European Journal of Echocardiography 2006 7(6):457-460; doi:10.1016/j.euje.2005.07.011
Copyright © 2005, The European Society of Cardiology
Noncompaction of ventricular myocardium involving both ventricles
Rifat Eralp Ulusoya,
Nezihi Kucukarslanb,*,
Ata Kirilmaza and
Ergun Demiralpa
aGATA Haydarpa
a Military Treaning Hospital, Department of Cardiology, Kadikoy, Istanbul, Turkey
bGATA Haydarpaa Military Treaning Hospital, Department of Cardiovascular Surgery, Kadikoy, Istanbul, Turkey
Received 20 April 2005; received in revised form 19 June 2005; accepted after revision 28 July 2005.
* Corresponding author. GATA Askeri Hastanesi Kalp ve Damar Cerrahisi ABD, 06018 Etlik, Ankara, Turkey. Tel.: +90 3123 045271/90 5335 185364/90 3123 045221; fax: +90 3123 045200. nkucukarslan{at}gata.edu.tr nezihimd{at}hotmail.com
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Abstract
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Aim We aimed to present a case with ventricular myocardial noncompaction
involving both ventricles.
Methods and results Noncompaction of ventricle is a rare and unclassified congenital cardiac malformation is due to an arrest in intrauterine endomyocardial morphogenesis. We presented a ventricular myocardial noncompaction case involving both left and right ventricles. The physical examination of this case is consistent with mitral regurgitation and the echocardiographic findings are consistent with noncompaction of ventricular myocardium involving both ventricles with left ventricular systolic failure.
Conclusion Transthoracic echocardiography is a useful clinical tool for diagnosing noncompaction of both the right and left ventricular myocardium. The LVNC definition can also be utilized for RVNC, which this diagnosis has never been reported in a Turkish patient.
Keywords: Noncompaction of ventricular myocardium; Cardiomyopathy; Heart failure
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Introduction
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Left ventricular noncompaction (LVNC) is a rare congenital anomaly
both in children and adults.
1,2 Some authors regarded this pathology
as a subvariety of the dilated cardiomyopathy (CMP) syndrome.
3 This unclassified cardiomyopathy results from an arrest in intrauterine
endomyocardial morphogenesis, and is diagnosed either sporadically
or with a familial tendency due to chromosomal anomalies
4,5–10 and the age of onset varies widely.
11,12 Its common clinical
presentations involve heart failure, ventricular tachyarrhythmia
and thromboembolic events. In the Australian Childhood Cardiomyopathy
trial, they have found a male predominance and 9.2% of patients
with unclassified cardiomyopathy have LVNC.
13
The diagnosis is based on Jenni and Stöllberger's LVNC/left ventricular hypertrabeculation definition.2,14 We present a patient with myocardial noncompaction of both ventricles. The diffuse right ventricular (RV) involvement is based on Jenni and Stöllberger's definition (RVNC).
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Case report and discussion
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A 26-year-old male was referred to our clinic for the evaluation
of mild effort dyspnea. Physical examination revealed a blood
pressure of 120/70mmHg with a regular pulse of 75/min. Cardiac
auscultation revealed normal first and second heart sounds without
any third and fourth heart sounds and a systolic murmur at the
fourth intercostal space without any other pathologic physical
findings. ECG was consistent with sinus rhythm and marked both
left and right ventricular hypertrophy by voltage criteria with
ST and T wave strain pattern.
Transthoracic echocardiography (TTE) was performed with a Vingmed System V (GE, Horton, Norway) and a 2.5-MHz probe according to American Society of Echocardiography (ASE) guidelines.15 The TTE was consistent with a mild dilated left ventricular (LV) chamber and diffuse LV hypokinesis with severe hypertrophy of both septal (IVS) and lateral LV walls. The RV wall thickness was 18.6mm (Fig. 1). The trabeculations with deep fissures and grooves were located in the LV inferior, apical, posterolateral and septal (IVS) walls. A bright echo reflectance was detected at the basal septal and lateral walls of both the LV and RV in the apical four-chamber view (Fig. 2). Blood flow was detected inside these deep fissures with color flow Doppler. The IVS thickness was thicker at certain sites (29.3mm) than the posterior (12mm) and lateral (12mm) LV walls. The LVEF and RVEF were measured as 35–40% and 55%, respectively. The Doppler examination of the mitral valve was consistent with mild degree mitral regurgitation. The pulmonary flow, which was recorded from the parasternal short axis at the aortic level, was also assessed normal (0.97m/s) without any gradient. We suggested cardiac catheterization to rule out the pulmonary hypertension but the patient refused this invasive procedure. There weren't any indirect TTE findings consistent with pulmonary hypertension. The patient complaints regressed with medical congestive heart failure therapy (digitalis, ACE inhibitor, diuretic) and regressed from NYHA class III to NYHA class II. The echocardiographic examination of family members showed normal echocardiographic examination for the parents and first degree relatives (uncle, aunt and brother). Medical treatment is one of the treatment modalities, which is effective in terms of relieving the heart failure symptoms besides implantable cardiac defibrillator as well as cardiac transplantation.16–18 The prognosis of LVNC differs according to authors, which we still follow our case for two years after the diagnosis.16,17
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Conclusion
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Transthoracic echocardiography is a useful clinical tool for
diagnosing noncompaction of the ventricular myocardium. Jenni
and Stöllberger's definition for the LVNC can also be utilized
for the RVNC.
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References
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Abstract
Introduction
