Académique Documents
Professionnel Documents
Culture Documents
heart for histopathologic study of the conduction system.'5 These tissue blocks included
the junction of the interatrial and interventricular septums, from the posterior margin of
the noncoronarv sinus of the aorta to the anterior margin of the coronary sinus. Serial
sections 6 p in thickness were cut perpendicular to the line of junction of the interatrial and
interventricular septums. Every 20th section was stained with either hematoxylin and
eosin or Masson's trichrome before examination.
Results
Clinical Fkidings and Ccurnstances of Death
Clinical findings in the 10 dogs with hvpertrophic cardiomvopathy are
summarized in Table 1. At death, ages ranged from 1 to 13 years (mean, 6
years). Eight dogs were male and 2 were female. Of the 10 dogs, 4 were
German Shepherds and 1 each was a Doberman Pinscher, Airedale, Great
Dane, Boston Terrier, Poodle, and Bulldog.
Four of the 10 dogs had evidence of congestive heart failure 1 week to 1
year prior to death. In 2 dogs (A14586 and A15978) cardiac decompensa-
tion was mild and was manifested by coughing, mild dyspnea, and radio-
graphic evidence of pulmonary venous congestion. In 2 other dogs (18109
and A18460) cardiac failure was marked, as evidenced by severe dyspnea,
cardiomegaly, hepatomegaly, and pleural or pericardial effusions. Three
of the 4 dogs with heart failure died unexpectedly while under anesthesia
during operation (1 each for repair of a skin laceration, pacemaker im-
plantation, and pericardiocentesis); the remaining dog was put to death
by request of the owner.
Six other dogs with hypertrophic cardiomyopathy had no evidence of
cardiac disease prior to death, including 2 dogs that died unexpectedly
during operations for noncardiac abnormalities (A17741 and A18065) and
3 that died suddenly (A15053, A16059, and A16456). Of the latter 3 dogs,
1 collapsed while being walked and the other 2 were found dead by their
owner. The remaining dog (A16020) died of causes apparently unrelated
to heart disease, ie, renal failure of undetermined etiology associated wvith
disseminated intravascular coagulopathy.
Electrocardiographic Findings
Electrocardiographic recordings were obtained in 5 of the 10 dogs with
hypertrophic cardiomyopathy. Three dogs showed complete heart block
(Text-figure 1), including 1 with a history of syncope, that died during
implantation of a pacemaker (A14586). This latter dog and 1 other
(A18460) with complete heart block also showed evidence of bifascicular
block with left axis deviation and right ventricular conduction delay
(Text-figure 2). In 1 other dog (A17741) complete heart block wi-as noted
500 LIU ET AL American Journal
of Pathology
TEXT-FIGURE 1-Rhythm strip of Lead II, recorded in Dog A18460 1 day prior to death, showing
complete heart block. Ventricular rate is 70 beats/min. ECG recorded at 50 mm/sec.
I II III
TEXT-FIGURE 2-Electrocar-
diogram recorded in Dog
A 14586 1 day prior to death,
showving left anterior hemi-
block and right ventricular
conduction delay (QRS dura-
tion = 0.09 second; normal
< 0.06 second). T waves are
peaked and upright in Leads
II, I1I, AVF, and V3. rV2
is obtained at right fifth inter-
space at chondrosternal junc-
tion.
A14586
Vol. 94, No. 3 HYPERTROPHIC CARDIOMYOPATHY 501
March 1979
0
S
0
0
0
S
0
0
.
I
ow - .*..._ -
-90-
0
S *
so
i_
a.
* 0
I I I I I I
Normal Acquired MV TV Other Congestive Hypertrophc
MVD Complex Dysplasia Congenital Cardio- Cdrdio-
Malformation Heart Diseases myopathy mVopdthV
TEXT-FIGLRE 3-Septalfree wall thickness ratios in 10 dogs with hypertrophic cardiomyopathy
and 95 dogs with normal hearts or with other acquired or congenital heart diseases. Group marked
with asterisk includes 4 with discrete subaortic stenosis, 6 with patent ductus arteriosus, 2 with
ventricular septal defect, 1 with atrial septal defect, and 1 with tetralogy of Fallot.
hearts (6.6 0.3 g/kg; P < 0.001); heart weights in each of the 10
individual dogs with hypertrophic cardiomyopathy were equal to or ex-
ceeded heart weights in each of the dogs with normal hearts. Heart
weights in the dogs with hypertrophic cardiomyopathy (9.6 0.3 g/kg)
did not differ significantly from those in dogs with acquired or congenital
heart diseases (12.2 0.5 g/kg).
In the 10 dogs with hypertrophic cardiomyopathy, ventricular septal
thickness ranged from 13 to 22 mm (mean, 19 mm) and posterior left
502 LIU ET AL American Journal
of Pathology
tum in the left ventricular outflow tract adjacent to the anterior mitral
leaflet (Figure 1). These endocardial plaques were similar in appearance
to those in patients wvith hypertrophic cardiomyopathy.4 In 2 other dogs
(A17741 and A18109) more diffuse fibrous tissue formation was present on
the ventricular septum in the left ventricular outflow tract. In each of the
10 dogs, the left ventricular cavity was moderately or severely reduced in
size; in 2 of these dogs the left atrium was moderately- dilated. The cardiac
valves appeared normal in each dog.
Histologic Findings
In 8 of the 10 dogs with hypertrophic cardiomyopathy and each of the
95 control animals studied, virtually all cardiac muscle cells in the ven-
tricular septum were in normal parallel alignment. However, in the
ventricular septum of the 2 remaining dogs, foci wvere present in which
cardiac muscle cells wvere arranged perpendicularly or obliquely to each
other (Figure 2). In quantitative terms, these areas of disorganized cardiac
muscle cells were marked, ie, occupied 14% (A17741) or 12% (A18460)
of the total area of mvocardium in which cardiac muscle cells were
viewved in longitudinal section.
All cardiac muscle cells in the left ventricular free wall of 9 of the 10
504 LIU ET AL American Journal
of Pathology
trophy due to other forms of heart disease.13 How%ever, the relative in-
frequency wvith which marked disorganization of ventricular septal archi-
tecture occurs in dogs with hypertrophic cardiomryopathy underlies the
fact that this disease does not resemble in all anatomic respects hyper-
trophic cardiomyopathy in humans.
Certain clinical features of hypertrophic cardiomyopathy in the dogs
described in this report were similar to those of human patients with this
disease.16" 7 These points of similarity include predominance of the disease
in males,'6 the occurrence of sudden and unexpected death 16-18 even in
patients without a previous symptomatic manifestation of cardiac dis-
ease,19 and the development of marked cardiac failure in some pa-
tients.6,"7 However, one feature of hypertrophic cardiomyopathy in dogs
that is distinctly uncommon in humans is complete heart block.20 Diffuse
degenerative changes were present in the conduction system of 2 of our
dogs w%ith hvpertrophic cardiomyropathy and may have been responsible
for the complete heart block in these animals. However, such histopatho-
logic abnormalities are not unique to dogs -ith hvpertrophic cardiomv-
opathv and complete heart block since similar alterations have also been
described in dogs with complete heart block that did not have a cardiomv-
opathyv 21 and in dogs without cardiac disease that either died suddenly or
were put to death by request of the owner.2
In conclusion, it is evident from the data presented in this report that a
primary mvocardial disease, with certain pathologic features similar to
those of hvpertrophic cardiomyopathy in humans, also occurs spontane-
ously in dogs. The existence of this canine disease may prove to be a
valuable aid in the investigation of human cardiomvopathies.
References
1. Epstein SE, Henry WL, Clark CE, Roberts WC, Maron BJ, Ferrans VJ, Redwood
DR, Morrow AG: Asymmetric septal hypertrophy. Ann Intern Med 81:650-680.
1974
2. Abbasi AS, MacAlpin RN, Eber LM, Pearce ML: Left ventricular hypertrophv
diagnosed by echocardiography. N Engl J Med 289:118-121, 1973
:3. Menges H, Brandenburg RO, Brown AL: The clinical, hemodynamic, and patho-
logic diagnosis of muscular subvalvular aortic stenosis. Circulation 24:1126-1136.
1961
4. Roberts WC: Valvular, subs al% ular and supravalvular aortic stenosis: Morphologic
features. Cardiovasc Clin 3:97-126, 1973
5. Abbasi AS, MacAlpin RN, Eber LM, Pearce ML: Echocardiographic diagnosis of
idiopathic hypertrophic cardiomyopathy without outflow obstruction. Circulation
46:897-904, 197-2
6. Henry WL, Clark CE, Epstein SE: Asymmetric septal hypertrophy: Echo-
cardiographic identification of the pathognomonic anatomic abnormality- of IHSS.
Circulation 47:225-233, 197:3
7. Ferrans NJ, Morrow AG, Roberts WC: Myocardial ultrastructure in idiopathic
506 LIU ET AL American Journal
of Pathology
4. - ;. W
Fgure 1-Heart of Dog 15053 showing disproportionate thickening of the ventricular septum (VS) with respect to
the left ventricular free wall (LV). Fibrous plaque is evident on the left ventricular outflow tract (arrow). RV, right ven-
cricular wall. Fgr 2-Histologic section of ventricular septal myocardium from Dog 18460 showing area of
cardiac muscle cell disorganization. (H&E, x 100)
508 LIU ET AL American Journal
of Pathology
[End ofArticle]