Right and left ventricular systolic function late after repair of tetralogy of Fallot

Right ventricular (RV) dysfunction has adverse effects on long-term outcome in patients with repaired tetralogy of Fallot (TOF). We employed serial radionuclide angiography (RNA) to examine RV and left ventricular (LV) systolic function in adults late after TOF repair and its relation to clinical outcome. We reviewed 10-year records of 95 patients (53 men) with TOF followed in our clinic (mean age at repair 12.6 ± 10.5 years, mean age at last follow-up 37.7 ± 9.8 years) who underwent at least 2 RNAs between 1987 and 1997. Most patients were well by the end of the study (80% were New York Heart Association class I, 17% were class II, and 3% were in class III). Sixteen patients experienced sustained tachyarrhythmias (8 had atrial; 8 patients had ventricular). One patient died suddenly. Fifteen patients underwent RV outflow reoperations (15 underwent pulmonary valve replacement; 7 had relief of RV outflow obstruction); RV systolic function during exercise in these 15 patients was significantly impaired before and returned to similar levels after surgery, compared with the rest of the patients. Overall, RV and LV function remained stable in the whole group at a mean interval of 5.7 ± 2.2 years between first and last RNA. This group of closely followed adults with TOF remained well over 10 years with a low incidence of sudden death and stable RV and LV systolic function, despite a relatively large number of RV outflow reoperations. Aggressive intervention for right-sided hemodynamic abnormalities may have contributed to this outcome. Preserved ventricular function may herald a favorable long-term outlook in this group.     

Age-related changes in right and left ventricular function in tetralogy of Fallot

Age-related changes in right (RV) and left (LV) ventricular myocardium in tetralogy of Fallot (TF) are analyzed with regard to pre- and postoperative ventricular function. In RV, preoperative function was better in patients under 4 years of age in terms of ejection fraction and compliance, but no significant change was seen in LV function. Postoperatively, patients who underwent corrective surgery at age less than 4 years showed no significant difference compared to those at operative age above 4 years in terms of RV and LV functions at rest. However, stress tests for RV by isoproterenol and LV using methoxamine revealed some masked ventricular dysfunction in both ventricles in the older groups. The relationships between these functional abnormalities and previously described histological changes of right and left ventricular myocardium in TF are discussed.     

Effects of left ventricular function on the exercise capacity in patients with repaired tetralogy of Fallot

Background: Tissue Doppler imaging has been recently used to evaluate ventricular function. Peak oxygen uptake (V^?O₂peak) has been demonstrated as a predictor for death in adults with repaired tetralogy of Fallot (TOF). The aim of this study was to determine which Doppler parameters correlated with V^?O₂peak in patients with repaired TOF. Method and Results: Doppler echocardiography, tissue Doppler imaging, and exercise test were performed in 30 patients with TOF after surgical repair. In 30 patients with repaired TOF (median age 14 years, range 9–25 years), 11 patients (37%) were female. Seven patients (median age 12 years) had normal left ventricular diastolic function, whereas the rest of the patients were classified as diastolic dysfunction grade II (median age 15 years; n = 15) and III and IV (median age 18 years; n = 8). The oxygen uptake at anaerobic threshold (V^?O_2AT) and peak exercise in patients with left ventricular diastolic dysfunction was significantly lower than that in those with normal diastolic function. Also, V^?O_2AT and V^?O₂peak in patients with diastolic dysfunction grade III and IV were significantly lower than that in those with diastolic dysfunction grade II. Left ventricular early diastolic myocardial velocity was most closely correlated to V^?O₂peak (r = 0.51; P = 0.005). Peak early ventricular filling velocity to early diastolic myocardial velocity ratio was significantly correlated with V^?O₂peak (r =?0.50; P = 0.006). Conclusion: Left ventricular diastolic dysfunction is correlated with V^?O₂peak. Left ventricular diastolic function should be a routine echocardiographic assessment in patients with repaired TOF. (Echocardiography 2011;28:1019‐1024)     

Factors affecting left ventricular function after correction of tetralogy of Fallot

To investigate the possible causes of left ventricular dysfunction after total correction of tetralogy of Fallot, 84 patients, aged 1 1/2 to 16 years, were studied by left ventricular cineangiography both before and a mean of 4.6 months after operation. Left ventricular ejection fraction and mean velocity of circumferential fibre shortening were calculated; using multivariate analysis the results were correlated with age at operation, the degree of hypoxia and polycythaemia before operation, occurrence of hypoxic spells, and the duration of operative procedures (cardiopulmonary bypass and aortic cross clamping). The postoperative left ventricular ejection fraction was decreased slightly or moderately in 46% of patients. The variable most significantly associated with altered left ventricular function was a history of hypoxic spells. Age, the degree of chronic hypoxia, and polycythaemia did not correlate significantly with left ventricular function indices. Although no correlation was found between the duration of cardiopulmonary bypass and left ventricular ejection fraction, bypass times exceeding 120 minutes were associated with decreased ejection fractions; this was statistically significant and independent of the variable "hypoxic spells". Thus repeated episodes of acute hypoxia and long operative procedures appear to have a deleterious effect on left ventricular function in tetralogy of Fallot.     

Echocardiographic study of right and left ventricular dimension and left ventricular function in patients with tetralogy of Fallot before and after surgery.

Right and left ventricular dimensions and function were determined by one-dimensional echocardiography in patients with tetralogy of Fallot before and after corrective surgery. Thirty-five children (mean age: 5.9 years) were examined; 5 of them died immediately after operation; 5 had palliative operations only. The remaining 25 had repeat echocardiography 2 to 4 and/or 8 weeks after total correction. Compared with normal values, preoperative left ventricular dimensions were smaller than expected for body surface area (mean = 85.4% +/- 1.9 SEM, range 65 to 105% of normal); 21 values were below the 5th centile. Postoperatively, left ventricular dimensions increased significantly and reached normal values in most cases (mean = 103.2 +/- 2.0% SEM, range 81 to 121%). The main increase took place in the first 4 weeks (P less than 0.001; mean difference 0.7 +/- 0.14 cm). The 5 children who died after operation had smaller left ventricular dimensions than the survivors (P less than 0.01). Left ventricular function was evaluated by measuring mean circumferential fibre shortening, per cent shortening, and ejection fraction; they were normal in most patients and diminished only insignificantly after corrective surgery. Right ventricular dimensions were increased preoperatively but decreased significantly (P less than 0.001) postoperatively. Septal movement was normal in direction and excessive in displacement in most patients before operation; immediately after operation it became flat or showed paradoxical motion. Two months after operation 50 per cent of the children showed a return to normal septal movement. Early appearance of normal septal movement could be related to the presence of significant pulmonary stenosis. It is concluded that a high percentage of patients with tetralogy of Fallot have underdeveloped but normally functioning left ventricles which adapt well to the new postoperative state.     

Left ventricular torsion and strain in patients with repaired tetralogy of Fallot assessed by speckle tracking imaging

Background: In patients with tetralogy of Fallot (TOF), left ventricular (LV) dysfunction is an important factor associated with poor clinical outcome. Objective: The purpose of this study was to investigate the torsion and multidirectional strain of the LV in patients with TOF. Methods: Echocardiographic images were prospectively acquired in 29 patients who underwent TOF repair (age range, 5–25 years) and in 29 normal controls. Torsion and circumferential and longitudinal strain of the LV were assessed using speckle tracking imaging. Results: The torsion in patients was smaller compared to that in the controls due to small apical rotation and/or inverse basal rotation (P < 0.01). Torsion and untwisting rates decreased with increasing age (R = 0.37, P < 0.05). Basal circumferential strain and strain rate (SR) at systole and diastole decreased with age (R = 0.58; R = 0.57; R = 0.57, all P < 0.001) and were smaller in patients compared to those in the controls (all P < 0.01). Septal longitudinal strain and SR at systole and diastole decreased with age (R = 0.52; R = 0.62; R = 0.71, all P < 0.001) and were smaller than those of the controls (P < 0.01), although lateral longitudinal strain and SR were relatively maintained. Conclusion: Abnormal torsion and strain pattern of the LV were observed in patients without symptoms of cardiac failure. Assessment of torsion and strain is a very sensitive tool to detect the early deterioration of LV function in patients with TOF. (Echocardiography 2011;28:720‐729)     

Echocardiographic study of right and left ventricular dimension and left ventricular function in patients with tetralogy of Fallot before and after surgery.

Right and left ventricular dimensions and function were determined by one-dimensional echocardiography in patients with tetralogy of Fallot before and after corrective surgery. Thirty-five children (mean age: 5.9 years) were examined; 5 of them died immediately after operation; 5 had palliative operations only. The remaining 25 had repeat echocardiography 2 to 4 and/or 8 weeks after total correction. Compared with normal values, preoperative left ventricular dimensions were smaller than expected for body surface area (mean = 85.4% +/- 1.9 SEM, range 65 to 105% of normal); 21 values were below the 5th centile. Postoperatively, left ventricular dimensions increased significantly and reached normal values in most cases (mean = 103.2 +/- 2.0% SEM, range 81 to 121%). The main increase took place in the first 4 weeks (P less than 0.001; mean difference 0.7 +/- 0.14 cm). The 5 children who died after operation had smaller left ventricular dimensions than the survivors (P less than 0.01). Left ventricular function was evaluated by measuring mean circumferential fibre shortening, per cent shortening, and ejection fraction; they were normal in most patients and diminished only insignificantly after corrective surgery. Right ventricular dimensions were increased preoperatively but decreased significantly (P less than 0.001) postoperatively. Septal movement was normal in direction and excessive in displacement in most patients before operation; immediately after operation it became flat or showed paradoxical motion. Two months after operation 50 per cent of the children showed a return to normal septal movement. Early appearance of normal septal movement could be related to the presence of significant pulmonary stenosis. It is concluded that a high percentage of patients with tetralogy of Fallot have underdeveloped but normally functioning left ventricles which adapt well to the new postoperative state.     

Right ventricular function in children with tetralogy of Fallot before and after aortic-to-pulmonary shunt.

Right and left ventricular volume variables were obtained in 43 tetralogy patients undergoing diagnostic cardiac catheterization. The patient population consisted of 25 preoperative patients (group 1) and 18 patients who had undergone aortic-to-pulmonary shunt procedure (group 2). Volumes were calculated from biplane cineangiocardiograms using Simpson's rule method for the right ventricle (RV) and the area-length methods for the left ventricle (LV). In group 1, RV end-diastolic volume (RVEDV) was not different from normal in the total group and averaged 93 +/- 4% (SEM) of normal. In patients with hemoglobin (Hgb) greater than or equal to 16 g%, however, this variable was significantly (P = 0.044) less than normal. Right ventricular ejection fraction was normal and RV systolic index was significantly (P less than 0.001) reduced, averaging 3.35 +/- 0.18 (SEM) L/min/m2. Left ventricular volume variables in this group were not significantly different from RV volume variables. In group 2, RVEDV in patients with Hgb greater than or equal to 16 g% was significantly (P = 0.037) less than normal, but was normal in patients with Hgb less than 16 g%. Right ventricular ejection fraction averaged 0.52 +/- 0.03 in this group and was significantly (P less than 0.001) less than normal. Right ventricular systolic index (RVSI) averaged 3.51 +/- 0.24 L/min/m2 and was significantly (P = 0.009) less than normal. RVSI in patients with Hgb less than 16 g% averaged 3.90 +/- 0.31 and was not different from normal. In contrast, this variable in patients with Hgb greater than or equal to 16 g% averaged 3.21 +/- 0.34 and was significantly (P = 0.005) less than normal. Left ventricular end-diastolic volume (LVEDV) and LV systolic output in group 2 were significantly higher than RVEDV and RV systolic output. Right ventricular and LV ejection fractions in group 2 were not different. The relatively decreased ejection fraction fraction in tetralogy patients, as compared with patients with valvular pulmonic stenosis and similar volumes and pressures, suggests that the decreased ejection fraction was not due to decreased preload or increased afterload and might be due to impaired ventricular function secondary to chronic hypoxia. Early corrective surgery in these patients might reverse this process. However, patients with severe tetralogy who have small ventricular volume and reduced output might benefit from shunt procedure rather than complete correction.     

Echocardiographic analysis of regional left ventricular wall motion in normal children and neonates

Because comprehensive normal data and the effect of observer variability for echocardiographic evaluation of regional left ventricular wall motion are not available in children or newborns, left ventricular wall motion was assessed by measuring regional area change. The study group comprised 55 infants and children with a normal heart: 15 neonates (greater than 1 week to less than 1 month old), 10 infants (greater than 1 month to less than 1 year old) and 30 children, 10 each in the age group greater than 1 year to less than 5 years, greater than 5 to less than or equal to 10 years and greater than 10 years. A combination of parasternal, apical and subcostal two-dimensional echocardiographic views was applied. After planimetry of an end-systolic and end-diastolic frame, the left ventricle was divided into eight equal segments and the percent area change calculated. Both a fixed reference and a floating system correcting for translation and rotation were applied. Intraobserver variability for percent area change measurements was 2.8 +/- 0.9% and 3.8 +/- 1% for observers 1 and 2, respectively. The mean interobserver difference of regional percent area change was 4.7 +/- 1.8%. Normal values for the eight anatomic segments were established in each echocardiographic imaging plane. The overall results were independent of the type of reference system utilized. The subcostal views yielded different results from their parasternal counterparts, probably because of differing imaging planes. These normal values establish a data base in the pediatric age range that can be used to detect abnormal segments in children at risk for developing regional left ventricular wall motion abnormalities.     

Global left ventricular function and regional wall motion in pure mitral stenosis-Left ventricular function in pure mitral stenosis

Global left ventricular function (LVF) and scgmental wall motion of the left ventricle are registered in 113 patients presenting a pure mitral stenosis (MS) and in a control group of 50 individuals. The segmental wall motion is measured on the end-diastolic-end-systolic frames of the left ventricle, obtained from right anterior oblique (RAO) monoplane cineangiography. Measurement of the segmental wall shortening is performed using the Stanford method. Group 1 includes 68 patients (60% of the total number of patients studied). These patients show no pathological contraction abnormality. In this group, the global LVF is not different from the control group. Group 2 includes 45 patients (40% of the total) for whom contraction abnormalities are present: anterior hypokinesis in 20% of the cases (anterior area mean shortening (AAS) = 18±8%; p<0.001 vs. group 1 and control group), and posterior hypokinesis in 20% of the cases (posterior area mean shortening (PAS) = 9.8 ±5.8%, p<0.001 vs. group 1 and control group). In this group, global LVF is impaired; ejection fraction (EF) = 0.57±0.1% (p<0.001 vs. group 1); velocity of circumferential fiber shortening (Vcf) = l±0.3 circ/s (p<0.001 vs. group 1); end-diastolic pressure (EDP) = 11±5 mmHg (p<0.01 vs. group 1). Segmental contraction abnormalities appear to be the main factor involved in the global LVF impairment. Segmental wall motion abnormalities could be related to subvalvular fibrosis, or LV filling difficulties, or principally, to a possible interplay between the right and the left ventricles.     

Temporal pattern of regional left ventricular wall motion in patients with segmental early relaxation

The purpose of this investigation was to describe the temporal pattern of regional left ventricular (LV) wall motion in patients with segmental early relaxation (SER). Segmental early relaxation was demonstrated angiographically in seven patients undergoing diagnostic cardiac catheterization because of chest pain. In all patients, early relaxation of only the anterolateral wall was observed. The temporal pattern of regional wall motion was assessed from ventriculograms, obtained in the right anterior oblique projection, by measuring the lengths of the anterolateral minor axis chord and the corresponding diaphragmatic minor axis chord. The onset of lengthening of the anterolateral chord preceded that of the diaphragmatic chord by 110 ± 10 msec. Maximal shortening of the anterolateral chord during systole was 43 ± 4% and was coincident with a 32 ± 4% shortening of the diaphragmatic chord. During early relaxation, shortening of the anterolateral chord diminished to 17 ± 4% (P <.01); and was coincident with a 35 ± 4% shortening of the diaphragmatic chord (P <.05). Early relaxation of the anterolateral wall was followed by a transient augmentation of shortening of the anterolateral chord to 29 ± 3% (P <.001), whereas shortening of the diaphragmatic chord diminished to 32 ± 4%. Subsequently both chords returned to their initial end-diastolic lengths. These preliminary observations indicate that early lengthening of the anterolateral segment is followed by a transient rebound of shortening which was only present in the segment that manifested early relaxation.     

法乐氏四联症患者左心功能有创、无创的对比研究

对２０例法乐氏四联症（ＴＯＦ）患者，行超声心动图、左室电影造影对比研究。超声胸骨旁长轴切面和剑下四腔切面的双面法与造影相关性最好（ｒ．ＬＶＥＤＶ＝０．９２３）；胸骨旁长轴切面与造影相关系数为ｒＬＶＥＤＶ＝０．９２１；超声结果均小于造影结果。ＴＯＦ的ＬＶＥＤＶＩ明显小于正常（Ｐ＜０．００１），ＥＦ无明显差别。ＥＦ与术前ＬＹＥＤＶＩ呈正相关（ｒ＝０．４６，Ｐ＜０．０５）；与术后血压呈正相关（ｒ＝０．４９．Ｐ＜０．０５）。     

Factors affecting left ventricular function after correction of tetralogy of Fallot.

To investigate the possible causes of left ventricular dysfunction after total correction of tetralogy of Fallot, 84 patients, aged 1 1/2 to 16 years, were studied by left ventricular cineangiography both before and a mean of 4.6 months after operation. Left ventricular ejection fraction and mean velocity of circumferential fibre shortening were calculated; using multivariate analysis the results were correlated with age at operation, the degree of hypoxia and polycythaemia before operation, occurrence of hypoxic spells, and the duration of operative procedures (cardiopulmonary bypass and aortic cross clamping). The postoperative left ventricular ejection fraction was decreased slightly or moderately in 46% of patients. The variable most significantly associated with altered left ventricular function was a history of hypoxic spells. Age, the degree of chronic hypoxia, and polycythaemia did not correlate significantly with left ventricular function indices. Although no correlation was found between the duration of cardiopulmonary bypass and left ventricular ejection fraction, bypass times exceeding 120 minutes were associated with decreased ejection fractions; this was statistically significant and independent of the variable "hypoxic spells". Thus repeated episodes of acute hypoxia and long operative procedures appear to have a deleterious effect on left ventricular function in tetralogy of Fallot.     

Analysis of left ventricular regional wall motion in normal neonates

Objective—To investigate neonatal circulatory change by quantitative analysis of left ventricular regional wall motion.Design—Random prospective study.Setting—Department of paediatrics in a teaching hospital.Participants—66 neonates born after a normal pregnancy, labour, and delivery.Interventions—Quantitative analysis of left ventricular regional wall motion was performed on cross sectional echocardiograms. M mode, cross sectional, and Doppler echocardiograms were obtained simultaneously.Main outcome measures—Manually traced endocardial contours at end diastole and at end systole were realigned by superimposing the centre of the ventricular mass and the axis. The contours were divided into 24 segments with 24 radii of equal arc from the centre. Then the ratio of the change in area between the outline of the contour and the two hemiaxes was calculated automatically.Results—There was hyperkinesis of the interventricular septum in the first 24 hours after birth which continued until the end of the first week. Simultaneous echocardiographic examination showed evidence of pulmonary hypertension, as indicated by an increase in the ratio of the right pre-ejection period to the right ventricular ejection time (RPEP/RVET) and of the diameter ratio of the pulmonary artery to the aorta and a shortening of the acceleration time of pulmonary arterial blood flow. These features disappeared within a week.Conclusions—Hyperkinesis of the interventricular septum may reflect circulatory changes that are characteristic of the early neonatal period.     

Development of left ventricular muscle in tetralogy of Fallot

Age-related changes of the muscle fiber diameter of the left ventricle were studied in 20 autopsied specimens of tetralogy of Fallot (TOF) and in 34 normal autopsied hearts. According to the amount of pulmonary blood flow, two groups were defined: In Group 1, TOF with a markedly decreased pulmonary blood flow. In Group 2, TOF with a moderately decreased pulmonary blood flow. In Group 1, the muscle fiber diameter was nearly normal at birth, but increased slightly and remained constant after one year, so that it was significantly less than normal. Moreover, histological observation revealed underdevelopment of the left ventricular muscle fibers in this group. In Group 2, the diameter increased with age in a manner similar to normal and, histologically, the muscle fibers were not different from normal ones except for those in one heart with left ventricular hypertrophy. The growth of muscle fibers in the left ventricle seems to depend on the volume of pulmonary blood flow, or the left ventricular inflow volume. If the left ventricular functional capacity correlates with the growth of muscle fibers, left ventricular dysfunction after the corrective operation may originate in the underdevelopment of muscle fibers. Surgical intervention is advisable before the retardation of muscle fiber growth has become apparent.     

Echocardiographic quantification of regional left ventricular wall motion with color kinesis

Echocardiographic assessment of regional systolic left ventricular function is usually performed qualitatively and depends on investigator experience. In this study, we investigated a new method for quantifying regional systolic wall motion based on color kinesis. In this study, regional systolic wall motion velocity (Vsys) was determined by dividing end-systolic color width by systolic time. High regional wall motion velocity (Vhigh) was determined by dividing the width of the widest color by its duration of 40 ms. First, in vitro measurements with an acrylic glass model were obtained; these demonstrated a high correlation between echocardiographically determined and real "wall motion velocities" (R = 0.99, p<0.001, R2 = 0.99). Then, 17 healthy, young persons were examined, and normal values for each left ventricular wall segment (16-segment model) were determined. The mean Vsys and Vhigh of all 272 wall segments were 2.3+/-0.6 and 7.4+/-1.8 cm/s, respectively. Finally, in 12 patients with coronary artery disease and prior myocardial infarction, Vsys and Vhigh of each left ventricular wall segment were determined and compared with conventional echocardiographic wall motion analysis using the usual 4-grade score system. Analysis of data showed that quantitative color kinesis measurements demonstrated significantly lower velocity values in pathologic than in normal wall segments (Mann-Whitney U test, p<0.05). Measurements discriminated between pathologic and normal wall motion, with an accuracy of 89% for Vsys and 83% for Vhigh (chi-square test, p<0.05). To summarize, in this first study, measurements of regional wall motion velocities with color kinesis demonstrated reliable results for the quantification of regional left ventricular systolic function.