European Journal of Echocardiography 2008 9(1):126-129; doi:10.1016/j.euje.2007.04.007
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2007. For permissions please email: journals.permissions@oxfordjournals.org
Left ventricular noncompaction: case of a heart transplant
Kursat Tigen*,
Tansu Karaahmet,
Gokhan Kahveci,
Bülent Mutlu and
Yelda Basaran
Kartal Kosuyolu Heart Education and Research Hospital, Cardiology Department, Istanbul, Turkey
Received 6 February 2007; accepted after revision 15 April 2007; online publish-ahead-of-print 30 June 2007.
* Corresponding author. Kartal Ko
uyolu Yüksek 
htisas Hastanesi Denizer Caddesi, Cevizli, Kartal, T ürkiye. Tel: +90 216 459 40 41; fax: + 90 216 459 63 21. E-mail address: mktigen{at}yahoo.com
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Abstract
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Noncompaction of the ventricular myocardium (NVM) is a rare
congenital cardiomyopathy characterized by multiple prominent
trabeculations with deep intertrabecular recesses resulting
from an arrest in normal embryogenesis of the endocardium and
myocardium. The major clinical manifestations are depressed
left ventricular systolic and diastolic function, systemic embolism,
ventricular tachyarrhythmias, conduction disorders and neurologic
abnormalities. We present a 21-year-old female who was diagnosed
as dilated cardiomyopathy due to isolated noncompaction of the
left ventricle and underwent cardiac transplantation.
Keywords: Noncompaction; Dilated cardiomyopathy; Transplantation
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Introduction
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Noncompaction of the ventricular myocardium (NVM) is a rare
congenital cardiomyopathy characterized by multiple prominent
trabeculations with deep intertrabecular recesses resulting
from an arrest in normal embryogenesis of the endocardium and
myocardium which can either be isolated or associated with complex
congenital heart disease.
1 The prevalence of NVM differs from
0.06 to 0.24%/y with a male dominancy.
2–4 The major clinical
manifestations are depressed left ventricular systolic and diastolic
function, systemic embolism, ventricular tachyarrhythmias, conduction
disorders and neurologic abnormalities.
5,6 Medical management
of heart failure and heart transplantation when clinically indicated,
management of arrhythmias, prevention of systemic embolism and
echocardiographic screening of first-degree relatives are treatment
strategies.
4,5,7,8 We present a 21-year-old female who was diagnosed
as dilated cardiomyopathy due to isolated noncompaction of the
left ventricle and underwent cardiac transplantation.
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Case history
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A 21-year-old female with heart failure during adolescence was
admitted to Kosuyolu Heart and Research Hospital with symptoms
of severe congestive heart failure. On physical examination
the patient was orthopneic and jugular venous distention and
bilateral leg edema were present. Blood pressure was 95/65 mm/Hg.
The cardiac apex was displaced laterally. On auscultation, there
was tachypnea and moderate retraction of the chest wall and
moist rales over the entire lung fields. The first and second
heart sounds were normal and a S3 sound was prominent. A grade
3/6 holosystolic murmur was heard at the apex. Her liver was
palpable 4 cm below the right costal margin. Sinus rhythm with
a heart rate of 102 pulse/min, normal QRS duration and nonspecific
ST–T wave changes were present on the electrocardiogram.
Chest X-ray showed mild cardiac enlargement with a cardiothoracic
ratio of 0.65, prominent central pulmonary arteries and pulmonary
venous congestion. Fasting blood glucose: 88 mg/dl, BUN: 45
mg/dl, creatinine: 0.97 mg/dL, AST: 44 U/L, ALT: 32 U/L, LDH:
388 U/L, haemoglobin: 9.1 g/dL, hematocrit: 27.9%, WBC: 4500/µ,
platelet count: 118 000/µ, INR: 1.1, total protein: 6.8
g/dL, and albumin: 3.5 g/dl were found in the biochemical assessment.
Plasma NTproBNP levels were also severely elevated (1595 pmol/L).
A transthoracic echocardiographic examination was performed
with a GE Vingmed Vivid System Five ultrasonographic unit. Echocardiography
revealed a markedly dilated left ventricle and severely impaired
left ventricular systolic function (left ventricular ejection
fraction by Teicholtz's method was 17%). Multiple, prominent
muscular trabeculations were present in the left ventricular
apex and lateral wall above the papillary muscle insertion (
Figure 1).
Parasternal long and short axis views revealed a spongy appearance
of the posteriobasal left ventricular wall (
Figure 2).
Deep intertrabecular recesses communicating with the left ventricular
cavity were evident on color Doppler imaging. A mild degree
of tricuspid and severe mitral regurgitation was present on
color Doppler examination. Estimated pulmonary artery systolic
pressure was 65 mmHg. Mitral inflow pattern was consistent with
restrictive physiology (mitral
E velocity of 85 cm/s, mitral
A velocity of 35 cm/s and deceleration time of 100 ms). A mild
degree of spontaneous echocontrast was also present in left
ventricular cavity. The right ventricle was mildly enlarged
with normal trabeculations and systolic function was reduced.
Both atrias were detected enlarged. Coronary vessels were found
normal at coronary angiography and heart catheterization revealed
moderately elevated chamber pressures. The findings were consistent
with isolated noncompaction of the ventricular myocardium. Neurological
consultation of the patient revealed completely normal. Echocardiographic
screening of the first-degree relatives was also within normal
ranges. During hospitalization period, despite vigorous treatment,
patient's condition progressively deteriorated requiring intravenous
inotropic support for adequate systemic perfusion. Due to poor
functional capacity (NYHA Class IV) despite intensive theraphy
with evidence of severe left ventricular dysfunction the patient
has been considered for heart transplantation and underwent
orthotopic heart transplantation within two months. The patient
is in her second year after transplantation and being followed-up
under appropriate manner. In pathologic examination the explanted
heart was dilated and weighed 420 g. The coronary arteries were
normal. Prominent trabeculations and invaginations especially
at left ventricular apex were found impressive (
Figure 3).
Areas of noncompaction were markedly thickened when compared
with compacted areas. Histologic examination shows endothelial
lining of the recesses communicating with left ventricular cavity.
Rhabdomyocyte hypertrophy, multinucleation, anisochromacy, interstitial
musinosis and fibrosis, subendocardial musinosis and fibrosis
were detected (
Figure 4). These pathologic findings confirmed
the diagnosis of isolated left ventricular noncompaction.
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Figure 4 Microscopic specimens of native heart: (A) Tricrom Masson stain showing fibrosis (green stripes), (B) Von Glesson stain showing elastic fibers (yellow islets), and (C) Giemsa stain showing rhabdomyocyte hypertrophy, multinucleation and anisochromacy.
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Discussion
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Noncompaction of the ventricular myocardium which was first
described in 1984 by Engberding and Bender,
9 is a rare congenital
cardiomyopathy characterized by multiple prominent trabeculations
with deep intertrabecular recesses resulting from an arrest
in normal embryogenesis of the endocardium and myocardium. This
abnormality can either be isolated, first described by Chin
et al in 1990, or associated with complex congenital heart disease.
1 In early embryonic period human myocardium is a loose meshwork
of interwoven myocardial fibers which is characterized by excessively
prominent trabeculations and deep intertrabecular recesses.
By gradual compaction, the fetal myocardium condenses causing
the large spaces within the trabecular meshwork to disappear.
This process typically progresses from epicardium to endocardium
and from the base of the heart toward the apex.
10,11 NVM is
the result of an arrest in the normal process of myocardial
compaction that is characterized by persistence of multiple,
excessively prominent ventricular trabeculations and deep intertrabecular
recesses.
12 The prevalence of NVM differs from 0.06 to 0.24%/y
with a male dominancy. Familial forms have been described.
2–4 The major clinical manifestations are impaired left ventricular
systolic and diastolic function, systemic embolism, ventricular
tachyarrhythmias, conduction disorders and neurologic abnormalities.
5,6 Our case also admitted with symptoms of severe left ventricular
systolic and diastolic dysfunction, no history of systemic embolisation
was present and we were not able to document an arrhythmia during
the hospitalization period. Neurological consultation also revealed
normal results. Echocardiographic examination revealed prominent
trabeculations that perfused from the left ventricular cavity
on color Doppler at apical and lateral left ventricular wall.
Confirmation of the diagnosed NVM was also made by pathologic
examination. Echocardiographic examinations of the first-degree
relatives of our case were within normal range. The prognosis
of NVM is quite poor. Ritter and colleagues reported that 59%
of patients with isolated left ventricular noncompaction either
died or underwent heart transplantation during 6-year follow-up
and observed worse prognosis in patients and with higher functional
classes.
13 Oechslin and colleagues reported that 35% of the
patients with isolated left ventricular noncompaction were dead
and 12% had undergone heart transplantation during 40 ±
40 months follow-up.
14 A review of the literature identified
only eight patients with isolated left ventricular noncompaction
who underwent cardiac transplantation. Our patient's clinical
status was also progressively deteriorated against aggressive
medical management and she had undergone cardiac transplantation
within two months. Because of high mortality rates, patients
with NVM should be followed-up closely with appropriate medical
management and considered for heart transplantation with high
priority. With all these data she is presented as a case report
because she is the first patient in our country and the 9th
patient in the literature who had undergone heart transplantation
due to isolated left ventricular noncompaction related dilated
cardiomyopathy.
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References
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- Carlson BM. Patten's foundations of embryology (1988) 5th ed. New York: McGraw-Hill Book Company.
- Taylor GP. Cardiovascular system. In: Development pathology of the embryo and fetus—Dimmick JE, Kalousek DK, eds. (1992) Philadelphia: Lippincott.
- Jenni R, Goebel N, Tartini R, Schneider J, Arbenz U, Oelz O. Persisting myocardial sinusoids of both ventricles as an isolated anomaly: echocardiographic, angiographic, and pathologic anatomical findings. Cardiovasc Intervent Radiol (1986) 9:127–31.[Web of Science][Medline]
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- Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol (2000) 36:493–500.[Abstract/Free Full Text]
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Abstract
Introduction
