Case report

Triple type of obstruction in hypertrophic cardiomyopathy

Georgios K. Efthimiadis, Despina G. Parcharidou, Vasilis Vassilikos,
Georgios Giannakoulas, Christodoulos Pliakos and Georgios E. Parcharidis
Journal of Cardiovascular Medicine 2008, 9:11561158
Keywords: hypertrophic cardiomyopathy, implantable cardioverter
defibrillator, left ventricular obstruction, right ventricular obstruction,
ventricular tachycardia
Cardiology Department, AHEPA Hospital, Aristotle University of Thessaloniki,
Thessaloniki, Greece
Correspondence to Georgios K. Efthimiadis, MD, Cardiology Department,
AHEPA Hospital, Aristotle University of Thessaloniki, 546 36, Thessaloniki,
Greece
Tel: +30 2310994830; fax: +30 2310994673; e-mail: efthymos@med.auth.gr
Received 12 January 2008 Revised 7 April 2008
Accepted 14 July 2008

Case description
A 19-year-old man with recently diagnosed hypertrophic
cardiomyopathy (HCM) was referred to our department
for sudden death risk assessment. The diagnosis was
made by a private cardiologist to whom the patient
was presented due to a history of cardiac murmur. The
patient had mild exertional dyspnea (New York Heart
Association functional class II), but without syncope or
chest pain. There was no family history of HCM or
sudden death. The patients blood pressure (BP) was
130/70 mmHg and a grade 34/6 systolic ejection murmur
was present between the left parasternal border and the
apex. The ECG showed a left ventricular (LV) hypertrophy with strain pattern. A two-dimensional echocardiogram revealed an asymmetrical type LV hypertrophy;
anterior ventricular septum equal to 35 mm; posterior
ventricular septum equal to 20 mm; anterolateral free
wall equal to 12 mm; and posterior free wall equal to
6 mm (Fig. 1). The patient had three types of obstruction;
LV outflow obstruction due to systolic anterior motion of
both mitral leaflets with a Doppler-estimated 34 mmHg
gradient at rest (Fig. 2a and b); a midventricular obstruction with a Doppler-estimated 55 mmHg gradient at rest
but without apical aneurysm or dyskinesia (Fig. 2a and c);
and right ventricular (RV) outflow tract obstruction with a
Doppler-estimated 40 mmHg gradient at rest (Fig. 3).
The LV end-diastolic dimension was 45 mm and the left
atrium was 45 mm. The RV dimension was 30 mm and
the RV free-wall thickness was 8 mm. No mitral or
tricuspid regurgitation was detected. The patient had a
normal BP response on exercise test with a VO2max of
20 ml/kg per minute and no episodes of nonsustained
ventricular tachycardia in 24-h ECG recording. Cardiac
magnetic resonance imaging (MRI) showed a gross late
enhancement at the septum. On the basis of the extreme
1558-2027 2008 Italian Federation of Cardiology

degree of LV hypertrophy, an implantable cardioverter

defibrillator (ICD) was implanted prophylactically for
primary prevention of sudden death. After an uneventful
6-month period, a routine ICD-device interrogation
revealed an episode of sustained monomorphic ventricular
tachycardia suppressed by ICD antitachycardia pacing.

Discussion
This is a very rare case of a triple type of obstructive
HCM (mid-LV, LV outflow tract, and RV outflow tract)
with severe LV hypertrophy, normal RV free-wall thickness and ventricular tachycardia suppressed by ICD
antitachycardia pacing. HCM is considered predominantly a disease of LV outflow tract obstruction in which
70% of patients present LV outflow obstruction at rest
and with exercise [1].
Midcavity obstruction is due to the systolic apposition of
hypertrophied papillary muscle and LV wall at the level
of the mid-LV, producing two distinct LV chambers. It
may be easily overlooked during echocardiography but a
modified left two-chamber apical view can detect the
narrow neck between basal and apical segments. It
should also be suspected when a turbulent flow is found
at mid-LV in a four-chamber view with an absent or
minor systolic anterior motion of the mitral valve.

Fig. 1

Two-dimensional echocardiogram from the left short axis view at the

level of the mitral valve, showing severe left ventricular asymmetric
hypertrophy with anterior septum thickness of 35 mm.

DOI:10.2459/JCM.0b013e3283108803

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Triple obstruction in HCM Efthimiadis et al.

1157

Fig. 2

Two-dimensional color echocardiogram from the five-chamber apical view, showing a turbulent flow in mid-left ventricular and left ventricular outflow
tract (a) with estimated gradients of 34 mm (b) and 50 mm (c).

RV outflow tract obstruction may infrequently occur,

usually in combination with LV outflow tract obstruction
[24]. Small RV outflow tract gradients (less than
30 mmHg) are very often present in patients with
HCM [5], but severe RV outflow tract obstruction combined with mid-LV and LV outflow tract obstruction is a
Fig. 3

very rare condition. In one study [3], RV obstruction at

any site (mid-RV, RV outflow tract, or RV apical trabecular obstruction) was present in approximately 15% of
patients with HCM. However, only 5.5% of patients had
significant gradients (30 mmHg) and none of them had
combined double LV obstruction (LV outflow and midLV) with significant RV obstruction. The mechanism of
RV outflow tract obstruction is not fully understood.
Although systolic anterior motion of the tricuspid valve
was hypothesized in the early echocardiographic era [6],
bulging of hypertrophied ventricular septum in RV outflow [7], and especially the excessive hypertrophy of RV
muscular bands [4,8,9] (especially of crista supraventricularis) seem to be the possible mechanisms of RV outflow tract obstruction. Apart from outflow tract, mid-RV
hypertrophy and intense RV apex trabeculation may
provoke RV obstruction. In our case, the bulging of
severe hypertrophied septum in RV outflow was the
possible explanation of obstruction. We hypothesize that
this type of obstruction, together with the severe LV
hypertrophy, may have contributed to the development
of ventricular tachycardia in our patient.

Conclusion
A triple type of obstruction in HCM, although rare, may
exist. This entity can be revealed easily by a simple
echocardiogram. Whether this form of HCM serves as
an independent prognostic factor of adverse outcome is a
topic for further investigation.

Acknowledgements
Dr Giannakoulas has received support from the Hellenic
Cardiological Society and the Propondis Foundation.
Two-dimensional color echocardiogram from the left short axis view at
the level of great vessels, showing a turbulent flow in the right
ventricular outflow tract with an estimated gradient of 40 mm.

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1158 Journal of Cardiovascular Medicine 2008, Vol 9 No 11

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