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CASE REPORTS IN CARDIOLOGY
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Case 1727 Orthotopic Heart Transplantation
for Ankylosing Spondylitis Masquerading
as Nonischemic Cardiomyopathy
Samarthkumar J. Thakkar, MDa, Paul A. Grayburn, MD
a,b
, and William C. Roberts, MD
MD
a,b,c,
*
a,b
, Shelley Anne Hall,
Described herein is a 48-year-old man who underwent orthotopic heart
transplantation because of severe heart failure considered clinically due to
idiopathic dilated cardiomyopathy, but examination of the operatively excised
native heart disclosed classic features of ankylosing spondylitis. Orthotopic
heart transplantation for this condition has not been reported previously.
© 2019 Elsevier Inc. All rights reserved.
(Am J Cardiol 2019;123:1732–1735)
We recently studied the heart of a patient who had undergone orthotopic
heart transplantation (OHT) for presumed idiopathic dilated cardiomyopathy and
examination of the operatively excised heart disclosed it to have classic morphologic
features of ankylosing spondylitis.
1, 2
The patient clinically had aortic regurgitation,
complete heart block, and periodic low back pain. Search of PubMed failed to
disclose any report of OHT for ankylosing spondylitis. Adescription of this patient
is the purpose of this report.
CASE DESCRIPTION
A 48-year-old male roofer, who was born in March1970, had been well until May2011
(age 41) when he developed the sudden onset of dyspnea and was hospitalized. His
systolic blood pressure was about 200mm Hg, his coronary arteries were free of
obstructive lesions, and his left ventricular ejection fraction was about 15%. He was
started on valsartan, carvedilol, isosorbide dinitrate, and amlodipine, but despite
these medicines, he had frequent episodes of acute heart failure. During one episode
in March2015, he was found to have abnormal kidney function and an atrophic left
kidney (cause unknown) that was excised. The main artery to the right kidney was
found to be stenotic and a stent was inserted. At that time, he developed complete
heart block and a dual chamber pacemaker was inserted. In July 2016, he was
started on peritoneal dialysis and 2 months later, hemodialysis. In July2017, cardiac
resynchronization therapy debrillator was inserted.
In April 2018, he developed cardiogenic shock and pulmonary edema and
was transferred to Baylor University Medical Center at Dallas. On arrival, his
blood pressure was 160/80mm Hg. Aprecordial murmur was not heard but his
respirations were extremely rapid. The electrocardiogram (Figure 1) showed
a
Baylor Scott and White Heart and Vascular Institute, Baylor University Medical Center,
Dallas, Texas;
Medical Center, Dallas, Texas; and
Center, Dallas, Texas. Manuscript received November 1, 2018; revised manuscript received
and accepted February11, 2019.
*
Corresponding author: Tel: (214) 820–7911; fax: (214) 820–7533.
E-mail address: william.roberts1@BSWHealth.org (W.C. Rob erts).
b
Department of Internal Medicine (Division of Cardiology), Baylor University
270 DOI: 10.1201/9781003409281-57
c
Department of Pathology, Baylor University Medical
CASE 1727 ORTHOTOPIC HEART TRANSPLANTATION FOR ANKYLOSING SPONDYLITIS
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Figure 1 Electrocardiogram, recorded at the time of presentation, showing atrialsensed ventricular-paced rhythm, biventricular pacemaker, and the total 12-lead
QRS voltage of 152mm.
Figure 2 Top left: Apical 4-chamber view showing severely dilated, elongated left
ventricle (LV) with normal right ventricular (RV) size and systolic function. An ICD
lead is seen in the RV (arrow). Top right: Apical 4-chamber end-diastolic frame with
ultrasound contrast. LV end-diastolic volume was 358 ml with LVEF 19% by biplane
Simpson’s method. Bottom left: Apical long-axis view showing severely thickened,
restricted anterior mitral leaet (yellow arrow). The posterior leaet (white arrow)
was of normal thickness and motion. Bottom right: Apical long-axis view with color
Doppler imaging showing severe aortic regurgitation (AR).
271
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Table 1: Pertinent admission laboratory ndings in the
patient described
B-type natriuretic peptide (pg/ml) 1895
Creatinine (mg/dl) 18
Blood urea nitrogen (mg/dl) 77
Estimated GFR (ml/min/1.73 m2) 3
Sodium (meq/L) 136
Potassium (meq/L) 5.2
Calcium (mg/dl) 8.7
Magnesium (mg/dl) 2.2
Phosphorous (mg/dl) 2.0
Total cholesterol (mg/dl) 219
Low density lipoprotein cholesterol (mg/dl) 151
High density lipoprotein cholesterol (mg/dl) 35
Triglyceride (mg/dl) 241
Hemoglobin A1c (%) 5.8
Rheumatic factor ([IU]/ml)* 8
ANA* Negative
HLA-B 27* Negative
C-reactive protein (mg/dl)* 0.5
GFR=glomerular ltration rate.
*Test performed 6 months after the orthotopic heart transplant.
atrial-sensed ventricular-paced rhythm and total 12-lead QRS voltage of 152mm
(10-mm standard).
3
The echocardiogram (Figure 2) showed the left ventricular
chamber to be severely dilated, the ejection fraction to be about 20%, and severe
aortic regurgitation to be present. At cardiac catheterization, the cardiac index was
1.5 L/min/m
2
. Certain laboratory ndings are listed in Table1.
He underwent combined heart and kidney transplant in May2018. The native
heart weighed 675 g (Figures3 and 4). The left ventricular cavity was considerably
dilated longitudinally: the distance from the base of the right aortic valve cusp to
the apex was 9.5cm. The anterior mitral leaet was severely thickened by dense
brous tissue, and the posterior mitral leaet was normal. The bases of each aortic
cusp were thickened by similar brous tissue which extended cephalad onto the
aorta in the areas of the commissures. The epicardial coronary arteries were free of
atherosclerotic plaques.
DISCUSSION
Described herein is a 48-year-old man who underwent OHT because of severe
heart failure attributed clinically to idiopathic dilated cardiomyopathy. Study
of his explanted native heart, however, disclosed classic (specic) morphologic
ndings of ankylosing spondylitis,
1, 2
distinctive and different from other cardiac
conditions (Figure 5). Before OHT, echocardiogram disclosed severe aortic
regurgitation. Although the degree of aortic regurgitation in our patient was
severe by echocardiogram, a precordial murmur was not detected while in severe
heart failure, probably the result of his rapid respiratory rate and his obesity (body
mass index 33 kg/m
2
). Aprecordial murmur had been present earlier when he was
not in heart failure. His pulse pressure when hospitalized at our institution was
80mm Hg.
Aortic regurgitation appears to occur in about 20% of patients with ankylosing
4
spondylitis
and it usually appears after the appearance of the orthopedic
consequences, although the reverse occurs, as in the present patient, on occasion.
272
CASE 1727 ORTHOTOPIC HEART TRANSPLANTATION FOR ANKYLOSING SPONDYLITIS
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Figure 3 Shown here is the heart of a 48-year-old man showing a dilated left ventricular cavity with thickened left ventricular walls, enlarged papillary muscles,
and thickened anterior mitral leaet. The posterior mitral leaet is normal (not
thickened).
Figure 4 Shown here is a closer view of the mitral and aortic valve showing the
remarkably thickened anterior mitral leaet which is extending into the base of the
posterior aortic valve cusp. The posterior mitral leaet is normal.
273
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The severe thickening of the anterior mitral leaet in ankylosing spondylitis in
the absence of thickening of the posterior mitral leaet as shown in the present
patient is diagnostic (Figure5). Although all 8 patients (all men) with ankylosing
spondylitis studied by Buckley and Roberts
regurgitation, only one of the 187 patients with ankylosing spondylitis studied
clinically by Klingberg et al
4
had “severe” aortic regurgitation; 24 others had “mild”,
1
at necropsy had extremely severe aortic
and 9 had “moderate” aortic regurgitation.
Interview of the patient and his wife 3 months after the OHT revealed that the
patient indeed had had low back pain periodically for years, but he attributed it to
Figure 5 Diagram showing the distinctive morphologic features of 4 different
cardiac conditions including ankylosing spondylitis. In cardiovascular syphilis, the aor-
tic wall behind the sinuses of Valsalva is spared and the adventitial scar tissue does
not extend below the aortic valve or involve mitral valve or ventricular septum.
Only the distal margins of the aortic valve cusps are thickened in syphilis, not the
proximal portions which are always involved in ankylosing spondylitis. In rheuma-
toid arthritis, the distinctive nodules similar to subcutaneous nodules, may inltrate
pericardium, myocardium and mural and valvular endocardium. If the valvular
tissue is involved, regurgitation usually of only mild degree results. In the Marfan
syndrome, aortic regurgitation is a consequence of disease of aortic wall, not of aortic
valve; the aorta is thinner, and usually contains intimal-medial tears. The ascending
aorta is diffusely involved, and dilatation of the aortic root causes the aortic regurgitation, which is usually severe. The mitral and rarely the aortic valve cusps may be
redundant in patients with the Marfan syndrome.
Abbreviations: Ao=aorta; A-V=atrioventricular; LA=left atrium; LV=left ventricle.
Reproduced with permission from the authors and the publisher.
274
1
CASE 1727 ORTHOTOPIC HEART TRANSPLANTATION FOR ANKYLOSING SPONDYLITIS
https://t.me/medicina_free
his kidney disease rather than to the arthritic problem. Thus, the cardiac features
of ankylosing spondylitis in this patient probably appeared after the clinical onset
of his orthopedic back problem. Lateral chest radiograph, however, did not show
changes of ankylosing spondylitis.
The dense brous tissue—characteristic of ankylosing spondylitis—was present
in the membranous ventricular septum just above the location of the atrioventricular
node and its presence in that location appears to be the cause of the patient’s complete
heart block diagnosed initially about 2years before the OHT.
We were unable to nd a previous publication of a patient with ankylosing
spondylitis having an OHT.
DISCLOSURES
The authors have no conicts of interest to disclose.
REFERENCES
1. Bulkley BH, Roberts WC. Ankylosing spondylitis and aortic regurgitation.
Description of the characteristic cardiovascular lesion from study of eight necropsy patients. Circulation 1973;48:1014–1027.
2. Roberts WC, Hollingsworth JF, Bulkley BH, Jaffe RB, Epstein SE, Stinson EB.
Combined mitra l and aortic regurgitation i n ankylosing spondylitis. Ang iographic
and anatomic features. Am J Med 1974;56:237–243.
3. Roberts WC, Filardo G, Ko JM. Comparison of total 12-lead QRS voltage in a vari-
ety of cardiac conditions and its usefulness in predicting increased cardiac mass.
Am J Cardiol 2013;112:904–909.
4. Klingberg E, Sveälv BG, Täng MS, Bech-Hanssen O, Forsblad-D’Elia H, Bergfeldt
L. Aortic regurgitation is common in ankylosing spondylitis: Time for routine
echocardiography evaluation? Am J Med 2015;128:1244–1250.
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Case 1729 Effect of Progressive Left Ventricular
Dilatation on Degree of Mitral Regurgitation
Secondary to Mitral Valve Prolapse
William C. Roberts, MD
Dan M. Meyer, MD
e,c
, and Shelley A. Hall, MD
a,b,c,
*, Paul A. Grayburn, MD
a,c
c
, Stuart R. Lander, MDd,
Descri bed herein is a 71-year-old man who at age 61 was found by echocard iogram
to have severe mitral regurgitation (MR) from mitral valve prolapse. During
the subsequent 9 years the MRprogressively lessened as his left ventricular
cavity dilated and his ejection fraction progressively fell such that just before
orthotopic heart transplantation the degree of MR was no longer severe, and
the prolapse of the mitral leaets had disappeared. This report describes this
unique patient. © 2019 Published by Elsevier Inc.
(Am J Cardiol 2019;123:1887–1888)
To our knowledge, the resolution of severe mitral regurgitation (MR) from mitral
valve prolapse (MVP) as the cardiac output progressively fell and the left ventricular
cavity progressively dilated has not been described. Such is the purpose of this
report.
CASE DESCRIPTION
A 71-year-old white man, who was born in December 1946, was told when in his
40s that he had a “heart murmur” from MVP. Because of the precordial murmur an
echocard iogram was done when he was 61years old (April2008), and it conrmed M VP
with marked leaet thickening, severe leaet prolapse, and severe MR; additionally,
the left ventricular size and function were normal. The tricuspid valve also had
evidence of prolapse. Thereafter, he was asymptomatic and working out regularly
with a trainer until age 70 (October2016), when experiencing an upper respiratory
infection, he also noted exertional dyspnea, orthopnea, and lower leg edema.
Examination in January 2017 disclosed no precordial murmur; echocardiogram
showed the left ventricular ejection fraction to be 20% (Figure 1). The thickened
mitral leaets were tented toward the left ventricu lar wall without prolapse and there
was moderate MR. The electrocardiogram showed atrial brillation, ventricular
premature complexes, left ventricular hypertrophy with strain, and prolonged
Q-T interval. Cardiac catheterization disclosed angiographically normal coronary
arteries and the following pressures (in mm Hg): mean pulmonary artery wedge 26;
right ventricle 35/3, mean right atrium 6; left ventricle 95/17, and aorta 105/75. The
cardiac index (Fick) was 1.3 L/min/m
2
. The left ventricular end-diastolic dimension
a
Baylor Scott & White Heart and Vascular Institute, Baylor Scott & White Health, Dallas,
b
Departments of Pathology, Baylor Scott & White Health, Dallas, Texas; cInternal
Tex a s;
Medicine (Division of Cardiology), Baylor University Medical Center, Baylor Scott& White
Health, Dallas, Texas;
White Health, Dallas, Texas; and
Health, Dallas, Texas. Manuscript received February26, 2019; and accepted February27, 2019.
*
Corresponding author: Tel: (214) 820–7911; fax (214) 820–7533.
E-mail address: William.Roberts1@bswhealth.org (W.C. Roberts).
d
Baylor Scott & White Heart and Vascular Hospital, Baylor Scott &
276 DOI: 10.1201/9781003409281-58
e
Department of Cardiac Surgery, Baylor Scott & White
CASE 1729 EFFECT OF PROGRESSIVE LEFT VENTRICULAR DILATATION
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Figure 1 Top left: Apical 4-chamber view at end-systole showing 4-chamber dila-
tion with thickened mitral leaets (arrows) that never prolapsed into the left atrium
(LA). The LA is bowed toward the right atrium (RA) consistent with high LA pressure. RV=right ventricle. Top right: Apical long-axis view showing thickened mitral
leaets (arrows) without prolapse. Bottom left: Apical long-axis view of centrally
directed mitral regurgitation (MR) jet. Bottom right: Continuous wave doppler of the
MRjet showing a low peak velocity—3.75m/s, suggesting very elevated LA pressure. The calculated EROA was 0.19cm
2
, a value suggesting only mild MR.
was 7.1cm, and its systolic diameter, 6.4cm. His B-type natriuretic peptide was 1440
pg/ml. He was placed for the rst time on full heart failure medications.
Repeat echocardiogram in November2017 showed the left ventricular ejection
fraction to be 10% and the MR was only of mild degree and no mitral prolapse
was seen. An intracardiac debrillator was inserted and the atrioventricular node
ablated. Heart failure medications and apixaban were continued. Because of lack of
improvement from either the medications or devices, orthotopic heart transplant
was performed in June2018. The explanted heart weighed 620 g. The epicardial
coronary arteries were devoid of any narrowing. The myocardium was devoid
of grossly visible lesions. The mitral valve leaets were classic for MVP (Barlow
syndrome type), and the tricuspid valve also had evidence of prolapse (Figure2).
When contacted in December2018, he was asymptomatic and back to work!
COMMENTS
Described herein is a patient with classic MVP known to be present for at least
3 decades. Several years before heart transplantation when the left ventricular
function was normal the echocardiogram showed severe MR. With time, the left
ventricular ejection fraction and cardiac output progressively fell, and the left
ventricular cavity progressively dilated such that just before heart transplantation
277
CASE REPORTS IN CARDIOLOGY
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Figure 2 Photograph of the mitral valve, typical of mitral valve prolapse, in the
explanted native heart. To p: View from the left atrium. Bottom: View of the opened
mitral valve.
there was no precordial murmur, no mitral valve prolapse, and only mild MRby
echocardiogram. As the left ventricular cavity dilated, the mitral chordae were
pulled laterally preventing the mitral leaets from prolapsing into the left atrial
cavity. Current guidelines support mitral valve repair before the left ventricular
ejection fraction falls below 60% to avoid the inevitable consequence of LV failure.
We are not aware of a similar published report describing the resolution of MVP
and severe reduction in MRfrom classic MVP with progressive worsening of left
ventricular function.
REFERENCE
1. Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP 3rd, Fleisher LA,
Jneid H, Mack MJ, McLeod CJ, O’Gara PT, Rigolin VH, Sundt TM 3rd, Thompson
A. 2017 AHA/ACC focused update of the 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College
of Cardiology/American Heart Association Task Force on Clinical Practice
Guidelines. J Am Coll Cardiol 2017;70:252–289.
278
1
CASE 1733 LIBMAN-SACKS ENDOCARDITIS INVOLVING A BIOPROSTHESIS
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Case 1733 Libman-Sacks Endocarditis Involving
a Bioprosthesis in the Aortic Valve Position
in Systemic Lupus Erythematosus
William C. Roberts, MD
Charles S. Roberts, MD
a,b,
*, Andy Y. Lee, MDb, Stuart R. Lander, MDb,
c
, and Baron L. Hamman, MD
c
Described herein is a 39-year-old man with systemic lupus erythematosus not
receiving corticosteroid therapy who developed Libman-Sacks endocarditis
causing stenosis of a bioprosthesis in the aortic valve position. © 2019 Elsevier
Inc. All rights reserved.
(Am J Cardiol 2019;124:316–318)
Libman and Sacks described what later became known as Libman-Sacks (L-S)
endocarditis in 1924,
described by Gross in 1940.
1
and its relation to systemic lupus erythematosus (SLE) was
2
Lipman and Sacks initially described 4 patients at
necropsy and noted t hat the endocarditis could i nvolve any of the 4 cardiac valves. The
“endocarditis” was described as deposits of brin not containing microorganisms
or leukocytes on either side of a valvular leaet but more commonly on the atrial
side (atrioventricular valve), or aortic side (semilunar valve). Once patients with SLE
were treated with corticosteroids the L-S brin lesions were uncommonly seen, the
medication presumably converting the brin deposits into brous thickenings.
3, 4
We recently encountered a man with known SLE not treated with corticosteroids
who developed aortic valve regurgitation, underwent replacement of that valve with
a bioprosthesis, which became stenotic because of development of L-S endocarditis
on both sides of the bioprosthetic cusps within 8 months of its implantation.
Adescription of this unusual patient is the purpose of this report.
CASE DESCRIPTION
A 39-year-old Hispanic man, who was born in April1979, had been well until age
26 when he noted tender nodules and a rash on his legs, and pain in some joints.
Adiagnosis of SLE was made. He was advised to take prednisone but during the
next 13years he failed to do so. At age 39, he developed signs of heart failure, was
found to have aortic regurgitation, and in May2018 underwent replacement of his
aortic valve with a bioprosthesis and insertion of a bypass conduit in his narrowed
left anterior descending coronary artery. About 6 months later, symptoms of heart
failure reoccurred and it rapidly progressed. On admission to Baylor University
Medical Center in December 2018, echocardiogram showed the velocity across
his bioprosthesis to be 3.9m/s; mean transbioprosthetic gradient 35mm Hg; left
a
Baylor Scott and White Heart and Vascular Institute, Departments of Internal Medicine
and Heart Surgery, Baylor University Medical Center, Baylor Scott and White Health, Dallas,
b
Departments of Internal Medicine (Division of Cardiology) and Cardiac Surgery,
Tex a s;
Baylor University Medical Center, Baylor Scott and White Health, Dallas, Texas; and
thoracic Surgery, Department of Cardiology, Baylor University Medical Center, Baylor Scott
and White Health, Dallas, Texas. Manuscript received and accepted April4, 2019.
*
Corresponding author: Tel: (214) 820–7911.
E-mail address: William.roberts1@bswhealth.org (W.C. Ro berts).
DOI: 10.1201/9781003409281-59 279
c
Cardio-
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