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Scaling left ventricular (LV) mass and other cardiac dimensions to account for individual body size is important. The traditional method of simple ratio scaling using, for example, body surface area (BSA) assumes a linear and proportional relationship and accurate measurement of both LV mass and BSA. These assumptions can be questioned; hence, we examined the appropriateness of methods and different indices using highly accurate magnetic resonance imaging scans. Cardiac and whole-body scans were performed in 172 young, healthy, male subjects (age range, 17-28 years) to assess LV mass, volume, linear dimensions, lean body mass and fat mass. Height, body mass and BSA were determined anthropometrically. Relationships were examined for linearity and closeness of fit using log-log least-squares linear regression to determine the slope exponent b (where 1.0 indicates linearity). The relationship between LV mass and lean body mass (b=.90+/-.15; r(2)=.66) was linear and geometrically consistent. This was also the case for LV end-diastolic volume (b=.70), although the confidence intervals were broader (+/-0.32) and the r(2) (.31) smaller. The relationships between LV mass, volume and other variables were generally not linear or geometrically consistent. LV linear dimensions did not demonstrate any linear relationships, and in particular, those with BSA were extremely poor (r(2)=.02-.09). In summary, the traditional scaling of LV measurements to BSA does not remove the influence of body size and other techniques should be considered. Lean body mass was the most appropriate variable for simple indexing of LV mass. No body size variable had a linear and proportional relationship with LV linear dimensions, and the use of simple ratio scaling for these is seriously questioned.

Cardiac amyloidosis describes clinically significant involvement of the heart by amyloid deposition, which may or may not be associated with involvement of other organs. The purpose of this review is to summarize the current state of evidence for the effective evaluation and management of cardiac amyloidosis. Acquired systemic amyloidosis occurs in more than 10 per million person-years in the U.S. population. Although no single noninvasive test or abnormality is pathognomonic of cardiac amyloid, case-control studies indicate that echocardiographic evidence of left ventricular wall thickening, biatrial enlargement, and increased echogenicity in conjunction with reduced electrocardiographic voltages is strongly suggestive of cardiac amyloidosis. Furthermore, newer echocardiographic techniques such as strain and strain rate imaging can demonstrate impairment in longitudinal function before ejection fraction becomes abnormal. Recent observational studies also suggest that cardiovascular magnetic resonance imaging yields characteristic findings in amyloidosis, offering promise for the early detection of cardiac involvement, and the presence of detectable cardiac troponin and elevated B-type natriuretic peptide in serum of affected patients portends an adverse prognosis. Management strategies for cardiac amyloid are largely based on nonrandomized single-center studies. One of the few published randomized studies shows the superiority of oral prednisolone and melphalan compared with colchicine in systemic AL amyloidosis. Intermediate-dose infusional chemotherapy regimes (such as vincristine, adriamycin, and dexamethasone) and high-dose chemotherapy with peripheral stem cell rescue have been used widely, but treatment-related mortality remains substantial with chemotherapy. Recent studies also indicate promising strategies to stabilize the native structures of amyloidogenic proteins; inhibit fibril formation; and disrupt established deposits using antibodies, synthetic peptides, and small-molecule drugs.

Atrial septal endocarditis can occur as an isolated event or in association with valvular endocarditis. It is also reported following percutaneous device closure of atrial septal defects. We present the echocardiography findings from a young man presenting with endocarditis of an abnormal mitral valve in whom endocarditis was demonstrated associated with an atrial septal defect.

The adult heart relies predominantly on fatty acids (FA) for energy generation, and defects in FA catabolism cause dramatic left ventricular (LV) growth in early age. Since lipoprotein lipase (LPL) is the key enzyme in plasma triglyceride catabolism and is highly expressed in the myocardium, we investigated an association between the functional LPL gene serine 447 stop (S447X) variant and exercise-induced LV growth. The S447X variant was genotyped in 146 British Army recruits undergoing a 10-week exercise programme. Over the training period, X447 allele carriers showed less LV growth than S447 homozygotes (SS, 5.8+/-0.7%; SX, 2.2+/-1.5%; P=0.03) and a decrease in systolic blood pressure (DeltaSBP: SS, 1.9+/-1.3 mmHg; SX, -5.7+/-2.2 mmHg; P=0.015). Although LPL genotype did not significantly predict LV growth with DeltaSBP in statistical modelling (LPL, P=0.14; DeltaSBP, P=0.06), regression analysis indicated that LPL S447X genotype effect on DeltaSBP accounted for only 20% of the effect on LV growth. In multivariate analysis, LPL, peroxisome-proliferator-activated receptor alpha and angiotensin-converting enzyme genotypes were independent predictors of cardiac growth. Thus, LPL S447X genotype influenced exercise-induced changes in LV mass and SBP. Change in blood pressure accounted for a proportion of LV growth. These data suggest that increased myocardial FA availability may reduce exercise-induced LV growth.

Background and Aim of the Study: Severe aortic stenosis in pregnancy creates several challenges for the physician. In recent years, balloon valvuloplasty has become more widely used, though the indications for its use in this setting are unclear. A review of the available evidence is presented, and a suggested management strategy illustrated., Methods and Results: Available literature on the subject was reviewed via Medline search and reference lists from the identified articles. Particular attention was paid to prediction of risk, management options and outcome. The data suggest the importance of early symptoms in determining management, as there is a high risk of complications if left untreated. This group should be considered for valvuloplasty, whereas asymptomatic patients are at low risk, and can be managed expectantly. This is illustrated with two contrasting cases from the authors' practice: the symptomatic patient underwent aortic balloon valvuloplasty as a palliative procedure, using transesophageal and minimal fluoroscopic guidance, with good medium-term results. Both patients required aortic valve replacement in the medium to long-term., Conclusion: The use of aortic balloon valvuloplasty in pregnancy is useful as a palliative procedure, allowing deferral of valve replacement until after birth. Echocardiographic features alone are not enough to decide on management, and symptoms play a vital role in determining risk. The use of transesophageal echocardiography during the procedure significantly reduces fluoroscopy time.

Purpose: Determination of the underlying etiology of left ventricular hypertrophy (LVH) is a common, challenging, and critical clinical problem. The authors aimed to test whether pathological LVH, such as occurs in hypertrophic cardiomyopathy (HCM), hypertensive heart disease, or aortic stenosis, and physiological LVH in athletes, can be distinguished by means of left ventricular volume and geometric indices, derived from cardiovascular magnetic resonance imaging., Methods: A total of 120 subjects were studied on a 1.5 Tesla MR (Sonata, Siemens Medical Solutions, Erlangen, Germany) scanner, comprising healthy volunteers (18), competitive athletes (25), patients with HCM (35), aortic stenosis (24), and hypertensive heart disease (18). Left ventricular mass index, ejection fraction, end-diastolic, end-systolic and stroke volume index, diastolic wall thickness, wall thickness ratio and diastolic and systolic wall-to-volume ratios were determined., Results: Left ventricular (LV) mass indices were similar for all forms of LVH (p > 0.05), which were at least 35% higher than those obtained in healthy volunteers (p < 0.05). Multiple logistic regression showed that the percentage of correctly predicted diagnoses was 100% for athlete's heart, 80% for hypertrophic cardiomyopathy, 54% for aortic stenosis, and 22% for hypertensive heart disease. Using a receiver operating curve-determined cut-off value for diastolic wall-to-volume ratio of less than 0.15 mm x m2 x ml(-1), athletes' hearts could be differentiated from all forms of pathological cardiac hypertrophy with 99% specificity., Conclusions: Athlete's heart can be reliably distinguished from all forms of pathological cardiac hypertrophy using CMR-derived LV volume and geometric indices, but pathological forms of LVH present with overlapping cardiac hypertrophy phenotypes. This capability of CMR should be of high clinical value.

Background and Aim of the Study: Cardiovascular magnetic resonance (CMR) assessment of mitral regurgitant volume from the subtraction of the right ventricular stroke volume (RVSV) from left ventricular stroke volume (LVSV) has commonly been performed using volumetric techniques. This is sensitive to errors in RVSV visualization and regurgitation of other heart valves, and therefore subtracting aortic flow volume from LVSV may be preferable. The study aim was to compare both techniques in a single CMR examination., Methods: Twenty-eight patients with isolated mitral regurgitation underwent left ventricular (LV) and right ventricular (RV) volumetry and aortic flow volume measurements. Mitral regurgitant fraction (RF) was calculated as either RF(VOL) = [LVSV - RVSV] or RF(FLOW) = [LVSV - aortic flow volume], both expressed as a fraction of LVSV. The agreement of the measurements was assessed as a measure of robustness in clinical practice., Results: There was good agreement between aortic and pulmonary flow (mean +/- SD difference -0.8 +/- 8.1 ml), and aortic flow volume and RVSV by volumetry (mean difference -2.6 +/- 11.8 ml). Intra- and interobserver variability (SD) of aortic flow volume (+/-6.6 ml and +/-5.3 ml) was superior to that of the RVSV (+/-8.5 ml and +/-12 ml). The intra- and inter-observer variability (SD) of RF(FLOW) was lower (+/-4.8% and +/-7.7%) than by RF(VOL) (+/-6.7% and +/-8.8%)., Conclusion: The RF(FLOW) technique maximized intra- and inter-observer agreement, and is the optimal CMR technique to quantify mitral regurgitation. RF(FLOW) also has the advantage of allowing correction for aortic regurgitation when it is present, and is potentially independent of the effects of tricuspid and pulmonary regurgitation.

Accumulating evidence suggests that athletic performance is strongly influenced by genetic variation. One such locus of influence is the gene for angiotensin-I converting enzyme (ACE), which exhibits a common variant [ACE insertion (I)/deletion (D)]. ACE can drive formation of vasoconstrictor ANG II but preferentially degrades vasodilator bradykinin. The ACE I allele is associated with higher kinin activity. A common gene variant in the kinin beta(2) receptor (B(2)R) exists: the -9 as opposed to +9 allele is associated with higher receptor mRNA expression. We tested whether this variant was associated with the efficiency of muscular contraction [delta efficiency (DE)] in 115 healthy men and women, or with running distance among 81 Olympic standard track athletes. We further sought evidence of biological interaction with ACE I/D genotype. DE was highly significantly associated with B(2)R genotype (23.84 +/- 2.41 vs. 24.25 +/- 2.81 vs. 26.05 +/- 2.26% for those of +9/+9 vs. +9/-9 vs. -9/-9 genotype; n = 25, 61, and 29, respectively; P = 0.0008 for ANOVA adjusted for sex). There was evidence for interaction with ACE I/D genotype, with individuals who were ACE II, with B(2)R -9/-9 having the highest DE at baseline. The ACE I/B(2)R -9 "high kinin receptor activity" haplotype was significantly associated with endurance (predominantly aerobic) event among elite athletes (P = 0.003). These data suggest that common genetic variation in the B(2)R is associated with efficiency of skeletal muscle contraction and with distance event of elite track athletes and that at least part of the associations of ACE and fitness phenotypes is through elevation of kinin activity.

Cardiac lipomatous hypertrophy is an unusual disorder that typically affects the interatrial septum. We report a case in which large subpericardial deposits of fat were initially mistaken for a pericardial effusion and the subsequent clinical picture resembled tamponade. The patient improved following a pericardiectomy.

The study of left ventricular (LV) hypertrophy is hindered by problems with LV mass measurement by echocardiography. Both the M-mode and 2D area-length formulas for calculating LV mass assume a fixed geometric shape, which may be a source of error. We examined this hypothesis by using cardiovascular magnetic resonance images to eliminate the confounding effects of acoustic access and image quality. LV mass was measured directly in 212 healthy subjects by means of a standard 3D cardiovascular magnetic resonance technique. LV mass was also calculated by using the cube-function and area-length formulas with measurements from the magnetic resonance images. A comparison of serial measurements was made by examining the changes in LV mass by all 3 techniques in those completing an exercise program (n=140). The cube-function technique showed a consistent underestimation of LV mass of 14.3 g, and there were wide 95% limits of agreement (+/-57.6 g and +/-46.3 g for cube-function and area-length techniques, respectively) when compared with 3D measurement. There were similarly wide limits of agreement for the change in mass (+/-55.2 g and +/-44.8 g for cube-function and area-length, respectively). The assumption of geometric shape in the cube-function and area-length formulas resulted in significant variation in LV mass estimates from direct measurement by using a 3D technique. The technique cannot be recommended either at a single time point or for serial studies in small populations; 3D imaging techniques, such as cardiovascular magnetic resonance, are preferable.

Left ventricular hypertrophy is associated with significant excess mortality and morbidity. The study and treatment of this condition, in particular the prognostic implications of changes in left ventricular mass, require an accurate, safe, and reproducible method of measurement. Cardiovascular magnetic resonance is a suitable tool for this purpose, and this review assesses the technique in comparison with others and examines the clinical and research implications of the improved reproducibility.

Background: Left ventricular hypertrophy (LVH) occurs as an adaptive response to a physiological (such as exercise) or pathological (valvular disease, hypertension, or obesity) increase in cardiac work. The molecular mechanisms regulating the LVH response are poorly understood. However, inherited defects in fatty acid oxidation are known to cause severe early-onset cardiac hypertrophy. Peroxisome proliferator--activated receptor alpha (PPARalpha) regulates genes responsible for myocardial fatty acid oxidation and is downregulated during cardiac hypertrophy, concomitant with the switch from fatty acid to glucose utilization., Methods and Results: The role of PPARalpha in left ventricular growth was investigated in 144 young male British Army recruits undergoing a 10-week physical training program and in 1148 men and women participating in the echocardiographic substudy of the Third Monitoring Trends and Determinants in Cardiovascular Disease (MONICA) Augsburg study. A G/C polymorphism in intron 7 of the PPARalpha gene significantly influenced left ventricular (LV) growth in response to exercise (P=0.009). LV mass increased by 6.7 +/- 1.5 g in G allele homozygotes but was significantly greater in heterozygotes for the C allele (11.8 +/- 1.9 g) and in CC homozygotes (19.4 +/- 4.2 g). Likewise, C allele homozygotes had significantly higher LV mass, which was greater still in hypertensive subjects, and a higher prevalence of LVH in the Third MONICA Augsburg study., Conclusions: We demonstrate that variation in the PPARalpha gene influences human left ventricular growth in response to exercise and hypertension, indicating that maladaptive cardiac substrate utilization can play a causative role in the pathogenesis of LVH.

Background: Local cardiac renin-angiotensin systems may regulate left ventricular (LV) hypertrophic responses. The absence (deletion [D]) of a 287-bp marker in the ACE gene is associated with greater myocardial ACE levels and exercise-related LV growth than is its presence (insertion [I]), an effect potentially mediated through either increased activity of the cellular growth factor angiotensin II on the angiotensin type 1 (AT(1)) receptor or increased degradation of growth-inhibiting kinins. We sought to confirm ACE genotype-associated exertional LV growth and to clarify the role of the AT(1) receptor in this association., Methods and Results: One hundred forty-one British Army recruits homozygous for the ACE gene (79 DD and 62 II) were randomized to receive losartan (25 mg/d, a subhypotensive dose inhibiting tissue AT(1) receptors) or placebo throughout a 10-week physical training program. LV mass, determined by cardiac magnetic resonance, increased with training (8.4 g, P:<0.0001 overall; 12.1 versus 4.8 g for DD versus II genotype in the placebo limb, P:=0.022). LV growth was similar in the losartan arm: 11.0 versus 3.7 g for DD versus II genotypes (P:=0.034). When indexed to lean body mass, LV growth in the II subjects was abolished, whereas it remained in the DD subjects (-0.022 versus 0.131 g/kg, respectively; P:=0.0009)., Conclusions: ACE genotype dependence of exercise-induced LV hypertrophy is confirmed. Additionally, LV growth in DD (unlike II) subjects is in excess of the increase in lean body mass. These effects are not influenced by AT(1) receptor antagonism with the use of losartan (25 mg/d). The 2.4-fold greater LV growth in DD men may be due to the effects of angiotensin II on other receptors (eg, angiotensin type 4) or lower degradation of growth-inhibitory kinins.