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Cibenzoline improves coronary flow velocity reserve in patients with hypertrophic obstructive cardiomyopathy.

The effect of cibenzoline, a class-Ia antiarrhythmic drug, on coronary flow velocity reserve (CFVR) was examined in patients with hypertrophic cardiomyopathy using transthoracic Doppler echocardiography. Coronary flow velocity reserve was assessed in 11 patients with hypertrophic obstructive cardiomyopathy (HOCM) and 12 patients with hypertrophic nonobstructive cardiomyopathy (HNCM), before and after the intravenous administration of cibenzoline (1 mg/kg). Coronary hyperemia was induced by an intravenous infusion of adenosine triphosphate and CFVR was calculated as the ratio of hyperemic to basal mean coronary diastolic flow velocity. At baseline, CFVR was significantly correlated with left ventricular outflow tract pressure gradient (LVPG) in patients with HOCM (r = 0.67, P < 0.03). In patients with HOCM, administration of cibenzoline significantly improved impaired CFVR (2.0 +/- 0.8 to 3.0 +/- 1.0, P < 0.001), and reduced LVPG (55 +/- 30 to 23 +/- 18 mmHg, P < 0.001), while CFVR remained unchanged in patients with HNCM (2.6 +/- 0.9 to 2.9 +/- 0.8, P not significant). Cibenzoline not only reduces LVPG but also improves CFVR in patients with HOCM. In addition left ventricular outflow obstruction plays an important role in impaired coronary circulation in patients with HOCM.

Adenosine Triphosphate↗

Systolic anterior motion of the mitral valve despite the sliding leaflet technique for repair of the mitral valve.

We report a systolic anterior motion of the anterior mitral leaflet despite employing the sliding leaflet technique for repair of mitral valve regurgitation. A 65-year-old man with chronic, symptomatic mitral regurgitation due to ruptured chordae tendineae underwent mitral valve repair by quadrangular resection of the posterior leaflet and sliding leaflet technique with ring annuloplasty. After weaning from cardiopulmonary bypass, left ventricular outflow obstruction developed and transesophageal echocardiography demonstrated systolic anterior motion of the mitral valve and severe mitral regurgitation. Non-operative treatment resolved the outflow tract obstruction, systolic anterior motion and mitral regurgitation. We conclude that post-repair systolic anterior motion can still occur after the sliding plasty procedure and that medical treatment can successfully resolve systolic anterior motion and outflow tract obstruction in most patients.

Aged↗

[Right ventricular outflow tract reconstruction with monocusp patch].

Two kinds of monocusp patch were used for reconstruction of right ventricular outflow tract in patients with a variety of congenital heart defects. A bovine pericardial patch with monocusp (MVOP) was used in 11 patients (group M), and a separate transannular outflow patch (STOP) with turned-down equine pericardial monocusp was used in 10 patients (group S). Mean age at operation was 9 years 5 months in group M, and 7 years 9 months in group S. There was one operative death in group M. Survivors have completed a mean follow-up of 19 months. There were no late deaths. In early postoperative period, mean peak systolic pressure gradient between right ventricle and main pulmonary artery was 21 +/- 11 mmHg in group M and 21 +/- 11 mmHg in group S, which were not statistically significant. During follow-up period, no significant increase of peak systolic pressure gradient was noted in both groups. In late postoperative period, pulmonary regurgitation was moderate in 7 and severe in 1 in group M, while it was non-significant in 1, mild in 6 and moderate in 2 in group S. The difference between two groups was significant (p < 0.005). In conclusion, both types of patches equally relieved right ventricular outflow obstruction, but STOP prevented postoperative pulmonary regurgitation more effectively.

Bioprosthesis↗

Significant intraoperative right ventricular outflow gradients after repair for tetralogy of Fallot: to revise or not to revise?

BACKGROUND: This study was performed to define alternative parameters for the management of intraoperative residual right ventricular outflow obstruction (RVOTO) after transatrial repair of tetralogy of Fallot (ToF) in order to differentiate those requiring immediate revision from those who do not. METHODS: Since October 1995, 166 patients of ToF underwent transatrial repair. Postbypass residual RVOTO was assessed by surgeon's subjective impression, direct intracardiac pressure measurements, and intraoperative echocardiography (IOE). RVOTO was labeled "significant" whenever it exceeded a gradient of 40 mm Hg on IOE or right ventricular to left ventricular pressure ratio (pRV/LV) exceeded 0.85. Further, on IOE, significant RVOTO was defined "fixed", if there was no change in RVOT dimensions during the cardiac cycle, along with the presence of anatomic substrate for obstruction, and "dynamic" if RVOT dimensions increased appreciably in diastole. Postoperative course and follow-up echocardiograms of all patients were analyzed. RESULTS: Significant RVOTO was detected in 58 (35%) patients (mean gradient 54 mm Hg). Seven (12%) of them with fixed obstruction (mean 46 mm Hg) underwent immediate surgical revision, while the remaining 51 patients with mean gradient of 78 mm Hg (including 10 patients with pRV/LV ratio of > or = 1.0) with dynamic obstruction did not undergo revision. There were six (3.6%) early deaths. Operative mortality and postoperative morbidity were not related to higher residual gradients, although the first 15 such patients had longer intensive care stay and inotropic support, in which this was done electively. On follow-up (mean 18.5 months), outflow gradients declined sharply (mean 16 mm Hg) irrespective of the severity of intraoperative gradients (p < 0.001). There were no reoperations or late deaths. CONCLUSIONS: This study shows that: 1) existing parameters for immediate revision of residual RVOTO possibly need to be reviewed; 2) intraoperative echocardiography helps in differentiating "fixed" from "dynamic" obstruction and helps obviate needless revisions; and 3) dynamic RVOT gradients decline significantly irrespective of their severity after transatrial repair of ToF.

Adolescent↗

Impact of gender on the left ventricular cavity size and contractility in patients with hypertrophic cardiomyopathy.

BACKGROUND: The aim of the study was to assess gender-specific differences in left ventricular cavity size, contractility and left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy. METHODS: We studied retrospectively 129 referred patients with hypertrophic cardiomyopathy (77 males and 52 females). The echocardiographically measured left ventricular end-systolic, end-diastolic dimensions, fractional shortening and occurrence of left ventricular outflow tract gradient > or =30 mmHg were compared between sexes. Logistic regression analysis was used to calculate the predictive values of left ventricular dimensions and contractility for left ventricular outflow tract obstruction for each gender separately. RESULTS: Left ventricular end-diastolic and end-systolic dimensions were significantly smaller in females than males (41.7+/-5.3 vs. 45.1+/-4.9 mm, P=0.0003; 23.1+/-44 vs. 25.6+/-5.3 mm, P=0.007 respectively). On the contrary, the value of fractional shortening was comparable in both sexes (44.7+/-7.3 vs. 43.6+/-7.9%, P>0.05). The left ventricular outflow tract gradient occurred in females as frequently as in males (28.8 vs. 33.8%, P>0.05). By logistic regression analysis the predictors of left ventricular outflow tract gradient in females were left ventricular end-systolic diameter (relative risk=0.74; confidence interval (CI) 0.61 to 0.91; P=0.0038), left ventricular end-diastolic diameter (relative risk=0.82; CI 0.72 to 0.96; P=0.0061) and fractional shortening (relative risk=1.11; CI 1.01 to 1.22; P=0.036). The most potent predictor appeared to be left ventricular end-systolic dimension. In males none of these parameters identified patients with left ventricular outflow tract obstruction. CONCLUSIONS: Females with hypertrophic cardiomyopathy featured smaller left ventricular cavity size, which predisposed to left ventricular outflow tract obstruction (the most potent predictor of left ventricular outflow tract obstruction was left ventricular end-systolic dimension). Higher left ventricular contractility also determined left ventricular outflow tract gradient occurrence in females with hypertrophic cardiomyopathy. In males despite a larger left ventricular cavity size the left ventricular outflow tract obstruction occurred with a similar frequency as in females. Left ventricular outflow tract obstruction was not predicted by left ventricular cavity size or contractility in males.

Adult↗

Prognostic significance of left atrial size in patients with hypertrophic cardiomyopathy (from the Italian Registry for Hypertrophic Cardiomyopathy).

This study assessed left atrial (LA) dimension as a potential predictor of outcome in hypertrophic cardiomyopathy (HC). From the Italian Registry for Hypertrophic Cardiomyopathy, 1,491 patients (mean age 47 +/- 17 years; 61% men; 19% obstructive), followed for 9.4 +/- 7.4 years after the initial echocardiographic evaluation, constituted the study group. The mean LA transverse dimension was 43 +/- 9 mm and was larger in patients with severe symptoms (48 +/- 9 mm for New York Heart Association classes III and IV vs 42 +/- 9 mm for classes I and II, p <0.001), atrial fibrillation (47 +/- 9 vs 42 +/- 8 mm in sinus rhythm, p <0.001), and left ventricular outflow obstruction (46 +/- 9 mm for >or=30 mm Hg at rest vs 42 +/- 9 mm for <30 mm Hg at rest, p <0.001). On univariate analysis, each 5-mm increase in LA size was associated with a hazard ratio (HR) of 1.2 for all-cause mortality (p <0.0001). On multivariate analysis, a LA dimension >48 mm (the 75th percentile) had a HR of 1.9 for all-cause mortality (p = 0.008), 2.0 for cardiovascular death (p = 0.014), and 3.1 for death related to heart failure (p = 0.008) but was unassociated with sudden death (p = 0.81). Similar results were obtained after the exclusion of patients with atrial fibrillation (HR 1.7, p = 0.008) or outflow obstruction (HR 1.8, p = 0.003). The predictive power of LA dimension >48 mm was also validated in an independent HC cohort from the United States, with similar HRs (1.8 for all-cause mortality, p = 0.019). In conclusion, in a large cohort of patients with HC from a nationwide registry, a marked increase in LA dimension were predictive of long-term outcome, independent of co-existent atrial fibrillation or outflow obstruction. LA dimension is a novel and independent marker of prognosis in HC, particularly relevant to the identification of patients at risk for death related to heart failure.

Adult↗

Echocardiographic findings in 83 patients 13-26 years after intracardiac repair of tetralogy of Fallot.

Doppler echocardiography was performed in 83 patients, 13-26 (median 19) years after repair of tetralogy of Fallot. Median age at repair was 7 years (range 9 months-42 years). An outflow patch restricted to the right ventricle was used in 29%, a transannular patch in 27% and the right ventricle was closed without a patch in 44%. Ninety-six percent of the patients were asymptomatic at follow-up. Patients with a transannular patch were younger, had a larger right ventricle and a higher right ventricle to pulmonary artery pressure difference than those without a patch. The average end-diastolic diameter of the right ventricle was larger than normal regardless of type of repair. The thickness of the right ventricular wall was increased in 50%. In 43% of the patients there was persistent hypertrophy of the right ventricular wall despite normalized systolic pressure. The average size and function of the left ventricle were normal although fractional shortening was reduced in 30% of the patients. The aortic root was abnormally large in 48%. Thirty-four percent had moderate or severe pulmonary valve regurgitation. Symptom-limited work capacity was 87% of predicted and was inversely related to age, systolic pressure in the right ventricle. E/A ratio of the left ventricle and use of the transannular patch. We conclude that right ventricular dysfunction and right ventricular outflow obstruction and/or incompetence were common even in asymptomatic long-term survivors after repair of tetralogy of Fallot.

Adolescent↗

Two-dimensional and Doppler echocardiographic evaluation of heart disease in the neonate and fetus.

Two-dimensional and Doppler echocardiography has had a major impact on the diagnostic and therapeutic management of the newborn infant and fetus with a cardiovascular disorder. This article illustrates the use of the ultrasound techniques in the diagnosis of heart disease in the acyanotic newborn infant with a left-to-right shunt or ventricular outflow obstruction and the cyanotic infant with increased or decreased pulmonary vascularity. In addition, echocardiography has proven invaluable in the in utero diagnosis of cardiovascular abnormalities (structural or rhythm disturbances). This article also reviews the indications for and technical aspects of the fetal echocardiographic examination.

Cyanosis↗

Two-dimensional echocardiography in complete transposition of the great arteries.

Sixty three patients with complete transposition of the great arteries (d-TGA) were studied by two dimensional echocardiography in order to assess: great artery relationships; ventriculo-arterial connections; presence and nature of the left ventricular outflow obstruction (LVOTO). From the parasternal short axis view at the great arteries level, four different relationships were imaged: the aorta was anterior and to the right in 35 patients, the two vessels were in a direct anterior-posterior position in 15 or side by side in 3 and the aorta was anterior and to the left in 10. Differently from normals, early pulmonary bifurcation of the artery arising from the left ventricle was imaged in 42 patients (84%) from the apical approach and in 36 (72%) from the subcostal longitudinal view. Left ventricular outflow tract obstruction was diagnosed in 29 patients (46%) and two distinct types of obstruction were differentiated: fixed and dynamic. We conclude that information derived from two-dimensional echocardiography is a valuable tool for the medical management, cardiac catheterization and surgical treatment of infants and children with d-TGA.

Adolescent↗

[Multiplane transesophageal echocardiography in the study of expansive mediastinal processes compressing the right heart: a report of clinical cases].

In order to assess the role of multiplane transesophageal echocardiography in detecting mediastinal masses and in evaluating the manifestations of cardiac compression due to tumoral growth, 3 cases of patients with right ventricular outflow obstruction are presented. In each of these patients transesophageal echocardiography provided an accurate evaluation of the hemodynamic consequences of the tumoral process.

Adolescent↗

Hemopericardium and cardiac tamponade as presenting findings of dexamethasone-induced hypertrophic cardiomyopathy complicated by transmural myocardial infarction.

This is the first report of dexamethasone-induced left ventricular outflow obstruction causing decreased coronary artery perfusion and apical transmural cardiac infarct that resulted in hemopericardium and rapid cardiac tamponade. The differential diagnosis of hemorrhagic pericardial effusion must now include dexamethasone-induced hypertrophic cardiomyopathy.

Anti-Inflammatory Agents↗

Doppler echocardiographic observations during percutaneous aortic balloon valvuloplasty.

To evaluate the hemodynamic changes occurring with percutaneous aortic balloon valvuloplasty for aortic stenosis, Doppler echocardiography was performed during the procedure in 16 patients. During balloon inflation, peak velocity and ejection time of the aortic valve systolic signals increased (26 and 30%, respectively; p less than 0.001). Aortic regurgitation deceleration time decreased from 1,337 to 625 ms (p less than 0.001). In three patients, aortic regurgitation stopped before end-diastole; in four patients, end-diastole forward flow across the aortic valve was documented. The deceleration time of the mitral valve inflow signal decreased from 303 to 194 ms (p less than 0.001) during balloon inflation, concurrently with an increase in left ventricular diastolic pressure. Mitral regurgitation signals became more prominent during inflation in 10 patients. Changes that occur during balloon inflation in the aortic valve include progressive left ventricular outflow obstruction, equalization of diastolic aortic and left ventricular pressures and changes in diastolic compliance.

Aged↗

B-type natriuretic peptide levels in congenital heart disease.

The objective of this study was to evaluate the potential role of B-type natriuretic peptide (BNP) levels in children with congenital heart disease undergoing cardiac catheterization. Measurement of plasma BNP concentration has been shown to be useful in the diagnosis, risk stratification, and management of adult patients with congestive heart failure, but little is known about the role of BNP in children with structural congenital heart disease. We measured plasma BNP levels using the Triage BNP test in patients with congenital heart disease referred for diagnostic or interventional cardiac catheterization. Plasma BNP concentration was measured in 96 children and 11 adults > or = 19 years old (7.9+/-8.3 years) undergoing heart catheterization for underlying congenital heart disease. BNP levels ranged from < 5 to > 1300 pg/ml, with a median BNP concentration of 19.0 pg/ml. Baseline BNP concentrations were > 100.0 pg/ml on 19 occasions in 17 patients. The pressure difference between the left ventricle and ascending aorta was 10-110 mmHg in 21 patients. BNP concentrations for this cohort ranged from < 5.0 to 1060.0 pg/ml and correlated with the degree of left ventricular outflow obstruction (correlation coefficient, 0.661; p = 0.001). This study suggests that with additional research, BNP concentration may prove to be a useful clinical tool in managing children and adults with congenital heart disease.

Adolescent↗

Comparison of computed tomography and cineangiography in the demonstration of central pulmonary arteries in cyanotic congenital heart disease.

PURPOSE: To assess the diagnostic accuracy of contrast-enhanced computed tomography (CT) for central pulmonary artery pathology in patients with cyanotic congenital heart disease (CCHD) and right ventricular outflow obstruction. METHODS: We compared contrast-enhanced CT and cine pulmonary arteriography in 24 patients including hte confluence. Both investigations were interpreted by a cardiac radiologist in a double-blinded manner at an interval of 3 weeks. Angiography was used as the gold standard for comparison. RESULTS: The sensitivity for visualization of main pulmonary artery (MPA), right pulmonary artery (RPA), left pulmonary artery (LPA), and confluence on CT was 94%, 100%, 92.8%, and 92.8%, respectively. Diagnostic specificity for the same entities was 28.5%, 100%, 80% and 50%, respectively. The positive predictive value for each was 76.2%, 100%, 94.1%, and 72.2%, respectively. The low specificity of CT in the evaluation of the MPA and the confluence is perhaps due to distorted right ventricular outflow anatomy in CCHD. Large aortopulmonary collaterals in this region were mistaken for the MPA in some patients with pulmonary atresia. CONCLUSION: CT is a useful, relatively noninvasive, imaging technique for the central pulmonary arteries in selected patients. It can supplement diagnostic information from angiography but cannot replace it. LPA demonstration on axial images alone is inadequate.

Adolescent↗