Download CT IMAGING OF A CASE WITH BIVENTRICULAR HYPERTROPHIC

CT IMAGING OF A CASE WITH
BIVENTRICULAR HYPERTROPHY
ABSTRACT ID- IRIA 1139
INTRODUCTION
HYPERTROPHIC CARDIOMYOPATHY (HCM)
Most common inherited cardiovascular disease, which manifests as
diffuse or segmental left ventricular (LV) hypertrophy with a nondilated
and hyperdynamic chamber.
HCM is an autosomal dominant disease caused by mutations in
genes encoding sarcomere proteins.
Common expression of HCM is during adolescence, however patients
may present at any point in life.
The common phenotypes are:
--asymmetric (septal) HCM
--apical HCM
--symmetric HCM (concentric HCM)
--mass-like HCM.
CASE REPORT
55 year old male
Chest pain for 2 years and breathlessness for 5 months.
Paroxysmal nocturnal dyspnea and orthopnea for 5 months.
Not a known case of Hypertension/ diabetes mellitus .
Family history- Nil relevant.
EXAMINATION
 GENERAL EXAMINATION:
BP – 146/98 mmHg,
Pulse Rate - 72/ min.
No pallor/icterus/cyanosis/clubbing/pedal edema/lymphadenopathy
 SYSTEMIC EXAMINATION:
CVS- S1, S2 +. No murmur heard.
OTHER SYSTEMS – normal.
 ELECTROCARDIOGRAM- Left ventricular hypertrophy with strain
pattern.
IMAGING
 CHEST X-RAY- Moneybag appearance due to pericardial effusion
Followed by Cardiac CT was done for the suspicion of pericardial
effusion and consolidation
CARDIAC COMPUTED TOMOGRAPHY
A
(A&B) 4 chamber and short axis view of interventricular septum seen with
thickness measuring 32.5 mm– suggestive of asymmetrical hypertrophy
B
C
D
C) Right ventricular free wall thickness measures 11.6mm.
D) Left ventricular posterior free wall thickness measures 23.4mm.
E) Left ventricular outflow tract shows no obstruction (orange arrow).
E
F
H
F) Main pulmonary artery measures- 40.4 mm.
G) Right pulmonary artery- 20.3 mm.
H) Left pulmonary artery – 23.1mm.
I) Right upper pulmonary vein measures 18.2mm.
Above findings are significant of pulmonary artery and venous hypertension.
G
I
DIAGNOSIS
Interventricular Septum thickened suggestive of asymmetrical
hypertrophic cardiomyopathy.
Biventricular hypertrophy.
Pulmonary artery and venous hypertension.
No left ventricular outflow tract obstruction.
CT imaging findings correlated with Echocardiography findings as
follows.
ECHOCARDIOGRAM:
 Hypertrophic cardiomyopathy concentric variant.
 Asymmetrical septal hypertrophy. Septum-30mm.
 Left atrium dilated.
 Moderate pericardial effusion; posteriorly 3.8cm thickness.
 No Systolic anterior motion.
 No Left ventricular outflow tract obstruction.
BACKGROUND
 A) Normal Heart
 B) asymmetric (septal) HCM with LVOT obstruction
 C) asymmetric (septal) HCM without LVOT obstruction
 D) apical HCM
 E) symmetric HCM (concentric HCM)
 F) mass-like HCM.
Asymmetric involvement of the interventricular septum is the most
common form (60%–70%).
 LV hypertrophy, with a thickened anterior mitral leaflet.
Asymmetric septal wall hypertrophy at anteroseptal myocardium with
and without LVOT obstruction.
Apical HCM is myocardial hypertrophy predominantly involves the
apex of the left ventricle (25%) of all patients .
Complicated by hypertension
Symmetric or concentric HCM, is characterized by concentric LV
hypertrophy with a small cavity dimension and no evidence of a
secondary cause.
42% occur in as many as of the cases of HCM.
Mass-like HCM –manifests as a mass-like hypertrophy because of the
focal segmental location of the myocardial disarray and fibrosis.
 CLINICAL FEATURES:
--Chest pain
--Dyspnea- PND and Orthopnea
--Palpitations
--Dizziness and Fatigue.
INVESTIGATIONS
 ECG
--Mild degrees of hypertrophy or LV hypertrophy- show strain pattern.
--Septal hypertrophy-Abnormal Q waves,
--Apical HCM- giant T-wave
 Chest X-ray
--Shows LV or LA and/or RA enlargement with or without vascular
redistribution in the lungs.
--Aorta is typically small.
--Bulge on the left heart border- reflects anteroseptal hypertrophy.
ECHOCARDIOGRAPHY
30-60% show Systolic anterior motion.
 Peak Left Ventricular Outflow Tract gradient >30 mmHg is significanct
in HCM
Systolic dysfunction occurs due to wall thinning, cavity dilation, and
fibrosis.
Diastolic dysfunction in reduced chamber compliance, increase in
chamber stiffness secondary to increased LV mass and myocardial
fibrosis.
Doppler echocardiography shows accuracy assessment of diastolic
function demonstrating impaired relaxation
CARDIAC MDCT
Asymmetric hypertrophy- septal thickness is greater than or equal to
15mm
The ratio of the septal thickness to the thickness of the inferior wall of the left
ventricle is greater than 1.5 at the midventricular level.
Apical hypertrophy- apical wall thickness of more than 15 mm
Ratio of apical to basal LV wall thicknesses of 1.3–1.5.
Symmetrical hypertrophy- LV wall thickening of about 14 mm.
Mass like hypertrophy - It has homogeneous appearance and perfusion of
adjacent normal myocardium.
ECG-gated CT
This test can be used to evaluate the patterns of LVH and wall motion in
HCM.
CARDIAC MRI
MRI can be used to accurately characterize the distribution and degree of
myocardial hypertrophy compared to Cardiac CT.
Its advantages are to evaluate:
Anatomical visualization of the entire LV wall morphology
Functional analysis of wall motion volume & ejection fraction calculation
--Left ventricular tract obstruction gradient
--Systolic anterior motion of anterior mitral leaflet
--Late Gadolinium Enhancement for identifying areas of fibrosis.
TREATMENT FOR VARIOUS TYPES OF HCM
 Surgical myectomy
 Septal alcohol ablation
 Ventricular pacing
 Cardiac transplantation
CONCLUSION
Hypertrophic cardiomyopathy even though being a common disease,
only a few cases with biventricular hypertrophy has been reported.
Cardiac MDCT provides good spatial resolution through 3-D
reformatted images.
It can offer comprehensive information on detailed anatomic and
functional information about the cardiac chambers.
However Cardiac MR is the standard modality for differentiating HCM
from other cardiomyopathies
REFERENCES
Hayashi S, Tojyo K, Uchikawa S, et al. Biventricular hypertrophic
cardiomyopathy with right ventricular outflow tract obstruction
associated with Noonan syndrome in an adult. Jpn Circ J
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Chirillo F, Zecchel R, Stritoni P. Biventricular hypertrophic
cardiomyopathy with alone obstruction to right ventricular outflow.
Heart 2002;87:565. [FREE Full text]
Fournier C, Bache R, Valette H, et al. Hypertrophic myocardiopathy
with isolated obstruction of the right ventricle. Ann Cardiol Angeiol
(Paris) 1985;34:71–4. [Medline]
Maron BJ, McIntosh CL, Klues HG, et al. Morphologic basis for
obstruction to right ventricular outflow in hypertrophic
cardiomyopathy. Am J Cardiol 1993;71:1089–94
Henry WL, Clark CE, Epstein SE. Asymmetric septal hypertrophy.
Echocardiographic identification of the pathognomonic anatomic c
abnormality of IHSS. Circulation 1973; 47: 225-33.