William A. Zoghbi

Recommendations for evaluation of the severity of native valvular regurgitation with two-dimensional and doppler echocardiography

A report from the American Society of Echocardiography’s Nomenclature and Standards Committee and The Task Force on Valvular Regurgitation, developed in conjunction with the American College of Cardiology Echocardiography Committee, The Cardiac Imaging Committee Council on Clinical Cardiology, the American Heart Association, and the European Society of Cardiology Working Group on Echocardiography, represented by:

Doppler Tissue Imaging: A Noninvasive Technique for Evaluation of Left Ventricular Relaxation and Estimation of Filling Pressures

OBJECTIVES This investigation was designed 1) to assess whether the early diastolic velocity of the mitral annulus (Ea) obtained with Doppler tissue imaging (DTI) behaves as a preload-independent index of left ventricular (LV) relaxation; and 2) to evaluate the relation of the mitral E/Ea ratio to LV filling pressures. BACKGROUND Recent observations suggest that Ea is an index of LV relaxation that is less influenced by LV filling pressures. METHODS One hundred twenty-five study subjects were classified into three groups according to mitral E/A ratio, LV ejection fraction (LVEF) and clinical symptoms: 34 asymptomatic subjects with a normal LVEF and an E/A ratio > or =1; 40 with a normal LVEF, an E/A ratio <1 and no heart failure symptoms (impaired relaxation [IR]); and 51 with heart failure symptoms and an E/A ratio >1 (pseudonormal [PN]). Ea was derived from the lateral border of the annulus. A subset of 60 patients had invasive measurement of pulmonary capillary wedge pressure (PCWP) simultaneous with Doppler echocardiographic DTI. RESULTS Ea was reduced in the IR and PN groups compared with the group of normal subjects: 5.8 +/- 1.5 and 5.2 +/- 1.4 vs. 12 +/- 2.8 cm/s, respectively (p < 0.001). Mean PCWP (20 +/- 8 mm Hg) related weakly to mitral E (r = 0.68) but not to Ea. The E/Ea ratio related well to PCWP (r = 0.87; PCWP = 1.24 [E/Ea] + 1.9), with a difference between Doppler and catheter measurements of 0.1 +/- 3.8 mm Hg. CONCLUSIONS Ea behaves as a preload-independent index of LV relaxation. Mitral E velocity, corrected for the influence of relaxation (i.e., the E/Ea ratio), relates well to mean PCWP and may be used to estimate LV filling pressures.

Recommendations for Evaluation of Prosthetic Valves With Echocardiography and Doppler Ultrasound. A Report From the American Society of Echocardiography's Guidelines and Standards Committee and the Task Force on Prosthetic Valves, Developed in Conjunction With the American College of Cardiology Cardiovascular Imaging Committee, Cardiac Imaging Committee of the American Heart Association

A Report From the American Society of Echocardiography’s Guidelines and Standards Committee and the Task Force on Prosthetic Valves, Developed in Conjunction With the American College of Cardiology Cardiovascular Imaging Committee, Cardiac Imaging Committee of the American Heart Association, the European Association of Echocardiography, a registered branch of the European Society of Cardiology, the Japanese Society of Echocardiography and the Canadian Society of Echocardiography, Endorsed by the American College of Cardiology Foundation, American Heart Association, European Association of Echocardiography, a registered branch of the European Society of Cardiology, the Japanese Society of Echocardiography, and Canadian Society of Echocardiography

Doppler Estimation of Left Ventricular Filling Pressure in Sinus Tachycardia A New Application of Tissue Doppler Imaging

BACKGROUND Doppler echocardiography is frequently used to predict filling pressures in normal sinus rhythm, but it is unknown whether it can be applied in sinus tachycardia, with merging of E and A velocities. Tissue Doppler imaging (TDI) can record the mitral annular velocity. The early diastolic velocity (Ea) behaves as a relative load-independent index of left ventricular relaxation, which corrects the influence of relaxation on the transmitral E velocity. METHODS AND RESULTS We evaluated 100 patients 64+/-12 years old with simultaneous Doppler and invasive hemodynamics. Mitral inflow was classified into 3 patterns: complete merging of E and A velocities (pattern A), discernible velocities with A dominance (B), or E dominance (C). The Doppler data were analyzed at the mitral valve tips for E, acceleration and deceleration times of E, and isovolumic relaxation time. In patterns B and C, the A velocity, E/A ratio, and atrial filling fraction were derived. Pulmonary venous flow velocities were also measured, and TDI was used to acquire Ea and Aa. Weak significant relations were observed between pulmonary capillary wedge pressure (PCWP) and sole parameters of mitral flow, pulmonary venous flow, and annular measurements. These were better for patterns A and C. E/Ea ratio had the strongest relation to PCWP [r=0.86, PCWP=1.55+1.47(E/Ea)], irrespective of the pattern and ejection fraction. This equation was tested prospectively in 20 patients with sinus tachycardia. A strong relation was observed between catheter and Doppler PCWP (r=0.91), with a mean difference of 0.4+/-2.8 mm Hg. CONCLUSIONS The ratio of transmitral E velocity to Ea can be used to estimate PCWP with reasonable accuracy in sinus tachycardia, even with complete merging of E and A velocities.

EAE/ASE recommendations for image acquisition and display using three-dimensional echocardiography.

Roberto M. Lang, MD, FASE*‡, Luigi P. Badano, MD, FESC†‡, Wendy Tsang, MD*, David H. Adams, MD*, Eustachio Agricola, MD†, Thomas Buck, MD, FESC†, Francesco F. Faletra, MD†, Andreas Franke, MD, FESC†, Judy Hung, MD, FASE*, Leopoldo Pérez de Isla, MD, PhD, FESC†, Otto Kamp, MD, PhD, FESC†, Jaroslaw D. Kasprzak, MD, FESC†, Patrizio Lancellotti, MD, PhD, FESC†, Thomas H. Marwick, MBBS, PhD*, Marti L. McCulloch, RDCS, FASE*, Mark J. Monaghan, PhD, FESC†, Petros Nihoyannopoulos, MD, FESC†, Natesa G. Pandian, MD*, Patricia A. Pellikka, MD, FASE*, Mauro Pepi, MD, FESC†, David A. Roberson, MD, FASE*, Stanton K. Shernan, MD, FASE*, Girish S. Shirali, MBBS, FASE*, Lissa Sugeng, MD*, Folkert J. Ten Cate, MD†, Mani A. Vannan, MBBS, FASE*, Jose Luis Zamorano, MD, FESC, FASE†, and William A. Zoghbi, MD, FASE*

Determination of parameters of left ventricular diastolic filling with pulsed Doppler echocardiography: comparison with cineangiography.

To determine the relationship between Doppler-derived flow velocity through the mitral anulus and angiographic parameters of left ventricular filling, 30 patients were studied by two-dimensional echocardiography combined with pulsed Doppler echocardiography followed within 1 hr by left ventricular angiography. The average heart rate for each test was 69 beats/min. Doppler-derived parameters included: early peak diastolic velocity (E) and peak atrial velocity, peak filling rate computed as E X cross-sectional area of the mitral anulus derived from the annular diameter, normalized peak filling rate computed as peak filling rate divided by the left ventricular end-diastolic volume determined by two-dimensional echocardiography, and half filling fraction derived from the time-velocity integral of the Doppler-determined velocity curve. Frame-by-frame left ventricular volumes were obtained throughout diastole from single-plane cineangiograms. A volume-time curve with its derivative was generated by computer processing from which peak filling rate, normalized peak filling rate, and half filling fraction were measured. Morphologically, the Doppler-derived velocity profile resembled the derivative of the angiographic volume curve. In patients with reduced angiographic peak filling rates, early peak diastolic velocity was often decreased less than 45 cm/sec with a relative increase in peak atrial velocity resulting in an early peak diastolic velocity to peak atrial velocity ratio less than 1.0. There were no significant differences in mean values for peak filling rate, normalized peak filling rate, and half filling fraction by Doppler echocardiography vs angiography (296 vs 283 ml/sec, 1.9 vs 2.0 sec-1 and 0.55 vs 0.55, respectively).(ABSTRACT TRUNCATED AT 250 WORDS)

Tissue Doppler Imaging Consistently Detects Myocardial Abnormalities in Patients With Hypertrophic Cardiomyopathy and Provides a Novel Means for an Early Diagnosis Before and Independently of Hypertrophy

Background—Left ventricular hypertrophy (LVH), the clinical hallmark of familial hypertrophic cardiomyopathy (FHCM), is absent in a significant number of subjects with causal mutations. In transgenic rabbits that fully recapitulate the FHCM phenotype, reduced myocardial tissue Doppler (TD) velocities accurately identified the mutant rabbits, even in the absence of LVH. We tested whether humans with FHCM also consistently showed reduced myocardial TD velocities, irrespective of LVH. Methods and Results—We performed 2D and Doppler echocardiography and TD imaging in 30 subjects with FHCM, 13 subjects who were positive for various mutations but did not have LVH, and 30 age- and sex-matched controls (all adults; 77% women). LV wall thickness and mass were significantly greater in FHCM subjects (P <0.01 versus those without LVH and controls). There were no significant differences in 2D echocardiographic, mitral, and pulmonary venous flow indices between mutation-positives without LVH and controls. In contrast, systolic and early diastolic TD velocities were significantly lower in both mutation-positives without LVH and in FHCM patients than in controls (P <0.001). Reduced TD velocities had a sensitivity of 100% and a specificity of 93% for identifying mutation-positives without LVH. Conclusions—Myocardial contraction and relaxation velocities, detected by TD imaging, are reduced in FHCM, including in those without LVH. Before and independently of LVH, TD imaging is an accurate and sensitive method for identifying subjects who are positive for FHCM mutations.

Doppler Estimation of Left Ventricular Filling Pressures in Patients With Hypertrophic Cardiomyopathy

BACKGROUND Conventional Doppler parameters are unreliable for estimating left ventricular (LV) filling pressures in hypertrophic cardiomyopathy (HCM). This study was undertaken to evaluate flow propagation velocity by color M-mode and early diastolic annular velocity (Ea) by tissue Doppler 2 new indices of LV relaxation, combined with mitral E velocity for estimation of filling pressures in HCM. METHODS AND RESULTS Thirty-five HCM patients (52+/-15 years) underwent LV catheterization simultaneously with 2-dimensional and Doppler echocardiography. Pulsed Doppler echocardiography of mitral and pulmonary venous flows was obtained along with flow propagation velocity and Ea. LV preA pressure had weak or no relations with mitral, pulmonary venous velocities and atrial volumes. In contrast, preA pressure related strongly to E velocity/flow propagation velocity (r=0.67; SEE=4) and E/Ea (r=0.76; SEE=3.4). In 17 patients with repeat measurements, preA pressure changes were well detected by measuring E velocity/flow propagation velocity (r=0.68; P=0.01) or E/Ea (r=0.8; P<0.001). PreA pressure estimation with these 2 methods was tested prospectively in 17 additional HCM patients with good results (E velocity/flow propagation velocity, r=0.76; E/Ea, r=0.82). CONCLUSIONS LV filling pressures can be estimated with reasonable accuracy in HCM patients by measuring E velocity/flow propagation velocity or E/Ea. These ratios also track changes in filling pressures.

Optimal Noninvasive Assessment of Left Ventricular Filling Pressures A Comparison of Tissue Doppler Echocardiography and B-Type Natriuretic Peptide in Patients With Pulmonary Artery Catheters

Background—Early transmitral velocity/tissue Doppler mitral annular early diastolic velocity (E/Ea) and B-type natriuretic peptide (BNP) have been correlated with left ventricular filling pressures, yet there are no data on how these 2 estimates of left ventricular filling pressures compare. Methods and Results—Patients admitted to intensive care underwent simultaneous tissue Doppler echocardiography, BNP measurement, and pulmonary capillary wedge pressure (PCWP) determination. The ability of mitral E/Ea and BNP to predict PCWP >15 mm Hg was assessed. Fifty patients were studied. Ln BNP had a correlation of r = 0.32 (P = 0.02) with PCWP compared with r = 0.69 (P < 0.001) between E/Ea and PCWP. E/Ea >15 was the optimal cutoff to predict PCWP >15 mm Hg (sensitivity, 86%; specificity, 88%), whereas the optimal BNP cutoff was >300 pg/mL (sensitivity, 91%; specificity, 56%). The correlation between change in PCWP and change in E/Ea at 48 hours was r = 0.87 (P = 0.003) compared with r = −0.59 (P = 0.39) for BNP. In the 36 patients with cardiac disease, E/Ea >15 (sensitivity, 92%; specificity, 91%) appeared more accurate than BNP >400 pg/mL (sensitivity, 92%; specificity, 51%), whereas in patients without cardiac disease, BNP (sensitivity, 81%; specificity, 83%) appeared more accurate than E/Ea >15 (sensitivity, 74%; specificity, 72%) for PCWP >15 mm Hg. Conclusions—In intensive care unit patients, mitral E/Ea has a better correlation than BNP with PCWP. Both BNP and mitral E/Ea have high sensitivity for PCWP >15 mm Hg; however, E/Ea appears more specific in this patient population. In patients without cardiac disease, BNP appears more accurate than E/Ea for PCWP >15 mm Hg, whereas E/Ea appears more accurate in patients with cardiac disease.

Desmin Mutation Responsible for Idiopathic Dilated Cardiomyopathy

BACKGROUND Idiopathic dilated cardiomyopathy, of which approximately 20% of cases are familial (FDCM), is a primary myocardial disorder characterized by ventricular dilatation and impaired systolic function. It is a common cause of heart failure and the need for cardiac transplantation. Although 6 chromosomal loci responsible for autosomal dominant FDCM have been mapped by linkage analysis, none of these genes have been identified. By use of the candidate-gene approach, actin was identified recently as being responsible for dilated cardiomyopathy. Considerable evidence suggests desmin, a muscle-specific intermediate filament, plays a significant role in cardiac growth and development. METHODS AND RESULTS To determine whether a defect of desmin induces dilated cardiomyopathy, 44 probands with FDCM underwent clinical evaluation and DNA analysis. Diagnostic criteria, detected by echocardiography, consisted of ventricular dimension of >/=2.7 cm/m(2) with an ejection fraction </=50% in the absence of other potential causes. After amplification by polymerase chain reaction, the exons of the desmin gene were sequenced. A missense desmin mutation, Ile451Met, which cosegregates with FDCM without clinically evident skeletal muscle abnormalities, was identified in a 4-generation family but was not detected in 460 unrelated healthy individuals. CONCLUSIONS A novel missense mutation of desmin, Ile451Met, was identified as the genetic cause of idiopathic dilated cardiomyopathy. This finding is of particular significance because this is the first mutation detected in the desmin tail domain, and the function of the desmin tail remains unknown. Because this mutation leads to a restricted cardiac phenotype in the family studied in the present report, it suggests that the tail of desmin plays an important functional role in cardiac tissue.