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					       Psoriasis, sacroiliitis, and aortitis: an
       echocardiographic mimic of aortic root
       D H Roller, W F Muna and A M Ross

       Chest 1979;75;641-643
       DOI 10.1378/chest.75.5.641

       The online version of this article, along with updated information and
       services can be found online on the World Wide Web at:

        CHEST is the official journal of the American College of Chest Physicians. It
        has been published monthly since 1935. Copyright 1979 by the American
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        (http://chestjournal.chestpubs.org/site/misc/reprints.xhtml) ISSN:0012-3692

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              © 1979 American College of Chest Physicians
                                                                                                                                                                  dissection           which                was         proven                 absent              by angiography        and
Psoriasis,                    Sacroiliitis,                                    and             Aortifis*
                                                                                                                                                                  at       surgery.       Rather,                      asymmetric                       flbrocalciflc          aortic    root
An Echocardiographic                                                 Mmic                    of Aortic                           Roof                             pathology            secondary                            to          systemic                connective      tissue   dis-
Dissecf           ion                                                                                                                                             ease was ultimately                             found                  to be etiologic.

Dean   H. Roller,              Walinjom
                  M.D.;#{176}#{176}                                                  F. Muna,                       M.D.;#{176}#{176}                                                                                  CASE                RBPORT
and Allan M. Ross, M.D.t                                                                                                                                              A 60-year-old      white man was hospitalized          following    an
                                                                                                                                                                  episode    of chest     pain, dyspnea,    and diaphoresis.        He was
                                                                                                                                                                  found   to be in pulmonary       edema    with an electrocardiogram
A patient             with          psoriasis,              HLA-B27-positlve                                          sacroililtis,
                                                                                                                                                                  showing    deeply    inverted  T waves in precordial         leads V2-V5
and    aortitls      presented      with     clinical       features      suggesting
                                                                                                                                                                  and periods       of A-V nodal      Wenckebach     rhythm.        He was
acute aortic dissecting            aneurysm.          Although        M-mode          and
                                                                                                                                                                  treated        as having            presumed            anterior       wall       subendocardial
two dimensional             echocardiography              supported         the diag-                                                                             myocardial            infarction,          although          cardiac      enzyme           levels      re-
nosis,     dissection       was excluded           by angiography              and by                                                                             mained         within normal limits. On the sixth day of hospitaliza-
direct     observation        at surgery.      Asymmetric            thickening         of                                                                        tion, a murmur               of aortic regurgitation                 was noted for the first
the right coronary           cusp secondary            to psoriatic      aortltis     was                                                                         time, and he was transferred                            to the West             Haven        Veterans
found to be etiologic              of both the regurgitation                   and the                                                                            Administration              Hospital         for further         evaluation.          There       was a
false-positive         echo for dissection.           It is emphasized            that in                                                                         past medical             history       of highly-expressed                psoriasis         including
the setting of any fibrocalcffic or inflammatory                           disease      of                                                                        peripheral           destructive           arthritis       treated       with       topical        corti-
the aorta,         the echo diagnosis              of dissection          is fraught                                                                              costeroid          creams,          as well          as systemic             salicylates,          indo-
                                                                                                                                                                  methacin,           and ibuprofen.                 There        was     a history           of labile
with        hazard.
                                                                                                                                                                  hypertension,            but no prior history                of heart disease. A 54-year-
                                                                                                                                                                  old brother            with       “spinal       arthritis”        and no known                  cardiac
E          chocardiography                     is proposed                          to be          an         accurate                pro-                         disease      had recently           died suddenly.
            cedure      for          establishing                    the           diagnosisof aortic     root                                                         Physical        examination            revealed         a mildly dyspneic                 middle-
dissection.1-6        Some investigators7                                        have suggested       that if                                                     aged man with normal body habitus.                                 The blood pressure                 was
                                                                                                                                                                   170/60        mm Hg in both anns and all peripheral                                    pulses      were
the echocardiographic               findings                                  are positive,  further    inva-
                                                                                                                                                                  brisk. The precordium                    was hyperdynaxnic                but without            thrills.
sive procedures            such    as angiography         may      be deferred.
                                                                                                                                                                  A grade           3/8      systolic        ejection        murmur          and a grade                2/6
However,         as with any new technologic                advance,        there                                                            is
                                                                                                                                                                  blowing          decrescendo              diastolic        murmur           were both heard
a need       for increased       awareness     of potential       false-positive                                                                                   along      the left sternal             border.        In addition,          a grade         1/6 low
and false-negative            diagnosis.                                                                                                                          frequency           diastolic         murmur          was audible             at the apex             and
    We report         an                unusual            case               which          both     clinically     and                                           interpreted          as an Austin-Flint                 murmur.        The extremities               and
echocardiographically                                  suggested                  the        diagnosis         of aortic                                          trunk      contained          numerous          well-demarcated                psoriatic       plaques
                                                                                                                                                                   and pitted,            discolored           nails      with      considerable            subungual
   #{176}Fromthe             Cardiology                   Section,                  Veterans                 Administration                                       hyperkeratosis.             Peripheral          arthritic       changes         were marked             by
         Hospital,    West    Haven,    Connecticut                                          and         Yale            University
                                                                                                                                                                  fusiform        swelling         of the phalanges,               and destructive               changes
         School    of Medicine,      New Haven.
   #{176}Post-Doctoral Fellow
#{176}                                             in Cardiology,                       Yale        University                   School                            at the proximal            and distal inter-phalangeal                    joints.
    of Medicine.                                                                                                                                                       Chest x-ray film showed                       moderate          cardiomegaly             (cardio-
   fAssociate    Professor                        of     Medicine,                    Yale         University                    School                            thoracic ratio = .55) and slight prominence                                    of the ascending
         of Medicine.
Reprint          requests:           Dr. Ross,              2150               Pennsylvania                         Avenue            NW,                          aorta. X-ray examination                     of the lumbo-sacral                spine and pelvis
Washington,             DC          20037                                                                                                                          revealed       sclerotic       sacroiliac        joints, but no abnormalities                    of the

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                               -lI_#{231}.                                                                                       ,


                                                                                                                                                                                       -‘-c-...              =#{231}                                  #{149}.

                                  Ficuna               1. Two             M-mode              echocardiograms       through       the base of the aortic   root. The second echo
                                  (B)        corresponds                      to     a more       cephalad     beam    angulation       than A, resulting  in different      aortic root
                                  and left atrial                 dimensions.                   The larger    arrows identify        an apparent  space between     parallel     anterior
                                  aortic echoes.                  The              smaller         arrows               identify         leaflet      motion                  in the    main               lunienal                    space       of the vessel.

CHEST, 75: 5, MAY, 1979                                                                                                                                                                                        PSORIASIS, SACROIIJITIS, AORTITJS 641

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                                                                 © 1979 American College of Chest Physicians
vertebral        bodies.       Peripheral            joint    x-ray films          disclosed        sub-
luxations,       flexion       contractures,            and     destructive          changes        sug-
gestive         of     psoriatic         arthritis.          A 99mTc-stannous                        pyro-
phosphate          myocardial           scintigram            displayed           no evidence             of
abnormal          uptake.       Histocompatibiity                   testing       showed        the pa-
tient      to be positive             for the HLA-B27                        antigen.         Serologic
testing      revealed          negative          VDRL,             rheumatoid             factor,       LE
preparation          and antinuclear             antibody.
    An M mode echocardiogram                              (Fig         1) revealed            a dilated
aortic root which on various views measured between 5 and 6
cm. There          was a persistent             additional           echo line parallel               with
and posterior           to the anterior             aortic wall, separated                   from that
wall by 1.5 cm. Most views showed                                 the space between                  these
parallel      lines to be echofree,                 although          with minimal             attenua-
tion some reflections                could be recorded                    from that area. The
aortic valve leaflet echoes                   were always               identified        behind       and
separate        from the two anterior                    aortic       echoes.       These       findings
were felt to be compatible                      with an anterior                 dissection         cham-
ber and a posterior              true lumen. Other features                         of the M mode
echo included              a normal         diameter           left atrium,            a dilated        left
ventricle        (diastole       6.8 cm) and a mitral                     valve demonstrating
 diastolic      fine flutter        and marked              premature             diastolic       closure
 consistent        with aortic         regurgitation             “A” waves             were seen on
 the mitral         echo       only during             the short            R-R       cycles       of the
Wenckebach              rhythm.
    Confirmation         of aortic dissection           was sought           by performing
a two dimensional             real-time     ultrasound         study       (Fig 2) using a
commercially          available       mechanical         sector      scanner.       Again     the
aorta appeared           as a dilated       “double        lumen”        structure       with a
persistent     bright      linear echo 1.5 cm behind                  the anterior        aortic
wall with fixed relationship                to that wall in systole.                Behind it,
fragments        of normally          moving       aortic leaflet tissue were re-
corded.     Long and short axis views were complimentary,                                    and
                                                                                                               Ficuna       3. Aortogram          in left anterior       oblique     position.        The
the image         interpreted         as a “false         lumen”        persisted        as the
                                                                                                               frame is chosen        from early systole          and shows the dilated             right
transducer         was angulated             superiorly         until     just     above      the
                                                                                                               coronary       cusp     (arrow)        which     remained       fixed    in diastolic
                                                                                                               configuration       throughout         the cardiac      cycle. Intense        filling of
                                                                                                               the left ventricle,         indicative      of severe aortic regurgitation,                is
                                                                                                               also evident.

                                                                                                               sinuses      of Valsalva         when      dropout        of all distinct         aortic    echoes
                                                                                                                   The patient         underwent          cardiac        catheterization             in prepara-
                                                                                                               tion for surgical           therapy       of acute severe             aortic       regurgitation
                                                                                                               and repair        of the presumably               responsible          dissection.          Biplane
                                                                                                               aortography,          however,        excluded          the diagnosis             of aortic       dis-
                                                                                                               section.     Instead,       a dilated       prolapsed         right coronary              cusp was
                                                                                                               demonstrated            (Fig 3) which             remained          fixed in its diastolic
                                                                                                               position      even during           ventricular         systole     when         left and non-
                                                                                                               coronary       leaflets     opened        normally.         Marked         incompetence              of
                                                                                                               the non-calcified            aortic    valve was noted.                The patient            subse-
                                                                                                               quently      underwent           uncomplicated              aortic     valve       replacement.
                                                                                                               At surgery,         there appeared             to be moderate              dilatation         of the
                                                                                                               aortic root, thickening              of the aortic walls above the sinuses                           of
                                                                                                               Valsalva       with loss of support                   of an enlarged                 right     aortic
                                                                                                               leaflet.   Pathologic         examination           of the excised            tissue revealed
                                                                                                               fibrous     scarring,        and inflammatory                changes         consistent          with
                                                                                                               the aortitis      of ankylosing          spondylitis.

Ficuna  2. A still frame                     taken       from     a two-dimensional                  real-                                             DISCUSSiON
time echo through     the                   aortic      root. A cardiac         silhouette         is              Echocardiography        has been shown’       to be a useful                               non-
sketched       to provide            orientation         for the sector     displayed.        The
larger     white      arrow            indicates        an apparent        anterior         aortic             invasive     tool in evaluating     aortic  valve     pathology,                                 and
space     between         the anterior                wall and a persistent                bright              several        reports        have       lauded         its    value       the
                                                                                                                                                                                           in   making
second     echo which         maintained               a fixed relationship          to the an-                diagnosis    of dissecting      aneurysms      affecting        the ascend-
terior    aortic     wall throughout                  the cardiac       cycle.      The small                  ing thoracic     aorta.    Diagnostic     criteria       that have been
double     arrow identifies        aortic           leaflet tissue in a more posterior
location.      These     two echoes               appropriately      merged        in diastole
                                                                                                               cited  as definitive       for aortic     dissection         include:     (1)
and parted        in systole.                                                                                  aortic root dilatation,       (2) widening        of the anterior       and/

642          ROLLER, MUNA, ROSS                                                                                                                                          CHEST, 75: 5, MAY, 1979

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                                               © 1979 American College of Chest Physicians
or posterior                  aortic
                         walls,      (3) maintenance          of parallelism                                                                              with      rupture      of the aneurysm                  into the right          atrium.        Am        J
between           separated
                      the           margins      of the walls.1,s         Other                                                                           Cardiol       30:427-431,              1972
findings    which    have been          suggested        as increasing       the                                                                    5 Krueger              SK, Starke H, Forker    AD, et al: Echocardio-
                                                                                                                                                      graphic              mimics of aortic root dissection. Chest 67:441-
specificity    of echocardiographic              diagnosis     are:    (4) in-
                                                                                                                                                      444, 1975
timal echo thinner          than outer        aortic    wall margin,         (5)
                                                                                                                                                    6 Drobinski           C, Nivet M, Kin C, et al: Echocardiographie
documentation                        of normal                   aortic        cusp         echoes           contained                    in
                                                                                                                                                          d’un      cas de dissection aortique. Nouv Presse  Med 5:570-
the       true       lumen.               Using           these           criteria,           Nanda               et al’          were                    572,      1976
able         to     make            the         correct            diagnosis                in      14       of         17      anglo-              7 Kronzon              I, Mehta         SS: Aortic           root   dissection.       Chest        65:88-
graphically-confirmed                                cases of aortic root dissection                                               with               89, 1974
three false negative                              and three false positive    results.                                             Mild             8 Gramiak              R, Shah             PM:       Echocardiography                of the       normal
fibrocalcific aortic valve disease was most often culpable                                                                                                and diseased           aortic         valve.     Radiology        96:1-8,       1970
for the latter error. Moothart        and colleagues,’0        Krueger                                                                              9 Nanda       NC, Lever         H, Gramiak         R, et al: Reliability              of
et al,’ and DiLuzio      and associates’1        have emphasized                                                                                      echocardiography            in the diagnosis          of aortic      root dissec-
                                                                                                                                                      tion. Circulation        56 (Suppl      III) :68, 1977
the additional  enhanced    specificity    engendered         by scan-
                                                                                                                                                   10 Moothart         RW, Spangler        RD, Blount SC: Echocardiog-
ning from the aorta to the mitral          valve,       if the double
                                                                                                                                                      raphy in aortic root dissection               and dilatation.         Am J Car-
aortic            echoes           can      be        shown          to be            discontinuous                       with         the
                                                                                                                                                      diol 36:11-16,        1975
septum              anteriorly              or the             mitral         anulus             posteriorly.                   Final-
                                                                                                                                                   11 DiLuzio       V, Purcaro        A, Boccanelli        A, et al: La diagnosi
ly, Krueger         et alh2                            and         Nicholson                  and           Coffs’3              have,                ecocardiografica          di dissezione       dell’aorta      toracica.       C Ital
respectively,         reported                             single      cases                  with                echocardio-                         Cardiol 6:677-685,            1976
graphic       demonstration                               of oscillating                    intixnal              flaps         in the             12 Krueger       SK, Wilson        CS, Weaver WF, et al: Aortic root
aortlc        root         secondary                  to aortic            dissections.                                                               dissection:       Echocardiographic           demonstration           of torn in-
       Our         patient           displayed                  many          of the     echocardiographic                                            timal flap. J Clin Ultrasound             4:35-39,       1976
signs         suggestive                   of         aortic         dissection.          The     aortitis  and                                    13 Nicholson        WJ, Cobbs        BW: Echocardiographic                 oscillating
                                                                                                                                                      flap in aortic root dissecting            aneurysm.        Chest 70:305-307,
consequent       aortic      regurgitation                                        which     were     noted   are
consistent      with      that     frequently                                       noted      in ankylosing
                                                                                                                                                   14     Bulkley   BH, Roberts WC: Ankylosing        spondylitis                                           and
spondylitis’4’”         In this condition,                                         the aortic      valve cusps
                                                                                                                                                          aortic regurgitation: Description of the characteristic                                           car-
and the aorta behind             and immediately        above    the sinuses                                                                              diovascular           lesion         from      study      of eight      necropsy          patients.
of Valsalva      are thickened           by adventitial     scar tissue    and                                                                        Circulation    48:1014-1027,                           1973
by intimal      fibrous      proliferation.      The scar tissue        in the                                                                     15 Zvaifler    NH, Weintraub                           AM:       Aortitis and          aortlc insuffi-
root of the aorta classically                                     extends   below the base of the                                                         ciency      in the      chronic            rheumatic       disorders.-A           reappraisal.
aortic  valve  to produce                                       a subaortic     fibrous ridge. The                                                        Arth Rheum             6:241-245,              1963
fibrous            thickening                of the             aortic         wall         behind              the          commis-
sures        causes           the         cusps          to sag          toward             the      left       ventricle.                It
is probable                  that the echo line on M mode                    and two-
dimensional                  scans thought      to represent       dissected    intima,
arose instead                   from the dilated,     thickened       and fixed right                                                              Endobronchial                                 Polyposis                     Secondary
coronary               leaflet;     the space     thought       to represent      “false                                                           to      Thermal                    Inhalational                          lnjury*
lumen”              was       in fact           the       sinus      of Valsalva.
    Thus,             not only   idiopathic                                  fibrocalcific                   degenerative                          Craig   Adams,     M.D.;   Ten-ence Moisan,                                 M.D.;
disease              of the aorta,     but                           in       addition                any              pathologic                  A. J. Chandrasekhar,        M.D.,  F.C.C.P.;                             and
entity             which           produces                    asymmetrical                      thickening                     of the             Raymond     Warpeha,     M.D.
right         or     non-coronary                         aortic           cusp         has        the       potential              for
mimicking                    the     echocardiographic                                stigmata               of aortic               dis-          A 28-year-old            man who sustained           inhalutlonal        injury      in
section.            Although                ultrasound                    remains             a valuable                      comple-              a house      fire developed          symptoms       of chronic         cough      and
ment          to      clinical             acumen                  and       angiography                      in        the       diag-            hemoptysis          requiring      bronchoscopy.          Two months            after
nosis        of dissecting                   aneurysm,             this               case again underscores                                       the initial      Injury,      numerous      endobronchial          polyps       were
the danger                   in ascribing                    an unduly                 high specificity  to the                                    found in the trachea              and throughout          the bronchial         tree.
technique.                                                                                                                                         His symptoms              have subsequently          improved         over a six.
                                                                                                                                                   month       period       while receiving        steroid      therapy.      To our
                                                       REFERENCES                                                                                  knowledge,         this delayed complication            of Inhalational         burn
                                                                                                                                                   Injury    has not been previously             reporteL
  1 Nanda              NC,      Cramiak     R, Shah                         PM:        Diagnosis                  of aortic root
         dissection            by echocardiography.                                   Circulation                  48:506-513,
         1973                                                                                                                                      p      ulmonary             injury    from thermal       accidents         contributes
  2 Yuste    P, Aza V,                                Minguez    I, et al:                           Dissecting                  aortic                   significantly             to the mortality       and morbidity                in the
    aneurysm      diagnosed                              by echocardiography.                                 Br              Heart            J   burn      patient.1          The acute pulmonary             complications             have
    36:111-113,      1974                                                                                                                          been       divided           on a temporal        basis     into    respiratory           in-
  3 Brown      OR, Popp                                RL,         Kloster            FE:          Echocardiographic
         criteria          for aortic             root         dissection.             Am         J Cardiol     36:17-20,                                   the
                                                                                                                                                   #{176}From Section                  of Pulmonary        Medicine  and Plastic Sur-
         1975                                                                                                                                       gery, Foster     C.               McGaw       Hospital    and Stritch  School    of
  4 Millward                   DK, Robinson    NF, Craig E:                                          Dissecting                   aortic            Medicine,    Loyola                University    of Chicago,    Maywood      IL.
                                                                                                                                                   Reprint          requests:            Dr.         Chandrasekhar,               2160       Sout1         First
    aneurysm                    diagnosed   by echocardiography                                                   in      a     patient            Avenue,          Maywood,             Illinois       60153

CHEST, 75: 5, MAY, 1979                                                                                                                                                                               ENDOBRONCHIAL                   POLYPOSIS             643
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                                                                       © 1979 American College of Chest Physicians
Psoriasis, sacroiliitis, and aortitis: an echocardiographic mimic of aortic root
                       D H Roller, W F Muna and A M Ross
                              Chest 1979;75; 641-643
                            DOI 10.1378/chest.75.5.641
              This information is current as of November 26, 2009

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