the absence of preexisting structural
heart disease. When ventricular tachycardia occurs
during pregnancy, it is most likely to originate
from the right ventricular outflow tract3 (ventricu-
lar tachycardia originating from such a location is
generally benign), and it does not typically result
in cardiac arrest. The superior axis of the ven-

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 Case Records of the Massachuset ts Gener al Hospital

I aVR V1 V4

II aVL V2 V5

III aVF V3 V6

V1

II

V5

Figure 2. Cardiac Studies.

An electrocardiogram (Panel A) shows normal sinus rhythm, right bundle‑branch block, borderline‑low QRS volt‑
age, and Q waves with T‑wave inversions in the anterior and lateral leads. A still image from a parasternal window
during transthoracic echocardiography (Panel B) that was performed while the patient was receiving ECMO support
shows left ventricular dilatation and a small pericardial effusion.

tricular tachycardia in this case does not support
origination from the right ventricular outflow
tract, because the axis of that type of tachycardia
would typically be directed inferiorly, traveling
from the base toward the apex.
The presence of a higher-than-expected heart
rate weeks before presentation suggests the ex-
istence of underlying structural heart disease. In
addition, sustained monomorphic ventricular
tachycardia with acute decompensation usually
occurs in patients who already have underlying
heart disease, and the marked dilatation of the
cardiac chambers suggests that the myopathic
process had been ongoing. These findings sug-
gest that the ventricular tachycardia was not the
primary problem but that there was an underlying
substrate — cardiomyopathy — that resulted in
ventricular arrhythmia.

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 The n e w e ng l a n d j o u r na l of m e dic i n e

Cardiomyopathy variable but can range from fatigue and dyspnea

We can rapidly rule out many causes of cardio- to cardiogenic shock and sudden death. This pa-
myopathy, including neuromuscular abnormali- tient’s presentation is consistent with a diagnosis
ties, genetic diseases, alcohol and drug use dis- of myocarditis, given her chest pain, abnormal
orders, and nutritional and endocrine disorders. troponin T level, and antecedent illness. There-
Could this patient have stress cardiomyopathy? fore, myocarditis remains a possible diagnosis
Despite being pregnant with twins and having in this case.
two young children at home, the patient does not
appear to have acute stressors that are causing Pregnancy-Associated Myocardial Infarction
stress cardiomyopathy.4 The pattern of wall-motion Pregnancy-associated myocardial infarction is an
abnormalities is also not consistent with stress increasingly recognized entity that occurs in 9.5
cardiomyopathy, which is typically characterized per 100,000 hospitalizations.8 The leading cause
by a hyperkinetic base and an akinetic midventricle of myocardial infarction during pregnancy is
and apex. Typical abnormalities on electrocardio- spontaneous coronary-artery dissection, followed
grams that are indicative of stress cardiomyopa- by atherosclerosis and coronary thrombosis.9
thy, such as ST-segment elevation, QT prolonga- Atherosclerosis is an unlikely diagnosis in this
tion, and T-wave abnormalities, were also absent patient, given the absence of traditional cardio-
in this case. In addition, stress cardiomyopathy vascular risk factors. The presence of a coronary
predominantly occurs in postmenopausal women, thrombus is possible and invokes the possibility
which makes this diagnosis relatively unlikely. that a paradoxical embolus had moved from the
In one study of a group of patients with ini- peroneal vein, across a patent foramen ovale, to
tially unexplained cardiomyopathy, the most com- the coronary artery. However, thrombi in the pero-
mon causes were myocarditis, ischemia, peripar- neal veins are unlikely to embolize, and a patent
tum cardiomyopathy, and infiltrative myocardial foramen ovale was not identified on echocardiog-
disease.5 This patient’s echocardiogram and labo- raphy in this patient. Pregnant women tend to
ratory test results do not support the diagnosis of have a more severe presentation of spontaneous
infiltrative myocardial disease; however, peripar- coronary-artery dissection than nonpregnant
tum cardiomyopathy, myocarditis, and ischemic women and are more likely to have multivessel
heart disease (pregnancy-associated myocardial coronary involvement, a reduced ejection frac-
infarction) are still considerations in this case. tion, and ST-segment elevation.10 These high-risk
features are consistent with this patient’s pre-
Peripartum Cardiomyopathy sentation; therefore, pregnancy-associated myo-
Given that this patient is pregnant, peripartum cardial infarction, possibly due to spontaneous
cardiomyopathy is an obvious consideration in coronary-artery dissection, is a possible diagnosis
this case. Peripartum cardiomyopathy is charac- in this case.
terized by a left ventricular ejection fraction below
45% that occurs toward the end of pregnancy or Amniotic-Fluid Embolism
during the months after delivery and without We also need to consider the possibility of am-
another identifiable cause.6 Twin pregnancy is a niotic-fluid embolism. This is a rare catastrophic
predisposing factor in this patient, but she had condition that occurs when amniotic fluid en-
no other known risk factors, such as Black race, ters the maternal circulation.11 This results in
advanced maternal age, traditional cardiovascular acute pulmonary hypertension with right ven-
risk factors, or preeclampsia.6 Peripartum cardio- tricular failure, followed by left ventricular systolic
myopathy rarely occurs before 36 weeks of gesta- dysfunction, hypoxemic respiratory failure, and
tion. Nevertheless, peripartum cardiomyopathy ultimately cardiovascular collapse. The embolus
remains a possible diagnosis in this case. develops during labor or immediately after delivery
and is associated with disseminated intravascular
Myocarditis coagulation. The sequence of events leading to this
Myocarditis is an inflammatory disease of the patient’s cardiovascular collapse, the presence of
myocardium that is caused by infectious or non- preterm labor, and the absence of disseminated
infectious processes and can result in myocardial intravascular coagulation do not support a diag-
dysfunction.7 The presentation of myocarditis is nosis of amniotic-fluid embolism in this case.

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 Case Records of the Massachuset ts Gener al Hospital
Further Differential Diagnosis
Any elevation in the troponin T level during preg-
nancy typically prompts further evaluation. At
the time of transfer to this hospital, the patient
had a troponin T level of 28.32 ng per milliliter
(high-sensitivity troponin T level, approximately
28,320 ng per liter). There is a broader differential
diagnosis with lower-range elevations in troponin
T levels; however, once the level rises to the level
seen in this patient, the differential diagnosis
becomes narrower,12 leaving us with two possible
diagnoses: myocarditis or a large myocardial in-
farction.
When all the features of this patient’s presen-
tation are considered, her primary diagnosis was
most likely biventricular cardiomyopathy due to
either myocarditis or pregnancy-associated myo-
cardial infarction. Owing to the severity of the
ventricular dysfunction, a myocardial infarction
would have probably involved the left main coro-
nary artery or multiple coronary arteries. To
further refine the differential diagnosis, I would
recommend coronary angiography to evaluate the
coronary arteries and, if possible, native endo-
myocardial biopsy, recognizing that the patient
is currently receiving anticoagulation for the ECMO
circuit.
Dr . Na ndi ta S . Sc o t t ’s Di agnosis
Cardiomyopathy most likely due to myocarditis
or myocardial infarction.
Discussion of M a nagemen t
Dr. Thomas: Our first goal in the management of
this patient’s condition was to provide adequate
hemodynamic support and resuscitate end-organ
function with ongoing vigilance for myocardial
recovery. At presentation to this hospital, the
patient was already receiving systemic perfusion
at a rate of 5.0 liters per minute through periph-
eral venoarterial ECMO. With this ECMO circuit
configuration, blood is returned to the arterial
circulation through the femoral arterial cannula,
and this retrograde flow can confer a predispo-
sition to higher left ventricular afterload, which
can lead to ventricular distention, particularly in
a nonpulsatile heart.
Clinically, increased left ventricular afterload
can cause ventricular arrhythmia, worsening mi-
tral regurgitation, and pulmonary edema, all of
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which were present in this patient. The fact that
the aortic valve was observed not to open on
echocardiography indicates that there was no
net outflow from the left ventricle, which can
worsen both mitral regurgitation and pulmo-
nary edema. To unload the left ventricle, we used
a percutaneous microaxial left ventricular assist
device (LVAD)13 to provide antegrade flow from
the left ventricle into the aorta. Once this device
was in place and the pulmonary edema and the
need for vasoactive medication support had de-
creased, the patient underwent coronary angiog-
raphy. We performed this procedure to investigate
the electrocardiographic changes and elevation
in the troponin T level, since we were considering
multiple causes, including ischemic disease, myo-
carditis, and peripartum cardiomyopathy. Given
the patient’s need for anticoagulation and the
presence of a pericardial effusion, we thought that
the risks associated with endomyocardial biopsy
were high and opted to defer this procedure.
Dr. DeFaria Yeh: Myocardial infarction is an im-
portant cause of severe maternal complications
and death. After careful review of this patient’s
Figure 3. Coronary Angiography.
Diagnostic coronary angiography, performed in the
right anterior oblique caudal projection, shows that
the proximal left anterior descending artery and proxi‑
mal left circumflex artery are smaller in caliber than
the distal portions of the arteries, findings that possi‑
bly suggest spontaneous coronary‑artery dissection in
the left main coronary artery. The percutaneous micro‑
axial left ventricular assist device is in place in the left
ventricle.
nejm.org January 21, 2021 279
 The n e w e ng l a n d j o u r na l
angiograms, we determined that the left main
coronary artery, the proximal left anterior de-
scending artery, and the proximal left circum-
flex artery were smaller in caliber than the more
distal portions of the vessels (Fig. 3). Given that
the diameter of normal coronary arteries tapers
distally, this proximal narrowing is abnormal and
arouses suspicion of a spontaneous coronary-
artery dissection of the left main territory. Spon-
taneous coronary-artery dissection in the left main
territory typically leads to an acute onset of severe
ventricular dysfunction due to coronary ischemia,
ventricular arrhythmias, and cardiogenic shock
or arrest. The event in this patient occurred after
the administration of anticoagulants for venous
thromboembolism.
Management of myocardial infarction during
pregnancy varies according to the underlying
cause. Conservative management with medical
therapy is preferred among patients with spon-
taneous coronary-artery dissection without hemo-
dynamic instability, since the majority of cases of
spontaneous coronary-artery dissection resolve with
time. However, if high-risk clinical features such
as involvement of the left main coronary artery,
cardiogenic shock, or ongoing ischemia are pres-
ent, careful percutaneous or surgical coronary
intervention may be considered, if feasible.14 As-
pirin and beta-blockers are safe to use if the
patient’s condition is hemodynamically stable.
Other causes of myocardial infarction during
pregnancy include paradoxical embolism to a
coronary artery in women who have an atrial-
level shunt; in such cases, anticoagulation is the
preferred therapy. Atherosclerotic acute plaque
rupture is rare but can be managed with dual
antiplatelet therapy and percutaneous interven-
tion, as appropriate.
Among women who have heart failure during
pregnancy, the assessment of volume overload
can be challenging, given the presence of symp-
toms that overlap with those of normal preg-
nancy. Loop diuretics may be used for volume
excess; beta-blockers, hydralazine, and digoxin
are generally safe to use. Angiotensin-convert-
ing–enzyme inhibitors, angiotensin-receptor
blockers, aldosterone antagonists, direct oral
anticoagulants, and statins are contraindicated
during pregnancy.15 Direct-current cardioversion
for the mother is safe if tachycardia is worsening
the hemodynamic status. Among women in car-
diogenic shock, inotropic and mechanical sup-
280 n engl j med 384;3
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of m e dic i n e
port with rapid delivery of the fetus is para-
mount. Of note, vaginal delivery is the preferred
method of delivery for women with cardiac dis-
ease, except in cases in which the mother has
received anticoagulation, has refractory heart
failure, or is in cardiogenic shock; in such cases,
urgent cesarean section is indicated for expedi-
tious delivery of the baby.
Dr. Thomas: Despite 7 days of temporary LVAD
use in combination with venoarterial ECMO sup-
port, the left ventricle failed to show any clini-
cally significant degree of myocardial recovery.
Consequently, the patient underwent implanta-
tion of a durable LVAD and removal of her ECMO
and temporary LVAD circulatory support devices.
Dr. R. Neal Smith: When a durable LVAD is
placed, a portion of the left ventricular apex is
removed and submitted for pathological analy-
sis. Histologic analysis of the specimen revealed
extensive myocardial infarction that was ap-
proximately 10 to 20 days old. Trichrome stain-
ing showed mild interstitial fibrosis. Congo red
staining, iron staining, periodic acid and peri-
odic acid–Schiff staining, and Luxol fast blue–
hematoxylin and eosin staining were all nega-
tive, and features of myocarditis were not
present.
Fol l ow-up
Dr. Thomas: On hospital day 35, the patient was
discharged with a durable LVAD as a bridge to
cardiac transplantation. Medications included
guideline-directed neurohormonal blockade and
ongoing antithrombotic therapy that consisted
of aspirin and warfarin that was adjusted to at-
tain a target international normalized ratio of
2.0 to 3.0. Nine months after LVAD implanta-
tion, the patient presented with worsening fa-
tigue and new-onset cola-colored urine due to
LVAD-related hemolysis. Interrogation of the
LVAD revealed increasing power requirements to
generate pump flow for the same fixed pump
speed. Echocardiography revealed a more dilated
left ventricle than was observed previously, with
worsening mitral regurgitation. This constella-
tion of findings was suggestive of possible
pump thrombosis. LVAD pump exchange was
performed after intensification of her anticoagu-
lation with intravenous heparin did not reduce
the LVAD-related hemolysis. Thrombolytic ther-
apy was not considered out of concern about the
nejm.org January 21, 2021
 Case Records of the Massachuset ts Gener al Hospital

A B

C D

E F

Figure 4. Explanted Heart.

Panel A shows a gross photograph of a mid–cross section of the explanted heart with a remote myocardial infarc‑
tion with thinning of the myocardium and scarring (white tissue). Panel B shows the infarction (arrow) with blue
scar tissue and with preserved epicardial and endocardial myocardium (trichrome staining). Panel C (left main coro‑
nary artery), Panel D (anterior descending coronary artery), Panel E (left circumflex coronary artery), and Panel F
(right coronary artery) show mild coronary artery disease without obvious vasculitis, remote thromboses, or remote
dissection (hematoxylin and eosin staining).

potential risk of catastrophic bleeding in a par- the patient underwent cardiac transplantation
ticularly young patient. with a heart from a hepatitis C virus–positive
Three months after the LVAD pump exchange, donor as a strategy to expedite her opportunity

n engl j med 384;3 nejm.org January 21, 2021 281

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 Case Records of the Massachuset ts Gener al Hospital

for transplantation. The surgical procedure went
well, with no clinically significant complications,
and her postoperative course was unremarkable.
Dr. Smith: The explanted heart weighed 660 g
with a securely inserted LVAD. Much of the atria
had been removed for implantation of the new
heart, and the foramen ovale was not patent.
The left ventricle was moderately dilated with
diffuse endocardial fibrous thickening. The
right ventricle showed mild endocardial fibrous
thickening. An extensive remote myocardial in-
farction that measured 15.2 cm in diameter and
occupied the anterior interventricular septum,
the anterior left ventricle, and the lateral left
ventricle was present (Fig. 4A and 4B). The epi-
cardial and endocardial myocardium was pre-
served (Fig. 4B). Histologic examination and
ultrastructural review showed no additional ab-
normalities. The valves were unremarkable.
The coronary circulation was found to be right
dominant. The coronary arteries were extensively
sampled, and only minimal atherosclerotic coro-
nary artery disease was identified (Fig. 4C through
4F). Vasculitis, myocarditis, remote coronary
thrombosis, pathologically significant atheroscle-
rosis, and remote dissection were not present.
The cause of the infarct was unclear on the basis
of the gross and histologic examination of the
explanted heart, but possibilities include tran-
sient thrombosis of the left main coronary ar-
tery, amniotic-fluid embolism, or healed sponta-
neous coronary-artery dissection.
Dr. Thomas: After cardiac transplantation, the
patient began treatment with the standard im-
munosuppressive regimen, which consisted of a
calcineurin inhibitor, an antimetabolite, and
glucocorticoids. Because she received her donor
heart from a hepatitis C virus–positive donor,
she was given an 8-week course of glecaprevir–
pibrentasvir treatment; after completing the treat-
ment, she had an undetectable viral load. The
post-transplantation course was notable for cyto-
megalovirus viremia and myocardial rejection,
but 2.5 years after transplantation, the patient is
doing well and the ejection fraction remains
normal.
A nat omic a l Di agnosis
Pregnancy-associated myocardial infarction,
probably due to spontaneous coronary-artery
dissection.
This case was presented at the Medical Case Conference.
No potential conflict of interest relevant to this article was
reported.
Disclosure forms provided by the authors are available with
the full text of this article at NEJM.org.

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