Steep left ventricle to aortic root angle and hypertrophic obstructive cardiomyopathy: study of a novel association using three-dimensional multimodality imaging.

BACKGROUND: Patients with hypertrophic cardiomyopathy (HCM) exhibit a difference in left ventricular outflow tract (LVOT) obstruction, independently of basal septal thickness (BST). Some patients with HCM have a steeper left ventricle to aortic root angle than controls. OBJECTIVE: To test the predictors of the LV-aortic root angle and the association between LV-aortic root angle and LVOT obstruction using three-dimensional imaging. PATIENTS: 153 consecutive patients with HCM (mean (SD) age 46 (14) years, 68% men) and 62 patients with hypertensive heart disease of the elderly (all >65 years of age, 73 (6) years, 34% men) who underwent whole-heart three-dimensional cardiac magnetic resonance (CMR) angiography (1.5 T) and Doppler echocardiography. Forty-two controls (age 43 (11) years, 38% men) who underwent contrast-enhanced multidetector computed tomography and were free of cardiovascular pathology were also studied. MAIN OUTCOMES: LV-aortic root angle, BST and maximal non-exercise LVOT gradient were measured in patients with HCM and in hypertensive-elderly patients. Additionally, LV-aortic root angle and BST were measured in controls. RESULTS: The mean (SD) LV-aortic root angle was significantly different (p<0.001) in the three groups: HCM (134 (10) degrees ), hypertensive-elderly (128 (10) degrees ), control (140 (7) degrees ). There was an inverse correlation between age and LV-aortic root angle in the three groups (all p<0.001): HCM (r = -0.56), hypertensive-elderly (r = -0.35), control (r = -0.48). On univariate analysis, in the HCM group, LV-aortic root angle (beta = -0.34, p<0.001), age (beta = 0.23, p = 0.01) and end-systolic volume index (beta = -0.20, p = 0.02), but not BST (beta = 0.02, p = 0.8), were associated with LVOT gradient. On multivariate analysis, only LV-aortic root angle was associated with LVOT gradient. CONCLUSIONS: Patients with HCM have a steeper LV-aortic root angle than controls. In patients with HCM, a steeper LV-aortic root angle predicts dynamic LVOT obstruction, independently of BST.

Abnormal papillary muscle morphology is independently associated with increased left ventricular outflow tract obstruction in hypertrophic cardiomyopathy.

BACKGROUND: Abnormal papillary muscles (PM) are often found in hypertrophic cardiomyopathy (HCM). OBJECTIVE: To assess the relationship between morphological alterations of PM in patients with HCM and left ventricular outflow tract (LVOT) obstruction, using magnetic resonance imaging (MRI) and echocardiography. METHODS: Fifty-six patients with HCM (mean age 42 years (interquartile range 27, 51), 70% male) and 30 controls (mean age (42 (30, 53) years, 80% male) underwent MRI on a 1.5 T scanner (Siemens, Erlangen, Germany). Standard cine images were obtained in short-axis (base to apex), along with two-, three- and four-chamber views. The presence of bifid PM (none, one or both) and anteroapical displacement of anterolateral PM was recorded by MRI and correlated with resting LVOT gradients obtained by echocardiography. RESULTS: Double bifid PM (70% vs 17%) and anteroapical displacement of anterolateral PM (77% vs 17%) were more prevalent in patients with HCM than in controls (p<0.001). Subjects with anteroapically displaced PM and double bifid PM had higher resting LVOT gradients than controls (45 (6, 81) vs 12 (0, 12) mm Hg (p<0.01) and 42 (6, 64) vs 11 (0, 17) mm Hg (p = 0.02), respectively. In patients with HCM, the odds ratio of having significant (>or=30 mm Hg) peak resting gradient was 7.1 (95% CI 1.4 to 36.7) for anteroapically displaced anterolateral PM and 10.4 (95% CI 1.2 to 91.2) for double bifid PM (both p = 0.005), independent of septal thickness, use of beta-blockers and/or calcium blockers and resting heart rate. CONCLUSIONS: Patients with HCM with abnormal PM have a higher degree of resting LVOT gradient, which is independent of septal thickness.

Outcome of patients with hypertrophic obstructive cardiomyopathy after percutaneous transluminal septal myocardial ablation and septal myectomy surgery.

OBJECTIVES: This study was conducted to evaluate follow-up results in patients with hypertrophic obstructive cardiomyopathy (HOCM) who underwent either percutaneous transluminal septal myocardial ablation (PTSMA) or septal myectomy. BACKGROUND: Controversy exists with regard to these two forms of treatment for patients with HOCM. METHODS: Of 51 patients with HOCM treated, 25 were treated by PTSMA and 26 patients via myectomy. Two-dimensional echocardiograms were performed before both procedures, immediately afterwards and at a three-month follow-up. The New York Heart Association (NYHA) functional class was obtained before the procedures and at follow-up. RESULTS: Interventricular septal thickness was significantly reduced at follow-up in both groups (2.3 +/- 0.4 cm vs. 1.9 +/- 0.4 cm for septal ablation and 2.4 +/- 0.6 cm vs. 1.7 +/- 0.2 cm for myectomy, both p < 0.001). Estimated by continuous-wave Doppler, the resting pressure gradient (PG) across the left ventricular outflow tract (LVOT) significantly decreased immediately after the procedures in both groups (64 +/- 39 mm Hg vs. 28 +/- 29 mm Hg for PTSMA, 62 +/- 43 mm Hg vs. 7 +/- 7 mm Hg for myectomy, both p < 0.0001). At three-month follow-up, the resting PG remained lower in the PTSMA and myectomy groups (24 +/- 19 mm Hg and 11 +/- 6 mm Hg, respectively, vs. those before procedures, both p < 0.0001). The NYHA functional class was also significantly improved in both groups (3.5 +/- 0.5 vs. 1.9 +/- 0.7 for PTSMA, 3.3 +/- 0.5 vs. 1.5 +/- 0.7 for myectomy, both p < 0.0001). CONCLUSIONS: Both myectomy and PTSMA reduce LVOT obstruction and significantly improve NYHA functional class in patients with HOCM. However, there are benefits and drawbacks for each therapeutic method that must be counterbalanced when deciding on treatment for LVOT obstruction.

Percutaneous transluminal alcohol septal myocardial ablation after aortic valve replacement.

When left ventricular outflow tract obstruction develops after aortic valve replacement, few treatment choices have been available until now. We present a patient with prior aortic valve replacement who developed left ventricle outflow tract obstruction that was successfully treated with a percutaneous transcoronary myocardial septal alcohol ablation. This technique is a useful tool for the treatment of obstructive hypertrophic cardiomyopathy, especially in those patients with prior heart surgery.

Homozygous mutation in cardiac troponin T: implications for hypertrophic cardiomyopathy.

BACKGROUND: Mutations in the gene that encode cardiac troponin T (cTnT) account for approximately 15% of cases of familial hypertrophic cardiomyopathy (HCM). These mutations are associated with a particularly severe form of HCM characterized by a high incidence of sudden death and a poor overall prognosis, despite subclinical or mild left ventricular hypertrophy. METHODS AND RESULTS: We evaluated a family with HCM and multiple occurrences of sudden death in children. DNA samples were isolated from peripheral blood or paraffin-embedded tissue, and all protein-encoding exons of the cTnT gene were sequenced. A mutation was identified in exon 11 and is predicted to substitute a phenylalanine-for-serine mutation at residue 179 (Ser(179)Phe) in cTnT. Both parents and 3 of 4 surviving and clinically unaffected children were heterozygous for this mutation; another clinically unaffected child did not carry the mutation. Genetic analysis of DNA from a child who died suddenly at age 17 years demonstrated he was homozygous for this mutation. A review of his echocardiogram revealed profound left and right ventricular hypertrophy. CONCLUSIONS: An homozygous Ser(179)Phe mutation in cTnT causes a severe form of HCM characterized by striking morphological abnormalities and juvenile lethality. In contrast, the natural history of the heterozygous mutation is benign. These studies emphasize the relevance of genetic diagnosis in hypertrophic cardiomyopathy and provide a new perspective on the clinical consequences of troponin T mutations.

Percutaneous transluminal septal myocardial ablation.

Percutaneous transluminal septal myocardial ablation (PTSMA) is a new, investigational, catheter-based treatment for severely symptomatic, medically refractory hypertrophic obstructive cardiomyopathy. A balloon catheter is used to cannulate and isolate the first or second septal perforator coronary artery. Following balloon inflation and intracoronary myocardial contrast echocardiography, ethyl alcohol is injected through the catheter lumen to cause proximal interventricular septum infarction and relief of outflow tract obstruction with improved patient symptoms. Septal scarring and thinning with reductions in the outflow tract gradients ensues over the following 6 to 12 weeks. Most patients have symptomatic improvement, at least moderate reductions in outflow tract gradients, and possibly improvement in exercise capacity. The most common procedural complication is the development of high-grade atrioventricular block necessitating implantation of a permanent pacemaker in 25% of patients. Compared with surgical myectomy, PTSMA has the advantage of being minimally invasive, easily repeated, and with relatively low major morbidity/mortality risk for patients with comorbid conditions. The findings from recently initiated international registries will be helpful in assessing the overall success and complication rates with PTSMA.

Mechanism of mitral regurgitation in hypertrophic cardiomyopathy: mismatch of posterior to anterior leaflet length and mobility.

BACKGROUND: In hypertrophic cardiomyopathy, a spectrum of mitral leaflet abnormalities has been related to the mechanism of mitral systolic anterior motion (SAM), which causes both subaortic obstruction and mitral regurgitation. In the individual patient, SAM and regurgitation vary in parallel; clinically, however, great interindividual differences in mitral regurgitation can occur for comparable degrees of SAM. We hypothesized that these differences relate to variations in posterior leaflet length and mobility, restricting its ability to follow the anterior leaflet (participate in SAM) and coapt effectively. METHODS AND RESULTS: Different mitral geometries produced surgically in porcine valves were studied in vitro. Comparable degrees of SAM resulted in more severe mitral regurgitation for geometries characterized by limited posterior leaflet excursion. Mitral geometry was also analyzed in 23 patients with hypertrophic cardiomyopathy by intraoperative transesophageal echocardiography. All had typical anterior leaflet SAM with significant outflow tract gradients but considerably more variable mitral regurgitation; therefore, regurgitation did not correlate with obstruction. In contrast, mitral regurgitation correlated inversely with the length over which the leaflets coapted (r= -0.89), the most severe regurgitation occurring with a visible gap. Regurgitation increased with increasing mismatch of anterior to posterior leaflet length (r=0.77) and decreasing posterior leaflet mobility (r= -0.79). CONCLUSIONS: SAM produces greater mitral regurgitation if the posterior leaflet is limited in its ability to move anteriorly, participate in SAM, and coapt effectively. This can explain interindividual differences in regurgitation for comparable degrees of SAM. Thus, the spectrum of leaflet length and mobility that affects subaortic obstruction also influences mitral regurgitation in patients with SAM.

Resting echocardiographic features of latent left ventricular outflow obstruction in hypertrophic cardiomyopathy.

We determined resting echocardiographic features predictive of latent left ventricular (LV) outflow obstruction in 50 consecutive patients with nonobstructive hypertrophic cardiomyopathy (26 provocable, 24 nonprovocable with amyl nitrite inhalation) to have a better understanding of the pathophysiology of this condition and to identify such patients without pharmacologic provocation. Measurements included wall thickness, type of hypertrophy, LV outflow tract diameter, degree of mitral systolic anterior motion, outflow pressure gradient, and ventricular volume. The direction of the ejection streamline was measured to assess the magnitude of the drag force acting on the mitral valve. Thirteen of 16 patients (81%) with proximal septal bulge were provocable, whereas only 3 of 8 patients (38%) with asymmetric septal hypertrophy and 10 of 26 (38%) with concentric hypertrophy were provocable (p < 0.05). LV outflow tract was significantly narrower and the angle between the ejection flow and the mitral valve was larger in provocable patients. The sensitivity for predicting provocable patients by a combination of a narrow outflow tract (< or = 2 cm) and a large angle (> or = 35 degrees) was 65%, with a specificity of 80% and a positive predictive value of 79%. When these criteria were combined with the presence of septal bulge, the sensitivity was 35%, but the specificity and the positive predictive value were both 100%. Patients with nonobstructive hypertrophic cardiomyopathy with proximal septal bulge, a narrow LV outflow tract, and an oblique angle between the ejection flow and the mitral valve appeared to be predisposed for latent outflow obstruction. These features are consistent with the presence of the large Venturi and drag forces. Thus, the left ventricle, which is capable of increasing both the Venturi and the drog forces on the basis of the morphologic change, contributes to the development of outflow obstruction with amyl nitrite inhalation.

Inspiratory muscle training and whole-body reconditioning in chronic obstructive pulmonary disease.

To examine the efficacy of targeted inspiratory muscle training (IMT), 25 patients with moderate COPD were randomly assigned to one of three groups. Eight patients received IMT along with general exercise reconditioning, GER+IMT; nine patients received general exercise reconditioning, GER; eight patients received sham breathing exercises, CONTROL. All groups used a spring-loaded inspiratory muscle trainer; however, the GER and CONTROL groups breathed through these devices at only 15% of their maximal inspiratory pressure. The GER+IMT group increased the load on these devices until at 6 wk the load was equal to 80% of their maximal inspiratory pressure. All patients exercised three times per week for a 12-wk period in supervised sessions. Analysis of covariance revealed no significant differences in spirometric measurements, maximal inspiratory pressure, or maximal oxygen consumption among any of the three groups after the intervention (p > 0.05). Twelve-minute walk distance was significantly greater in the GER+IMT and GER groups than in the CONTROL group (p = 0.03). After the intervention, there was a trend (p = 0.08) for treadmill time to be greater for the GER+IMT and GER groups than for the CONTROL group. Dyspnea ratings at different exercise intensities were not found to be significantly different among the three groups after the intervention. These results demonstrate that GER+IMT and GER alone are equally effective in improving exercise performance in patients with COPD. Additionally, the combination of GER and IMT does not appear to provide any clinically significant improvements in exercise performance or perceptions of dyspnea during exercise when compared with GER alone.

Dynamic magnetic resonance imaging assessment of the effect of ventricular wall curvature on regional function in hypertrophic cardiomyopathy.

We hypothesized that contraction within the ventricular septum in hypertrophic cardiomyopathy (HC) may be related to its abnormal morphology because ventricular wall stress is related to wall curvature by the Laplace equation. To test this, we studied 17 HC patients with various septal morphologies using dynamic magnetic resonance imaging techniques. Short- and long-axis curvatures of the basal septal and basal lateral walls were determined on cine images as the reciprocal of the radius of the arc best fit to the endocardial contour, which was negative if the wall was convex to the cavity of the left ventricle. Endocardial and epicardial intramyocardial circumferential shortening (% circumferential shortening) was measured in the septal and lateral walls on basal short-axis myocardial tagging images. Septal walls were flatter in the short-axis plane and more convex toward the left ventricular cavity in the long-axis plane than lateral walls, as indicated by smaller short- and long-axis curvatures. Septal percent circumferential shortening was significantly lower than the lateral percent circumferential shortening, suggesting reduced septal contraction. Endocardial and epicardial percent circumferential shortening showed significant positive correlations with wall curvatures. Multiple stepwise linear regression analysis revealed that both short- and long-axis curvatures significantly contributed to percent circumferential shortening (r=0.87 for endocardial and r=0.70 for epicardial, both p<0.0001). In conclusion, wall curvature is related to wall function in HC; the more convex toward the left ventricular cavity the wall is, the less it contracts. Reduced contraction of the septum in HC may be partly due to its abnormal curvature.

New insights into the reduction of mitral valve systolic anterior motion after ventricular septal myectomy in hypertrophic obstructive cardiomyopathy.

To determine the mechanism of reduction of mitral valve systolic anterior motion by myectomy, we examined 33 patients with hypertrophic obstructive cardiomyopathy echocardiographically before and after myectomy. Measurements included outflow tract diameter, the direction of ejection streamline (the angle between the ejection flow and the mitral valve), midventricular fractional area change, and papillary muscle inward excursion in the short-axis image. After myectomy, the outflow tract was enlarged (from 1.2 +/- 0.3 cm to 2.1 +/- 0.4 cm; p < 0.001), and the ejection flow became more parallel to mitral leaflets (from 51 +/- 10 degrees to 28 +/- 8 degrees; p < 0.001), whereas hyperdynamic midventricular fractional area change was reduced (81% +/- 14% to 62% +/- 14%; p < 0.001), and papillary muscle excursion decreased (1.3 +/- 0.3 cm to 0.8 +/- 0.3 cm; p < 0.001). Outflow enlargement and reduced ventricular contraction would decrease the Venturi force. Change of ejection streamline and reduced contraction would decrease the drag force onto the mitral leaflets. Blunted papillary motion would increase the mitral leaflet tension and decrease the effect of drag force on both leaflets. Thus myectomy decreases Venturi and drag forces and appears to reduce systolic anterior motion of the mitral valve.

Left ventricular outflow tract obstruction in hypertrophic cardiomyopathy: presurgical and postsurgical evaluation by computed tomography magnetic resonance imaging.

Along with hypertrophy of the left ventricle (LV), hypertrophic cardiomyopathy (HC) is characterized by LV outflow tract (LVOT) obstruction, partly from systolic anterior motion (SAM) of the anterior mitral leaflet; if obstruction is significant, excision of excess subaortic septal myocardium may be indicated. In this study, the ability of computed tomography magnetic resonance imaging (Cine MRI) to provide information about LVOT obstruction was assessed in a series of 37 HC cases undergoing evaluation, including echocardiography (0 to 14 days before), for possible septal myectomy; in 4 cases, Cine MRI was used postsurgically (5 to 25 months after). Blinded to echocardiography results, 3 reviewers analyzed by consensus the Cine-MRI LVOT-long-axis image-loops for SAM grade (none, mild, severe) and "aorta:LVOT signal ratio" (intensity in descending aorta/intensity in LVOT at maximum systolic-flow disturbance). Resting LVOT flow velocities were separately determined using Doppler analysis, permitting differentiation between insignificant (< 30 mm Hg) and significant (< or = 30 mm Hg) gradients. With echocardiography, significant resting obstruction was found in 62% of cases, including 92% treated surgically. A significant association between SAM grade and obstruction was found; all cases with a SAM grade of none had insignificant ([-] predictive value: 100%) and most with a severe grade had significant ([+] predictive value: 78%) obstruction. Signal ratio in the absence of a significant gradient was significantly lower than in its presence; a significant linear relationship between aorta:LVOT signal ratio and resting gradient was found: LVOT gradient = ([2.9] x [signal ratio]) + 22.8. SAM grade did not contribute significantly in obstruction categorization when signal ratio was known. In all cases studied after surgery, SAM grade had decreased from severe to insignificant levels and aorta:LVOT signal ratio had been significantly reduced. Insights into the dynamic nature of the LVOT in HC patients can be provided by Cine MRI, either during their evaluation for surgery or after their septal myectomy.

Surgical management of hypertrophic obstructive cardiomyopathy. Early and late results.

From 1975 through 1993, 178 patients underwent surgical management of hypertrophic obstructive cardiomyopathy. Operations included isolated septal myectomy (n = 95), septal myectomy and coronary artery bypass grafting (n = 41), septal myectomy plus a valve procedure (n = 25), septal myectomy, valve procedure, and coronary artery bypass grafting (n = 14), and mitral valve replacement without septal myectomy (n = 3). Recent myectomy results were monitored with transesophageal echocardiography. After initial myectomy, 32 patients (20%) underwent a second pump run for more extensive myectomy only (n = 22), mitral valve replacement only (n = 5), or both (n = 2). In-hospital mortality was 6% (n = 11) and 4% (n = 6) for patients undergoing septal myectomy or septal myectomy plus coronary artery bypass grafting, respectively. Heart block occurred in 17 patients (10%). Left ventricular outflow tract systolic gradients decreased from a mean of 93 mm Hg to 21 mm Hg after myectomy. Late survival was 86% and 70% at 5 and 10 postoperative years, respectively, and 93% and 79% for patients undergoing septal myectomy alone or septal myectomy plus coronary artery bypass grafting, respectively. Only 3 of 131 in-hospital survivors of septal myectomy or septal myectomy plus coronary artery bypass grafting died late cardiac deaths, for a yearly mortality of 0.6%. However, the 5-year late survival of patients undergoing valve operation plus septal myectomy was 51%, and multivariate testing confirmed the adverse influence on late survival (p = 0.008), as well as adverse influences of increasing age (p = 0.016) and return to cardiopulmonary bypass for mitral valve replacement (p = 0.038). At follow-up 136 patients (94%) had New York Heart Association class I or II symptoms. For patients with hypertrophic obstructive cardiomyopathy, septal myectomy alone or in combination with coronary artery bypass grafting produces effective symptom relief, excellent long-term survival, and a low risk of late cardiac death.

Provocation of latent left ventricular outflow tract gradients with amyl nitrite and exercise in hypertrophic cardiomyopathy.

Amyl nitrite may be used to provoke latent gradients in patients with hypertrophic cardiomyopathy (HC) without significant resting outflow tract gradients, but afterload reduction may not be comparable to a more physiologic stressor such as symptom-limited exercise testing. This study compared the ability of amyl nitrite and exercise testing to provoke outflow tract gradients in 57 patients (40 men and 17 women, mean age +/- SD 49 +/- 16 years) with HC (septal thickness 19 +/- 5 mm, average resting gradient 13 +/- 10 mm Hg) who underwent echocardiography at rest, after amyl nitrite inhalation, and after maximal exercise. No significant gradient (< 50 mm Hg) was induced after either provocation in 26 patients (46%); in 15 patients (26%), inducibility was achieved after both stressors, in 6 (11%) after exercise only, and in 10 (18%) after amyl only. Patients with amyl-induced gradients differed from those in whom gradients were noninducible on the basis of smaller outflow tract dimensions (p < 0.001), larger resting gradients (p < 0.001), and a greater prevalence of "septal bulge" morphology (p = 0.02). Those with exercise-induced gradients were able to attain a greater workload (p = 0.07), have larger resting gradients (p = 0.02), and also tended to have a septal bulge morphology (p < or = 0.01). Although outflow tract obstruction increased to similar levels after amyl nitrite (49 +/- 39 mm Hg) and symptom-limited exercise (47 +/- 39 mm Hg), gradients induced by exercise and amyl correlated poorly (r = 0.54).(ABSTRACT TRUNCATED AT 250 WORDS)

Anatomic explanation of mobile systolic clicks: implications for the clinical and echocardiographic diagnosis of mitral valve prolapse.

An echocardiogram (echo) is often ordered for suspected mitral valve prolapse (MVP). Using echo as the gold standard, we conducted a meticulous physical examination on 61 patients with this referral diagnosis. Ninety percent of patients with negative physical examination and echo results for MVP had physical examination findings likely to have been misinterpreted as MVP by the referring physician. Redundant portions of the mitral valve apparatus were found in 57% of patients with MVP on our physical examination but not on echo. A carefully performed physical examination (including dynamic auscultation) can exclude MVP. Not all mobile systolic clicks are associated with anatomic echo prolapse; they can be generated by redundant chordae tendineae and, in the absence of echo prolapse, probably by redundant leaflets. Patients with mobile systolic clicks should have an echo to determine the portion of the spectrum of echo prolapse present and to determine risk stratification and management.

The role of echocardiography in assessing the morphological response of left ventricular thrombus to anticoagulation.

We present a patient with idiopathic dilated cardiomyopathy and a large left ventricular apical thrombus in which serial echocardiography over a 1-month period documented progressive enlargement of this mural thrombus. This case illustrates the dramatic progression of left ventricular thrombus size despite aggressive anticoagulation. In addition, the critical role of echocardiography in the diagnosis and follow-up of patients with left ventricular thrombus is emphasized.

Treatment of hypertrophic cardiomyopathy by mitral valve repair and septal myectomy.

This report describes surgical treatment consisting of mitral valve repair and septal myectomy in a young patient with a combination of hypertrophic cardiomyopathy and intrinsic mitral valve disease. The posterior mitral valve leaflet was elongated and was subject to systolic anterior motion, creating left ventricular outflow tract obstruction. With the described treatment, it was possible to avoid mitral valve replacement in this patient.

Mechanism of outflow tract obstruction causing failed mitral valve repair. Anterior displacement of leaflet coaptation.

BACKGROUND: Systolic anterior motion of the mitral valve causing left ventricular outflow tract obstruction occurs in 1% to 2% of patients having mitral valve repair, in some cases requiring further surgery to relieve the obstruction, but the mechanism and the geometry involved are not certain. METHODS AND RESULTS: We studied 14 patients who developed systolic anterior motion and left ventricular outflow tract obstruction, all after posterior leaflet resection and annuloplasty, in whom a second repair eliminated systolic anterior motion by complete (n = 6) or partial (n = 8) ring removal. Intraoperative transesophageal echocardiography was recorded before pump, after failed repair during left ventricular outflow tract obstruction, and after a corrective second pump run to relieve the systolic anterior motion. Systolic anterior motion occurred when the mitral valve coaptation to septum distance was reduced (before, 26.5 +/- 4.3; during systolic anterior motion, 17.4 +/- 4.4 versus after second pump, 23.4 +/- 6.9 mm) and the mitral valve coaptation to posterior mitral annulus distance was greater (before, 18.9 +/- 3.4; during systolic anterior motion, 22.2 +/- 4.6 versus after second pump, 17.4 +/- 3.6 mm), both P < .01. Comparing dimensions before pump, during systolic anterior motion, and after the second pump, there were no differences in left ventricular cavity diameter in systole or diastole, the septum to posterior annulus distance, or the angle between the aortic and mitral annular planes. CONCLUSIONS: After mitral repair, left ventricular outflow tract obstruction occurs when the mitral coaptation line is displaced anteriorly. When systolic anterior motion occurs, reduction of the amount of annuloplasty or use of the posterior leaflet sliding procedure may eliminate this problem. Understanding the geometry of this phenomenon may facilitate preoperative echo selection of high-risk patients (those with large redundant posterior leaflets and relatively normal ventricular size) and modification of surgical technique to avoid the problem of outflow tract obstruction after mitral valve repair.

Benefits of intraoperative echocardiography in the surgical management of hypertrophic cardiomyopathy.

OBJECTIVES: The purpose of this study was to determine the role of intraoperative echocardiography in planning the site and extent of myectomy and in ensuring adequate control of the left ventricular outflow tract gradient. BACKGROUND: Although intraoperative echocardiography has been found to be beneficial in patients undergoing valve repair, its impact on surgical decisions in patients undergoing septal myectomy for hypertrophic cardiomyopathy has not been described. METHODS: In 50 patients undergoing septal myectomy over a 5-year period, epicardial echocardiography was performed before cardiopulmonary bypass to establish the extent of outflow tract obstruction, locate its site and plan the myectomy. In 30 patients, transesophageal echocardiography was also used to corroborate data on outflow tract anatomy and examine the mitral valve. RESULTS: In 40 patients (80%) the initial myectomy resulted in a reduction of the maximal outflow tract gradient from 88 +/- 45 to 24 +/- 11 mm Hg, measured by epicardial continuous wave Doppler echocardiography. Ten patients (20%) were shown by postbypass intraoperative echocardiography to have an unsatisfactory result, based on a persistent gradient > 50 mm Hg (n = 7) or persistent mitral regurgitation of greater than moderate severity (n = 3). The postbypass two-dimensional echocardiogram was then used to direct the surgeon toward the most likely site of continued obstruction, and cardiopulmonary bypass was reinstituted to permit further myectomy (n = 9) or mitral valve repair (n = 1). After the second or subsequent period of cardiopulmonary bypass, the outflow tract gradient (26 +/- 14 mm Hg) was substantially reduced and was not significantly different from the postbypass gradient (24 +/- 11 mm Hg) in the group with initial surgical success. At postoperative follow-up (20 +/- 37 weeks), the maximal measured outflow tract gradient (22 +/- 21 mm Hg) showed no difference between patients with immediate surgical success and those requiring a second period of cardiopulmonary bypass for further resection. CONCLUSIONS: Intraoperative echocardiography proved a useful tool to guide the site and extent of septal myectomy, leading to more adequate surgical resection and to persistence of satisfactory control of the outflow tract obstruction into the early follow-up period.