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Annulo-aortic ectasia with cystic medial necrosis
Annulo-Aortic
Ectasia
Medial Diagnosis DON W
with Cystic
Necrosis
and Surgical
Treatment
*
CHAPMAN, M D , F A c c , H LISTON BEAZLEY, M D , PAUL K PETERSON, M D . JOHN A WEBB, M D and DENTON A COOLEY, M D , F 4 c c Houston,
A
Texas
cendmg aorta and aortlc valve that demonstrate the characterlstlc clmlcal or pathologic process All patients were brought to olu- attention by the appearance of aortlc regurgrtatton resulting from dllatatlon and dlstortlon of the annulus of the aortlc valve The slgmficant cardiovascular chmcal findings, unique mformatlon obtamed from catheterlzatlon and anglogr aph), pathologic material, and suggested surgical treatment form the basis for this report (Table I)
LTHOUGH most aneurysms of the thoraclc aorta result from arterlosclerosls, syphlhs or trauma, some occur because of an mherent structural defect In the aortlc wall Itself Such lesions are frequently associated with skeletal and ophthalmic abnormahtles of the so-called ‘Marfan’s disease ” These aneurysms are generally considered to be a cardiovascular mamfestatlon of this syndrome The development of thoraclc aortlc aneurysms m assoclatlon with Marfan’s disease was first described by Baer etal’ln 1943 Mlcroscoplcally the basic process 1s one of medial degeneration previously described by Erdhelm2 as cystic medial necrosis Grossly these lesions usually begm at the annulus of the aortlc valve and terminate proximal to the origin of the mnommate artery 3 In some mstances the aortlc lesion 1s more extensive and involves the remainder of the thoraclc and abdominal aorta Several cases are reported m which the typical aortlc lesion was present m patients havmg a family hlstory of Marfan’s disease but lacking the tvplcal skeletal and ophthalmic findings This lesion has been described as the “forme fruste” or mcomplete form of Marfan’s disease 4 5 Ellis et al fi reported that m the maJorlty of the patients they had operated on with this pathologic lesion, the annulus was usually involved Therefore, the\ suggested the term “annuloaortic ectasia ” We would like to amend the termmology to designate It “annul0-aortlc ectdsla with cystic medial necrosis ” We encountered 16 cases lmolvmg the as-
AN~IYSIS
01 (:~SES
CLINICAL M4NIFEST4TIONS
Svmptomatzcally the most common mamfestatlons, were left ventricular failure and either exertlonal shortness of breath or nocturnal dy spnea , they were present m 14 of the cases Angina with typical substernal distress occurred m 11 cases Edema, palpltatlon and fatigue were noted m 5 cases Dlssectlon of the aorta was encountered In 3 cases and uas ushered m with a severe back pain located Inltlally between the scapulae On examznatzon, all 16 revealed left 1 entrlcular hypertrophy and all had an aortlc ejection systohc murmur usually transmitted to the carotid vessels Aortlc decrescendo dlastohc murmurs were umformly present together with all the usual peripheral signs of aortlc requro;ltation In 1 of the patients with dlssectmg aneurysm, a systolic murmur of a “mooing” t\ pe \Ias present Although Stembridge et al 7 attributed a slmllar murmur (Hodgkm-Kevs) to central weakemng
* From the Departments of Medune and Pathology and the Cora and Webb Madlng Department of surgery, Baylor Unlverslty College of MedIcme, and the Methodist and St Luke’s Hospital, Houston Texas This qtudv was supported m part by the T L Miller Cardmvascular Research Fund VOLUME
16,
NOVEMBER
1965
679
680
Chapman
et al
-I ABLEI
Caw 1
2
‘ige 8 Sex 35 F
4X M
Skeleton
Obe
e
Normal
STS
Nee,
NW
S\ mptoms
s, ncope-3 L\‘F-l
8. D,,r ,t,“o
>r vr
45 M
4rachnodactyIy
Neg
L\ F-2
4
36 F
Arachnoddct,ly and pectus ewavatutn
Neg
Faugue-2 \r LVF-1 yr 4ngma-1 yr Palmt‘it1on-1 RtiF-1 m” Fatigue-6 m” Angma-3 mo LVF-2 m”
m”
5 \r
’
5
35 F
Arachnodactyly
Neg
6
35 M
Norm11
Neg
Fattgue-2 “r LVF-1 m” Palpltatlon-1
7
46
Normal
Nets
Angma-3 LVF-1
\I r yr
8
6X F
NOtXidl
Neg
Angma-2 LHF-1 RHF-6
yr yr m”
9
44
M
Normal
Neg
Diss aneur Apr LVF-10 davs Angma-1 day
10
30 M
Normal
Neg
LHF-1
11
47
Normal
Neg
Angma-5 \ r Pam v.lth radtatmn legs-3 n h
F
M
12
71
M
13
33 M
yr
1963
yr
Ectomorph
Neg
Angma-4 LHF-2 RHF-6
Normal
Neg
Fattgue-1 yr DOE-6 m” Pam substernally between shoulders Famtmg Palpltatlon-1 yr Fattgue-2 m” DOE-l m”
Neg
44 M
NOrlIla
N-x
Angma-3
33 M
Normal
Neg
Fatigue-1 yr DOE-3 yr Parox noct dysp Palpttatmn-3 yr Edema-6 wk Angma
48
15
16
F
to
yr yr mo
Norm4
14
LCG
LHB 44L AShf I\ .‘.DM I\ BP = 114 6X LHB h14L AShi 111 4DM III BP = 14010
Fatigue-2 \r D\spnei-1 >r Palp1tatton-2 yr Angma-I vr
3
He‘lrt
wk
-1 yr
Intraventrrculnr bloch
LVH
& str L,”
LGH DI
ascendmg 1ort 1
LHB AAL ASM 111 ADM 111
Nonapecthc T nave changes
L\/H
LHB MAL ASM III 4Dhl IV BP = 140/20
LVH
LVH D&ted ascendmg aorta
LHB AAL ASM I ADM ‘11 BP = 130,‘50/20 LHB-AAL SM III DM 111 BP = 140/70 LHB-AAL ASM v 4DM III BP = 170/50 LHB-MAL ASM III ADM I\ BP = 180/70 LHB MAL 4SM III 4DM I\ BP = 90/46 Combmed fatlure LHB-AAL ASM 111 ADM III BP = 166150 LHB AAL ASM tt1 AD&f 111 BP = 156/60
Normnl
LVH
LVH
LVH
L\Ii
LVH Large aneurysm m ascendmg aorta & arch LVH Dtlated ascend mg aorta
LHB MAL AShl 1t1 ADM I” BP = 145/70 LHB MAL SM III DM w BP = 160/‘70
LVH
LVH
& stram
LBBB
LVH D&ted ascend mg aorta
LVH
LVH Dtlated asccndmg aorta
LVH
LVH
LVH (Normal root)
aorttc
Normal
Borderhoe
LVH
Mmor ST changes
Dtlated aorta
LVH
LVH Large aortlc pulsations
LAD Nonspectfic wave changes
LHB AAL ASM III ADM I” BP = 135/65 LHB MCL + 2 cm ASM III ADM IV BP = 145/75 LHB-MCL + 3 cm ASM II ADM IV BP = 160/50/40
LVH Pulmonar) edema
& stram
T
ascendmg
ABBREVIATIONS STS - semloglc test for syphllts hypertrophy LVH = left ventncular AAL = antenor aullary Ime
MAL = mid-axtllary lme LHB _ left heart border LBBB = left bundle branch
ASM = aorhc systohc murmur ADM = aorttc dtastohc murmur AI = a”r”c rosuffiaency
block
THE
AMERICAN
JOURNAL
OF
CARDIOLOGY
Annulo-;lortlc T4BLE
Ectasla
I (&~ntznued)
Operanon
Parhologu
& Course
Fmdmq,
4neur dddtanon up to mnomm~te *rtery Sclerouc \‘d\es ,ncompetent
4/29~64 ti”\en tu No 5 Magovern v ~l\e bular Dacron% graft Died 9 19 64
C\qnc me&l1 necrobl?. 4utopay Intact value 0 rhythm chstur bmce Post mroc hemorrhage
Not don?
Furlform, 10 cm rysm ascendmg ,nate artery 41
Cysuc medul necrosis 411top\\ renal mLeft cerebral mfdrctmn pulmonary congest,“” iarcuon wsceral congestion
Pear-shaped aneuqsm of ascrndmg aorta 41
Fwform
11/4/64 No 9 Starr-Edwards valve, 25 mm graft Died Wove” tubular right hermplegm com1 l/8/64 bmed fadure 5/20/64 30 mm No 3 Magovem valve Ahve woven Dacron tubular graft & norL1ng 11/23/64 No 11 Starr-Edwards \al\.e, 25 Died mm woven Dacron graft 11/24/64 hemomechastmum 24 hr
4utopsy Cysuc medial necro~~b Hemomedmstmum cdrdlomegalv
7 cm aneurysmal dllatatmn of ascendmg aorta to ,ust short of mValve thlckened ““inmate arteq & retracted 7 cm aneur ddatatlon ascendmg ~“rta bhort of mnommate arrery D&ted annulus AI
6/28/64 No 4 Magovern r&e 22 mm woven Dacron graft Ah\e & worL1ng 10/16/64 No 13 Starr-Edwards val\e DaAhve & w”rl\mg cron woven graft
Cysuc
medml
necro~ls
Cysnc
mrchal
ne~r”hl’
Large aneurysm awendmg & aoruc Supermr aspect of arch arch strip tram which 3 great \easels arose was normal AI -Ineur\sm ascenchnq aorta 41
4/29/64 No 4 Magwern valre 25 mm Alne woven Dacron graft
9 5 cm dlametrr m&al necrosis leaflet
10/26/64 13 No 11 Starr Ed**ards \Irl~e mm wore” Dacron prosthea Ahve 7/l/64 25 mm No 4 Magovern valve Dted 9/20/64 woven Dacron graft
Annulo noruc ectau necro~u cnlahed
PI tr ,haped ,rcwhng
ddatatlon ?“rtl AI
aorta
ennal spht of mtlma and media Prolapse of right posterior commlsam-e AI Fwform aneurvsm ascendmg aorta AI
chlatatlon aorta AI
Not don?
Huge ~neur”bm ascendmg aorta and arch Marked AI Not done
Not
ascendmg
-fake fypconnrrysm ~/IOcucumfer-
Not done
Pear shaped ascendmg
aneurysm
m dmmeter aneuaorta up to mnomCusps shortened
double lumen D~~sectrng onuryrm mldpomt ascendmg aorta stoppmg below mnommate artery
done
aneuqsm 41
7/g/64 No 4 Magovern valve, w”\cn DaAhbe & workmg cron graft
Not done
Foalform torta
of ascendmg
Large, 10 7 cm m dmmeter pear-shaped aneurysm m\o1wng the root and proumal a0rt.l 41
Fwform aneurysm clrssectmg \*ah a double lumen distally mrolvmg a/~ DlSSeCtl”” of the cncumference prox to wlthm 1 cm of v&e V&e cusps prolapsed AI
Not done
Fuslform aneurysm ascendmg aorta ends proximal to mnommate artery 41 Dlssectmn-aorta Mlth hemoperl cardmm & &tally
Pear shaped annulo a”rt,c aneurysm
AI
Pear-shaped annuloa”rt,c aneurysm
AI
Pear-shaped ddatatlon of Sm”seS of Valsalva LVH Masswe a”rt,c regurgltatlon
LHF = left heart fallore RHF = right heart falure BP = blood pressure
VOLUME
16,
NOVEMBER
DOE = dypsnca DI = d,lated
1965
7/31/64 Resection from 3 mm distal I” annulus 1” 2 cm below mnommate arter) two co %ts muted cbst~lly 19 mm woven Dacron graft, No 5 Magovern valve Ahve & w”rLmg No 7 Starr Edwards valve woven 4h\ e Dacron graft
No 13 Starr Edwvds \al\e Dacron grafr Alive
Not operated
upon
Not operated
upon
Not operated
upon
on exert,“”
MCL Parox
woven
-
1
Cyatlc
me&al
necroblh
to annulus cvsuc hbrotlc valve
c)snc medial hbrous valve
hbraals & hplds of u?ll card~omegaly = 900 Partml occlusl”” of pros gm thesu +2’ dnteroseptal myocar dml mfarcuon Dissect aneuqsm C\stx medml necro~lr Dlssect~on Autopsy
Cysuc medkd necroals sxcular lated aneurysm with dw.ectmn
dl-
hbrorls
of
Cystic me&al necrosis 1oruc Cd1b.e
Cystw medlal necrow, (hemoperrcdrdmm aneurysm)
mid clav~ular lme noct dysp = paroxysmal
hbrotlc valve & dtssectmg
nocturnal
dypsnra
Chapman
et al.
A
B
FIG. 1. Case 3. Angiocardiograms show pear-shaped aneurysmal dilatation and aortic regurgitant jet. A, posteroanterior view. B, lateral view.
and rolling down of the thickened right anterior aortic cusp, McKusick et a1.8 suggested that murmurs of this description may be produced either by fiddle-string bands traversing the false channel in the ascending aorta or by vibrations of the lips of the cusps created at the site of the initiating intimal tear. This musical murmur when located at the base of the neck and below the clavicle on the right, especially if it develops abruptly or changes, may be a sign suggesting dissecting aneurysm. Of the 16 cases with proved cardiovascular abnormalities, only 3 patients displayed the skeletal manifestations of Marfan’s typical syndrome. LABORATORY
FINDINGS
The eleztrocardiograns were not diagnostic and in general reflected evidence of left ventricular hypertrophy secondary to aortic insufficiency. In 9 of the 16 cases electrocardiographic evidence of left ventricular hypertrophy was present, and in 4 of these there was associated left ventricular strain. Left bundle branch block and intraventricular conduction disturbance each occurred once. Nonspecific S-T segment and T wave changes were seen in 3. All patients had sinus rhythm when initially seen. Bowers9 on reviewing the electrocardiographic
of the ascending aorta
abnormalities in Marfan’s syndrome also noted nonspecific electrocardiographic changes. These consisted mainly of left ventricular hypertrophy, incomplete right bundle branch block, nonspecific S-T and T wave changes, and atria1 fibrillation. He attributed these changes to a degenerated aorta with or without an aneurysm and secondary aortic insufficiency, diffuse myocardial hypertrophy and fibrosis, or to the cardiovascular complications secondary to kyphoscoliosis and pectus excavatum. Teleroentgenograms revealed some degree of left ventricular hypertrophy in all cases ; a dilated ascending aorta was present in nine of the sixteen A brisk pulsation of the ascending aorta, cases. a “rocking chair” phenomenon, was noted in 9 patients. Retrograde catheterization studies, employing the percutaneous femoral method of Seldinger’O or the direct brachial cut-down technic of Sones Injecand Shirey, I1 were made of 9 of 16 cases. tion of Angio-Conray @ dye was made into the root of the aorta, and in all 9, marked aneurysmal dilatation of the ascending aorta was The pear-like dilatation’* noted (Fig. 1 and 2). started in the intrapericardial region at the annulus and extended upward proximal to the innominate artery except in 1 case in which a dissection extended into the aortic arch. The THE
AMERICAN
JOURNAL
OF
CARDIOLOGY
Annulo-Aortic
A
Ectasia
683
B
B, angiogram shows pear-shaped dilatation of the ascending aorta with aortic regurgitation of dye into the left ventricle. FIG. 3.
Case 5.
A, teleoroentgenogram shows aneurysmal dilatation of the ascending aorta.
dyt:
frequently demonstrated the annular dilatacould not have been seen on the ro1:rtine roentgenogram and was thought to be dia gnostic of the conditionr” (Fig. 3). This was the most common and the earliest cardiovascular ectasia with cystic am lmaly in annulo-aortic
tiol n which
VOLUME
16,
NOVEMBER
1965
medial necrosis. Aortic regurgitation in var ‘Ying degrees was present in all. Although the lesion may not be detectable in the routine roentgenograms, anterior and later -al displacement of the pulmonary artery segme :nt should arouse the physician’s suspicion. T he
684
Chapman et al.
’ stromal substance.
displacement may result from dilatation of the aortic root and the ascending aorta. All serologic tests for syphilis were negative. PATHOLOGIC
FINDINGS
The pathologic cardiovascular ““abnormalities5J4-‘6 seen with the so-called Marfan’s syndrome may include : Cystic medial necrosis of the aorta and pulmonary artery; dilatation of saccular and dissecting aneuthe aortic root; rysms of the ascending aorta (more rarely of the arch or distally) ; sacculation’, rolling or redundancy of aortic cusps ; upward displacement of aortic cusps ; upward displacement of coroor cardiomegaly associated with nary ostia; other congenital defects such as atria1 or ventricular septal defects or patent ductus arteriosus: According to Weaver and associates5 changes other than cystic medial necrosis of the aorta are not seen in so-called idiopathic aortic dissection or aortic dissection with pregnancy.
Cystic medial necrosis in varying degrees of advancement was seen in all 13 patients operated on. In some cases the degeneration consisted merely of fragmentation and loss of elastic fibers with accumulation of basophilic ground substance or mucopolysaccharides within these areas. Usually, however, these cystic areas within the media were extensive, with degeneration and loss of elastic fibers (Fig. 4, A and B). Most of the cases revealed almost no atherosclerosis, although some had a slight amount. Histochemical studies of the mucoid ground substance in the aortic cystic areas demonstrated this material to be acid mucopolysaccharides; it is strongly positive with Alcian blue stain. The heart valves in every instance revealed considerable thickening throughout their length with laminated mature fibrous tissue, as well as considerable nodular areas of “basophilic” degeneration of the ground substance (Fig. 4C). These nodular areas contained accumulations THE AMERICAN
JOURNAL
OF
CARDIOLOGY
Annulo-Aortlc In none of these valves was of stellate cells calclficatlon or osslficatlon observed M o,f the dzssectznguneurysms had severe medlal c\stlc necrosis of the aorta with rupture m the central portIon In 1 case It was rupturmg slowly, as evidenced by granulation tissue wlthm The remammg aortas, away from the rent the dlssectlon sites, were mdlstmgulshable from those of other cases m which dlssectlon had not occurred 5LRGICAL
CONSIDER4TIONS
Technzc Operation upon the ascending aorta and aortlc valve was performed durmg total cardlopulmonary bypass In every case m the study the slmphfied method previously reported by Cooley and assoclates’7 was employed use of plastic disposable ox\genators (Travenol Laboratones), pnmmg the extracorporeal system exclusively with 5% dextrose m dlstllled water, and maintenance of normothermla In most patients with annulo-aortic ectasla requmng prolonged bypass of one hour or longer, body temperature gradually fell to 94 or 92” at the completion This level of hypothermia probably did of bypass not influence slgnlficantly the effectiveness of the Full heparmlzatlon of the patient tissue perfuslon was mamtamed durmg bypass with 3 0 mg /kg bode weight At the termmatlon of bypass, the heparm was counteracted with protamme sulfate, 3 5 mg /kg body weight A median sternotomy provided excellent exposure Venous outflow was of the aorta and aortlc valve obtained by mdlvldual cannulatlon of the venae Lavae and artenal return by a cannula m the common A sump-type suction placed m the femoral artery apex of the left ventricle provided a “dry” operative field by asplratmg bronchial and coronary return Only the left coronary artery was perfused durmg the period of open cardlotomy or aornc cross-clamping 4 small metal-tipped catheter was placed m the coronarv or&ice from mslde the open aorta and secured m place with a tourmquet placed around the artery outside the aorta posteriorly Rate of coronary perfuslon was controlled entirely by momtormg presbure m the tubing at 8&100 mm Hg Usually the heart maintained smus rhythm throughout the operation, but m some instances ventncular fibnllatlon occurred Defibnllatlon was easily achieved after the aorta was closed when a direct current defibnllator was employed After complete cardiopulmonary bypass was started, an artenal clamp was placed across the ascendmg aorta proxunal to the ongm of the mnommate artery The aneurysm was opened widely and excmon was performed Removal of the entire sac from the adjacent pulmonary artery was not always possible, and often a portion was left attached to the artery to avoid unnecessary arterial damage requn-mg 1epalr and prolongation of the operation The aortlc VOLUME
16,
NOVEMBER
1965
Ectasla
685
leaflets were removed completely, and a ball valve prosthesis of the Starr-Edwards or Magovern-Cromle type was used Our preference IS for the StarrEdwards prosthesis which depends upon suture fixation After the valve was replaced, a segment of woven, lmpervlous Dacron@ tubmg was inserted m the ascending aorta UsualI, the proximal anastomosls was done first, using a diagonal dlvlslon of the graft to accommodate the wide mouthed annulus Meticulous suture techmc Nlth 3-O Mersllene sutures (Ethlcon Laboratones) was used to reduce bleeding after operation The distal anastomosls was completed last After bypass was dlscontmued, hemostasls was obtamed with addltlonal sutures m the anastomoses reThe thorainforced by absorbable cellulose sponge cotomy mclslon was closed and waterseal dramage provided In 2 patients with aneurysmal dllatatlon extending mto the transverse aortlc arch, It was necessary to apply the occludmg aortlc clamp distal to the ongm of the left subclavlan arter) Smce this excluded the carotid arteries from the cannulatlon, it was necessary to perfuse the mnommate and left common carotid arteries with a separate pump A combined flou mto both carotid arteries of 400-500 cc /mm was sufficient to mamtam a normal electroencephalographic pattern In both patients, an earlv recovery from anesthesia was noted, and no cerebral comphcatlons occurred after operation Replacement of the transverse aortlc arch was performed durmg temporary carotid perfusion
Complzcatlons followmg operation were mfrequent Persistent bleedmg with hemoperlcardmm made emergency thoracotomy necessary in 2 patients In another patlent complete heart block developed owing to Injury to the A-V conducting system A pacemaker was implanted SIX weeks later because of the onset of Stokes-Adams episodes, and she has made excellent recovery Operatzve Results Of 13 patients operated on, One pa9 are hvmg and domg well (Fig 5) tlent died wlthm 24 hours of postoperative hemorrhage, mcludmg left hemlthorau, right mtrapulmomc hemorrhage and hemomedlasUnum A second patient died on the fourth postoperatlve day of multiple emboh to the left cerebral hemaphere, large and small Intestines, and kidneys These emboh arose from the prosthetic valves, which were well seated m both of these patients Two other patients died suddenly m their third postoperatlbe months Autopsy of the first patlent revealed a partial occlusion of the ball valve prosthesis with thrombosis extendmg upward and partially occludmg the left coronary
Chapman
686
FIG. 5. Case 5. Postoperative film shows disappearance of aneurysmal dilatation and emplacement of the prosthetic valve (cf. with Fig. 3A).
artery. This patient had a recent anteroseptal myocardial infarction. In the second patient no evidence of occlusion of the coronary artery was noted at autopsy, but a soft posterior surface of the heart was present with some hemorrhage in it. It is postulated that the coronary orifice may have been temporarily occluded leading to possible myocardial infarction, and a sudden rhythm disturbance may have been the final cause of death.
DISCUSSION Although knowledge of the manifestations of the Marfan’s syndrome are widely known, too often the syndrome is diagnosed only in its classic form. One must be alerted to the lesser manifestation, the forme fruste feature of aortic insufficiency with a dilated aortic root. Annulo-aortic ectasia with aortic insufficiency ,should be considered in the differential diagnosis of all cases of aortic insufficiency. The lack of skeletal deformities, ectopic lens or a family history of Marfan’s syndrome need not deter the diagnosis, and these may be absent also with the so-called forme fruste of Marfan’s cystic medial syndrome. The 1characteristic
et al. necrosis was universally present. Aortic regurgitation was also seen in all of our patients, resulting from saccular dilatation of the annulus, thickened nodulation or bacterial endocarditis of the CUSPS leading to dilatation and distortion of the aortic valve. Physical examination revealed predominantly the findings of aortic regurgitation. Routine roentgenograms may not demonstrate the lesion, but if there is anterior or lateral displacement of the pulmonary artery segment, it should arouse suspicion of a dilated aortic root. Retrograde arteriography may demonstrate dilatation of the annulo-aortic region. Differentiation before surgery between this lesion and pure aortic valvular insufficiency is of importance to facilitate technical preparation for the more complicated procedure. The lesion of annulo-aortic ectasia is unusual in that it may present a double threat to life. Death may result from intractable cardiac failure initially manifesting itself as left heart failure and ultimately progressing to combined failure secondary to aortic insufficiency. Secondly, the thin-walled aortic aneurysm may rupture or dissect, resulting in fatal hemorrhage into the pericardium, pleura, right atrium, right ventricle, pulmonary artery, or left ventricle. Surgical treatment should be reserved for those patients in whom the lesion is life-threatening. If there is progressive dilatation of the ascending aorta with potential rupture or if severe aortic regurgitation with progressive congestive failure develops, surgery may be indicated. In early cases of ectasia, operation should be withheld until progress of the disease can be demonstrated.
Annulo-aortic ectasia is a term first suggested by Ellis and Cooley for an aneurysmal dilatation of the ascending aorta and aortic annulus frequently associated with aortic regurgitation. The basic structural defect in the aortic wall is cystic medial necrosis which may or may not be accompanied by stigmata of Marfan’s syndrome. Thirteen cases fulfilling the above criteria were operated on with four deaths, and three additional cases have been studied. When the patient’s diagnosis was uncertain, retrograde angioaortography demonstrated characteristic pear-shaped dilatation of the annulo-aortic region. Surgical treatment consisted of excision of the aneurysm with replacement by a woven Dacron THE AMERICAN
JOURNAL
OF CARDIOLOGY
Annulo-Aortlc yraft and correction of the aortlc regurgitation bv pi osthetlc 1 alve REFERENCES 1 BOER, R E
H
aorta
ii’,
T~USSIG, H
Congemtal associated
G
9 10
and OPPENHEIMER,
aneurysmal dllatatlon wth arachnodactyly
of the
Bull Johns Hopktns Hasp, 72 309, 1946 2 ERDHEIM, J Medlonecrosls aortae ldlopathlca cystlca Vmhows 4rch Path Anat, 276 187, 1930 3 \VAGENVOORT, C 4 NEUFELD, H N and EDWARDS, J E Cardiovascular system m Marfan’s syndrome and m ldlopathm dllatatlon of the ascendmg aorta Am J Card&, 9 496, 1962 The forme fruste m 4 GOLDEN,R L and LochIN, H Marfan’s syndrome New England J Med, 260 797, 1959 5 WEAVER, W F , EDWARDS,J E and BRANDENBURG, R 0 Idlopathlc dllatatlon of the aorta with aortic valvular msufficlency A possible forme fruste of Marfan’s syndrome Pm Meet Mayo Cltn , 34 518, 1959 6 ELLIS, P R , COOLEY, D A and DEBAKEY, M E Chmcal conslderatlon and surgmal treatment of annulo-aortic ectasla J Thoram & Cardaovas Surg , 42 363, 1961 7 STEMBRIDGE, V A, HEJTMANCIK, M R and HERRMANN, G Unusual musmal murmurs of anterior aortic origin Am Heart J, 48 163, 1954 8 McKusnx, V A, LOCUE, R B and BAHNSON,H T 4ssoclatlon of aortm valvular disease and cystm
VOLUME 16, NOVEMBER1965
Ectasla
11
12
13
14
15 16
17
687
( I?,ulrillon medlal necrosn of ascendmg aortrl 16 188 1957 The eleCtrOCdrdlOgrdm m M,nfdn’\ BONERS, D syndromt rln1 J Cardml, 7 661 1961 SELDIUGER, S I Catheter replacement of the needle in percutaneous artenogrdph\ lrtu mdzol 39 368 1953 C21ucLOlOSONES, F M , JR and SHIREY E K nary arterlography Mod Concepts C ardmr us Drs , 31 735, 1962 BARRETT, J S , HEL~IG, J , JR KA\ C F and JOHNSON, J Cme-aortographlc e\ dludtion of aortlc msufficlency , unsuspected ldlopathlc aneurysmal dllatatlon of the aortlc root as a posstble mdlcatlon of the Marfan syndrome Ann Znt &fed, 61 1071 1964 STEINBERG,I, MANGIARDI, J L dnd NOBLL \li J Aneurysmal dllatatlon of the dortlc smuses m Marfan’s syndrome (anglocardlographlc and cardiac catheterlzatlon studies m ldentlcal twins) Cmulatton, 16 368, 1957 4cld mucopolyBUNTING,C H and BUNTING H saccharides of the aorta Arch Path 55 257, 1953 GORE I Dlssectmg aneurysm of the dorta under forty years of age Arch Path, 55 1, 1953 PE4RSE, 4 G E Hlstochemlstry, Theoretmal and Applied, p 106 Boston, 1953 Little Brown and Company COOLER, D A BEALL, 4 C , JR and GRONDIN, 0 M Open heart surgery by a slmphlied technic using disposable oxygenators, 5 percent dextrose solution prime and normothermm Ma1 Cardzouas , 4 455, 1963
Ectasia
Medial Diagnosis DON W
with Cystic
Necrosis
and Surgical
Treatment
*
CHAPMAN, M D , F A c c , H LISTON BEAZLEY, M D , PAUL K PETERSON, M D . JOHN A WEBB, M D and DENTON A COOLEY, M D , F 4 c c Houston,
A
Texas
cendmg aorta and aortlc valve that demonstrate the characterlstlc clmlcal or pathologic process All patients were brought to olu- attention by the appearance of aortlc regurgrtatton resulting from dllatatlon and dlstortlon of the annulus of the aortlc valve The slgmficant cardiovascular chmcal findings, unique mformatlon obtamed from catheterlzatlon and anglogr aph), pathologic material, and suggested surgical treatment form the basis for this report (Table I)
LTHOUGH most aneurysms of the thoraclc aorta result from arterlosclerosls, syphlhs or trauma, some occur because of an mherent structural defect In the aortlc wall Itself Such lesions are frequently associated with skeletal and ophthalmic abnormahtles of the so-called ‘Marfan’s disease ” These aneurysms are generally considered to be a cardiovascular mamfestatlon of this syndrome The development of thoraclc aortlc aneurysms m assoclatlon with Marfan’s disease was first described by Baer etal’ln 1943 Mlcroscoplcally the basic process 1s one of medial degeneration previously described by Erdhelm2 as cystic medial necrosis Grossly these lesions usually begm at the annulus of the aortlc valve and terminate proximal to the origin of the mnommate artery 3 In some mstances the aortlc lesion 1s more extensive and involves the remainder of the thoraclc and abdominal aorta Several cases are reported m which the typical aortlc lesion was present m patients havmg a family hlstory of Marfan’s disease but lacking the tvplcal skeletal and ophthalmic findings This lesion has been described as the “forme fruste” or mcomplete form of Marfan’s disease 4 5 Ellis et al fi reported that m the maJorlty of the patients they had operated on with this pathologic lesion, the annulus was usually involved Therefore, the\ suggested the term “annuloaortic ectasia ” We would like to amend the termmology to designate It “annul0-aortlc ectdsla with cystic medial necrosis ” We encountered 16 cases lmolvmg the as-
AN~IYSIS
01 (:~SES
CLINICAL M4NIFEST4TIONS
Svmptomatzcally the most common mamfestatlons, were left ventricular failure and either exertlonal shortness of breath or nocturnal dy spnea , they were present m 14 of the cases Angina with typical substernal distress occurred m 11 cases Edema, palpltatlon and fatigue were noted m 5 cases Dlssectlon of the aorta was encountered In 3 cases and uas ushered m with a severe back pain located Inltlally between the scapulae On examznatzon, all 16 revealed left 1 entrlcular hypertrophy and all had an aortlc ejection systohc murmur usually transmitted to the carotid vessels Aortlc decrescendo dlastohc murmurs were umformly present together with all the usual peripheral signs of aortlc requro;ltation In 1 of the patients with dlssectmg aneurysm, a systolic murmur of a “mooing” t\ pe \Ias present Although Stembridge et al 7 attributed a slmllar murmur (Hodgkm-Kevs) to central weakemng
* From the Departments of Medune and Pathology and the Cora and Webb Madlng Department of surgery, Baylor Unlverslty College of MedIcme, and the Methodist and St Luke’s Hospital, Houston Texas This qtudv was supported m part by the T L Miller Cardmvascular Research Fund VOLUME
16,
NOVEMBER
1965
679
680
Chapman
et al
-I ABLEI
Caw 1
2
‘ige 8 Sex 35 F
4X M
Skeleton
Obe
e
Normal
STS
Nee,
NW
S\ mptoms
s, ncope-3 L\‘F-l
8. D,,r ,t,“o
>r vr
45 M
4rachnodactyIy
Neg
L\ F-2
4
36 F
Arachnoddct,ly and pectus ewavatutn
Neg
Faugue-2 \r LVF-1 yr 4ngma-1 yr Palmt‘it1on-1 RtiF-1 m” Fatigue-6 m” Angma-3 mo LVF-2 m”
m”
5 \r
’
5
35 F
Arachnodactyly
Neg
6
35 M
Norm11
Neg
Fattgue-2 “r LVF-1 m” Palpltatlon-1
7
46
Normal
Nets
Angma-3 LVF-1
\I r yr
8
6X F
NOtXidl
Neg
Angma-2 LHF-1 RHF-6
yr yr m”
9
44
M
Normal
Neg
Diss aneur Apr LVF-10 davs Angma-1 day
10
30 M
Normal
Neg
LHF-1
11
47
Normal
Neg
Angma-5 \ r Pam v.lth radtatmn legs-3 n h
F
M
12
71
M
13
33 M
yr
1963
yr
Ectomorph
Neg
Angma-4 LHF-2 RHF-6
Normal
Neg
Fattgue-1 yr DOE-6 m” Pam substernally between shoulders Famtmg Palpltatlon-1 yr Fattgue-2 m” DOE-l m”
Neg
44 M
NOrlIla
N-x
Angma-3
33 M
Normal
Neg
Fatigue-1 yr DOE-3 yr Parox noct dysp Palpttatmn-3 yr Edema-6 wk Angma
48
15
16
F
to
yr yr mo
Norm4
14
LCG
LHB 44L AShf I\ .‘.DM I\ BP = 114 6X LHB h14L AShi 111 4DM III BP = 14010
Fatigue-2 \r D\spnei-1 >r Palp1tatton-2 yr Angma-I vr
3
He‘lrt
wk
-1 yr
Intraventrrculnr bloch
LVH
& str L,”
LGH DI
ascendmg 1ort 1
LHB AAL ASM 111 ADM 111
Nonapecthc T nave changes
L\/H
LHB MAL ASM III 4Dhl IV BP = 140/20
LVH
LVH D&ted ascendmg aorta
LHB AAL ASM I ADM ‘11 BP = 130,‘50/20 LHB-AAL SM III DM 111 BP = 140/70 LHB-AAL ASM v 4DM III BP = 170/50 LHB-MAL ASM III ADM I\ BP = 180/70 LHB MAL 4SM III 4DM I\ BP = 90/46 Combmed fatlure LHB-AAL ASM 111 ADM III BP = 166150 LHB AAL ASM tt1 AD&f 111 BP = 156/60
Normnl
LVH
LVH
LVH
L\Ii
LVH Large aneurysm m ascendmg aorta & arch LVH Dtlated ascend mg aorta
LHB MAL AShl 1t1 ADM I” BP = 145/70 LHB MAL SM III DM w BP = 160/‘70
LVH
LVH
& stram
LBBB
LVH D&ted ascend mg aorta
LVH
LVH Dtlated asccndmg aorta
LVH
LVH
LVH (Normal root)
aorttc
Normal
Borderhoe
LVH
Mmor ST changes
Dtlated aorta
LVH
LVH Large aortlc pulsations
LAD Nonspectfic wave changes
LHB AAL ASM III ADM I” BP = 135/65 LHB MCL + 2 cm ASM III ADM IV BP = 145/75 LHB-MCL + 3 cm ASM II ADM IV BP = 160/50/40
LVH Pulmonar) edema
& stram
T
ascendmg
ABBREVIATIONS STS - semloglc test for syphllts hypertrophy LVH = left ventncular AAL = antenor aullary Ime
MAL = mid-axtllary lme LHB _ left heart border LBBB = left bundle branch
ASM = aorhc systohc murmur ADM = aorttc dtastohc murmur AI = a”r”c rosuffiaency
block
THE
AMERICAN
JOURNAL
OF
CARDIOLOGY
Annulo-;lortlc T4BLE
Ectasla
I (&~ntznued)
Operanon
Parhologu
& Course
Fmdmq,
4neur dddtanon up to mnomm~te *rtery Sclerouc \‘d\es ,ncompetent
4/29~64 ti”\en tu No 5 Magovern v ~l\e bular Dacron% graft Died 9 19 64
C\qnc me&l1 necrobl?. 4utopay Intact value 0 rhythm chstur bmce Post mroc hemorrhage
Not don?
Furlform, 10 cm rysm ascendmg ,nate artery 41
Cysuc medul necrosis 411top\\ renal mLeft cerebral mfdrctmn pulmonary congest,“” iarcuon wsceral congestion
Pear-shaped aneuqsm of ascrndmg aorta 41
Fwform
11/4/64 No 9 Starr-Edwards valve, 25 mm graft Died Wove” tubular right hermplegm com1 l/8/64 bmed fadure 5/20/64 30 mm No 3 Magovem valve Ahve woven Dacron tubular graft & norL1ng 11/23/64 No 11 Starr-Edwards \al\.e, 25 Died mm woven Dacron graft 11/24/64 hemomechastmum 24 hr
4utopsy Cysuc medial necro~~b Hemomedmstmum cdrdlomegalv
7 cm aneurysmal dllatatmn of ascendmg aorta to ,ust short of mValve thlckened ““inmate arteq & retracted 7 cm aneur ddatatlon ascendmg ~“rta bhort of mnommate arrery D&ted annulus AI
6/28/64 No 4 Magovern r&e 22 mm woven Dacron graft Ah\e & worL1ng 10/16/64 No 13 Starr-Edwards val\e DaAhve & w”rl\mg cron woven graft
Cysuc
medml
necro~ls
Cysnc
mrchal
ne~r”hl’
Large aneurysm awendmg & aoruc Supermr aspect of arch arch strip tram which 3 great \easels arose was normal AI -Ineur\sm ascenchnq aorta 41
4/29/64 No 4 Magwern valre 25 mm Alne woven Dacron graft
9 5 cm dlametrr m&al necrosis leaflet
10/26/64 13 No 11 Starr Ed**ards \Irl~e mm wore” Dacron prosthea Ahve 7/l/64 25 mm No 4 Magovern valve Dted 9/20/64 woven Dacron graft
Annulo noruc ectau necro~u cnlahed
PI tr ,haped ,rcwhng
ddatatlon ?“rtl AI
aorta
ennal spht of mtlma and media Prolapse of right posterior commlsam-e AI Fwform aneurvsm ascendmg aorta AI
chlatatlon aorta AI
Not don?
Huge ~neur”bm ascendmg aorta and arch Marked AI Not done
Not
ascendmg
-fake fypconnrrysm ~/IOcucumfer-
Not done
Pear shaped ascendmg
aneurysm
m dmmeter aneuaorta up to mnomCusps shortened
double lumen D~~sectrng onuryrm mldpomt ascendmg aorta stoppmg below mnommate artery
done
aneuqsm 41
7/g/64 No 4 Magovern valve, w”\cn DaAhbe & workmg cron graft
Not done
Foalform torta
of ascendmg
Large, 10 7 cm m dmmeter pear-shaped aneurysm m\o1wng the root and proumal a0rt.l 41
Fwform aneurysm clrssectmg \*ah a double lumen distally mrolvmg a/~ DlSSeCtl”” of the cncumference prox to wlthm 1 cm of v&e V&e cusps prolapsed AI
Not done
Fuslform aneurysm ascendmg aorta ends proximal to mnommate artery 41 Dlssectmn-aorta Mlth hemoperl cardmm & &tally
Pear shaped annulo a”rt,c aneurysm
AI
Pear-shaped annuloa”rt,c aneurysm
AI
Pear-shaped ddatatlon of Sm”seS of Valsalva LVH Masswe a”rt,c regurgltatlon
LHF = left heart fallore RHF = right heart falure BP = blood pressure
VOLUME
16,
NOVEMBER
DOE = dypsnca DI = d,lated
1965
7/31/64 Resection from 3 mm distal I” annulus 1” 2 cm below mnommate arter) two co %ts muted cbst~lly 19 mm woven Dacron graft, No 5 Magovern valve Ahve & w”rLmg No 7 Starr Edwards valve woven 4h\ e Dacron graft
No 13 Starr Edwvds \al\e Dacron grafr Alive
Not operated
upon
Not operated
upon
Not operated
upon
on exert,“”
MCL Parox
woven
-
1
Cyatlc
me&al
necroblh
to annulus cvsuc hbrotlc valve
c)snc medial hbrous valve
hbraals & hplds of u?ll card~omegaly = 900 Partml occlusl”” of pros gm thesu +2’ dnteroseptal myocar dml mfarcuon Dissect aneuqsm C\stx medml necro~lr Dlssect~on Autopsy
Cysuc medkd necroals sxcular lated aneurysm with dw.ectmn
dl-
hbrorls
of
Cystic me&al necrosis 1oruc Cd1b.e
Cystw medlal necrow, (hemoperrcdrdmm aneurysm)
mid clav~ular lme noct dysp = paroxysmal
hbrotlc valve & dtssectmg
nocturnal
dypsnra
Chapman
et al.
A
B
FIG. 1. Case 3. Angiocardiograms show pear-shaped aneurysmal dilatation and aortic regurgitant jet. A, posteroanterior view. B, lateral view.
and rolling down of the thickened right anterior aortic cusp, McKusick et a1.8 suggested that murmurs of this description may be produced either by fiddle-string bands traversing the false channel in the ascending aorta or by vibrations of the lips of the cusps created at the site of the initiating intimal tear. This musical murmur when located at the base of the neck and below the clavicle on the right, especially if it develops abruptly or changes, may be a sign suggesting dissecting aneurysm. Of the 16 cases with proved cardiovascular abnormalities, only 3 patients displayed the skeletal manifestations of Marfan’s typical syndrome. LABORATORY
FINDINGS
The eleztrocardiograns were not diagnostic and in general reflected evidence of left ventricular hypertrophy secondary to aortic insufficiency. In 9 of the 16 cases electrocardiographic evidence of left ventricular hypertrophy was present, and in 4 of these there was associated left ventricular strain. Left bundle branch block and intraventricular conduction disturbance each occurred once. Nonspecific S-T segment and T wave changes were seen in 3. All patients had sinus rhythm when initially seen. Bowers9 on reviewing the electrocardiographic
of the ascending aorta
abnormalities in Marfan’s syndrome also noted nonspecific electrocardiographic changes. These consisted mainly of left ventricular hypertrophy, incomplete right bundle branch block, nonspecific S-T and T wave changes, and atria1 fibrillation. He attributed these changes to a degenerated aorta with or without an aneurysm and secondary aortic insufficiency, diffuse myocardial hypertrophy and fibrosis, or to the cardiovascular complications secondary to kyphoscoliosis and pectus excavatum. Teleroentgenograms revealed some degree of left ventricular hypertrophy in all cases ; a dilated ascending aorta was present in nine of the sixteen A brisk pulsation of the ascending aorta, cases. a “rocking chair” phenomenon, was noted in 9 patients. Retrograde catheterization studies, employing the percutaneous femoral method of Seldinger’O or the direct brachial cut-down technic of Sones Injecand Shirey, I1 were made of 9 of 16 cases. tion of Angio-Conray @ dye was made into the root of the aorta, and in all 9, marked aneurysmal dilatation of the ascending aorta was The pear-like dilatation’* noted (Fig. 1 and 2). started in the intrapericardial region at the annulus and extended upward proximal to the innominate artery except in 1 case in which a dissection extended into the aortic arch. The THE
AMERICAN
JOURNAL
OF
CARDIOLOGY
Annulo-Aortic
A
Ectasia
683
B
B, angiogram shows pear-shaped dilatation of the ascending aorta with aortic regurgitation of dye into the left ventricle. FIG. 3.
Case 5.
A, teleoroentgenogram shows aneurysmal dilatation of the ascending aorta.
dyt:
frequently demonstrated the annular dilatacould not have been seen on the ro1:rtine roentgenogram and was thought to be dia gnostic of the conditionr” (Fig. 3). This was the most common and the earliest cardiovascular ectasia with cystic am lmaly in annulo-aortic
tiol n which
VOLUME
16,
NOVEMBER
1965
medial necrosis. Aortic regurgitation in var ‘Ying degrees was present in all. Although the lesion may not be detectable in the routine roentgenograms, anterior and later -al displacement of the pulmonary artery segme :nt should arouse the physician’s suspicion. T he
684
Chapman et al.
’ stromal substance.
displacement may result from dilatation of the aortic root and the ascending aorta. All serologic tests for syphilis were negative. PATHOLOGIC
FINDINGS
The pathologic cardiovascular ““abnormalities5J4-‘6 seen with the so-called Marfan’s syndrome may include : Cystic medial necrosis of the aorta and pulmonary artery; dilatation of saccular and dissecting aneuthe aortic root; rysms of the ascending aorta (more rarely of the arch or distally) ; sacculation’, rolling or redundancy of aortic cusps ; upward displacement of aortic cusps ; upward displacement of coroor cardiomegaly associated with nary ostia; other congenital defects such as atria1 or ventricular septal defects or patent ductus arteriosus: According to Weaver and associates5 changes other than cystic medial necrosis of the aorta are not seen in so-called idiopathic aortic dissection or aortic dissection with pregnancy.
Cystic medial necrosis in varying degrees of advancement was seen in all 13 patients operated on. In some cases the degeneration consisted merely of fragmentation and loss of elastic fibers with accumulation of basophilic ground substance or mucopolysaccharides within these areas. Usually, however, these cystic areas within the media were extensive, with degeneration and loss of elastic fibers (Fig. 4, A and B). Most of the cases revealed almost no atherosclerosis, although some had a slight amount. Histochemical studies of the mucoid ground substance in the aortic cystic areas demonstrated this material to be acid mucopolysaccharides; it is strongly positive with Alcian blue stain. The heart valves in every instance revealed considerable thickening throughout their length with laminated mature fibrous tissue, as well as considerable nodular areas of “basophilic” degeneration of the ground substance (Fig. 4C). These nodular areas contained accumulations THE AMERICAN
JOURNAL
OF
CARDIOLOGY
Annulo-Aortlc In none of these valves was of stellate cells calclficatlon or osslficatlon observed M o,f the dzssectznguneurysms had severe medlal c\stlc necrosis of the aorta with rupture m the central portIon In 1 case It was rupturmg slowly, as evidenced by granulation tissue wlthm The remammg aortas, away from the rent the dlssectlon sites, were mdlstmgulshable from those of other cases m which dlssectlon had not occurred 5LRGICAL
CONSIDER4TIONS
Technzc Operation upon the ascending aorta and aortlc valve was performed durmg total cardlopulmonary bypass In every case m the study the slmphfied method previously reported by Cooley and assoclates’7 was employed use of plastic disposable ox\genators (Travenol Laboratones), pnmmg the extracorporeal system exclusively with 5% dextrose m dlstllled water, and maintenance of normothermla In most patients with annulo-aortic ectasla requmng prolonged bypass of one hour or longer, body temperature gradually fell to 94 or 92” at the completion This level of hypothermia probably did of bypass not influence slgnlficantly the effectiveness of the Full heparmlzatlon of the patient tissue perfuslon was mamtamed durmg bypass with 3 0 mg /kg bode weight At the termmatlon of bypass, the heparm was counteracted with protamme sulfate, 3 5 mg /kg body weight A median sternotomy provided excellent exposure Venous outflow was of the aorta and aortlc valve obtained by mdlvldual cannulatlon of the venae Lavae and artenal return by a cannula m the common A sump-type suction placed m the femoral artery apex of the left ventricle provided a “dry” operative field by asplratmg bronchial and coronary return Only the left coronary artery was perfused durmg the period of open cardlotomy or aornc cross-clamping 4 small metal-tipped catheter was placed m the coronarv or&ice from mslde the open aorta and secured m place with a tourmquet placed around the artery outside the aorta posteriorly Rate of coronary perfuslon was controlled entirely by momtormg presbure m the tubing at 8&100 mm Hg Usually the heart maintained smus rhythm throughout the operation, but m some instances ventncular fibnllatlon occurred Defibnllatlon was easily achieved after the aorta was closed when a direct current defibnllator was employed After complete cardiopulmonary bypass was started, an artenal clamp was placed across the ascendmg aorta proxunal to the ongm of the mnommate artery The aneurysm was opened widely and excmon was performed Removal of the entire sac from the adjacent pulmonary artery was not always possible, and often a portion was left attached to the artery to avoid unnecessary arterial damage requn-mg 1epalr and prolongation of the operation The aortlc VOLUME
16,
NOVEMBER
1965
Ectasla
685
leaflets were removed completely, and a ball valve prosthesis of the Starr-Edwards or Magovern-Cromle type was used Our preference IS for the StarrEdwards prosthesis which depends upon suture fixation After the valve was replaced, a segment of woven, lmpervlous Dacron@ tubmg was inserted m the ascending aorta UsualI, the proximal anastomosls was done first, using a diagonal dlvlslon of the graft to accommodate the wide mouthed annulus Meticulous suture techmc Nlth 3-O Mersllene sutures (Ethlcon Laboratones) was used to reduce bleeding after operation The distal anastomosls was completed last After bypass was dlscontmued, hemostasls was obtamed with addltlonal sutures m the anastomoses reThe thorainforced by absorbable cellulose sponge cotomy mclslon was closed and waterseal dramage provided In 2 patients with aneurysmal dllatatlon extending mto the transverse aortlc arch, It was necessary to apply the occludmg aortlc clamp distal to the ongm of the left subclavlan arter) Smce this excluded the carotid arteries from the cannulatlon, it was necessary to perfuse the mnommate and left common carotid arteries with a separate pump A combined flou mto both carotid arteries of 400-500 cc /mm was sufficient to mamtam a normal electroencephalographic pattern In both patients, an earlv recovery from anesthesia was noted, and no cerebral comphcatlons occurred after operation Replacement of the transverse aortlc arch was performed durmg temporary carotid perfusion
Complzcatlons followmg operation were mfrequent Persistent bleedmg with hemoperlcardmm made emergency thoracotomy necessary in 2 patients In another patlent complete heart block developed owing to Injury to the A-V conducting system A pacemaker was implanted SIX weeks later because of the onset of Stokes-Adams episodes, and she has made excellent recovery Operatzve Results Of 13 patients operated on, One pa9 are hvmg and domg well (Fig 5) tlent died wlthm 24 hours of postoperative hemorrhage, mcludmg left hemlthorau, right mtrapulmomc hemorrhage and hemomedlasUnum A second patient died on the fourth postoperatlve day of multiple emboh to the left cerebral hemaphere, large and small Intestines, and kidneys These emboh arose from the prosthetic valves, which were well seated m both of these patients Two other patients died suddenly m their third postoperatlbe months Autopsy of the first patlent revealed a partial occlusion of the ball valve prosthesis with thrombosis extendmg upward and partially occludmg the left coronary
Chapman
686
FIG. 5. Case 5. Postoperative film shows disappearance of aneurysmal dilatation and emplacement of the prosthetic valve (cf. with Fig. 3A).
artery. This patient had a recent anteroseptal myocardial infarction. In the second patient no evidence of occlusion of the coronary artery was noted at autopsy, but a soft posterior surface of the heart was present with some hemorrhage in it. It is postulated that the coronary orifice may have been temporarily occluded leading to possible myocardial infarction, and a sudden rhythm disturbance may have been the final cause of death.
DISCUSSION Although knowledge of the manifestations of the Marfan’s syndrome are widely known, too often the syndrome is diagnosed only in its classic form. One must be alerted to the lesser manifestation, the forme fruste feature of aortic insufficiency with a dilated aortic root. Annulo-aortic ectasia with aortic insufficiency ,should be considered in the differential diagnosis of all cases of aortic insufficiency. The lack of skeletal deformities, ectopic lens or a family history of Marfan’s syndrome need not deter the diagnosis, and these may be absent also with the so-called forme fruste of Marfan’s cystic medial syndrome. The 1characteristic
et al. necrosis was universally present. Aortic regurgitation was also seen in all of our patients, resulting from saccular dilatation of the annulus, thickened nodulation or bacterial endocarditis of the CUSPS leading to dilatation and distortion of the aortic valve. Physical examination revealed predominantly the findings of aortic regurgitation. Routine roentgenograms may not demonstrate the lesion, but if there is anterior or lateral displacement of the pulmonary artery segment, it should arouse suspicion of a dilated aortic root. Retrograde arteriography may demonstrate dilatation of the annulo-aortic region. Differentiation before surgery between this lesion and pure aortic valvular insufficiency is of importance to facilitate technical preparation for the more complicated procedure. The lesion of annulo-aortic ectasia is unusual in that it may present a double threat to life. Death may result from intractable cardiac failure initially manifesting itself as left heart failure and ultimately progressing to combined failure secondary to aortic insufficiency. Secondly, the thin-walled aortic aneurysm may rupture or dissect, resulting in fatal hemorrhage into the pericardium, pleura, right atrium, right ventricle, pulmonary artery, or left ventricle. Surgical treatment should be reserved for those patients in whom the lesion is life-threatening. If there is progressive dilatation of the ascending aorta with potential rupture or if severe aortic regurgitation with progressive congestive failure develops, surgery may be indicated. In early cases of ectasia, operation should be withheld until progress of the disease can be demonstrated.
Annulo-aortic ectasia is a term first suggested by Ellis and Cooley for an aneurysmal dilatation of the ascending aorta and aortic annulus frequently associated with aortic regurgitation. The basic structural defect in the aortic wall is cystic medial necrosis which may or may not be accompanied by stigmata of Marfan’s syndrome. Thirteen cases fulfilling the above criteria were operated on with four deaths, and three additional cases have been studied. When the patient’s diagnosis was uncertain, retrograde angioaortography demonstrated characteristic pear-shaped dilatation of the annulo-aortic region. Surgical treatment consisted of excision of the aneurysm with replacement by a woven Dacron THE AMERICAN
JOURNAL
OF CARDIOLOGY
Annulo-Aortlc yraft and correction of the aortlc regurgitation bv pi osthetlc 1 alve REFERENCES 1 BOER, R E
H
aorta
ii’,
T~USSIG, H
Congemtal associated
G
9 10
and OPPENHEIMER,
aneurysmal dllatatlon wth arachnodactyly
of the
Bull Johns Hopktns Hasp, 72 309, 1946 2 ERDHEIM, J Medlonecrosls aortae ldlopathlca cystlca Vmhows 4rch Path Anat, 276 187, 1930 3 \VAGENVOORT, C 4 NEUFELD, H N and EDWARDS, J E Cardiovascular system m Marfan’s syndrome and m ldlopathm dllatatlon of the ascendmg aorta Am J Card&, 9 496, 1962 The forme fruste m 4 GOLDEN,R L and LochIN, H Marfan’s syndrome New England J Med, 260 797, 1959 5 WEAVER, W F , EDWARDS,J E and BRANDENBURG, R 0 Idlopathlc dllatatlon of the aorta with aortic valvular msufficlency A possible forme fruste of Marfan’s syndrome Pm Meet Mayo Cltn , 34 518, 1959 6 ELLIS, P R , COOLEY, D A and DEBAKEY, M E Chmcal conslderatlon and surgmal treatment of annulo-aortic ectasla J Thoram & Cardaovas Surg , 42 363, 1961 7 STEMBRIDGE, V A, HEJTMANCIK, M R and HERRMANN, G Unusual musmal murmurs of anterior aortic origin Am Heart J, 48 163, 1954 8 McKusnx, V A, LOCUE, R B and BAHNSON,H T 4ssoclatlon of aortm valvular disease and cystm
VOLUME 16, NOVEMBER1965
Ectasla
11
12
13
14
15 16
17
687
( I?,ulrillon medlal necrosn of ascendmg aortrl 16 188 1957 The eleCtrOCdrdlOgrdm m M,nfdn’\ BONERS, D syndromt rln1 J Cardml, 7 661 1961 SELDIUGER, S I Catheter replacement of the needle in percutaneous artenogrdph\ lrtu mdzol 39 368 1953 C21ucLOlOSONES, F M , JR and SHIREY E K nary arterlography Mod Concepts C ardmr us Drs , 31 735, 1962 BARRETT, J S , HEL~IG, J , JR KA\ C F and JOHNSON, J Cme-aortographlc e\ dludtion of aortlc msufficlency , unsuspected ldlopathlc aneurysmal dllatatlon of the aortlc root as a posstble mdlcatlon of the Marfan syndrome Ann Znt &fed, 61 1071 1964 STEINBERG,I, MANGIARDI, J L dnd NOBLL \li J Aneurysmal dllatatlon of the dortlc smuses m Marfan’s syndrome (anglocardlographlc and cardiac catheterlzatlon studies m ldentlcal twins) Cmulatton, 16 368, 1957 4cld mucopolyBUNTING,C H and BUNTING H saccharides of the aorta Arch Path 55 257, 1953 GORE I Dlssectmg aneurysm of the dorta under forty years of age Arch Path, 55 1, 1953 PE4RSE, 4 G E Hlstochemlstry, Theoretmal and Applied, p 106 Boston, 1953 Little Brown and Company COOLER, D A BEALL, 4 C , JR and GRONDIN, 0 M Open heart surgery by a slmphlied technic using disposable oxygenators, 5 percent dextrose solution prime and normothermm Ma1 Cardzouas , 4 455, 1963