noncompaction cardiomyopathy ( nccm ) is a recently recognized rare disorder [ 1 , 2 ] . it is characterized by prominent myocardial trabecularizations , and deep intertrabecular recesses leading to the spongy appearance of the myocardium . the disease is frequently associated with systolic and diastolic heart failure ( hf ) , ventricular arrhythmias , and systemic embolization . alterations in arterial function have been demonstrated in patients with hf [ 5 , 6 ] . with two - dimensional transthoracic echocardiography ( tte ) , ascending aortic diameter changes during a heart cycle can be measured . when blood pressure data are also available , aortic elasticity can be characterized [ 7 , 8 ] . this study was designed to examine aortic stiffness in nccm patients and to compare these results to age- and gender - matched controls . a total of 20 patients with typical echocardiographic features of nccm were investigated [ 3 , 9 ] . clinical assessment included medical and family history , physical examination , electrocardiography , two - dimensional echocardiography , and , in most cases , contrast echocardiography . their results were compared to 20 age- and gender - matched controls without apparent cardiovascular disease . informed consent was obtained from each patient , and the study was approved by the institutional review board and complied with the declaration of helsinki . table  1.clinical and demographic data of nccm patientsnccm patientsage ( years)38    16male ( % ) 8 ( 40)diabetes ( % ) 1 ( 5)index eventarrhythmia ( % ) 6 ( 30)heart failure ( % ) 8 ( 40)screening ( % ) 6 ( 30)electrocardiogramatrial fibrillation ( % ) 2 ( 10)lv hypertrophy ( % ) 3 ( 15)left bundle branch block ( % ) 6 ( 30)nccm noncompaction cardiomyopathy clinical and demographic data of nccm patients nccm noncompaction cardiomyopathy previously proposed echocardiographic diagnostic criteria for nccm by jenni et al .   were used : ( 1 ) absence of coexisting cardiac anomalies , ( 2 ) segmental , excessive thickening of the left ventricular ( lv ) wall with a two - layered structure : a thin , compacted epicardial layer and a much thicker , noncompacted layer with the characteristic appearance of numerous , prominent trabeculations ( meshwork ) and deep intertrabecular recesses , ( 3 ) color doppler evidence of deeply perfused intertrabecular recesses , and ( 4 ) predominant localization of thickening in the lv apical , midlateral , and midinferior walls . hypertensive heart disease was excluded by clinical and echocardiographic examinations ( septal thickness  <  13  mm ) . systolic and diastolic blood pressures ( sbp and dbp , respectively ) were measured in the supine position with an automatic mercury cuff sphygmomanometer from the left arm after 10  min of rest . none of the patients or controls used coffee or tea within 1  h before blood pressure measurements . all patients underwent a complete two - dimensional tte and doppler study using a philips sonos 7500 echocardiography equipment ( philips , best , the netherlands ) in the left lateral decubitus position from multiple windows . the lv wall segments were analyzed according to the 9-segment model as described by jenni et al . . systolic and diastolic ascending aortic diameters ( sd and dd , respectively ) were recorded in m - mode at a level of 3  cm above the aortic valve from a parasternal long - axis view ( fig .  1 ) . the sd and dd were measured at the time of maximum anterior motion of the aorta and at the peak of the qrs complex , respectively . 1measurements of systolic ( sd ) and diastolic ( dd ) diameters of the ascending aorta are shown on the m - mode tracing obtained at a level 3  cm above the aortic valve measurements of systolic ( sd ) and diastolic ( dd ) diameters of the ascending aorta are shown on the m - mode tracing obtained at a level 3  cm above the aortic valve aortic stiffness index (  ) was defined as ln(sbp data are reported as means    standard deviation . for variables , student s t test and analysis of variance ( anova ) test the reproducibility of the aortic diameter measurements was tested in all nccm patients at both systole and diastole by two independent , blinded observers . a total of 20 patients with typical echocardiographic features of nccm were investigated [ 3 , 9 ] . clinical assessment included medical and family history , physical examination , electrocardiography , two - dimensional echocardiography , and , in most cases , contrast echocardiography . their results were compared to 20 age- and gender - matched controls without apparent cardiovascular disease . informed consent was obtained from each patient , and the study was approved by the institutional review board and complied with the declaration of helsinki . 
 table  1.clinical and demographic data of nccm patientsnccm patientsage ( years)38    16male ( % ) 8 ( 40)diabetes ( % ) 1 ( 5)index eventarrhythmia ( % ) 6 ( 30)heart failure ( % ) 8 ( 40)screening ( % ) 6 ( 30)electrocardiogramatrial fibrillation ( % ) 2 ( 10)lv hypertrophy ( % ) 3 ( 15)left bundle branch block ( % ) 6 ( 30)nccm noncompaction cardiomyopathy clinical and demographic data of nccm patients nccm noncompaction cardiomyopathy were used : ( 1 ) absence of coexisting cardiac anomalies , ( 2 ) segmental , excessive thickening of the left ventricular ( lv ) wall with a two - layered structure : a thin , compacted epicardial layer and a much thicker , noncompacted layer with the characteristic appearance of numerous , prominent trabeculations ( meshwork ) and deep intertrabecular recesses , ( 3 ) color doppler evidence of deeply perfused intertrabecular recesses , and ( 4 ) predominant localization of thickening in the lv apical , midlateral , and midinferior walls . hypertensive heart disease was excluded by clinical and echocardiographic examinations ( septal thickness  <  13  mm ) . systolic and diastolic blood pressures ( sbp and dbp , respectively ) were measured in the supine position with an automatic mercury cuff sphygmomanometer from the left arm after 10  min of rest . none of the patients or controls used coffee or tea within 1  h before blood pressure measurements . all patients underwent a complete two - dimensional tte and doppler study using a philips sonos 7500 echocardiography equipment ( philips , best , the netherlands ) in the left lateral decubitus position from multiple windows . the lv wall segments were analyzed according to the 9-segment model as described by jenni et al . . systolic and diastolic ascending aortic diameters ( sd and dd , respectively ) were recorded in m - mode at a level of 3  cm above the aortic valve from a parasternal long - axis view ( fig .  1 ) . the sd and dd were measured at the time of maximum anterior motion of the aorta and at the peak of the qrs complex , respectively . 1measurements of systolic ( sd ) and diastolic ( dd ) diameters of the ascending aorta are shown on the m - mode tracing obtained at a level 3  cm above the aortic valve measurements of systolic ( sd ) and diastolic ( dd ) diameters of the ascending aorta are shown on the m - mode tracing obtained at a level 3  cm above the aortic valve aortic stiffness index (  ) was defined as ln(sbp / dbp)/[(sd  -  dd)/dd ] , where ln is the natural logarithm . data are reported as means    standard deviation . for variables , student s t test and analysis of variance ( anova ) test the reproducibility of the aortic diameter measurements was tested in all nccm patients at both systole and diastole by two independent , blinded observers . the presenting symptoms were hf in eight ( 40% ) and arrhythmias in six ( 30% ) patients . the remaining six ( 30% ) asymptomatic patients were nccm relatives and were diagnosed after family screening ( table  1 ) . three hf patients were in nyha - class iii hf and the other five were in nyha - class ii . cardiac medication used in nccm patients were : angiotensin - converting enzyme inhibitors in 13 ( 65% ) , beta - blockers in 11 ( 55% ) , oral anticoagulant therapy in 10 ( 50% ) , diuretics in 8 ( 40% ) , digitalis in 2 ( 10% ) , and nitrate in 1 ( 5% ) . the number of noncompacted segments in the nccm patients was 4.6    2.0 in the nccm group and ( as seen in table  2 ) these patients had significantly increased lv dimensions and reduced lv ejection fraction . aortic stiffness index (  ) was significantly increased in nccm patients compared to controls . for controls , nccm patients with moderate hf , and nccm patients with severe hf , values were 3.5    1.1 , 7.9    5.5 , and 10.4    1.8 , respectively ( p  <  0.001 ) . aortic stiffness indices of all individual patients and controls are presented in fig .  2 . table  2.transthoracic echocardiographic and blood pressure data in nccm patients and normal subjectsgroup 1 ( normal subjects)group 2 ( nccm patients)lv end - diastolic diameter ( mm)47.4    3.661.0    10.9lv end - systolic diameter ( mm)29.9    2.948.2    12.3lv ejection fraction ( % ) 67.5    5.936.0    17.6systolic aortic diameter ( mm)26.7    4.126.6    4.4diastolic aortic diameter ( mm)23.3    3.824.6    4.3pulsatile change in aortic diameter ( mm)3.4    1.12.0    1.2systolic blood pressure ( mmhg)125.2    12.9120.4    17.4diastolic blood pressure ( mmhg)77.8    8.574.1    9.6aortic pulse pressure ( mmhg)47.4    8.746.3    11.7aortic stiffness index ( )3.5    1.18.3    5.2continuous variables are given as mean    standard deviationlv left ventricular , nccm noncompaction cardiomyopathyp  <  0.001fig . nccm noncompaction cardiomyopathy transthoracic echocardiographic and blood pressure data in nccm patients and normal subjects continuous variables are given as mean    standard deviation lv left ventricular , nccm noncompaction cardiomyopathy individual  indices of nccm patients and control subjects . nccm noncompaction cardiomyopathy the mean    standard deviation difference in values obtained by two observers for the measurements of aortic diameter at systole was 0.7    2.2  mm , with a correlation coefficient between these independent measurements of 0.88 ( p  <  0.01 ) ( figs . the difference between these observations was 0.05    1.95  mm , with a correlation coefficient between observations of 0.9 ( p  <  0.01 ) . the difference in values that were detected by observers was within twofold of the standard deviation of the mean ( figs . 3a interobserver correlation ( r  =  0.88 , p  <  0.01 ) between aortic systolic diameters in nccm patients . b interobserver correlation ( r  =  0.9 , p  <  0.01 ) between aortic diastolic diameters in nccm patients . nccm noncompaction cardiomyopathy a interobserver correlation ( r  =  0.88 , p  <  0.01 ) between aortic systolic diameters in nccm patients . b interobserver correlation ( r  =  0.9 , p  <  0.01 ) between aortic diastolic diameters in nccm patients . the presenting symptoms were hf in eight ( 40% ) and arrhythmias in six ( 30% ) patients . the remaining six ( 30% ) asymptomatic patients were nccm relatives and were diagnosed after family screening ( table  1 ) . three hf patients were in nyha - class iii hf and the other five were in nyha - class ii . cardiac medication used in nccm patients were : angiotensin - converting enzyme inhibitors in 13 ( 65% ) , beta - blockers in 11 ( 55% ) , oral anticoagulant therapy in 10 ( 50% ) , diuretics in 8 ( 40% ) , digitalis in 2 ( 10% ) , and nitrate in 1 ( 5% ) . the number of noncompacted segments in the nccm patients was 4.6    2.0 in the nccm group and ( as seen in table  2 ) these patients had significantly increased lv dimensions and reduced lv ejection fraction . aortic stiffness index (  ) was significantly increased in nccm patients compared to controls . for controls , nccm patients with moderate hf , and nccm patients with severe hf , values were 3.5    1.1 , 7.9    5.5 , and 10.4    1.8 , respectively ( p  <  0.001 ) . table  2.transthoracic echocardiographic and blood pressure data in nccm patients and normal subjectsgroup 1 ( normal subjects)group 2 ( nccm patients)lv end - diastolic diameter ( mm)47.4    3.661.0    10.9lv end - systolic diameter ( mm)29.9    2.948.2    12.3lv ejection fraction ( % ) 67.5    5.936.0    17.6systolic aortic diameter ( mm)26.7    4.126.6    4.4diastolic aortic diameter ( mm)23.3    3.824.6    4.3pulsatile change in aortic diameter ( mm)3.4    1.12.0    1.2systolic blood pressure ( mmhg)125.2    12.9120.4    17.4diastolic blood pressure ( mmhg)77.8    8.574.1    9.6aortic pulse pressure ( mmhg)47.4    8.746.3    11.7aortic stiffness index ( )3.5    1.18.3    5.2continuous variables are given as mean    standard deviationlv left ventricular , nccm noncompaction cardiomyopathyp  <  0.001fig . nccm noncompaction cardiomyopathy transthoracic echocardiographic and blood pressure data in nccm patients and normal subjects continuous variables are given as mean    standard deviation lv left ventricular , nccm noncompaction cardiomyopathy individual  indices of nccm patients and control subjects . the mean    standard deviation difference in values obtained by two observers for the measurements of aortic diameter at systole was 0.7    2.2  mm , with a correlation coefficient between these independent measurements of 0.88 ( p  <  0.01 ) ( figs . the difference between these observations was 0.05    1.95  mm , with a correlation coefficient between observations of 0.9 ( p  <  0.01 ) . the difference in values that were detected by observers was within twofold of the standard deviation of the mean ( figs . 3a interobserver correlation ( r  =  0.88 , p  <  0.01 ) between aortic systolic diameters in nccm patients . b interobserver correlation ( r  =  0.9 , p  <  0.01 ) between aortic diastolic diameters in nccm patients . nccm noncompaction cardiomyopathy a interobserver correlation ( r  =  0.88 , p  <  0.01 ) between aortic systolic diameters in nccm patients . b interobserver correlation ( r  =  0.9 , p  <  0.01 ) between aortic diastolic diameters in nccm patients . nccm is a recently recognized disorder characterized by a loosened , spongy myocardium associated with a high incidence of progressive systolic and diastolic hf . in recent studies , alterations have been demonstrated in arterial function in patients with chronic hf [ 1114 ] . to the best of authors s knowledge , this is the first time that the aortic distensibility in a series of nccm patients was examined . in this study , increased aortic stiffness index (  ) was found in nccm patients compared to age- and gender - matched controls . in prior studies , it has been shown that pulsatile changes in ascending aortic vessel diameter can be indirectly measured during routine tte . stefanadis et al .   found that noninvasive measurements of aortic distensibility were as accurate as invasive methods . aortic stiffness index (  ) relies on aortic and blood pressure data and is one of the most frequently used parameters to characterize arterial stiffness [ 7 , 8 ] . increased aortic stiffness is an independent risk factor , predictor of cardiovascular mortality , and a contributor to lv afterload [ 15 , 16 ] . increased aortic stiffness occurs early during the development of pacing - induced congestive hf in animals [ 17 , 18 ] and has also been described in clinical patients with hf [ 5 , 6 ] , with comparable changes in arterial distensibility in ischemic and idiopathic dilated cardiomyopathy . .   demonstrated that the distensibility of the proximal aorta is markedly reduced in older patients with hf due to lv systolic dysfunction beyond the changes from the aging process . giannattasio et al .   described that arterial compliance is impaired in congestive hf , and although more marked in severe congestive hf , the impairment is manifested in mild congestive hf as well . nakamura et al .   described vascular hypertrophic remodeling and endothelial dysfunction - associated alterations in vascular wall elastic properties in limb muscle conduit arteries in patients with congestive hf . poelzl et al .   described an intriguing relationship between phenotype changes and functional impairment in peripheral conduit arteries of hf patients . neurohormonal changes associated with hf include an increase in sympathetic drive and an activated renin  angiotensin system resulting in increased plasma norepinephrine levels causing vasoconstriction and sodium retention [ 2023 ] . early atheromatous changes and endothelial dysfunction can also be associated with hf , and can affect vascular elasticity . however , there is also an interaction between aortic stiffening and hf . aortic stiffening can increase lv load causing lv stiffening with increased wall tension , early impairment in diastolic lv relaxation , and contractility inducing lv hypertrophy and fibrosis [ 26 , 27 ] . these results suggest that aortic stiffness may contribute to the progression of systolic and diastolic lv dysfunctions . during this study , brachial cuff pressure measurement instead of a direct assessment of aortic pulse pressure by catheter was used . however , previous studies demonstrated an excellent correlation in aortic distensibility measured by invasive and noninvasive methods . coronary artery disease is correlated with increased aortic stiffness and was not excluded in most but not in all our patients by coronary angiography . the aortic elastic properties of control patients were somewhat larger than those described in the literature . the reason for this can be the larger body mass index ( 29.0    5.1  kg / m ) in our controls . however , a relatively higher  in the normal subjects in our study only strengthens the abnormal findings in the nccm patients . increased aortic stiffness can be observed in patients with nccm with moderate to severe hf .
backgroundnoncompaction cardiomyopathy ( nccm ) is a recently recognized disorder frequently associated with systolic and diastolic heart failures . 
 this study was designed to examine aortic stiffness in nccm patients and to compare these results to age- and gender - matched controls.methodsa total of 20 patients with typical echocardiographic features of nccm ( age 38    16  years , eight males ) were investigated . 
 their results were compared to 20 age- and gender - matched controls . 
 all subjects underwent a complete two - dimensional transthoracic echocardiographic examination . 
 systolic ( sd ) and diastolic ( dd ) ascending aortic diameters were recorded in m - mode at a level of 3  cm above the aortic valve from a parasternal long - axis view . 
 aortic stiffness index (  ) was calculated as a characteristic of aortic elasticity , as ln(sbp / dbp)/[(sd  -  dd)/dd ] , where sbp and dbp are the systolic and diastolic blood pressures , respectively , and ln is the natural logarithm.resultsthe number of noncompacted segments in the nccm patients was 4.6    2.0 . 
 nccm patients had significantly increased left ventricular dimensions and reduced left ventricular ejection fraction . 
 compared to controls , aortic stiffness index (  ) was significantly increased in nccm patients ( 8.3    5.2 vs. 3.5    1.1 , p  <  0.001).conclusionincreased aortic stiffness can be observed in patients with nccm with moderate to severe heart failure . 
 these alterations may be due to neurohormonal changes in heart failure .