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Chapter 11: Hypertrophic Cardiomyopathy  145


              to accurately quantify LV mass in animal models of LV   cellular (interstitial) space and has a slow washout period
              hypertrophy including experimental canine aortic ste-  (6–20 minutes). In cardiac fibrosis, the extracellular space
              nosis and a transgenic mouse model of LV hypertrophy   is expanded due to the loss of myocytes and the presence
              (Franco et al. 1998; Wang et al. 1990). Results of cMRI   of collagen. The expanded extracellular space results in
              are also highly reproducible, with much lower inter- and   pooling  of  the  gadolinium  and  slow  washout  in  this
              intraobserver  variability  than  echocardiography  (7.8   region. Using T1-weighted images, delayed enhancement
              grams vs 19.2 grams mean weighted interstudy standard   (DE) is a hyperintense (bright) appearance of the myo-
              deviation) in normal people and patients with LV hyper-  cardium where contrast medium has accumulated.
              trophy (Grothues et al. 2002). Studies that quantified LV   DE-cMRI has been used in human patients as well as
              mass in dogs and normal people have found similar low   cats with HCM to noninvasively assess the presence of
              intraobserver  error  (0.4%  and  4%,  respectively)  and   myocardial  fibrosis  (Figure  11.23)  (Moon  et  al.  2004;
              interobserver error (5.8% and 4%, respectively) (Fieno   Choudhury et al. 2002; Wilson et al. 2002). A majority
              et al. 2002). Similarly, there was extremely low intraob-  of asymptomatic to mildly symptomatic human patients   Cardiomyopathies
              server  and  interobserver  variability  of  2%  for  cMRI   (80%) have evidence of DE of the hypertrophied regions
              quantification of left ventricular mass in cats (MacDonald   of the LV, with a patchy to multifocal distribution, pre-
              et al. 2004). With the higher accuracy and reproducibility   dominantly involving the middle third of the LV wall
              of cMRI, the number of subjects needed to obtain mean-  (Choudhury et al. 2002). Histologic studies have con-
              ingful data is dramatically reduced. For example, in one   firmed  that  regions  of  DE  correspond  to  regions  of
              study the sample size necessary to demonstrate a 10 gram   myocardial fibrosis in people with HCM (Moon et al.
              change in LV mass in people with LV hypertrophy was   2004).  Given  the  spatial  resolution  of  contrast  cMRI,
              15 patients with cMRI versus 152 patients with echocar-  replacement fibrosis is the most likely type of fibrosis
              diography (Grothues et al. 2002).                  detected. Detection of diffuse interstitial fibrosis by DE
                  An unexplored area of research is the use of cMRI to   is more limited since the technique is sensitive to regional
              evaluate LV variables over time to assess whether phar-  differences in gadolinium accumulation. DE is seen in
              maceutical  interventions  cause  significant  changes  in   fewer than 50% of patients with dilated cardiomyopathy
              patients with cardiomyopathy (Hoffmann et al. 2001).   where  there  is  diffuse  interstitial  fibrosis  (McCrohon
              cMRI has been proposed as the gold standard for mea-  et al.  2003). DE-MRI has also been performed  in Bio
              suring regression of LV mass in people (Hoffmann et al.   14.6 cardiomyopathic hamsters, which have regions of
              2001; Rajappan et al. 2003). Regression of LV mass fol-
              lowing aortic valve replacement in patients with aortic
              stenosis  was  evaluated  in  one  study  by  cMRI  and  by
              echocardiography using M-mode measurements. cMRI
              identified a regression of LV mass in all patients 1 year
              after valve replacement, while M-mode echocardiogra-                   RV
              phy failed to accurately identify the regression in these
              individuals.  Using  cMRI  for  LV  mass  quantification,               LV
              there was no regression in concentric hypertrophy in 26
              asymptomatic Maine coon cats with familial HCM that
              were treated with ramipril for 12 months (MacDonald
              et al. 2006b). The use of cMRI provides more accurate
              assessment of LV mass with less variability in measure-
              ments  than  echocardiography  and  allows  for  fewer
              patients needed while maintaining a powerful study.
                                                                 Figure 11.23.  Delayed	enhancement	seen	on	cardiac	magnetic
              Assessment of myocardial fibrosis with             resonance	imaging	in	a	cat	with	severe	hypertrophic	cardiomyopathy.
              cardiac MRI                                          Approximately	7–10	minutes	after	administering	intravenous
                                                                 gadolinium,	postcontrast	cross-sectional	images	are	obtained	of
              Contrast enhancement cMRI is a noninvasive technique
              used to identify myocardial fibrosis or necrosis in various   the	left	ventricle	to	evaluate	for	delayed	enhancement	(DE).	There
                                                                 is	a	large	region	of	DE	(i.e.,	a	bright,	hyperintense	region)	of	the
              cardiac diseases. A low molecular weight paramagnetic   anterior	left	ventricular	free	wall,	indicating	myocardial	fibrosis
              contrast agent is administered intravenously, distributes   (arrow).	The	DE	occurred	at	the	most	severely	hypertrophied	re-
              to the myocardium, and is reabsorbed by the capillary bed   gion	of	the	left	ventricle	(LV)	in	this	cat	with	severe	HCM.	The
              to be excreted by the kidneys. In regions of abnormal   hyperintense	circle	to	the	right	is	a	reference	marker.	RV	=	right
              myocardium, the contrast agent accumulates in the extra-  ventricle.
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