Quadricuspid Semilunar Valve
LARRY E . HURWITZ, MD
WILLIAM C . ROBERTS, MD, FACC
Bethesda, Maryland
From the Section of Pathology, National
Heart and Lung Institute, National Institutes
of Health, Bethesda, Md . Manuscript re-
ceived July 11, 1972, accepted September
14, 1972 .
Address for reprints : William C . Roberts,
MD, Section of Pathology, National Heart
and Lung Institute, National Institutes of
Health, Bethesda, Md . 20014 .
Clinical and necropsy findings are described In 10 patients with quad-
ricuspid semilunar valves, and previous reports of 197 cases are
reviewed . Quadricuspid semilunar valves occur infrequently, and
usually the pulmonic valve is affected . The quadricuspid pulmonic
valve typically functions normally . The quadricuspid aortic valve was
functionally abnormal In 5 of 11 cases studied .
The most common major congenital anomaly involving the heart or
great vessels is malformation of one or both semilunar valves .' Of
the various congenital abnormalities affecting these valves, the most
frequent by far is the bicuspid aortic valve (occurring in approxi-
mately 2 percent of the general population') ; next in frequency is the
unicuspid aortic valve . 2 Semilunar valves with 4 cusps are much less
common than those with either 1 or 2 cusps . The bicuspid and uni-
cuspid malformations of these valves have received considerable at-
tention in recent years .l ,2 In contrast, the occurrence of 4 cusps in
either the pulmonic or aortic valve has received relatively little at-
tention . This report focuses on the quadricuspid semilunar valve .
Clinical and necropsy observations in 10 patients studied by us are
summarized, and observations previously recorded by others are re-
viewed .
Patients Studied
Pertinent information on the 10 patients is summarized in Table I .
In 2 patients the aortic valve was quadricuspid (Fig . 1 and 2), and in
the other S the pulmonic valve was quadricuspid (Fig. 3) . Function
of the quadricuspid valve appeared to have been normal in 8 pa-
tients and abnormal in 2 . One of the latter, a 51 year old woman,
had severe aortic regurgitation necessitating aortic valve replace-
ment (Fig . 1) . The other patient, a 3 day old boy, had a dysfunction-
ing quadricuspid valve with severe valvular pulmonic stenosis . He
died shortly after operative alleviation of the right ventricular out-
flow obstruction . Other congenital cardiovascular anomalies-name-
ly atrial septal defect of the fossa ovale type, partial atrioventricular
canal and ventricular septal defect-were present in 3 of the 10 pa-
tients . Two other patients, each with a quadricuspid pulmonic valve,
had rheumatic mitral and aortic valvular stenosis, but there was no
evidence in either patient that the rheumatic process had involved
the pulmonic valve .
Of the 2 patients with a quadricuspid aortic valve, 1 had a valve
with 3 equal-sized cusps and I smaller cusp (Fig . 4), and the other
had a valve with 2 equal larger and 2 unequal smaller cusps . In 5
cases, the pulmonic quadricuspid valve had 3 equal larger cusps and
1 smaller cusp ; 1 valve had 4 equal-sized cusps, 1 had 2 equal larger
and 2 equal smaller cusps, and 1 valve had 1 larger, 2 intermediate
and I smaller cusp .
May 1973 The American Journal of CARDIOLOGY Volume 31 623


QUADRICUSPID SEMILUNAR VALVE-HURWITZ AND ROBERTS

TABLE I
Data on 10 Patients with Quadricuspid Semiluna r Valves

Case Age (yr) Quadricuspid Functional History Other Cardiac

no. & Sex Valve Status RF IE* Abnormalities

1 51F Aortic Incompetent 0 0 0

2 11F Aortic Normal 0 + VSD
3 40M Pulmonic Normal 0 0 0
4 3daysM Pulmonic Stenotic 0 0 ASD
5 14M Pulmonic Normal ? ? PartiaIA-V
FIGURE 1 . Case 1 . Schematic diagram of he incompetent qua- canal
dricuspid aortic valve in a 51 year old woman with angina pec- 6 57M Pulmonic Normal + + Rheumatic
toris and refractory congestive failure necessitating aortic valve
MS, AS
replacement . At operation, the aortic valve was quadricuspid
with 2 smaller and 2 larger cusps . A prosthesis was inserted . 7 46F Pulmonic Normal 0 0 0
She died suddenly 17 days later . At necropsy, the heart weighed 8 81M Pulmonic Normal 0 0 0
520 g . Both ventricles were markedly dilated, and the left ven- 9 41F Pulmonic Normal 0 0 HC
tricular free wall measured up to 1 .7 cm in thickness . Death 10 34M Pulmonic Normal + 0 Rheumatic
was due to acute myocardial infarction secondary to an embo- M S, AS
lus to a coronary artery .
' Although 2 and possibly 3 patients had a positive history of
infective endocarditis, none had vegetations involving the
quadricuspid valve .
AS = aortic stenosis ; ASD = atrial septal defect ; A-V = atrio
ventricular ; HC = hypertrophic cardiomyopathy without sub-
aortic stenosis ; IE = infective endocarditis; MS = mitral steno
sis; RF = acute rheumatic fever ; VSD = ventricular septal de-
fect .

FIGURE 2 . Case 2 . A normally tunctioning quadricuspid aortic

valve in an 11 year old girl with a known ventricular septet de-
fect. Six months before death she was successfully treated for
alpha streptococcus endocarditis ; however, she died of progres-
sive renal failure . At necropsy, the heart weighed 270 g . Both
ventricles were dilated . The septal leaflet of the tricuspid valve
was bound down to the ventricular septum by fibrous tissue, and
a jet lesion (2 cm in diameter) was evident on the free wall of
the right ventricle . There was no evidence of endocarditis on the
quadricuspid aortic valve . The "closed" ventricular septal defect FIGURE 3 . Quadricuspid pulmonic valves observed as incidental
is outlined by the dashed circle . A = accessory valve cusp ; necropsy findings in 2 patients . a, Patient 3, who died of carci-
A .M .L . = anterior mitral leaflet ; L = left aortic valve cusp ; L .V . _ noma of the pancreas . b, Patient 7, who died of acute leukemia .
left ventricle; N = noncoronary valve cusp ; R = right valve cusp . Neither patient had symptoms of cardiac dysfunction .

cuspid pulmonic valves and 2 quadricuspid aortic

Comments valves during examination of 6,000 necropsies . Oth-
A quadricuspid semilunar valve is an uncommon ers have shown a similar smaller incidence of quadri-
condition . Review of previous reports yielded 197 cuspid aortic valves . Neither Simonds 4 nor Simpson 8
cases : in 184 cases (93 percent) the affected valve found a quadricuspid aortic valve during examina-
3-8
was pulmonic and in 13 cases (7 percent) was aor- tion of 6,252 necropsies ; deVries 5 observed only 1 in
tic . 5,7, e -14 It is unclear why the pulmonic valve is 3,600 necropsies . These reports may underestimate
more commonly affected, since the aortic valve is the actual frequency of this anomaly since, unless
more commonly bicuspid or unicuspid . 1,2 The quad- they are specifically sought, minor abnormalities of
ricuspid pulmonic valve has been found in from 1 in the semilunar valves may be easily overlooked at
4001 to 1 in 2,000 necropsies . 4 We found 8 quadri- necropsy examination .

824 May 1973 The American Journal of CARDIOLOGY Volume 31

 QUADRICUSPID SEMILUNAR VALVE-HURWITZ AND ROBERTS

Sex distribution : The quadricuspid pulmonic

valve more commonly occurs in male subjects : of 79 a Four Equal Cusps
affected patients whose sex was known, 52 (65 per- GD
cent) were male and 27 (35 percent) were female .
Unlike other malformations of the aortic valve that Aft Three Equal Cusps,
primarily affect male subjects,' • 2 the quadricuspid One Smaller Cusp
aortic valve appears to have an equal sex distribu- ',
tion . Of affected patients whose sex was known, 4
were male and 3 were female .
Valve function : The quadricuspid pulmonic valve
nearly always appears to function normally . Conse-
C (~~ tu:o Two Equal Larger Cusps,
Two Equal Smaller Cusps

quently, most quadricuspid pulmonic valves repre-

sent incidental findings at necropsy . Why one quad-
ricuspid valve functions normally and why another
functions abnormally is uncertain . Of 158 quadri-
0 One Large, Two Intermediate,
One Small Cusp

cuspid pulmonic valves available for study or review,

6 (4 percent) functioned abnormally ; of these, 2 were
stenotic and 4 were purely incompetent . In contrast,
the quadricuspid aortic valve frequently functions
'S Three Equal Cusps,
One Larger Cusp

abnormally . Of 11 quadricuspid aortic valves, 1 was Two Equal Larger Cusps,

stenotic and 4 were incompetent . Valvular incompe- Two Unequal Smaller Cusps

tence, the most common hemodynamic abnormality,

was not observed in infants or young children, thus
suggesting that the quadricuspid valve is not incom-
petent from birth . In no case was incompetence a re-
sult of infective endocarditis . The finding of a steno-
•• UJU Four Unequal Cusps

F IGURE 4 . Seven described anatomic variations of quadricuspid

tic quadricuspid valve in a 3 day old infant (our valves . The most common variation in either aortic or pul-
monic valve consisted of 3 equal-sized cusps and 1 smaller
Case 4) suggests that the quadricuspid valve, unlike cusp (b) . No correlation was found between anatomic variation
the congenitally bicuspid valve,' may be stenotic and functional status .
from birth.
Other cardiac anomalies : The quadricuspid
semilunar valve is rarely associated with other con-
genital cardiac anomalies . Seven patients (4 percent)
with quadricuspid pulmonic valves had other abnor- Normal and Abnormal Development
malities : 4 had atrial septal defect of the fossa ovate of the Aortic Valve
type, 1 had ventricular septal defect, 1 had partial NORMAL
COMMON TRUNK
atrioventricular canal and 1 had patent ductus arte-
riosus . One patient with a quadricuspid aortic valve
had ventricular septal defect .
Endocarditis : The normally functioning quadri-
cuspid valve does not appear to be more likely to NORMAL
have infective endocarditis than the normally formed SEPTATION `,

valve . The only reported case of endocarditis involv-

ing a quadricuspid valve occurred on a previously
stenotic valve . 1 3 Our Patient 2, who had a ventricu-
lar septal defect, had endocarditis of the right ven-
tricular endocardium but no evidence of vegetation
on the normally functioning quadricuspid aortic
valve .
Although the rheumatic process has been demon-
strated to involve the congenitally bicuspid aortic ABNORMAL
COMMON TRUNK
valve, it does not appear to involve quadricuspid
semilunar or any other malformed valve with in-
creased frequency . Although 2 of our patients had
rheumatic disease of both mitral and aortic valves,
the quadricuspid pulmonic valves were unaffected by
the rheumatic process . \'
Anatomic variations : The anatomic variations of Ouodricuspid AV
FIGURE 5 . Diagrammatic portrayal of the probable mechanism
the quadricuspid valves are schematically presented
of development of both normal and abnormal semilunar valves .
in Figure 4 . Of 121 quadricuspid pulmonic valves re- Abnormalities may occur from either abnormal septal division or
viewed, 72 valves (60 percent) had 3 equal-sized from abnormalities of mesenchymal proliferation in the common
cusps and 1 smaller cusp ; 18 valves (15 percent) had trunk . A .V . = aortic valve ; P .V . = pulmonic valve .

May 1973 The American Journal of CARDIOLOGY Volume 31 625


OUADRICUSPID SEMILUNAR VALVE-HURWITZ AND ROBERTS
2 equal larger and 2 equal smaller cusps ; 15 valves
(12 percent) had 4 equal cusps ; 7 valves (6 percent)
had 1 larger, 2 intermediate and 1 smaller cusp ; 4
valves (3 percent) bad 3 equal and 1 larger cusp ; 3
valves (2 percent) had 2 equal and 2 smaller unequal
cusps and 2 valves (2 percent) had 4 unequal-sized
cusps, Of 7 quadricuspid aortic valves reviewed, 3
valves had 3 equal and 1 small cusp ; 2 valves had 4
equal cusps ; 1 valve had 3 equal and 1 larger cusp,
and 1 valve bad 4 unequal cusps .
Multiple small fenestrations were commonly ob-
servetj in both quadricuspid aortic and pulmonic
valves . In no patient were both aortic and pulmonic
valves quadricuspid . lp contrast, several patients
with congenitally bicuspid pulmonic and aortic
valves have been observed .
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6 26 May 1973 The American Journal of CARDIOLOGY Volume 31
Embryology : Semilunar valve formation appears
to occur as follows : By the fourth week of gestation,
a pair of mesenchymal ridges have formed in the
cephalad portion of the truncus arteriosus . With fur-
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