HCM

2.  myocardial hypertrophy without an identifiable
cause
 Second most common cardiomyopathy in
children
 Sporadic or familial forms.
 most common cause of cardiac death in children
and young adults.
 Marked heterogeneity in clinical
manifestations with varied presentations.

3.  0.2 % of the population.
 Incidence is likely higher as it goes undetected
in those without symptoms.

4.  PRIMARY HCM- genetic disease with AD
inheritance.
 SECONDARY HCM-
 metabolic, mitochondrial and syndromic
diseases like Noonan, Leopard syndromes.
 usually present in infancy(15% of infants
with HCM).
 Develop concentric HCM, often accompanied by
involvement of the right ventricle.
 Metabolic screening
 muscle biopsy

5.  diffuse or segmental LVH with a nondilated and
hyperdynamic chamber, in the absence of
another cardiac or systemic disease capable of
producing the magnitude of hypertrophy
 mutations in one of the sarcomeric genes
 myocyte dis-array and interstitial fibrosis

6.  1.ASH-(predominantly basal septum)-70%
 2.concentric-25%
 3.posterior LV hypertrophy-3-5%
 4.apical-2%
OBSTRUCTIVE OR NONOBSTRUCTIVE VARIETY.

7. 7MANAGEMENT OF HOCM-CURRENT PRACTISE

8. PARAMETERS REPORTED AS RISK FACTORS FOR
SUDDEN DEATH IN CHILDREN:
 Young age at presentation
 Previous aborted SCD
 Malignant family history of HCM
 Syncope
 VT on ambulatory monitoring.
 Marked LVH> 30 mm.
 Elevated E/e ratio
 LV dilation
 Decreased EF.

9.  Age < 1 year at presentation
 Massive hypertrophy of metabolic cause.
 Troponin T mutations
 Abnormal B.P response to exercise.
all these carry poor prognosis

11.  Asymmetrical septal hypertrophy(ASH)
 Systolic anterior motion of the mitral valve
(SAM)
 Small LV cavity
 Septal immobility
 Premature closure of the aortic valve.

12.  LV thickness- abnormal when ≥ 15 mm,
 asymmetrical in presence of a septal to free wall
thickness ratio between 1.3 and 1.5.
 SAM - an abrupt anterior movement of the MV
reaching its peak before maximum movement of
the posterior wall

13.  Grade1-distance between AML and
septum>10mm
 Grade2-distance between AML and septum<10
mm
 Grade3-a.contact of AML with septum<40% of
systole.
 Grade3-b.contact of AML with septum>40% of
systole.

15.  1.flow drag-hydrodynamic pushing force of
flow behind the mitral leaflets- dominant
role.
 2.venturi effect-suctioning from LVOT
tunnel- minor contribution
 3.Obstruction begets more obstruction.

16.  Abnormalities contribute to SAM.
reduced posterior leaflet restraint.
Anterior displacement of the papillary muscles - shifts
the mitral leaflets anteriorly toward the LVOT and
leads to chordal and leaflet laxity.
 coaptation point between the AML and PML is
typically eccentric because of the greater AML
motion relative to the PML.

17.  30– 60% -SAM
 25–50% -LVOTO
 Haemodynamic consequences of SAM --
prolongation of ejection time and a reduction in
stroke volume.
 Coaptation of the mitral leaflets may be disrupted
resulting in MR
 Mid-systolic notching of the aortic valve.
 drag forces that create SAM play an important
role in LVOT gradient.
 extent of septal hypertrophy and resultant
narrowing of the LVOT also contribute to the
LVOT gradient.

19.  gradient is (x/y)*25+25 mmHg.
 positive correlation between the severity of
SAM and the severity of obstruction
 a contact between SAM and the septum =
obstruction≥30 mmHg.

20. 1. prolapse
2. excessive leaflet tissue
3. chordal elongation
4. elongation of the mitral leaflets which coapt at
the leaflet body rather than the tip
5. anterior displacement of the mitral apparatus
6. insertion of the papillary muscle directly into
the anterior mitral valve leaflet.

21.  risk of AF
 heart failure
 cardiac mortality .
 high LA diameter > 48 mm.
 LA FS ([maximal diameter –minimum
diameter]/maximal diameter*100), is an estimate
of end-diastolic pressure in HCM
 <16% an independent risk factor for AF

23. 1.Basal septal hypertrophy
2.Anomalous papillary muscle.
3.After AVR with LVH and hyperdynamic EF.
4apical ballooning.(takatsubo syndrome
5.After MV repair.
6.positive inotropic usage.
7.Apical MI with with hyperdynamic function of
basal myocardial segments.
8.Massive MAC
9.Hypovolemia with small LV cavity.

24. 1. unexplained maximal wall thickness >15 mm
in any myocardial segment
2. septal/posterior wall thickness ratio >1.3 in
normotensive patients
3. septal/posterior wall thickness ratio >1.5 in
hypertensive patients

25. Distribution of hypertrophy
 any pattern and at any location, including the RV.
 Septal predominance is more common.
 LV free wall or apex
 Apical HCM and apical aneurysms can be missed
without contrast.

33.  The most clinical important method is the
measurement of the maximal wall thickness
(MWT) at any LV level
 Spirito et al showed that a maximum thickness of
30 mm or more, present in approximately 10% of
HCM patients, resulted in a substantial long-term
risk.

34.  normal or supranormal in both obstructive and
non-obstructive HCM
 EF is typically preserved (or increased) despite
an impairment of long-axis function
Systolic dysfunction
 10–15%
 end-stage or ‘burnt-out HCM (wall thinning,
cavity dilation, and fibrosis)

35.  LV and LA filling abnormalities - patients with
HCM irrespective of the presence and extent of
LVH.
Transmitral inflow pattern-
 Reduced E-wave velocity
 Increased A-wave velocity
 Decreased E/A-wave ratio.
 Prolonged DT
 Prolonged IVRT.

36.  Progressive decrease in systolic flow
 Increase in A-wave reversal
 Atrial reversal velocity and its duration -
significant correlation with LVEDP.
 reasonable correlations between E/e¹ ratio and
LV filling pressures

37.  HCM with elevated LVEDP but normal LA pressure.
 short mitral A duration
 Ar velocity in pulmonary venous flow is increased in amplitude and duration.
 Lateral annular e velocity is normal
 ratio of peak E velocity (at the level of mitral tips) to e¹ velocity is <8

39.  The E/e¹ ratio -exercise tolerance
 septal e¹ velocity - independent predictor of
death and ventricular dysrhythmia

40.  prognostic
 multifactorial
severity of MR
diastolic dysfunction
 LA volume indexed to body surface area

41. Patients with HCM and a maximal LA volume
index 34 mL/m2
 higher incidence of abnormal diastolic filling,
 a higher mitral inflow/ annular velocity (E/e‘)
ratio,
 a higher calculated LA pressure
 less favorable outcome

42.  presence and degree of MR
 MR occurs in almost all patients with
obstructive HCM as a consequence of SAM
which induces abnormal mitral leaflet
coaptation.
 Direct relation between the pressure gradient
and the severity of MR

44.  SAM induces a mitral regurgitation jet directed
posteriorly,
 intrinsic mitral valve disease due to annular,
papillary or leaflet disease, patients with
obstruction and mitral regurgitation can show a
systolic mitral anterior directed jet

45.  25% - significant resting pressure gradient, i.e.
≥30 mmHg
 provoked by physiological and pharmacological
interventions that diminish LV end-diastolic
volume or augment LV contractility.

46. 46MANAGEMENT OF HOCM-CURRENT PRACTISE

47.  labile obstruction - spontaneous appearance and
disappearance of obstruction
 latent obstruction - gradients that only appear with
provocation
 Valsalva maneuver,amyl nitrite, and dobutamine
 Exercise - provoking latent LVOTG
 50% of HCM patients without significant outflow
tract obstruction at rest - outflow gradients over 30
mmHg with exercise
 upright exercise-greatest resemblance to daily
physiologic activities, should be used

50.  Pulsed Doppler at mitral and pulmonary vein
level
 all phases of diastole are altered
 Isovolumetric relaxation is slowed and prolonged
 rate of rapid filling is diminished
 atrial contribution to filling is increased as well
as LV chamber stiffness.
 localising the site of LVOT obstruction

52.  enhance endocardial definition, Doppler
signals, and to evaluate myocardial perfusion
during percutaneous transluminal septal
myocardial ablation (PTSMA).
 PTSMA – absolute alcohol into a septal
perforator branch of the LAD to produce a MI
within the proximal IVS
 selects the appropriate septal perforator
branch determining the precise area of septum
targeted for alcohol ablation and evaluates
whether selected septal perforator also
perfuses other distant and unwanted areas of
LV or LV myocardium or papillary muscles

56.  ratio of E to e’ of lateral mitral annulus -
quantified LV pressures, in particular the LV
pressure before atrial contraction
 E/e’ ≥10 =best sensitivity and specificity for
identifying LV pre-A pressure > 15 mmHg
 identifies patients with low exercise capacity.

57.  baseline LMSa <,4 cm/s had an increased risk
of clinical deterioration
 best value of LMSa with the highest sensitivity
(75%) and specificity(88%) was 4 cm/s

59.  Diastolic TD -risk of sudden death, ventricular
tachycardia, or cardiac arrest
 transmitral E/septal Ea ratio -death, cardiac
arrest, and VT.
 Decrease in systolic TD parameters (LMSa ) -
marker of occult systolic dysfunction

60.  regional differences in wall motion at rest
 despite normal global systolic function
 longitudinal and radial systolic myocardial
deformation are heterogeneously reduced
 more pronounced in the more severely
hypertrophied myocardial segments
 FS & EF are known to overestimate systolic
function in the presence of LVH

61.  TDI-examines myocardial motion relative to
the transducer
 strain -myocardial motion relative to the
adjacent myocardium

62.  Systolic myocardial strain
 dimension-less quantity
 measure of tissue deformation.
 When the left ventricle contracts, the
myocardium shortens longitudinally and
circumferentially (negative strain) and
lengthens or thickens in the radial direction
(positive strain).
 Strain rate (SR) -local rate of myocardial
deformation
 SRI after ablation accurately identified areas
of discrete regional infarction, reiterating its
superiority over TDI for the objective
quantification of regional dysfunction.

63.  optimal cut-off value of strain, i.e.systolic
longitudinal strain by 4 and 2 chamber views, for
discrimination between HCM and hypertensive
LVH Is -10.6%;
 sensitivity, specificity, and predictive accuracy of
85, 100,and 91.2%

67. 3D-ECHO
 diagnosis, assessing systolic function, and understanding the
mechanics of SAM and LVOTO
 volumetric data
 accurate assessment of systolic function
 distribution of hypertrophy

72. Surgical Myectomy.
 Intraoperative TEE
 surgical planning,
 adequacy of repair
 complications.
 maximum thickness of the septum
 distance of maximum thickness from the aortic annulus, the
location of the endocardial fibrous plaque
 apical extent of the septal bulge.

73.  Functional and intrinsic MV abnormalities
 direct insertion of papillary muscles into the
middle or base of the AML.

75. Alcohol Septal Ablation.
 Alternative to surgery when medical therapy has
failed or is not tolerated.
 MCE -contrast agent into the proposed target septal
arteries to delineate the vascular distribution of the
individual perforator branches

76.  MCE - demarcated area with increased echo density
in the basal septum.
 document the absence of perfusion of myocardial
segments remote from the targeted areas for
ablation, including the LV anterior wall, right
ventricular (RV) free wall, and papillary muscles.

77.  referred for surgery-target septal perforator also
supplies papillary muscles or in settings when it is not
possible to cannulate the target septal vessel

79. 
 The region of the basal septum, which is infarcted by the
alcohol infusion, is typically intensely echo dense.
 reduced thickening and excursion.
 reduction or elimination of MR when it is due to SAM.
 elimination or reduction of dynamic obstruction.

82. 83MANAGEMENT OF HOCM-CURRENT PRACTISE