Download Isolated Non-Compacted Right Ventricular Myocardium

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© JAPI • may 2012 • VOL. 60
Case Report
Isolated Non-Compacted Right Ventricular Myocardium
Monika Maheshwari*, RK Gokroo**, SK Kaushik***
Abstract
Ventricular non-compaction or spongy myocardium is a rare type of cardiomyopathy resulting from arrested
myocardial development during embryogenesis. This rare entity is characterized by excessive prominent
trabeculations and deep inter-trabecular recesses in the ventricular wall. The clinical manifestations include
heart failure, arrhythmias and cardioembolic events. The usual site of involvement is the left ventricle. Rarely
the right ventricle (RV) is affected. Here we report a case of 40 year old male patient presenting with isolated
RV non–compaction.
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Introduction
orld Health Organisation /International and Federation
of Cardiology Task Force in 1995 categorised noncompacted ventricular myocardium (NCVM) as an ‘unclassified
cardiomyopathy’.1 It has an estimated prevalence of 0.05% in
adults and 0.14% in pediatric population with a male to female
ratio of 5.7 to 1.2 The usual site of involvement is the left ventricle.
Involvement of both ventricles or isolated non-compaction of
RV has been described rarely.3-5 Hence it was worth reporting
this unusual entity.
Case – Report
A 40 year male presented in emergency department with
complains of exertional dyspnoea, palpitation and swelling
over both lower extremities and abdomen since 1 month. On
examination he was afebrile with pulse 102/minute regular,
blood pressure-110/70 mmHg and respiratory rate-20/minute.
There was pallor, bilateral pitting edema over lower extremities
and raised jugular venous pressure 6 cm above sternal angle with
a prominent ‘v’ wave. There was no cyanosis, clubbing, icterus
or lymphadenopathy. On cardiac auscultation there was loud P2,
prominent S3 gallop with a grade 3/6 systolic murmur at left lower
sternum. His lung fields were clear. Chest Xray demonstrated
cardiomegaly. Cardiac enzymes and electrocardiogram were
normal. However, 2D transthorasic echocardiogram revealed
spongy RV myocardium with heavy trabeculations and deep
intertrabecular recesses prominent at the RV apex and free
wall diagnostic for non-compaction. Left ventricle was normal.
(Figures l and 2) The patient was treated with angiotensin–
converting enzyme inhibitors, thiazide diuretics, aldosterone
antagonist and warfarin and was discharged with symptomatic
relief after 7 days.
Discussion
Myocardial non-compaction is a rare disorder with uncertain
etiology.6 Both familial and sporadic forms of NCVM have been
described. The diagnostic criteria for NCVM have been described
as follows:
1.
The absence of co - existing cardiac anomalies
2. A two layered structure of the ventricular wall,with
end-systolic ratio of the non-compacted to compacted
myocardial layer >2
3. Finding this structural abnormality predominantly in the
apical and midventricular areas.
Fig. 1 : Trans thoracic echocardiogram showing trabeculations and
deep intertrabecular recesses prominent at the RV apex and free
wall.
Resident, ** Professor, *** Professor and HOD, Department of
Cardiology, J.L.N. Hospital, Ajmer.
Received: 08.04.2011; Accepted: 03.06.2011
*
Fig. 2 : Trans thoracic echocardiogram showing trabeculations and
deep intertrabecular recesses prominent at the RV apex and free
wall.
© JAPI • may 2012 • VOL. 60 4.
The blood flow directly from the ventricular cavity into deep
inter-trabecular recesses.
Normally during 5 to 8 gestational weeks compaction of the
developing myocardium occurs along with the commencement
of coronary circulation in the myocardium; during compaction
the recesses in the trabecular network are reduced to capillaries.
Arrest in endocardial morphogenesis and failure of regression
of embryonic ventricular sinusoids results in non compaction.7
However the exact etiology still remains unknown. Morbidity
and mortality is substantial at an early age with five year
survival less than 50%. The main complications are heart failure,
arrhythmias and embolism. Endomyocardial morphology in
NCVM is responsible for the development of mural thrombi
within the inter-trabecular spaces. All adult patients are
recommended oral anticoagulation irrespective of ventricular
functions.8 Perhaps the most controversial management option
is ICD implantation. Although studies demonstrating potential
benefit of ICD implantation has not been performed, it seems
intuitive that these devices may protect patients from sudden
cardiac death.
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