Low EF: the best marker of poor prognosis in patients affected by non compaction
McMahon et al reported in a recent article a reduction of TD velocities in children with noncompaction of the left ventricle, compared with normal controls. The authors concluded their work saying that the reduction of lateral mitral Ea velocity helps to predict children with LVNC who are at risk of adverse clinical outcomes including death and need for cardiac transplantation. In a precedent report our group reported a strong correlation between pathological tissue Doppler and reduction of ejection fraction. In our report the tissue Doppler analysis was performed segment by segment, and a correlation had been ob served only in patients with a low EF.
Noncompaction of the ventricular myocardium (LVNC) is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis; it is characterized by a thin compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep intertrabecular recesses (1-6). In this rare cardiomyopathy clinical presentation and outcome is variable, but heart failure, arrhythmias and sudden cardiac death are described like common clinical findings (1-6). Unfortunately the pathophysiological mechanisms are unknown. In 2002 Jenni et al. (7) reported a microvascular dysfunction in 12 patients affected by non compaction: areas of restricted myocardial perfusion have been documented by scintigraphy, suggesting a reduction of Coronary flow reserve (CFR). The decreased of CFR is not confined to noncompacted segments, but extends to most segments with wall motion abnormalities, thus global coronary microcirculatory dysfunction could be associated with IVNC (7-12). McMahon et al (1) reported in a recent article a reduction of TD velocities in children with noncompaction of the left ventricle, compared with normal controls. In a precedent report our group reported a strong correlation between pathological tissue Doppler and reduction of ejection fraction. In this study we performed a Tissue Doppler analysis, segment by segment, in a series of 15 children affected by non-compaction. The bidimensional echocardiogram showed a strong correlation between systolic function and diastolic dysfunction (2). Alterations of the diastolic function compared in 7 (49%) patients: in 2 cases, a reduction of the Ea wave was present in all segments. In 3 patients the diastolic dysfunction was limited to apical and lateral segments. In the last 2 children a reduction of the Ea wave interested only the apical segments. Every patient with diastolic dysfunction also presented a severe reduction of the systolic function (less then 40%). In our opinion the late enhancement can be depend on a CFR, and is the determinant of the tissue Doppler alterations. So the TD alteration is associated with EF, and is an indirect index of poor clinical outcome, like EF (1-12). McMahon et al (1) concluded their work saying that the reduction of lateral mitral Ea velocity helps to predict children with LVNC who are at risk of adverse clinical outcomes including death and need for cardiac transplantation. In our opinion, more then the Em, the EF at the admission can help to predict the mortality in these patients.
1)McMahon CJ, Pignatelli RH, Nagueh SF, Lee VV, Vaughn W, Valdes SO, Kovalchin JP, Jefferies JL, Dreyer WJ, Denfield SW, Clunie S, Towbin JA, Eidem BW
Left ventricular non-compaction cardiomyopathy in children: characterisation of clinical status using tissue Doppler-derived indices of left ventricular diastolic relaxation
Heart. 2007; 93:676-81.
2)Fazio G, Pipitone S, Iacona MA, Marchì S, Mongiovì M, Zito R, Sutera L, Novo G, Novo S
Evaluation of diastolic function by the Tissue doppler in children affected by non-compaction
Int J Cardiol. 2007;116:e60-2.
3)Fazio G, Sutera L, Corrado G, Novo S
The chronic heart failure is not so frequent in non-compaction
Eur Heart J. 2007; 28:1269.
4) Fazio G, Corrado G, Pizzuto C, Zachara E, Rapezzi C, Sulafa AK, Sutera L, Stollberger C, Sormani L, Finsterer J, Benatar A, Di Gesaro G, Novo G, Cavusoglu Y, Baumhakel M, Drago F, Carerj S, Pipitone S, Novo S.
Supraventricular arrhythmias in noncompaction of left ventricle: Is this a frequent complication?
Int J Cardiol. 2007; [Epub ahead of print]
5)Fazio G, Corrado G, Zachara E, Rapezzi C, Sulafa AK, Sutera L, Pizzuto C, Stollberger C, Sormani L, Finsterer J, Benatar A, Di Gesaro G, Cascio C, Cangemi D, Cavusoglu Y, Baumhakel M, Drago F, Carerj S, Pipitone S, Novo S.
Ventricular tachycardia in non-compaction of left ventricle: is this a frequent complication?
Pacing Clin Electrophysiol. 2007; 30:544-6
6) Fazio G, Sutera L, Vernuccio F, Fazio M, Vernuccio D, Pizzuto C, Di Gesaro G, Cascio C, Novo S
Heart failure and cardiomyopathies: a case report
G Ital Cardiol. 2007; 8:129-32
7) Jenni R, Wyss CA, Oechslin EN, Kaufmann PA
Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction.
J Am Coll Cardiol. 2002; 39:450-4
8) Hamamichi Y, Ichida F, Hashimoto I, Uese KH, Miyawaki T, Tsukano S, Ono Y, Echigo S, Kamiya T
Isolated noncompaction of the ventricular myocardium: ultrafast computed tomography and magnetic resonance imaging.
Int J Cardiovasc Imaging. 2001;17:305-14
9) Sato Y, Matsumoto N, Matsuo S, Kunimasa T, Yoda S, Tani S, Kasamaki Y, Kunimoto S, Saito S.
Myocardial perfusion abnormality and necrosis in a patient with isolated noncompaction of the ventricular myocardium: Evaluation by myocardial perfusion SPECT and magnetic resonance imaging.
Int J Cardiol. 2007; [Epub ahead of print]
10) Jassal DS, Nomura CH, Neilan TG, Holmvang G, Fatima U, Januzzi J, Brady TJ, Cury RC.
Delayed enhancement cardiac MR imaging in noncompaction of left ventricular myocardium.
J Cardiovasc Magn Reson. 2006;8:489-91
11) Korcyk D, Edwards CC, Armstrong G, Christiansen JP, Howitt L, Sinclair T, Bargeois M, Hart H, Patel H, Scott T
Contrast-enhanced cardiac magnetic resonance in a patient with familial isolated ventricular non-compaction.
J Cardiovasc Magn Reson. 2004;6:569-76
12) Soler R, Rodríguez E, Monserrat L, Alvarez N
MRI of subendocardial perfusion deficits in isolated left ventricular noncompaction.
J Comput Assist Tomogr. 2002; 26:373-5