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14 Severe Eccentric Mitral Regurgitation Misdiagnosed asSevere Aortic Stenosis
93
What WasMisdiagnosed inThis Case andWhy?
In this case, the patient’s severe mitral regurgitation was misdiagnosed and reported
as severe aortic stenosis. This was because of the nature and direction of the regurgitant jet which was toward the anterior mitral leaet causing left ventricle outow
gradients. The authors didn’t consider transesophageal echocardiography with multiple views in this patient preoperatively which could probably have assisted in
reaching the proper diagnosis.
Discussion
Multivalvular heart diseases possess a diagnostic challenge in terms of their correct assessment of the severity of their hemodynamic intermingling causing variable effects on the heart. Rheumatic AS is commonly associated with MR of
varying degrees. Mostly, severe MR causes low-ow-low-gradient aortic stenosis
which falsely underpredicts the aortic valve gradients [1]. On the other hand,
severe eccentric mitral regurgitation can mislead us to high trans-aortic gradients
which may be normal [5]. The inaccuracy in proper diagnosis in patients with
multivalvular heart disease is a challenge. The complexity of pathophysiology of
the affected valves causes the valves to behave differently in different patients,
affecting the heart function variably and often misleading the diagnosis. In this
particular case, the MR jet was eccentric, hugging the anterior mitral leaet and
interfering with the reading of the aortic valve gradient. Abnormally high gradients were observed on CW which picked up the MR jet toward the LV outow
tract. Additionally, this patient had long-standing asthma causing a poor echocardiographic window contributing to the error in interpretation. The CW Doppler
picks up systolic signals which are away from the probe, and both MR and AS
have a systolic murmur which makes the assessment difcult. Electrocardiography
gating during echocardiography is essential for excellent correlation with the cardiac cycle. Numerous views were obtained to establish the correct diagnosis and
for accurate quantication of the images. A mid-esophageal long-axis view
revealed smooth blood ow across the aortic valve without any turbulence. The
short-axis images showed good coaptation of the aortic leaets. Deep trans-gastric ndings stated the peak and mean gradients as 4 mmHg and 2 mmHg, respectively [5]. Degenerative AS is frequently associated with MR in the west. The
mixed lesions affect the dynamics of the heart chambers in a complex way. The
antagonistic effects on the left ventricle with AS and MR dubiously estimate the
ejection fraction as normal. The CW Doppler overestimates the MR and underreads the AS severity [6]. MR causes increased volume load on the LV, whereas
AS causes pressure overload resulting in LV hypertrophy and reduced stroke volume. Consequently, there is a false estimation of low ow and low gradients with
conserved LV function [7]. Transesophageal or transthoracic real-time
94
three-dimensional echocardiography with color Doppler images may deliver better judgment than 2D echocardiography [8]. Measurement of regurgitant volume
can be more specic when Doppler quantication lacks accuracy. Though the
“gold- standard” imaging type is still not dened, volumetric analysis is considered better than 2D Doppler as it overestimates the MR [9]. Cardiac magnetic
resonance imaging (CMR) can be used as an additional modality for the proper
identication of the structure and function of the cardiac chambers and heart
valves. The immediate and long-term outcomes are poor if moderate to severe
mitral regurgitation is left behind during surgical aortic valve replacement [10].
MR magnitude post-SAVR is unpredictable. SAVR reduces LV pressure load
thereby reducing the transmitral pressure and gradient. There are multifactorial
predictors of MR severity improvement post- SAVR such as LV dimensions and
volumes, ejection fraction, wall motion abnormalities, bundle branch block, etc.
[11] A heart team approach for the best treatment plans and better short- and long-
term outcomes should be recommended.
S. Shrivastava
Plan ofAction: ThePoints Clinician Should Consider–
Pitfalls toAvoid
This patient was misdiagnosed with severe aortic stenosis in presence of moderate
mitral regurgitation. The transthoracic echocardiography couldn’t pick up the
mitral regurgitation severity due to the eccentricity of the jet toward the left ventricle outow tract. In this patient, multiple views of echocardiographic images
with different measurements should have been contemplated to arrive at a certain
diagnosis. Moreover, whenever such situations arise, a transesophageal echocardiography should always be considered before wheeling the patient to the OR.This
will avoid surprises that are cumbersome and inappropriate for the patient and the
family. Also, sudden changes in operating plans may not turn up with the desired
results.
Pearls ofKnowledge toConsider
In severe aortic stenosis associated with varying degrees of mitral regurgitation, a
denitive diagnosis should be sought. Multiple views, several readings, and combinations of various measurements must always be observed. A detailed examination
with transesophageal echocardiography with different views should be mandatory
to avoid a misleading diagnosis. The direction of the MR jet should be analyzed
intricately, especially when toward the anterior mitral leaet to avoid misinterpretations as left ventricle outow gradients.
14 Severe Eccentric Mitral Regurgitation Misdiagnosed asSevere Aortic Stenosis
95
Conclusion
Mixed valvular pathology is a common occurrence in rheumatic or degenerative
diseases. Unerring quantication of the severity of stenotic or regurgitant lesions is
a diagnostic predicament. Surgeons often land up in surprises in the OR which
causes the change of operating plans. The precise determination of aortic stenosis
gradients with concomitant severe mitral regurgitation can be misjudged. The position of the transducer probe during TTE can also lead to a false evaluation of the
disease. Sometimes the transducer picks up the MR jet velocity and reads it as
abnormally high aortic gradients. In this case, a similar thing happened as the regurgitant jet was directed eccentrically toward the anterior mitral leaet close to the left
ventricular outow tract. Hence the initial TTE was reported as severe aortic stenosis along with moderate mitral regurgitation. TEE in the OR on various views ruled
out any AS and reported a normally functioning aortic valve. On the contrary, the
MR turned out to be severe, and the treatment approach was switched to mitral
valve replacement. These diagnostic shortcomings can be overcome with a combination of various paradigms of echocardiography images. The application of 3D
imaging techniques and transesophageal echocardiography with color Doppler
quantication can curb the difculties to a large extent. The use of CMR and the
recent advances in technology can serve better results. This can provide meticulous
and detailed information about LV function and volumes, valve morphology and
their diseases, and other necessary calculations. In this case report, the patient
underwent mitral valve replacement. The postoperative TEE showed good prosthetic valve function and a normally functioning aortic valve. Mixed lesions need to
be evaluated carefully for better patient outcomes.
References
1. Bonow R, Carabello B, Chatterjee K, de Leon A, Faxon D, Freed M, etal. ACC/AHA 2006
guidelines for the management of patients with valvular heart disease. J Am Coll Cardiol.
2006;48(3):e1–e148. https://doi.org/10.1016/j.jacc.2006.05.021.
2. Mantovani F, Fanti D, Tafciu E, Fezzi S, Setti M, Rossi A, Ribichini F, Benfari G.When aortic
stenosis is not alone: epidemiology, pathophysiology, diagnosis and management in mixed and
combined valvular disease. Front Cardiovasc Med. 2021;8:744497. https://doi.org/10.3389/
fcvm.2021.744497.
3. Iung B.A prospective survey of patients with valvular heart disease in Europe: The Euro Heart
Survey on Valvular Heart Disease. Eur Heart J. 2003;24(13):1231–43. https://doi.org/10.1016/
s0195- 668x(03)00201- x.
4. Leong D, Pizzale S, Haroun M, Yazdan-Ashoori P, Ladak K, Sun Y, etal. Factors associated
with low ow in aortic valve stenosis. J Am Soc Echocardiogr. 2016;29(2):158–65. https://doi.
org/10.1016/j.echo.2015.10.007.
5. Kumar A, Rajanikant K, Negi S, Singla A.Misdiagnosis of severe aortic stenosis instead of
severe eccentric mitral regurgitation: possible causes and ways for prevention. Saudi J Anaesth.
2019;13(4):391–3. https://doi.org/10.4103/sja.SJA_92_19.
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6. Mantovani F, Barbieri A, Albini A, Bonini N, Fanti D, Fezzi S, etal. The common combination of aortic stenosis with mitral regurgitation: diagnostic insight and therapeutic implications
in the modern era of advanced echocardiography and percutaneous intervention. J Clin Med.
2021;10(19):4364. https://doi.org/10.3390/jcm10194364.
7. Benfari G, Clavel M, Nistri S, Maffeis C, Vassanelli C, Enriquez-Sarano M, Rossi
A.Concomitant mitral regurgitation and aortic stenosis: one step further to low-ow preserved
ejection fraction aortic stenosis. Eur Heart J Cardiovasc Imaging. 2017;19(5):569–73. https://
doi.org/10.1093/ehjci/jex172.
8. Grayburn P, Carabello B, Hung J, Gillam L, Liang D, Mack M, etal. Dening “severe” secondary mitral regurgitation. J Am Coll Cardiol. 2014;64(25):2792–801. https://doi.org/10.1016/j.
jacc.2014.10.016.
9. Uretsky S, Argulian E, Gudiwada S, Cohen R, Jang J, Chaudhry F, et al. Discordance
between MRI and echocardiography in the assessment of mitral regurgitation severity: prediction of surgical response suggests MRI may be more accurate. J Cardiovasc Magn Reson.
2015;17(S1):P262. https://doi.org/10.1186/1532- 429x- 17- s1- p262.
10. Harling L, Saso S, Jarral O, Kourliouros A, Kidher E, Athanasiou T. Aortic valve replacement for aortic stenosis in patients with concomitant mitral regurgitation: should the mitral
valve be dealt with? Eur J Cardiothorac Surg. 2011;40:1087. https://doi.org/10.1016/j.
ejcts.2011.03.036.
11. Matsumura Y, Gillinov A, Toyono M, Oe H, Yamano T, Takasaki K, etal. Echocardiographic
predictors for persistent functional mitral regurgitation after aortic valve replacement in
patients with aortic valve stenosis. Am J Cardiol. 2010;106(5):701–6. https://doi.org/10.1016/j.
amjcard.2010.04.030.
S. Shrivastava
Part III
Emergency Department
Chapter 15
A Case ofMisdiagnosis ofHepatic
Encephalopathy
MohammadJ.Ghosheh
Learning Objectives
By the end of this presentation, the clinician should be able to:
1. Discuss the classical presentation of hepatic encephalopathy and its variations in
clinical practice.
2. Enumerate the processes of how to diagnose hepatic encephalopathy in the
emergency department and know what the most appropriate investigations are.
3. Manage the different causes of hepatic encephalopathy and the precipitating
factors.
4. Describe the grading of hepatic encephalopathy and know how to implement it
in a clinical setting.
5. Summarize the pathophysiology of hepatic encephalopathy and apply the knowledge clinically.
6. Learn how to avoid misdiagnosing hepatic encephalopathy to facilitate a better
treatment approach to reduce complications and get better outcomes.
Introduction
Hepatic encephalopathy (HE) is a disorder that presents with reversible neuropsychiatric impairment, usually accompanied by severe liver dysfunction. It manifests
as uctuations in mental and cognitive function that may progress to stupor and
coma [1]. A diagnosis of hepatic encephalopathy may only be made after the
M. J. Ghosheh (*)
Al-Faisal University College of Medicine, Riyadh, Saudi Arabia
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
H. Tohid et al. (eds.), The Misdiagnosis Casebook in Clinical Medicine,
https://doi.org/10.1007/978-3-031-28296-6_15
99
100
Fig. 15.1 Causes of hyperammonemia
M. J. Ghosheh
exclusion of any other cause of cerebral dysfunction. Hepatic encephalopathy is
believed to be caused by an abnormal accumulation of neurotoxic metabolites (i.e.,
ammonia, NH3) in the systemic circulation [2]. Several studies have suggested that
increased concentrations of ammonia have been directly associated with astrocyte
swelling and cerebral edema, due to the excess glutamine produced as a result.
Cerebral edema would cause an increase in the intracranial pressure and subsequently result in the neurological deterioration seen in hepatic encephalopathy
patients [1, 3]. The diagnosis of hepatic encephalopathy is primarily based on both
the patient’s medical history and clinical presentation. A psychiatric assessment can
be made by performing a mini-mental state examination. Moreover, laboratory
studies should be done to evaluate serum ammonia levels. Hepatic encephalopathy
most commonly presents due to a precipitating factor in patients with underlying
liver dysfunction (i.e., cirrhosis). Avoidance and correction of the underlying factor
and preventing the development of further complications are generally the mainstay
of treatment. When considering the appropriate treatment for patients with hepatic
encephalopathy, other serious concurrently present medical conditions such as hepatorenal syndrome, portal vein thrombosis, and portal hypertension should also be
investigated. General treatment measures for hepatic encephalopathy patients
depend on the precipitant factor. Hepatic encephalopathy due to hyperammonemia
can be precipitated by two main factors [3–7]. Figure15.1 illustrates the causes of
hyperammonemia.
15 A Case ofMisdiagnosis ofHepatic Encephalopathy
Table 15.1 Patient’s vital signs
Parameters Results
Blood pressure 106/48 mmHg
Heart rate 122
Temperature (Celsius) 37.2
Respiratory rate 20
O2 saturation % 95
101
Clinical Case Presentation
A 53-year-old gentleman was admitted to the emergency department accompanied by his wife due to epigastric abdominal pain, confusion, and irritability that
has been increasing for the past 2 days. His wife states that her husband has been
acting strange recently and is unable to care for himself anymore and spends most
of his time sleeping. She also mentions that he has been shouting at her recently
and is “getting very frustrated with things we would normally enjoy doing
together.” The patient is drowsy, oriented to person only, and only arousable in
response to pain. His Glasgow coma scale score is 11/15. The patient’s vital signs
are in Table15.1.
The patient has lost his appetite for food and refuses to eat. He has lost 3kg in
the past 2 weeks. He has a known medical history of hypertension, gastroesophageal reux disease, peptic ulcer disease, and alcoholic cirrhosis. His medications
include esomeprazole, ramipril, and a laxative for when he occasionally gets constipated. He is a 20-pack-year smoker and has been drinking 4–6 beers a day for
the past 30 years. He works in construction and is married with 2 children. His
family history is only signicant for diabetes and hypertension in his mother.
General physical examination shows jaundice and multiple small bruises on his
arms and legs. Neurological examination shows an altered level of consciousness,
increased muscle tone, and rigidity in all extremities. His gait is abnormal, and he
falls to the side when attempting to walk (Tables 15.2 and 15.3) [8].
102
Table 15.2 Patient’s laboratory results
Parameters Results Reference range
Hemoglobin 9.4 g/dL 13.5–17.5
Hematocrit 31% 41%–53%
ESR 25 4.3–5.9
CRP 8 <10
Platelet count 131,000/mm
Leukocyte count 3800/mm
3
3
150,000–400,000
4500–11,000
Albumin 3.5 g/dL 3.5–5.5
Total bilirubin 2.2 mg/dL 0.1–1.0
AST 96 12–38
ALT 68 10–40
α-Fetoprotein 8 10–20
Creatinine 1.1 0.6–1.2
BUN 22 7–18
Anti-HAV IgG antibody Positive Negative
Anti-HBs antibody Positive Negative
Sodium 138 136–146
Potassium 3.6 3.5–5.0
Chloride 99 95–105
Bicarbonate 27 22–28
Urinalysis Results Reference range
Blood 1+ Negative
WBC 0 0–5 per high-power eld
Protein Negative Negative
RBC N/A 0–4 per high-power eld
Nitrite Negative Negative
Ketone Negative Negative
M. J. Ghosheh
Table 15.3 Differential diagnosis and associations
Differential diagnoses Associations
Infections Meningitis, encephalitis, intracranial abscess
Metabolic encephalopathy Hypoglycemia, electrolyte imbalance, anoxia, hypercarbia,
uremia, ketoacidosis
Alcohol-related
encephalopathy
Acute alcohol intoxication, alcohol withdrawal, Wernicke’s
encephalopathy
Toxic encephalopathy Sedatives, antidepressants/antipsychotics, salicylates
Hyperammonemia Hepatic encephalopathy, urea cycle disorders
Intracranial lesions Intracranial bleeds, stroke, tumor
Differential Diagnosis
Considering this patient’s presentation, a devised list of differential diagnoses
should have been formulated. An accurate list of differentials is a vital factor in
15 A Case ofMisdiagnosis ofHepatic Encephalopathy
103
determining an accurate diagnosis and the most appropriate next step in management [9]. The table below summarizes the list of differential diagnoses and their
associations based on the patient’s history, physical exam, and laboratory evaluations [1, 2, 4, 9, 10].
Discussion
Based on the patient’s medical/surgical history, clinical presentation, and laboratory
evaluation, a diagnosisof hepatic encephalopathy remains only one of several other
possible diagnoses. This patient’s hemodynamic instability with cognitive disturbances and a history of chronic alcohol abuse may hold the emergency physician in
doubt. Further investigations are the next best step in determining an accurate
diagnosis.
This patient was suspected to have an episode of Wernicke’s encephalopathy due
to vitamin B1 deciency. The patient was initially stabilized with IV uids and a
banana bag containing thiamine, folic acid, and magnesium sulfate. The patient
showed slight improvement, and a diagnosis of hepatic encephalopathy was missed.
The slight initial cognitive improvement might have been due to the nutritional and
vitamin deciencies that might have precipitated the encephalopathy in this patient.
After further investigations, elevated serial ammonia measurements with an abdominal CT scan conrming alcoholic liver cirrhosis were sufcient to diagnose hepatic
encephalopathy. Imaging of the brain may also be necessary for ruling out any
important intracranial lesions that might cause similar symptoms [4, 5].
There are many important hepatic encephalopathy clinical features that every
physician should be aware of, to prevent a potential misdiagnosis. Disorientation,
alterations in consciousness, confusion, irritability, fatigue, memory loss, abnormal
sleeping patterns, cognitive disturbances, slurring of speech, asterixis, and muscle
rigidity are important clinical features that can support diagnosing hepatic encephalopathy [3, 4, 6, 7, 10].
The symptoms of hepatic encephalopathy have been graded into a well-known
classication system. The West Haven classication system is divided into ve
grades, Grade 0 to Grade 4in ascending order of severity [2, 3, 7]. After the diagnosis was established, this patient was assigned a Grade 2 on the West Haven scale.
Subsequent to investigations, it was evident that the reason behind this patient’s
exacerbated hepatic encephalopathy was a bleeding peptic ulcer that increased the
production of ammonia by the gut bacteria and ultimately its absorption into the
systemic circulation [1]. The increase in ammonia levels with a cirrhotic liver that is
unable to degrade the excess ammonia through the urea cycle precipitated the symptoms observed in this patient [4].
Ammonia is believed to induce hepatic encephalopathy in several ways. Increased
levels of ammonia alter the transport of amino acids and impair their metabolism in
the brain [3]. The altered amino acid metabolism results in increased levels of glutamine that ultimately results in astrocyte swelling and cerebral edema [3, 4]. The