Review

Diagnosis of the Anatomic Types of

Single or Common Ventricle*
RICHARD VAN PRAAGH, M.n.,t STELLA VAN PRAAGH, M.D.,$ PETER VLAD, M.D.~ and
JOHN D. KEITH, M.D., F.A.C.C.

Toronto, Canada and Buffalo, New York

I
F A PATIENT with a single or common ven- DEFINITION AND ANATOMICTYPES
tricle is submitted to open ventriculotomy- A single or common ventricle may be defined
usually because of diagnostic error or uncer- in a general, nonmorphologic way as one ven-
tainty-death almost invariably results soon tricular chamber which receives both the tri-
postoperatively. For this reason it is most im- cuspid and mitral valves or a common atrio-
portant not to mistake a single ventricle for a ventricular valve. This definition excludes tri-
malformation which currently is correctable cuspid and mitral atresia. A rudimentary
with cardiopulmonary bypass. outlet chamber may or may not be present.
Following the initial clinical examination, The great arteries may or may not be trans-
the presence of a single or common ventricle posed, and the atria1 septum may be normal,
usually is not suspected. Diagnostically, one deficient or absent.
tends to focus upon findings produced by other The terms “single” and “common” are em-
associated malformations. Thus, the initial ployed interchangeably because both are general
clinical impression (based upon the physical designations, without specific morphologic
examination, chest roentgenograms and electro- meaning.
cardiogram) is likely to be one of the following: From the morphologic standpoint, there are four
isolated ventricular septal defect with pulmo- types of single ventricle’: single left ventricle
nary hypertension, “corrected” transposition (absence of the right ventricular sinus or inflow
with ventricular septal defect, patent atrioven- tract) ; single right ventricle (absence of the left
tricular canal, preductile coarctation of the ventricular sinus) ; undivided ventricles (absence
aorta, acyanotic tetralogy of Fallot, “complete” of the ventricular septum); and infundibulum
transposition with ventricular septal defect, or only (absence of both ventricular sinuses).
tricuspid atresia with transposition. MATERIAL AND METHODS
Can one accurately diagnose the various This study was based upon a correlation of the clin-
anatomic types of single or common ventricle by ical and pathologic data in 39 necropsied cases* of
clinical and laboratory methods? This is the
* The Hospital for Sick Children, Toronto, Canada, 23
question which the present report seeks to cases; Children’s Hospital, Buffalo, N. Y., 15 cases;
answer. and Boston Floating Hospital, Boston, Mass., 1 case.

* From the Department of Pediatrics, University of Toronto, and the Research Institute of The Hospital for Sick
Children, Toronto, Canada, aided by grants from the Ontaric Heart Foundation and from the Department of National
Health and Welfare; and from the Children’s Hospital of Buffalo and the Department of Pediatrics, School of Medi-
cine, State University of New York at Buffalo. Aided also by the M. S. Goode and R. W. Goode Gift, by the Pat-
terson-wheeler Fund and by the National Institutes of Health grant TlHD39.
t Present address: Congenital Heart Disease Research and Training Center, Hektoen Institute for Medical Re-
sea-ch, Chicago, 111. Aided in part by Grant HE-07605-02.
$ Present address: Children’s Memorial Hospital, Chicago, Ill.
Q Present address: State University of Iowa, Department of Pediatrics, Iowa City, Iowa

VOt.UME 15, MARCH 1965 345

 346 Van Praagh, Van Praagh, Vlad and Keith

TABLE I
Clinical and Postmortem Findings in 39 Cases of “Single” or “Common” Ventricle

1 .J.dcS. 7 )‘. 5. 8, 3,‘4. 2 1.1s + + 0 RZ57U 58.62 T

(Fig. 11 F (pc,wp., 78

2 I1.P. ? <l‘i\., s. $, 1 ‘4. 4 1.1s (1 0 + 55 t

3% (posop. 1

S..\ 0 + 76 61 XR 5 56 t
(F3ig. 2) k

4 Single LV, norm,ill!_ relalrd C;1Z’s, + + 62 t

solilua V & A
5 Single LV, norm.rll\ wl.ltrd (g.\‘\, 0 + 42 60 65 55 4
solitus V & .4
6 Single LV. (absent KV smus) cl- + + 69 70 75 68 t
TGA’s, solitus V & A
7 Single LV, d-TGA’s, solitms V & 3 + 2t 67 t

8 ii ,rk. 0 0 + + RI 62 90 63 f
(pow,,. ,
‘I d‘l”., s. L(Tz/4. LLSK 0 + 2t 72 t
(hp. 3 )

10 11,s. + 2t 67 t
hl
11 <;.I 1 .s 1110. s. <I’ 3’4, 4 1.1s 0 + + 60 t
M (poctop )
17. ‘I‘.F’. 7 7.5 yr. s, $r 2 4. LLSB 0 0 + 48,57 t
M (p0wp.l I), gr 2 ‘4, LLSB (I

13 \$..I.. 10.7; y, s, c,- 3 4, LLSB + 0 i ‘11 86 ‘14 60 t

(Fig. 4) hl (posmp. / I), (MIIM) apex +
14 .I.‘\ 3 yr, S,qr? 4,2 41.1s + 0 3f 50 4
F
I).I). &;w! s. g’ I, 4, ULSB 0 0 33 31 45 50
15 3+ 4
F
16 H.V. 1 (I.)\. 0 0 + 3+ 65 f
M

17 Single LV, /-TGA’s. solilua V h I\ I..:%. S’, 1 )“. S.$r3,‘4,2 4LlS + 0 + RR 79 95 60 t

F fp0smp.j I> (MDM), apex 0

18 Single I,V, I-TGA’s, sol~tus V & A .z.c. 4’, I? vr. S, qr- 3 ‘4, LJLSB NK + f 70 t
F

I‘.% 0
+ 66 f
M

Smgle LV, I-TC:A’y. \~IIIUS V & .\ .l.H. ‘I S. qt- 3,‘4, LLSB 0 0 47

20 1110. + 4
M C, disappeared .%I
8 mo.
21 Single LV, I-TGA’s. aolirus V & A n.u. 7 Mk. C. ULSB 0 I) 3f 43 31 56 50 4
M (powp.)

22 Single LV, I-TGA’s. solrtur V Pr A T.S. I, mo. S. $v 2,‘4. LLSB + + + 80 81 93 64 t

(Fig. 6) M

23 Single LV, I-TG.A’s, sobrus V & A ?,%.J. 3 mo s. gr 3 4. I.I.SB + + + 62 t

M (poslop. 1

24 Single LV, I-TGA’s, solitus V & A P.R. 2 l”0. s, 8,. 3,‘4, LLSB 0 + 0 66 t
M D (MDM), gr
z/4, apex 0
25 Single LV, /-TC;A’s, soliius V & A J.M. 8 1x10. s, gr 3/4, LLSB 0 + f 62 t
(Fig. 5) M

THE AMERICAN JOURNAL OF CARDIOLOGY

 Diagnosis of Single Ventricle 347

TABLE I (continued)

Diagnoses at Autopsy
ECG Clinical Diagnoses (Associated Malformations)

-750, counter, CAH, Ostium primum, ASD with M.I. Common A-V valve (patent A-V canal)
CVH, WPW

--45’=, counter, LVH A-V communis, or large VSD Secundutn ASD, common A-V valve, muscular subaortic stenosis with
hypoplasia of AoV, ascending Ao and severe preductile coarc, huge
distal PDA, aberrant right subclavian a. from desc Ao, hare lip,
posterior choanal atresia (bilat.), T-E fistula
-40°, counter, LAH, CVH Single ventricle with mitral stenosis Supravalvular and valvular mitral stenosis, PDA

0°, counter, CAH, LVH Large VSD Pneumonia. Common A-V valve, muscular sub Ao stenosis, hypoplasia
of Ao
+25’, clock, RAH, LVH Tricuspid atresin Pulmonary atresia, PDA

+9”, counter, LVH Transposition, preductile coax, PDA, tri- Subaortic stenosis due to small bulboventricular foramen, preductile
cuspid atresia coax, PDA, overriding tricuspid valve
+60”, clock, LVH Truncus, or EFE, or .40 stenosis, or trans- Subaortir stenosis (small foramen), atresia of Ao isthmus, PDA
position with tricuspid atresia. (All pulses
weak )
+30”, clock, RAH, LVH Tricuspid atresia, transposition, preductile Subaortir stenosis (small foramen), atresia of Ao isthmus, PDA
coax
+60”, clock, RAH, LVH Tricuspid atresia with transposition, or Subaortic stenosis (small foramen, and partly occluded by tricuspid
single v. with transposition, and coarc of valve), preductile coax, PDA
A”
+‘)o”, clock, equiphasic Coarc of Ao with congestive heart failure Suhaortic stenosis (small foramen), bicuspid AoV, preductile coarc,
R/S, Katz/Wachtel sign PDA
+llO”, clock, LVH Coarc of Ao (“irdult” type) Subaortic stenosis (small foramen), preductile coarc, PDA

+ilO’, clock, CAE, RVH, Atypical tetralogy of Fallot Severe stenosis of left-sided tricuspid valve, marked leaflet fusion with
A-V block (1st & 2nd several small openings: “watering can” stenosis of the tricuspid valve
degree)
+80’, clock, CAE, CVH VSD with pulmonary hypertension, “pera- Regurgitation of left-sided tricuspid valve
ble (large L-R shunt)
+ 1 30°, clock, RAE, RVH Corrected transposition, pulmonary stenosis, Pulmonary stenosis (valvular and subvalvular), and mild regurgitation
with large VSD or SV of left-sided tricuspid valve
+ 1 loo, clock, RAE, RVH Corrected transposition, pulmonary stenosis, Pulmonary stenosis, valvular (dome) and subvalvular
sv
+115’, clock, complete Cyanotic CHD with congen complete A-V Subaortic stenosis (small bulbovcntricular foramen), atresia of Ao
A-V block (congen), block and CHF isthmus, small distal PDA (? closing), stenotic left-sided tricuspid
atria1 rate 130, ventric valve
rate 70/min. No ore-
cordial leads available
+lOO’,clock, CAE, RVH, Atypical tetralogy of Fallot Regurgitation of left-sided tricuspid valve, and subpulmonary stenosis,
transient 2nd degree produced by abnormal insertion of anterior papillary muscle of left
A-V blork ventricle (right-sided) just beneath transposed PV
+lOO”, clock, RAE, CVH, Corrected trnnsponition, VSD, pulmonary Subpulmonary stenosis (muscular) with bicuspid PV; subaortic stenosis
intermittent WPW stenosis (small bulboventricular foramen), without preductile coax; and re-
gurgitation of left-sided tricuspid valve
+ 1 OO”, clock, CAE, RVH Corrected transposition, coarc of Ao, large Suhaortic stenosis (small bulboventricular foramen), preductile coax
VSD or SV (ductus closed), regurgitation of left-sided tricuspid valve. Following
aortogram, tbromboembolism of desc. Ao with multiple renal infarcts
(thromhi from coax)
+ 1 OO’, clock, RVH Cyanotic CHD Pulmonary atresia; PDA, long, probably closing at PA end; left-sided
tricuspid valve hypoplastic

+1050, clock, relatively VSD with pulmonary atresia Pulmonary atresia; probe patent PDA; probe patent Blalock-Taussig
equiphasic R-S from anastomosis; mild stenosis of left-sided tricuspid valve; and staphylo-
VIR to Vs coccal pneumonia
+95”, clock, CAE, CVH Complete transposition with VSD or SV Subaortic stenosis (muscular), with bypoplasia of Ao and preductile
coarc; left coronary artery arises above aortic septal commissure;
right A-V valve overrides ventricular septum
+lOO”, clock, CVH VSD with pulmonary hypertension Subaortic stenosis (small bulboventricular foramen) and moderate
hypoplasia of ascending Ao (no preductile coarc); regurgitation of
left-sided tricuspid valve; complete heart block occurred when sur-
gical attempt made to enlarge bulboventricular foramen, to relieve
subaortic stenosis
+lOO”, clock, CAE, RVH L-transposition, single LV and infund “ut- Stenosis of left-sided tricuspid valve
let chamber

+lSO”, clock, RVH Isolated dextrocardia with corrected trans- PFO, valve incompetent; dextrocardia
position and ASD, or total anomalous
pulmonary venous drainage into coronary
sinus

VOLUME 15, MARCH 1965

 Van Praagh, Van Praagh, Wad and Keith

Ca5.Z -------Physical Findiq------% Oxirnetry Chest X-Ray

No. Anaromic Type of Nnmr Cyano- (7%Sat.) CTR
(Fig. ) “Single” Ventricle Sex Murmur Thrill CHF sis R Ex 02 (“c,) Vast.

26 Single I.V, I-TG.4’5. solituc V & .\ D.J 7.5 mo. C, gr 3,‘4, ULSB 0 0 r+ 54
t, (postop.) S :LLSB 0

27 Single l.V, I-TGA’s, asptenia \\KN. I6 d‘lya 0 0 2+ 45

(Fig. 7) M (POS’OP.)

2x Single LV, I-TG.4’s, nsplenia B.D 7 days 0 r+ 57

M

29 Single LV, I-TGA’s, asplenia S.K. 3 M-h S, gr 1,‘4, RLSB 0 + + 71

!vl

30 Single LV, d-TGA’s in an i-loop, soil- n.v 7 wk s, g’ 3/4, 3 I,IS + + + so 32 90 72

tus V & A M

31 Sin& LV. d-TGA’s in an i-loop, soil- G.M. to d<lys 0 + 3+ 75

tus v & A F
32 Single RV (absent LV sinus), I-TGA’s, T.O. 22 1’. S, gr 3,‘4, LLSB + + 2f 54
(Fig. 8) solitus V & A M
33 Single RV, d-TGA’s, inversus V & A I’.C. X’;‘,r yr. S, gr 3/4. LLSB + + 3f 74
hl C, gr 1,‘4, 2 RIS 0

34 Llndlvidcd vrn~ricles (abwnt vcn- c;.tr 4 d.IW S, gr 214, LLSB 0 + + 57 Nor-

(Fig. 9) trirular seprum), d-TGA’s, soliluc F D (MDM), apex 0 mat
v & A
35 L’ndivided ventricles, d-TGA’s, soli- 1’) days 0 0 0 3+ 40 31 42 57
c
1”s v & .4 (PO-P
36 Infundibulum only (abscnrc of RV and 4 mo. S, VLSB 0 + 2+ 79 77 82 62 t
LV sinusrs. and of ventric. septum),
d-TGA’s. sotitus V & A
37 Infundibulum only, d-TGA’s. solitur S. g“ 3 ‘4. I.IzSB + 0 + 02 J-
(Fit’. I I ) V&A
38 Infundibulum only, &TGA’s, .qleni;l 0 0 0 2+ . .
(Fig. 101

39 S. er 3;4. LLSB + 0 2+ 52 3

single ventricle; 26 were male and 13 female (males, or d-. relative to the transposed pulmonary valve,
females, 2/l). ‘The ages at death ranged from 2 days ‘icomplete” transposition) ; tYpe 111, /-transposition
to 22 years; the mean was 2 years 3 months, and the (transposed aortic valve to the left, i.e., levo- or I-,
median, 3 months. .411 patients had at least one relative to the transposed pulmonary valve, “cor-
electrocardiogram with 11 or more leads (except for rected” transposition); type A, single left ventricle
Case 16 in which chest leads were not obtained). (absent right ventricular sinus); type B, single right
Our electrocardiographic criteria for atria1 and ventricle (absent left ventricular sinus) ; t_ypeC, undivi-
ventricular overloading were those employed by ded ventricular sinuses (absent or rudimentary ventric-
Keith and associates2 Cardiac catheterization data ular septum) ; type ll: infundibulum only (both sinuses
were available in 12 patients, and 17 had angio- absent) ; s&us; situs solitus (normal locations) of the
cardiograms. viscera and atria; inversus, situs inversus (apparent
The anatomic findings were reported previously as mirror-image of normal locations) of the viscera and
part of a larger morphologic study.’ Some of these atria ; and heterotayy, the abnormal visceral locations
cases also were reported by Keith and associates.” with asplenia. in which the basic type of visceroatrial
situs (solitus or inversus) often is anatomically uncer-
CLASSIFICATION AND TERMINOLOGY
tain or indeterminate.
A brief and convenient classijication, proposed prr- Other brief and convenient terms proposed previously’,4
viously,’ is used in the electrocardiographic figures and used in this paper are: d-loop, a cardiac loop
of this paper: t@e Z, the “normal” (solitus) relation- which has protruded initially to the right (dextro-
ship between the great arteries; type ZZ, d-transposi- or d-), and hence, the morphologically right ventricle
tion (transposed aortic valve to the right, i.e., dextro- lies to the right, and the morphOlogically left ventricle

THE AMERICAN JOURNAL OF CARDIOLOGY

 Diagnosis of Single Ventricle 349

TABLE I (continued)

Diagnoses at Autopsy
ECG Clinical Diagnoses (Associated Malformations)

$1300, counter, RVH Isolated dextrocardia with corrected trans- Rudimentary spleen (polysplenia); dextrocardia; severe muscular sub-
oosition. PDA. oulmonarv stenosis. SV, valvular Dulmonary stenosis with bicuspid PV; PDA (probe patent);
&iD, absent I’VC, with aiygos draining overriding left-side2 tricuspid valve; v&e incompeteni PFO; absent
into SVC (R) and with LSVC draining IVC with azwos extension emotvine
IV ., ”
into RSVC: LSVCemoties into
into coronary sinus coronary sinus; hepatic veins drain directly into RA ’
+llO’, clock, RVH Corrected transposition, pulmonary atresia, Pulmonary atresia; right Ao arch; right PDA; patent A-V canal;
PDA, right Ao arch Pott’s anastamosis not patent. Basic type of viscera-atria1 situs un-
certain
+lm”, counter, CAE, Isolated dextrocardia with cyanotic CHD Dextrocardia; pulmonary atresia; rt. Ao arch; left PDA; patent A-V
RVH, PI neg. with canal; bilat&al SVC; total anomalous pulmonary vcno& return to
“dome and dart” P RSVC: RIVC: absent coronarv sinus: and 2 left hcoatic wins drain
waves in VI and VP into leit-sided a&n. Prohabl; basicaiiy a sofitus iidividual
- YO”, clock, RVH Isolated dextrocardia with pulmonary Dextrocardia; absent right cor&mry o&m; pulmonary stenosis;
stenosis oatent A-V canal: LSVC: LIVC: and right-sided common DUI-
monary vein. Probably ao’inversus’individual basically 1
+136’, clock, RAE,RVH Taussig-Bing anomaly with probable pre- d-TGA’s occurring in an l-loop is an exception to the usual bulboven-
ductile coax of Ao tricular interrelationships; subaortic stenosis, muscular; preductilc
coax; distal PDA, large
+102’, clock, RVH Preductile coarc An exception to the usual bulboventricular interrelationships; muscular
subaortic stenosis; preductile coarc; large distal PDA
- 1 ZOO, counter, RVH, in- SV, corrected transposition of great vessels Pulmonary stenosis, valvular and subvalvular
termittent WPW and pulmonary stenosis
WPW since 5t/2 yr. of age Cyanotic CHD: levocardia with situs in- Pulmonary atresia; absent right coronary ostium (#I ); two left coro-
~crsus, probably increased pulmonary nary osria (#3); left Ao arch; huge bronchial arteries; patent A-V
flow, WPW syndrome, severe CHF, ter- canal (spleen present)
minal tachycardia
-40°, counter, LVH Transposition with tricuspid atresia, or Aortic stenosis (subvalvular and valvular); hypoplasia of the ascending
truncus arteriosus Ao and preductile coax; large distal PDA; and common A-V valve

+170’, rlock, RVH Transposition, pulmonary atresia, large Pulmonary atresia; absence of MPA; PDA supplying RPA and LPA;
VSD or SV PDA ligated surgically
+1200, clock, RVH Pure pulmonary stenosis Subaortic stenosis, muscular, moderate; and left A-V valve regurgita-
tion

+ 1 ZOO, clock, RAE, RVH Transposition of the great vessels, SV, and Pulmonary stenosis, subvalvular and valvolar
subpulmonary stenosis
- 115’, counter, relatively Cyanotic CHD, or congenital methemoglo- Pulmonary atresia; common A-V valve; coronary sinus absent; bi-
equiphasic R/S from binemia bilateral SVC
VaR to Vs
-lZO’=, clock, RVH Dextrocardia with transposition of great Dextrocardia; pulmonary atresia; PDA; absent left coronary ostium
vessels, pulmonary atresia, PDA, SV or (#3); common A-V valve; total anomalous pulmonary venousdrain-
large VSD, and ASD age, infradiaphragmatic; IVC into left-sided atrium; coronary sinus
into right-sided atrium; bilateral SVC with absence of left innomi-
nate vein

lies to the left (a “normal” or solitus loop) ; l-loop, a findings are summarized in Table I (Cases l-5).
cardiac loop which has protruded initially to the left A representative electrocardiogram (Case 1) is
(levo- or l-), and hence, the morphologically right presented in Figure 1, and a typical angiocardio-
ventricle lies to the left, and the morphologically left gram (Case 3) is shown in Figure 2.
ventricle lies to the right (a mirror-image or inversus
All but Case 1 displayed cyanosis of mild to
loop) ; discordant cardiac look, the type of the cardiac
moderate degree, and even in Case 1 oximetry
loop inappropriate to the type of visceroatrial situs,
revealed distinct systemic unsaturation (resting,
e.g., an Z-loop with situs solitus of the viscera and
atria, or a d-loop with situs inversus. 82.5% and with exercise, 78%). All 5 cases
In single ventricle, the terms “complete” and displayed a systolic murmur, usually maximal
“corrected” transposition often are extremely in- along the upper or mid left sternal border.
accurate, and hence are not used. The intensity varied from grade l/4 to grade 3/4,
Abbreviations: See appended List of Abbreviations. but only 2 patients exhibited a systolic thrill.
An apical diastolic murmur and thrill were
FINDINGS
observed only in Case 3, which exhibited severe
SINGLE LEFT VENTRICLE (ABSENCE OF RV SINUS) congenital mitral stenosis (supravalvular dia-
Single left ventricle with normally related great phragm and valvular thickening).
arteries and situs solitus of the viscera and atria oc- The clinical picture of congestive heart failure
curred in 5 of the 39 patients (13%). The was found in only 2 of the 5 patients, and definite
salient clinical, laboratory, and postmortem cardiomegaly, as judged by the cardiothoracic

VOLUME15, MARCH1965
 Van Praagh, Van Praagh, Wad and Keith

FIG. 1. Case 1. SW& left wntrdr (RI’ .mus afmnt) WI//Ino~mdl~intmrlatrd ,ymt urt~rm and situs sditus of the
viscera and atria: electrocardiogram at 7 years of age. \VPW (Wolff-Parkinson-White) and normally conducted
QRS complexes alternate, producing bigeminy (lead II). Only normally*conducted complexes are shown in
the other leads. Patent A-V canal is accurately suggested by the frontal AQRS ( -75 “). by the frontal QRS
vector loop (counterclockwise, largely above the lead I axis) and by the evidence of combined atria1 and ven-
tricular overloading. However, the presence of a single left ventricle is not evident electrocardiographically
for two reasons: (1) absence of the right ventricular sinus (inflow) is electrically “silent,” being masked by
the signs of hypertrophy of the right ventricular infundibulum (rsR’ in V,R. V,R and VI, and the deep S in
Va of 23 mm.); and (2) the spatial orientation of the ventricular septum is normal, as is the distribution of the
“septal” q waves (Vb-Vs).

ratio, likewise was confined to these 2 cases. axis of lead I) strongly suggested this diagnosis
The pulmonary vascuIarity in the chest roent- in Cases 1 and 2 but were equivocal in Case 4.
genograms appeared increased in all patients The electrocardiogram presented the findings
except one (Case 5) who had pulmonary atresia of left ventricular overloading in 3 cases and of
and a patent ductus arteriosus. combined in 2. Electrocardiographic evidence
It is noteworthy that a patent A-V canal of pure right ventricular overloading was not
(common A-V valve) occurred in 3 of the 5 found. Note that the large left ventricle is
cases. Electrocardiographically, the frontal located to the left and posteriorly (see diagrams,
AQRS (markedly left) and the frontal QRS Fig. 1).
vector loop (counterclockwise, largely above the Only in Case 3 was angiocardiography per-

THE AMERICAN JOURNAL OF CARDIOLOGY

 Diagnosis of Single Ventricle

FIG. 2. Cast 3. Normally related pat arteries, sin,& left mztrtclc and situs solitus of the viscera and atria:
angiocardiogram. A, PA view. B, right lateral view. In both views it is clear that the great ar-
teries are normally interrelated. The locations and planes of the pulmonary valve (PV) and of the
aortic valve ( AoV) are indicated by arrows in B. The large ventricle appears coarsely trabeculated.
Morphologically, however, this large ventricle must be a left ventricle (LV) because a normally re-
lated aorta ( Ao) arises therefrom.4 Similarly, the small outlet chamber from which the normally lo-
cated pulmonary artery (PA) originates must be the right ventricular infundibulum (RV II&).~
The right ventricular sinus was not visualized at any time. The catheter passed from the superior
vena cava (SVC), through the right atrium (RA) into the left ventricle (LV). Contrast medium,
selectively injected into the large LV, passed directly out the normally located Ao, and also entered
the RV infundibulum via a large defect (the bulboventricular foramen), and thence out the normally
located PA. Dye has returned to the large left atrium (unlabelled in A) and is held there by con-
genital mitral stenosis (Table I).

formed (Fig. 2), and only in this case was an the viscera and atria, and in 3 patients (8%) with
anatomically accurate clinical diagnosis asplenia (basic type of visceroatrial situs uncertain).
achieved (Table I). Salient findings are summarized in Table I
Single left ventricle with d-transposition and situs (Cases 12-29). Representative electrocardio-
solitus of the viscera and atria occurred in 6 of the grams are presented in Figures 4 and 5 and
39 patients, 15 per cent of the series (Table I, characteristic angiocardiograms in Figures 6
Cases 6-11 and Fig. 3). and 7.
All of these patients were dead by 3 months of Cyanosis was the rule (16/18). So was a
age. Only half had a murmur and none ex- systolic murmur (14/18), but a thrill was rela-
hibited a thrill. All displayed relative smallness tively infrequent (5/18). The clinical picture
of the bulboventricular foramen, i.e., the aper- of congestive heart failure was observed in
ture leading into the infundibular chamber slightly less than half the cases (8/18), whereas
from which the d-transposed aorta originates. cardiomegaly (a cardiothoracic ratio greater
Thus, all had subaortic stenosis which invariably than 55%) was found in the chest films of two-
was associated with hypoplasia of the ascending thirds (12/l 8).
aorta, coarctation or atresia of the aortic isthmus With I-transposition, the large left ventricle is
and a large distal patent ductus arteriosus. right-sided and somewhat posterior relative to
b All of these patients also exhibited cyanosis, the right ventricular infundibulum (see heart
congestive heart failure, cardiomegaly and in- diagrams in Fig. 4 and 5). As will be con-
creased pulmonary vascularity. The electro- sidered in the Discussion, an understanding of
cardiogram showed left ventricular overloading these positional interrelationships helps in the
in 5 cases (e.g., Fig. 3) and combined in 1 pa- comprehension of the electrocardiographic find-
tient. None showed pure right ventricular ings : right axis deviation of the mean frontal
overloading. QRS vector (> 90°) in 17/18; clockwise
Single left ventricle with l-transposition occurred in frontal QRS vector loop in 16, and counter-
75 patients (3w0 of th e series) zcith situs solitus of clockwise in only 2 (Cases 26 and 28, Table I);

VOLUME 15, MARCH 1965

352 Van Praagh, Van Praagh, Vlad and Keith

FIG. 3. Case 9. Type IIA-solitus. Single left wntricle, d-transposition and situs soiitus of uiscua und atria: elec-
trocardiogram at 5 days of age. This tracing was interpreted as showing right atria1 enlargement (P,, 4 mm..
peaked) and left ventricular overloading (S in Vr 22 mm.) which, however, did not appear marked (frontal
AQRS + 60”. and frontal QRS vector loop clockwise, largely below the axis of lead 1). Note that the large
left ventricle is posterior and only mildly to the left of the right ventricular infundibulum (see heart diagram
within thorax). No infundibular complexes 5.6 (late R’, R notching or slurring) were recorded. The infundib-
ulum was relatively small, associated with a small foramen leading into the infundibulum, which produced
subaortic stenosis (Table II). Thus, the electrocardiogram reflects the ventricular anatomy with consider-
able accuracy but certainly is not diagnostic of single left ventricle.

and the patterns of right ventricular overloading to the usual bulboventricular interrelation-
in 12, combined in 4, equiphasic RS complexes ships4 Usually, d-transposition occurs in a
in 1, and incomplete electrocardiogram (no d-loop and l-transposition occurs in an l-loop.
chest leads) in 1 (Case 16, Table I). None dis- These 2 cases (30 and 31, Table I) displayed a
played the picture of pure left ventricular over- clinical picture very similar to that of single left
loading. ventricle with d-transposition (cf. Cases 6-11,
Clinical diagnoses were anatomically accurate Table I). Both had preductile coarctation,
and complete only when selective ventricular hypoplasia of the ascending aorta, and smallness
angiocardiography was performed (e.g., Fig. 6 of the bulboventricular foramen producing
and 7). subaortic stenosis. Both died at an early age,
Single left ventricle, witfl d-transposition in an and during life displayed cyanosis, congestive
1-bulboventricular loop occurred in 2 patients (570 of heart failure, marked cardiomegaly and in-
the series), bath with situs solitus of the viscera and creased pulmonary vascularity. The severity
atria (Cases 30, 31, Table I). Such cases are of the clinical picture and the very bad prognosis
decidedly infrequent’ and constitute exceptions appeared to be determined principally by the

THE AMERICAN JOURNAL OF CARDIOLOGY

 Diagnosis of Single Ventricle 353

FIG. 4. Case 13. Single lef; ventricle with I-transfiositionand situs solitus of the viscera and atria: electrocardiogram at lOi/s
years of age. The frontal AQRS is +80”, with a clockwise frontal QRS vector loop largely below the axis of lead I;
this tracing suggests combined ventricular overloading (R in Vi 25 mm. and in Vg 35 mm.). In earlier tracings there
was pure right ventricular hypertrophy, without q wave in VG or V,, but all records showed deep Q waves in the right
precordial leads, suggesting an I-loop (with Z-transposition). The appearance of combined ventricular overloading
and q in VS and Vr should suggest that one is not dealing with a usual E-loop. Indeed, this observation might correctly
suggest the presence of a large left ventricle, being recorded by both right and left precordial leads. The clinical
diagnosis was Eisenmenger’s complex ; in this condition, the late appearance of q waves in Vs and V, should be re-
garded as extraordinary and should indicate the wisdom of selective ventricular angiocardiography to clarify the
diagnosis.

presence of subaortic stenosis and preductile coarcta- ings of single left ventricle with I-transposition.
tion.
These 2 patients exhibited electrocardiographic SINGLE RIGHT VENTRICLE (ABSENCE OF LV SINUS)

jndings typical of single left ventricle in an l-bulbo- This rare anomaly occurred in 2 patients, or
ventricular loop (cf. Cases 12-29, Table I, and 5% of the series (Table I, Cases 32 and 33).
Fig. 5): right axis deviation of the mean Both had discordant cardiac loops: an l-loop
frontal QRS vector, clockwise frontal QRS with Z-transposition in a solitus patient (Case 32,
vector loop, and the pattern of right ventricular Fig. 8), and a d-loop with d-transposition in an
overloading. inversus patient (Case 33). Both also displayed
Thus, these 2 exceptional cases displayed the cyanosis, pulmonary outflow tract obstruction, a
clinical picture of single left ventricle with systolic murmur and thrill, the clinical picture
d-transposition, and electrocardiographic find- of congestive heart failure and the Wolff-Parkin-
VOLUME 15, MARCH 1965
 \'an Praagh, Van Praagh, Vlad and Keith

FIG. 5. Case 25. Lkxtrocardia zelth I-t,-ampusitun. (y,ylc I?// vnt~~cle jnqht-sided) md sztrrs sclitus of the viscera and &a:
electrocardiogram at 3 months of age. The frontal AQRS is +150 a and the frontal QRS vector loop is clockwise and
almost all below the lead I axis. This tracing could be interpreted as showing right ventricular overloading, i.e., over-
loading of the right-sided ventricle (S in VL 40 mm. and R in V,R 16 mm. with S in V& 0 mm.). However, the mor-
phology of the precordial leads suggests ventricular inversionTa*: rS and KS in the left chest leads, and qR in V&R
and VsR. Hence, the electrocardiogram correctly indicates overloading of the left ventricle (right-sided). Absence
of the right ventricular sinus (left-sided) is electrically “sil?nt”: thus, this electrocardiogram is not diagnostic of single
left ventricle.

son-White syndrome. The electrocardiogram (clinical, radiologic and electrocardiographic,

of Case 32 is presented in Figure 8. Table differed markedly, apparently due to
I)
different associated anomalies. The electro-
UNDIVIDED VENTRICLES (ABSENT OR RUDIMENTARY cardiogram of Case 34 is shown in Figure 9.
VENTRICULAR SEPTUM)

This infrequent malformation was found in 2 INF~NDIB~L~M ONLY (FAILURE OF DEVELOPMENT

patients, or 5 per cent of the series (Cases 34 and OF BOTHVENTRICULAR SINUSES AND OF THE VEN-

35, Table I). Both exhibited situs solitus of the TRICULARSEPTUM)

viscera and atria, d-transposition of the great Four patients (Cases 36-39, Table I, and Fig.
arteries and cyanosis. However, other findings 10 and 11) displayed this severe anomaly (lOyO

THE AMERICANJOURNAL OF CARDIOLOGY

 FIG. 6. Case 22. Single 1eJt ventricle, l-tronrposition and situs solitus of the viscera and atria: selec-
tive angiocardiogram. The catheter was passed down the superior vena cava (SVC), through
the right atrium (RA), into the single left ventricle (LV). The catheter tip fortunately pointed
directly at the defect (D), i.e., at the bulboventricular foramen, leading into the right ven-
tricular infundibulum (RV Inf.) from which the I-transposed aorta (Ao) originates. The Ao
and aortic valve (AoV) are left, anterior and superior to the PA and pulmonary valve (PV).
Ao is much smaller than PA, reflecting the subaortic stenosis produced by the small defect
(D). The preductile coarctation is not well seen. The RV sinus (inflow) was never visua-
lized at any time. The infundibular outlet chamber (RV Inf.) is the hallmark of single LV.

TABLE II
Catheterization Findings in 12 Patients With “Single” Ventricle

7-p 01 Determinations PTSSW.S

(% Sat.1 (mm. Hg)
Case Age at “Single” Syrt. “ Single” Syst.
No. Cath. WC IVC RA V PA Art. V PA Art.

1 4 yr., 3 mo. 43 55 78 89 92 105/15 130/75

13 6 yr., 5 mo. 63 77 62 91,97,96 ;4 97 80/O 7;;25 85/45
15* 3 wk. 34 44 33,30 44 60/ 0 9/3 65/25
17 4 yr., 10 ma &t 52 55 77,87 82 70/ 2 75/37
1st 2 yr., 11 ma 46 34 98,68, 85 8; 95 143/3 66;24 100/50
19 11 mo., 2 wk. 48 j; 42 79,81, 54 70 88 113/3 125/48 150/75
94/ 67
23 2 mo., 3 wk. 52 51 54 65,74,95 82 109/o 110/56
88 127/O
24 7 wk. 61,60 70, 68 91,76,79 88, 86 92 80/6 70/30 SO/ 50
58 71 74, 89, 941
25 3 ma, 3 wk. 51 58 85, 88 86 84 89 67/12 60/31 74/52
26: 5 mo., 2 wk. 46 38 . 51 96/12 96/42
32 20 yr., 1 mo. 70 65 77 ss 87 112/5 15;6 106/75
21 yr., 2 mo. ki 46. 53,753 98 68 102/3 102/63
37 7 mo. 36 ii 32 50,73,80 50 7R/3 . go/57
100/3

* Pulmonary stenosis, severe.

t Aortic stenosis, subvalvular(smallbulboventricular foramen).
$ In the infundihular outlet chamber.

VOLUME 15, MARCH 1965

356 Van Praagh, Van Praagh, Wad and Keith

FIG. 7. Case 27. Sin& leftventricle with I-transpositionand aspleniu: angiocardio-

gram. The catheter has passed through the inferior vena cava (WC), right-sided
atrium, common A-V valve, single left ventricle (LV) and through the large
bulboventricular foramcn (D), lodging in the right ventricular infundibulum
(KV Inf.). Dye outlined the infundibular chamber well (u) and regurgitated
through the large defect (D) into the single LV, after which the large aortic valve
(AoV) and the dilated aorta (Ao) were clearly seen. Pulmonary atresia is evi-
dent (c and d), the small pulmonary branches tilling via a small patent ductus
arteriosus (PDA). Despite the fact that the pulmonary artery is not well demon-
strated. I-transposition obviously is present because the .40 originates anteriorly
from the left side of the ventricular base, arising from the infundibulum or distal
bulbus cordis, as transposed aortas always do. There is a right aortic arch.

of this series). Two had situs solitus of the DISCUSSION

viscera and atria, whereas 2 displayed the

DIAGNOSIS OF SINGLE VENTRICLE
asplenia syndrome. All 4 exhibited cyanosis
and d-transposition. The electrocardiogram of Our experience may be summarized as fol-
Case 38 (Fig. 10) showed relatively equiphasic lows: The history, physical examination and
RS complexes in the precordial leads, and the chest roentgenograms are not specific. The
other 3 patients had a pattern of right ven- electrocardiogram may suggest this possibility,
tricular overloading. The angiocardiogram of but often it does not. Cardiac catheterization
Case 37 is presented in Figure 11. often provides the first clue that a single ven-
tricle is present, and angiocardiography is diag-
CATHETERIZATION FINDINGS
nostic.
Hemodynamic data in 12 patients with single Two major types can be distinguished angio-
ventricle are summarized in Table II. cardiographically: (1) single ventricle with an

THE AMERICAN JOURNAL OF CARDIOLOGY

 Diagnosis of Single Ventricle 357

FIG. 8. Case 32. Type IIIB-solitus. of viscera and atria.

Single ri,$zt oentricle (L V absent) with l-transpositionand situs soli~uus
electrocardiogram at 21 years of age. The combination of severe left axis deviation (frontal AQRS, -120”), the
counterclockwise frontal QRS vector loop (all above the axis of lead I), and marked right ventricular overloading in
the chest leads (R in Vi 21 mm., S in Vi 0 mm., R in Vs 2 mm., S in Ve 15 mm.) might well erroneouslysuggest the
presence of a patent A-V canal. Many leads show the picture of “incomplete” right bundle branch block. The pre-
cordial electrocardiogram accurately indicates the presence of extreme right ventricular hypertrophy. However, in
view of the severe left axis deviation, it would be difficult to deduce from this tracing alone that the left ventricle is
absent. Angiocardiography disclosed l-transposition, indicating that an l-loop (right ventricle left-sided and left
ventricle right-sided) is very probable. It is important to note that all leads display a right ventricular type of QRS
morphology,r** the R or S being broad, slurred or notched, suggesting that all leads are recording a huge right ventricle.
In an I-loop, left ventricle and the ventricular septum should be rightward and posterior to the right ventricle. If the
main A-V bundle is right and posterior (which requires histologic verification), and since the right-sided left ventricle
is absent, the frontal plane QRS loop is likely to be inscribed counterclockwise. This hypothesis seems to provide a
reasonable explanation of the counterclockwise loop occurring with a pattern of right ventricular hypertrophy, in the
absence of a patent A-V canal. None of the leads display a left ventricular type of QRS morphology.r,s This should
suggest that the left ventricle is small or absent, but it is not regarded as diagnostic of the specific anomaly present in
this case.

outlet chamber, and (2) single ventricle without an left ventricle. The outlet chamber is the right
outlet chamber. An outlet chamber is present in ventricular infundibulum. The principal ven-
Figures 2, 6 and 7 but is absent in Figure 11. tricular malformation is absence of the right
From the anatomic standpoint, single ven- ventricular sinus (body or inflow tract). This
tricle with an outlet chamber is one entity, results in a single left ventricle.
whereas single ventricle without an outlet Single ventricle without an outlet chamber
chamber is a heterogeneous group of three un- includes single right ventricle (absent left ven-
related anomalies.’ tricular sinus), undivided ventricles (absent or
In single ventricle with an outlet chamber, rudimentary ventricular septum), and infundib-
the single ventricle morphologically is a large ulum only (both sinuses absent).

VOLUME 15, MARCH 1965

 Van Praagh, Van Praagh, Vlad and Keith

FIG. 9. Case 34. Cndioided omtricles (rudimentaryventricularsepturn)_mzthd-transpositzonand situs solitus of viscera and atria:
electrocardiogram at 2 days of age. Left axis deviation (frontal AQRS, -40 “) and a counterclockwise QRS loop in
the frontal plane largely above the axis of lead I correctly suggest the presence of a patent A-V canal. The precordial
leads suggest left ventricular overloading. The common A-V valve opens principally into the left ventricular sinus
(diagram) which is better developed than that of the right. Concerning ventricular localization, the slurring and notch-
ing of the R waves in aVL and in V,-V, could erroneously suggest that the right ventricle is left-sided. Within the
limitations of our present understanding, this tracina does not suggest virtual absence of the ventricular septum.

Single left ventricle with an infundibular LS SINGLE VENTRICLE A HUGE VSD?

chamber is the frequent type (79yo of this series), The widespread view”-14 that single ven-
single ventricle without an outlet chamber being tricle results from virtual absence of the ven-
the infrequent group (21yo). tricular septum is almost always erroneous. As
Diagnostically, single ventricles with outlet the primary malformation, absence of the ven-
chambers can be distinguished from those with- tricular septum is rare (5% of this series). It is
out, as noted above. However, at the present one of the three types of single ventricle without
time we do not know how to separate and an outlet chamber. Almost invariably, how-
identify each of the three different anomalies ever, involvement of the ventricular septum
lacking an outlet chamber. Our experience appears to be secondary to absence of either or
with the latter group is very limited (8 cases). both ventricular sinuses.

THE AMERICAN JOURNAL OF CARDIOLOGY

 Diagnosis of Single Ventride

FIG. 10. Case 38. Type IID heterotaxy. Infundibulum only (failure of development of both ventricular sinuses and of the ven-
tr@&zr septum), with d-transpositiorr and asplenia: electrocardiogram at 3 days of age. The marked left axis deviation
(AQRS, - 115 “) and the counterclockwise frontal QRS vector loop accurately suggest patency of the A-V canal.
The precordial electrocardiogram displays relatively equiphasic R/S complexes from VaR to Vs, which has been re-
garded as suggestive of “single” ventricle2*sf10 but which occurred in only 3 of the 38 cases with chest leads (80/c of the
series, Cases 10, 21, 38, Table I). There is no electrocardiographic evidence of selective ventricular overloading,
This patient had asplenia, pulmonary atresia, patent A-V canal and bilateral SVC’s. Note that the AP is normal
(PI+, PaVR-, etc.), this being relatively .%
infrequent in our experience with asplenia. 4 Bilaterality of S-A node tissue”
may well underlie the variability of the AP in asplenia.

In text books, single ventricle often appears RELATIONSHIPS BETWEEN THE GREAT ARTERIES
in the chapter on ventricular septal defects, for Transposition of the great arteries, to our
obvious hemodynamic reasons. However, from knowledge, always occurs in single ventricle
the developmental and anatomic standpoints, without an outlet chamber, and it frequently
single ventricle belongs in a chapter concerned occurs in single left ventricle with an infundib-
with failure of ventricular development. The ular chamber (84% of this series).
usual problem, in fact, is ventricular absence
(of right, left, or both ventricles), not a huge AORTIC AND PULMONARY STENOSIS

ventricular septal defect. Indeed, the ven- It has been statednJ3 that the great artery
tricular septal defect (i.e., the bulboventricular arising from the outlet chamber usually is
foramen) not infrequently is obstructively small, stenotic, and that the great artery originating
producing outflow tract obstruction : subaortic from the single ventricle usually is not ob-
or subpulmonary stenosis, depending on which structed. This clear-cut relation was not found
great artery arises from the infundibular cham- in our 31 cases of single left ventricle.
ber. A normally located pulmonary artery arose from

VOLUME 15, MARCH 1965

 Van Praagh, Van Praagh, Wad and Keith

FIG. 11. Case 37. rnfundibulum only, rc,iUs d-trnnsposition and s&s solitus of the cisczra and atria: angiocardio-
gram. The right-sided location of the inferior vena cava (IVC) accurately indicates situs solitus of the
viscera and atria (0). The aorta (Ao) is to the right of the pulmonary artery (PA) (a, c), and Ao is
anterior to PA (b. d), indicating d-transposition of the great arteries. The prominent crista supra-
veutricularis (CS) separating the outflow tracts is well seen (b), as is the moderate subvalvular and
valvular pulmonary stenosis (PS) (6, d). The ventricular cavity opacifies rapidly and completely,
there being no suggestion of a ventricular septum. It is important to note that beneath the d-transposed
aorta there is no discrete infundibular chamber. There is a well developed subaortic infundibulum, this
being typical of transposition of the great arteries, extending from the crista supraventricularis (CS)
inferiorly to the aortic valve (AoV) superiorly. However. this subaortic infundibulum does not form a
discrete outlet chamber, the hallmark of absent right ventricular sinus or single left ventricle (cf. Fig. 2,
6, 7).

the infundibular chamber in 5 casts; 4 had in t? (all had I-transposition), subaortic stenosis
no pulmonary stenosis and 1 had pu’monary in 14 (8 had d- and 6 had I-transposition).
atresia. However, all 3 cases recently reported Subaortic stenosis occurred in all of our cases of
by Elliott and associates15 did have pulmonary single left ventricle with d-transposition.
stenosis. Thus, pulmonary stenosis is a variable Thus, the presence or absence of aortic or
in such cases. pulmonary stenosis appeared unrelated to
A transposed pulmonary nrtvry originated from whether or not the great artery arose from the
the single left ventricle in 26 cases; there was outlet chamber or from the single left ventricle.
no obstruction in 17, stenosis in 5 and atresia in However, in our cases of single left ventricle,
4. These 9 cases with pulmonary obstruction all the type of relation between the great arteries
occurred with I-transposition. did seem to be related to the state of the outflow
A normally related aorta arose from the single left tracts. With d-transposition, all had muscular
ventricle in 5 cases; there was no obstruction subaortic stenosis, hypoplasia of the ascending
in 3, and muscular subaortic stenosis in 2. aorta, preductile coarctation or atresia, and
A transjwsed aorta originated ,from the infundibular distal patent ductus arteriosus. None had pul-
chamber in 26 cases no obstruction occurred monary obstruction. With l-transposition, one-

THE AMERICAN JOURNAL OF CARDIOLOGY

 Diagnosis
third had mild to moderate subaortic stenosis,
without coarctation. But one-half exhibited
subpulmonary stenosis or atresia. Thus, single
left ventricle with d-transposition was prone to aortic
obstruction (700%), w he reas single left ventricle with
l-transposition was moderately prone to pulmonary
obstruction (50%).
HEART SIZE
It has been statedlZJ3 that the heart usually is
not enlarged in single ventricle. However, if
cardiomegaly is considered present when the
cardiothoracic ratio in chest roentgenograms is
55 per cent or greater, the heart was enlarged in
30 of our 38 cases (79a/c, Table I). Relatively
normal heart size (cardiothoracic ratio less than
55%) occurred only in patients with reduced
pulmonary blood flow (pulmonary atresia or
severe stenosis). Thus, cardiomegaly is usually
present in patients with single ventricle.
INFANTS WITH CYANOSIS, COARCTATION,
CREASED PULMONARY FLOW AND LEFT
TRICULAR OVERLOADING
When a young infant displays cyanosis, coarcta-
tion(established by arm and leg blood pressures),
increased pulmonary vascularity (in the chest roent-
genograms) and left ventricular overloading (in the
electrocardiogram), this unusual clinical picture
should suggest two diagnostic possibilities: (1)
single left ventricle with d-transposition, subaortic
stenosis, preductile coarctation and distal patent
ductus arteriosus ; or (2) tricuspid atresia with
d-transposition, subaortic stenosis, preductile
coarctation and distal patent ductus arteriosus.
Angiocardiography will establish the state of the
tricuspid orifice, thereby distinguishing these
two entities. This distinctive clinical picture
was displayed by Cases 6-11 (Table I).
In the more usual case of preductile coarcta-
tion in an infant with two ventricles, the electro-
cardiogram shows right ventricular overloading.
However, the electrocardiogram in these cases
of single left ventricle almost always displays the
picture of left ventricular overloading, very
probably because the large left ventricle is left-
sided and posterior, relative to the ventricular
septum (cf. Fig. 3).
ECG FINDINGS
The electrocardiographic findings usually are
not specific for single ventricle. Hence, the
electrocardiogram as a rule is not diagnostic,
and often is not even suggestive of single ven-
tricle. Paradoxically, however, these tracings
VOLUME 15, MARCH 1965
of Single Ventricle 361
usually do reflect the ventricular anatomy well,
a fact which frequently is understood only
retrospectively following angiocardiography or
autopsy. Clinically, one must search for “diag-
nostic” electrocardiographic and vectorcar-
diographic patterns, realizing ,a11 the while that
probably no pattern is entirely specific for only
one pathologic state; Wilson and associates’
adagel that “electrocardiographic abnormal-
ities are not diseases” is well demonstrated by
this “entity.”
A basic fact is that single ventricle is not one
entity anatomically. E ectrocardiographically,
if one treats single ventricle as an entity (as is
done in current cardiologic text books), the only
picture which can emerge is one of extreme
variation. This has been a widespread experi-
ence.g,lO.17-19 A key to the understanding of
the electrocardiogram in these different anom-
alies is to study each individually, just as one
would with any other group of dissimilar mal-
IN-
formations.
VEN- In single left ventricle with an infundibular outlet
chamber, the type of cardiac loop appeared largely to
determine both the frontal LQRS and the type of ven-
tricular overloading pattern.
When the single left ventricle was left-sided
(because of a d-bulboventricular loop), the
frontal AQRS almost always showed no devia-
tion, or left deviation (Fig. 12 left, 1 and 3).
The patterns of ventricular overloading were
left in 8, combined in 2, and equiphasic RS in 1.
The picture of right ventricular overloading
was not observed when the large left ventricle
was left-sided (Table I).
When the single left ventricle was right-sided
(because of an I-bulboventricular loop), the
frontal AQRS almost always showed right axis
deviation (Fig. 12 center, 4 and 5). The pat-
terns of ventricular overloading were : “right”*
in 14, combined in 4, equiphasic R/S in 1 and
unknown in 1 (Case 16, no precordial leads).
The pattern of left ventricular overloading was
not found when the large left ventricle was right-
sided (Table I).
Outflow tract obstructions such as pulmonary
stenosis, pulmonary atresia or aortic stenosis had
no obvious affect upon the frontal AQRS or the
ventricular overloading pattern. However, pa-
tent A-V canal was associated with typical
changes of the frontal AQRS in 6 of the 8 cases
(Fig. 1 and 12).
* This “right” ventricular overloading in fact is left
ventricular overloading, right-sided (cf. Fig. 5 ).
 362 Van Praagh, Van Praagh, Wad and Keith

FIG. 12. The mean manifEst AQRS in the frontal plane in

38 GDS~Sof single ventricle. Type A, d-loop: single left
ventricle with an infundibular chamber (absent RV
sinus) in a d-loop (large left ventricle is left sided).
Solid circles indicate 2 A-V valves. Open circles indi-
cate a common A-V valve (patent A-V canal). Type
A, I-loop: single left ventricle with an infundibular
chamber in an I-loop (large left ventricle is right-sided).
Types B, C and D: single ventricle without an outlet
chamber (see classification).

High voltage mid-&cordial RS complexes (the served in 4 patients (Cases 1, 18, 32 and 33,
Katz-Wachtel sign) occurred in our Case 10 Table I). Cases 1 and 18 had absence of the
(Table I) and also was observed by Shaher” and right ventricular sinus, whereas Cases 32 and 33
by Elliott and associates (their Fig. 6B).l5 displayed absence of the left ventricular sinus.
Since this sign also has been found in hearts Three of these 4 patients exhibited discordant
with two ventricles and combined ventricular cardiac loops. Similar conduction disturb-
overloading,20 it clearly is not diagnostically ances were found by Shaher.” Diagnostically
specific. they are not specific for single ventricle.
Right ventricular Iypertrophy in the electrocardiogram Dome and dart P waves, as described by
with absence of the right ventricular sinus is note- Mirowski and co-workers,22 were found only in
worthy in our Figure 1, and in Figure 1 of Case 28 (Table I). They were seen best in Vr
Mandel and Hirsch.21 This picture probably is and Vz, and Pr was negative. Anatomically,
related to hypertrophy of the infundibular we were certain that the atria were in situs
chamber. solitus in this asplenic individual; (with as-
Heart block was recorded in 3 patients (Cases plenia, the situs of the viscera and atria often
12, 16 and 17, Table I). All had discordant remains anatomically uncertain or indeter-
Z-loops, with absence of the right ventricular minate) .l s4 As is usual with asplenia, bilat-
sinus. eral superior venae cavae were present. Left
The WolfJ-Parkinson- White syndrome was ob- to right activation of these solitus atria may

THE AMERlCAN JOURNAL OF CARDIOLOGY

 Diagnosis of Single
well have been initiated by a left S-A node, the
presence of which recently has been demon-
strated in asplenic individuals by Van Mierop
and associates. *I1
Regarding the electrocardiogram in single ventricle
without an outlet chamber, our experience with
these three different anomalies is much too
limited to permit general conclusions. It is
noteworthy that the location of the ventricular
septum usually cannot be established anatomi-
cally in this group, except when the ventricular
septum is rudimentary, as in Figure 9. Hence,
the probable location of the conduction system
usually cannot be deduced from gross anatomic
findings. Thus, the findings in this group of 8
patients are presented without discussion (Cases
32-39, Table I, and Fig. 8-10 and 12). Studies
of the conduction system are needed in all types
of single ventricle to clarify the sequences of
ventricular activation.
Q waves in the precordial electrocardiogram cannot
be used to plot the plane of the abnormally
located ventricular septum in single ventricle,
contrary to our earlier interpretation.2” Sub-
sequent experience24 has demonstrated the
considerable variability of precordial q waves,
which makes the above-mentioned approach
unreliable.
The literature concerning the electrocardiogram in
single ventricle is now somewhat easier to under-
stand. Keith and associates3 described three
patterns as suggestive of single ventricle:
(1) right ventricular hypertrophy with a qR
pattern in the right chest leads and a relatively
equiphasic RS pattern in the left chest leads;
(2) negative complexes across the precordium
(also described by Freireich and Nicolson25
and by Taussign) ; and (3) equiphasic com-
plexes across the precordium (also reported by
Nadass and by Elliott and associateslO).
The tracing of right.ventricular hypertrophy
(pattern 1) is like our Figure 5: the qR mor-
phologies in V& and VsR suggest that the
left ventricle is right-sided;‘,8 the RS pattern in
the left chest leads suggest that the right ven-
tricle is left-sided;7.8 and the S&P is normal
(solitus). Hence, this is the cardiogram of an
l-loop (with l-transposition) in a solitus indi-
vidual. It is important to appreciate that
usually one can not distinguish with certainty,
by means of the electrocardiogram alone, be-
tween l-transposition with two ventricles, and
* In patients with asplenia, the large left lobe of a
relatively symmetric liver may be mistaken for a palpable
spleen, as occurred in Case 29.
VOLUME 15, MARCH 1965
Ventricle 363
l-transposition w th absence of the left-sided
right ventricular sinus (single left ventricle).
Negative complexes across the precordium
(pattern 2) were not found in the present study.
This precordial QS pattern appears to be dis-
tinctly infrequent.
Relatively equiphasic complexes across the
chest (pattern 3) were observed in only 3 of the
38 patients with precordial tracings (8oj,, Cases
10, 21 and 38, Table I and Fig. 10). Such
“single pattern” complexes occurred with ab-
sence of the right ventricular sinus in a d-loop
(Case 10) and in an Z-loop (Case 21) and ab-
sence of both sinuses in a d-loop (Case 38).
Thus, the pattern of relatively equiphasic RS
complexes across the precordium was both in-
frequent and anatomically nonspecific.
CATHETERIZATION FINDINGS
Often the first clue to the presence of a single
ventricle was provided by a marked increase in
oxygen content at the “right” ventricular level,
an increase which seemed far too large for a
patient with systemic pressure in the “right”
ventricle. This led to repeated sampling
which revealed an extraordinarily wide range
of oxygen content from different sites within
the “right” ventricle, and even from the same
site. Some degree of systemic unsaturation
(less than 95%) was the rule, occurring in 10 of
the 12 patients (Table II).
The sharp increase in oxygen content at the
ventricular level can be masked by a large left
to right shunt at the atria1 level, as in Case 25
(Table II). Or, the large left to right shunt
at the ventricular level, in fact, may not occur
in two situations: (1) when the pulmonary blood
flow is greatly reduced, due to pulmonary steno-
sis or atresia with inadequate collateral circula-
tion (e.g., Case 15, Table II), and (2) with totally
anomalous pulmonary veins, both of which are
to be anticipated in cases of single ventricle with
asplenia. Similar findings have been reported
by Taussigi3 and Nadas.g
In cases of single left ventricle with infundib-
ular chamber and transposition of the great
arteries (d- or l-), if the systolic pressure in the
ascending aorta is distinctly less than in the
single left ventricle, subaortic stenosis is very
probable. Such stenosis usually is produced by
a small ventricular septal defect (the bulbo-
ventricular foramen) which leads from the
single left ventricle into the infundibular cham-
ber from which the transposed aorta originates
(e.g., Case 18, Table II).
 Van Praagh, Van Praagh, Vlad and Keith

Hemodynamically, we do not know how to discrete infundibular chamber is the hallmark of

distinguish reliably between a pulmonary flow single left ventricle (absence of the right ven-
gradient and mild pulmonary stenosis. When- tricular sinus) ; it is well seen in Figures 2, 6 and
ever we thought we had found a case with a 7. The right ventricular infundibulum forms a
pulmonary flow gradient, reexamination of the discrete chamber because the right ventricular
heart specimen always revealed a mild organic sinus, which should be located beneath the in-
stenosis, usually subvalvular and muscular, fundibulum, is absent. This results in the
with or without valvular stenosis. Thus, pul- ventricular septum being displaced towards
monary flow gradients seemed to be infrequent the side of the absent right ventricular sinus
with single ventricle. (‘just as the mediastinum is shifted towards the
side of an absent lung). Hence, the ventricular
ANGIOCARDIOGRAPHICFINDINGS septum forms a ledge or shelf beneath the
Selective ventricular angiocardiography is the infundibulum, creating the appearance of a
diagnostic method of choice in single ventricle. chamber. The chamber’s inlet is the space above
In this series, there were venous, atrial, ventricu- the ventricular septum, and the outlet may be
lar and outlet chamber injections available for either or both great arteries.
study. As expected, ventricular and outlet In conclusion, absence of the right ventricular
chamber injections delineated the ventricular sinus can be diagnosed angiocardiographically
anatomy best. by the infundibular chamber which remains.
With venous angiocardiography, if there is a However, we do not know how to separate
defect in the atria1 septum, dye may opacify diagnostically the three comparatively rare
both atria and enter the single ventricle approxi- types of single ventricle without an infundibular
mately simultaneously from each atrium. chamber.
Streaming of contrast can create the false ap-
pearance of a normally located ventricular sep- SUMMARY
tum beneath the atria1 septum, as occurred in The present report seeks to determine whether
Case 21. Dye in the auricular appendages may or not one can accurately diagnose the various
obscure the relation between the great arteries anatomic types of single ventricle by clinical
in the lateral films. However, even with a and laboratory methods.
venous injection, it is often possible to demon- The history, physical examination, chest
strate the outlet chamber but usually not well roentgenograms and electrocardiogram usually
(e.g., Fig. 8B of Elliott and associates’“). were diagnostically nonspecific. Despite its
With left atria1 injection, the single left ventri- lack of specificity, the electrocardiogram in
cle and the infundibular chamber may be seen single left ventricle with an infundibular outlet
somewhat more clearly (e.g., Fig. 3B of Anselmi chamber usually could be understood in terms
et a1.26). of the type of bulboventricular loop which was
With injection into the single ventricle, the present.
anatomy of the large ventricle and of the outlet Cardiac catheterization often provided the
chamber, if present, usuall), is well visualized first clue to the presence of a single ventricle:
(Fig. 2 and 11). If the catheter tip happens to a large left to right shunt at the ventricular level,
be located at the defect (Fig. 6) or within the despite systemic pressure in the “right” ventri-
outlet chamber (Fig. 7), demonstration of the cle : and an extraordinarily wide range of oxygen
subaortic anatomy is excellent. content from different sites in the ventricle, and
The reason that clear visualization of the even from the same site.
subaortic anatomy is so important, particularly Selective ventricular angiocardiography is the
with l-transposition, is that the aorta may origi- diagnostic method of choice. Two groups can
nate from an infundibular chamber or from a be distinguished : (1) single ventricle with an
completely formed, left-sided right ventricle. outlet chamber, and (2) single ventricle without
The latter is amenable to open heart surgery, an outlet chamber.
whereas the former at present is not. Those with an outlet chamber have absence
The presence or absence of the right ventricu- of the right ventricular sinus (inflow tract).
lar sinus with I-transposition also may be clearly This results in a single left ventricle with an
established 1,~ subaortic injection via a retro- infundibular outlet chamber, the relatively
grade aortic catheter, as i-1 Case 24. frequent type of single ventricle (79yc of this
An angiocardiographically and anatomically series).

THE AMERICAN JOURNAL OF CARDIOLOGY

 Diagnosis of Single Ventricle 365

APPENDIX
Key to Abbreoiations
A = anterior LVH = left ventricular hypertrophy
Ao = aOrta MDM = mid-diastolic murmur
AoV = aortic valve Ml = mitral insufficiency
Art = artery mos = months
ASD = atria1 septal defect MPA = main pulmonary artery
A-V = atrioventricular NK = not known
Bilat = bilateral P = posterior
C = continuous (murmur) PA = pulmonary artery
CAE = combined avial enlargement PDA = patent ductus arteriosus
CAH = combined atria1 hypertrophy PFO = patent furamen ovate
Cath = catheterizadon ._ Postop = postoperatively
CHD = congenital heart disease PV = pulmonary valve
CHF = conrestive heart failure R = right, rest
Clock = clockwise RA = right atrium
Coarc = coarctation RAH = right atria1 hypertrophy
Conga = congenital RPA = right pulmonary artery
Cot = coronary artery RSVC = right superior vena cava
Counter = counterclockwise RV = right ventricle
CVH = combined ventricular hypcrtrophy RVH = right ventricular hypertmphy
CTR = cardiothoracic ratio S = systolic
D = dextro, diastolic, defect Sat = saturation
EFE = endocardial fibroelastosis SV = single ventricle
Ex = exercise SVC = superior vena cava
GA’s = great arteries Syst = systemic
Gr = grade T-E = trachco-esophageal
Infund = infundibulum TGA’s = transposition of the great arteriu
IVC = inferior vena cava ULSB = upper left sternal border
L = levo, left V & A = viscera and atria
LAH = left atria1 hypertrophy Vast = vascularity
LIS = left interspace Ventric = ventricular
LLSB = lower left sternal border VSD = ventricular septal defect
LPA = left pulmonary artery Wk$ = weeks
LSVC = left,superior vena CaYa WPW = Wolff-Parkinmn-White
LV = left ventricle Yrs = years

Those without an outlet chamber comprise a epicardial potentials in right ventricular prepon-
derance. Am. Heart J., 57: 578, 1959.
comparatively infrequent group (21aj,), made up
7. PORTILLO, B., ANSELMI, G., SODI-PALLARES,D. and
of three unrelated malformations: (1) absence MEDRANO, G. A. Importance of the unipolar
of the left ventricular sinus, resulting in a single leads in the diagnosis of dextrocardias, levo-
right ventricle; (2) absence or rudimentary de- cardias, dextropositions and dextrorotations. Am.
velopment of the ventricular septum, resulting Heart J., 57: 396, 1959.
8. SODI-PALLARES, D., BISTENI, A., FISHLEDER, B. L.
in undivided ventricles; and (3) absence of and MEDRANO, G. A. Importance of the uni-
both ventricular sinuses and of the ventricular polar morphologies in the interpretation of the
septum, leaving the infundibulum only. HOW- electrocardiogram: The theoretical basis of the
ever, from the angiocardiographic standpoint, unipolar morphologies and its correlation with
vectorial analysis, with cardiac activation and
we are unable to distinguish these three different
with the potential variations at the epicardial
types of single ventricle without an outlet surface of the heart. Am. Heart J., 57: 590, 1959.
chamber. 9. NADAS, A. S. Pediatric Cardiology, p. 453. Phila-
delphia, 1963. W. B. Saunders Company.
REFERENCES 10. ELLIOTT, L. P., ANDERSON, R. C., ADAMS, P. and
1. VAN PRAAGH, R., ONGLEY, P. A. and SWAN, H. J. C. EDWARDS, J. E. Vectorelectrocardiogram in
Anatomic types of single or common ventricle in single ventricle (Abstr.). Circulation, 26: 711,
man. Morphologic and geometric aspects of 60 1962.
necropsied cases. Am. J. Cardiol., 13: 367, 1964. 11, VAN MIEROP, L. H. S., PATTERSON, P. R. and
2. KEITH, J. D., ROWE, R. D. and VLAD, P. Heart REYNOLDS, R. W. Two cases of congenital
Disease in Infancy and Chiidhood, p. 31. New asplenia with isomerism of the cardiac atria and
York, 1958. Macmillan Company. the sinoatrial nodes. Am. J. Cardiol., 13: 407,
3. Ref. 2., p. 512. 1964.
4. VAN PRAAGH, R., VAN PRAAGH, S., VLAD, P. and 12. BROWN, J. W. Congenital Heart Disease, p. 227.
KEITH, J. D. Anatomic types of congenital London, 1950. Staples Press.
dextrocardia. Diagnostic and embryologic im- 13. TAUSSIG, H. B. Congenital Malformations of the
plications. Am, J. Cardiol. 13: 510, 1964. Heart. 11. Specific Malformations, p. 325. Cam-
5. BARBATO, E. et al. Study of the sequence of VW-I- bridge, 1960. Harvard University Press.
tricular activation and the QRS complex of the 14. KJELLBERG, S. R., MANNHEIMER, E., RUDHE, U.
pathologic human heart, using direct epicardial and JONSSON, B. Diagnosis of Congenital Heart
leads. Am. Heart J., 56: 340, 1958. Disease, p. 404. Chicago, 1959. The Year Book
6. WASSERBURGER, R. H., &BECKER, K., FREEMAN, Publishers, Inc.
D. J., KING, J. T. and YOUNG, W. P. Direct 15. ELLIOTT, L. P., AMPLATZ, K., ANDERSON,R. C. and

VOLUME 15, MARCH 1965

 Van Praagh, Van Praagh, Vlad and Keith

EDWARDS, J. E. Car triloculare biatriatum with triatum. Report of a case with survival to the
pulmonary stenosis and normally related great age of 29 years. Am. Heart J., 66: 104, 1963.
vessels. Clinicopathologic observations in three 22. MJROWSKJ, M., NEILL, C. A. and TAUSSIG, H. B.
cases. Am. J. Cardiol., 11: 469, 1963. Left atria1 ectopic rhythm in mirror-image dextro-
16. WILSON, F. N., ROSENBAUM,F. F. and JOHNSTON, cardia and in normally placed malformed hearts.
F. D. Interpretation of the ventricular complex of Report of twelve cases with “dome and dart” P
the electrocardiogram. Advances Int. Med.. 2: 1. waves. Circulation, 27: 864, 1963.
1947. 23. VAN PRAAGH, R., ZACHARIOUDAKIS, S. and KEITH,
17. SHAHER, R. M. The electrocardiogram in single J. D. Electrocardiogram in common ventricle
ventricle. Brit. Heart J., 25: 465, 1963. (Abstr.). Circulation, 26: 798, 1962.
18. ROGERS, H. M. and EDWARDS,J. E. Cor triloculare 24. VAN PRAAGH, R., VAN PRAAGH S., VLAD, P. and
biatrium: An analysis of the clinical and patho- KEITH, J. D. Diagnosis of the anatomic types of
logic features of nine cases. Am. Heart J.: 41 : congenital dextrocardia. Am. J. Cardiol., 15 : 234
299, 1951. 1965.
19. CAMPBELL,M., REYNOLDS, G. and TROUNCE, J. R. 25. FREIREJCH,A. W. and NJCOLSON,G. B. A rare
Six cases of single ventricle with pulmonary electrocardiographic finding occasionally seen in
stenosis. Guy’s Hosp. Rep., 102: 99, 1953. single ventricle hearts. Am. Heart J., 43 : 526,
20. ELLIOTT, L. P., TAYLOR, W. J. ~~~SCHIEBLER,G. L. 1952.
Combined ventricular hypertrophy in infancy. 26. ANSELMI,G., MUNOZ, S., MACHADO, I., BLANCO, P.
Vectorcardiographic observations with special and ESPINO-VELA, J. Complex cardiovascular
reference to the Katz-Wachtel phenomenon. Am. malformations associated with the corrected type
J. Cardiol., 11: 164, 1963. of transposition of the great vessels. Am. Heart J.,
21. MANDEL, A. and HIRSCH, V. Cor triloculare bia- 66: 614, 1963.

THF. AMERICANJOURNAL OF CARDIOLOGY