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Background Patients with repaired aortic coarctation (CoA) are prone to develop early left ventricular (LV) and left atrial (LA) dysfunction due to chronic abnormal LV afterload. Clinical and prognostic relevance of LA function, including LA strain analysis, is increasingly recognized particularly for predicting incident atrial fibrillation and stroke. This study examined the prevalence of left atrial dysfunction (LAD) in patients with corrected CoA and the relationship between LA function and patient characteristics. Methods 56 patients (34 males, age: 31 ± 16 years) who underwent CoA repair (46 post ‘end-to-end anastomosis/sub-clavian flap’, 10 post ‘stenting/bypass/Teflon patch’) and 56 controls were studied. 2D strain imaging was performed to assess left ventricular (LV) and LA functions including peak positive LA strain (LAS), early diastolic and late diastolic LA strains, global longitudinal LV strain (LV-GLS), and circumferential strain (LV-GCS). LAD was defined as LAS value lower than the mean value of the control group minus 2 SDs. Results LAS, early and late diastolic LA strains, and LV-GLS were significantly lower in the CoA group while LV-GCS did not differ. No significant correlation was found between LAS and either current age, age at initial repair or blood pressure values. Among echocardiographic parameters, Ea and LV-GLS were moderately correlated to LAS (r = 0.49, p Conclusions Based on LAS analysis, LAD is prevalent after CoA repair. Post-operative aortic arch anatomy may impact peak positive LA strain. LA strain may help screening high-risk patients for atrial arrhythmia and stroke after CoA repair.

Severe aortic stenosis (AS) causes left ventricular (LV) afterload and subendocardial ischemia. Despite this, most infants with AS have normal LV ejection fraction (EF). Strain analysis using two-dimensional speckle tracking echocardiography (2DSTE) may identify more sensitive markers of systolic dysfunction. We sought to show changes in LV strain after balloon aortic valvuloplasty (BAV) in infants with AS. Twenty-seven infants ≤ 1 year of age with AS who underwent BAV from 2007 to 2017 were included. Echocardiograms before/after BAV were retrospectively analyzed with 2DSTE. Median age was 29 days (interquartile range 3–52) and LV EF was 64 ± 10%. Global longitudinal strain (GLS) significantly improved post-BAV (− 17 ± 5 vs. − 20 ± 4%, p = 0.001) with no difference in global circumferential strain. Peak longitudinal strain was abnormal at the inferoseptal base and mid-ventricle (− 15 ± 6 and − 17 ± 5 = 7%, respectively) and significantly improved in the basal and mid-anterolateral segments (− 17 ± 5 vs. − 21 ± 5%, p

Systemic ventricular end-diastolic pressure (SVEDP) is an important determinant of pulmonary artery pressure in those with a Fontan circulation. Predictors of an elevated SVEDP have been incompletely identified in this population. All who underwent the Fontan operation at our center between 1/2009 and 12/2013 were retrospectively identified. SVEDP at the pre-Fontan catheterization and other patient variables were extracted. We identified 61 patients. Pre-Fontan SVEDP was positively associated with systemic ventricular systolic pressure (β = 0.4, p = 0.004), aortic systolic pressure (β = 0.3, p = 0.007), aortic mean pressure (β = 0.3, p = 0.02), and decreased ventricular systolic function (p = 0.03). Compared to those with pre-Fontan SVEDP ≤ 7 mmHg, patients with SVEDP > 7 mmHg had higher average ventricular systolic pressure (85.0 ± 7.5 vs. 78.7 ± 8.3 mmHg, p = 0.003), higher average descending aorta mean pressure (62.4 ± 4.9 vs. 58.6 ± 8.1 mmHg, p = 0.03), and a higher incidence of decreased ventricular systolic function (36 vs. 15%, p = 0.07). For those with a systemic right ventricle, the SVEDP decreased significantly from the pre-Stage 2 to pre-Fontan measurements (8.7 ± 2.6 vs. 7.3 ± 2.0 mmHg, p = 0.02), but not for those with a systemic left ventricle (7.8 ± 2.0 vs. 7.2 ± 1.8 mmHg, p = 0.3). At pre-Fontan catheterization, decreased ventricular systolic function and markers of systemic afterload were positively associated with the SVEDP. SVEDP decreased significantly after Stage 2 for those with a systemic right ventricle, but not for those with a systemic left ventricle; the systemic right ventricle may be particularly vulnerable to pre-Stage 2 volume loading.

Right ventricular (RV) failure is a significant cause of morbidity and mortality in patients with pulmonary hypertension (PH). Myocardial performance index measured by tissue Doppler imaging (TDI-MPI) has been useful in assessing RV dysfunction in adults with PH. However, TDI-MPI as a marker for RV dysfunction or disease severity has not been evaluated in pediatric PH. The aim of this study was to investigate TDI-MPI and correlate with invasive hemodynamics in pediatric PH patients. Eighty pediatric PH patients undergoing cardiac catheterization and simultaneous transthoracic echocardiography were analyzed. RV TDI-MPI was averaged over three cardiac cycles and measured under each condition of vasodilatory testing during the catheterization. TDI-MPI was compared between PH patients and age-matched controls and correlated to invasive hemodynamics. RV TDI-MPI was increased in PH patients compared to controls (0.49 vs. 0.35, p

The purpose of the study is to analyze the impact of hybrid stage 1 palliation on right ventricular myocardial pathology in hypoplastic left heart syndrome. Sufficient amount of right ventricular biopsies could be obtained from 16 of 32 patients who underwent Norwood operation between 2007 and 2013. Histopathological findings of right ventricle in patients who underwent primary Norwood operation (primary group, n = 5), patients with aortic atresia (HS1P AA group, n = 6) or aortic stenosis (HS1P AS group, n = 5) who underwent staged Norwood palliation following hybrid stage 1 palliation were compared. To eliminate the influence of right ventricular pressure afterload, right ventricular biopsies were obtained from patients with truncus arteriosus communis (TAC group, n = 6) at total correction. The percentage of myocardial fibrosis was significantly higher in both HS1P groups than in TAC group; moreover, it was significantly higher in HS1P AA group than in primary group. Capillary vascular density was significantly lower in all hypoplastic left heart syndrome groups than in TAC group. At the sub-endocardial layer, collagen type I/III ratios were higher in HS1P AA group than in other hypoplastic left heart syndrome groups. The proportions of N-cadherin immunolocalized to myocyte termini were lower in all hypoplastic left heart syndrome groups than in TAC group. Right ventricle in hypoplastic left heart syndrome showed more significant ischemic change and myocardial immaturity than that in truncus arteriosus communis. Hybrid stage 1 palliation for aortic atresia would be a risk factor for further right ventricular myocardial ischemia.

Children born with a functional single ventricle who undergo Fontan palliation are prone to early pump failure. Whether they develop early arterial stiffness with resultant increase in afterload is not well known. We hypothesized that the aortic stiffness is higher in pediatric Fontan patients when compared to healthy controls. A prospective study was conducted at the Children's Hospital of Michigan. Twenty-two Fontan patients (aged 6-21 years) were compared with 22 healthy controls (aged 9-17 years) selected from children referred to our clinic who had normal cardiac anatomy and function on the echocardiogram. Aortic stiffness was assessed noninvasively by measuring the aortic augmentation index (AAI) using applanation tonometry (Sphygmocor, Atcor, IL). AAI was calculated as AP/PP where augmentation pressure (AP) is the increase in aortic systolic blood pressure (BP) and pulse pressure (PP) is the difference between aortic systolic and diastolic BP. Ten patients (45 %) had hypoplastic left ventricle, and 11 (50 %) had undergone aortic arch surgery. The median AAI was significantly higher in Fontan patients when compared to controls (12.5, IQR 4.8, 17.3 vs 0, IQR -6.3, 5.8; p = 0.0003). History of aortic arch surgery and single ventricle morphology did not have a significant impact on AAI. Pediatric patients who undergo Fontan palliation have significantly higher AAI, a marker of aortic stiffness and increased afterload, compared to healthy controls. Larger longitudinal studies are warranted to elucidate the possible contribution of elevated AAI on pump failure in these patients.

This study aimed to clarify the relationship between onset of protein-losing enteropathy (PLE) and Fontan circulation, with special reference to the development of contractility–afterload mismatch. The PLE group comprised 9 patients who experienced PLE after undergoing the Fontan operation, and the control group consisted of 32 patients had did not experienced PLE more than 10 years after the Fontan operation. The study compared the pre- and postoperative values of arterial elastance (Ea), end-systolic elastance (Ees), and contractility–afterload mismatch (Ea/Ees). Furthermore, the variations in the values were examined during the preoperative, postoperative, and midterm postoperative periods in seven PLE patients who underwent cardiac catheterization at the onset of PLE and during the pre- and postintervention periods in three PLE patients who underwent surgical intervention to improve the Fontan circulation after the onset of PLE. Comparison of the values obtained before and after Fontan operations showed that the Ea values increased significantly in the PLE group. However, the pre- and postoperative Ees values did not differ in the two groups. During the postoperative period, Ea/Ees increased significantly, and the Ea and Ea/Ees values increased continuously until the onset of PLE in the PLE group. In the patients who underwent surgical intervention to improve the Fontan circulation after the onset of PLE, the Ea/Ees decreased significantly, and the serum albumin levels improved after the intervention. Contractility–afterload mismatch, mainly caused by the increase in the afterload of the systemic ventricle, may have an important role in the development of PLE after the Fontan operation.

The prevalence of obesity continues to increase in the developing world. The effects of obesity on the cardiovascular system include changes in systolic and diastolic function. More recently obesity has been linked with impairment of longitudinal myocardial deformation properties in children. We sought to determine the effect of increased body mass index (BMI) on cardiac deformation in a group of children taking part in the population-based Southampton Women’s Survey to detect early cardiovascular changes associated with increasing BMI before established obesity. Sixty-eight children at a mean age of 9.4 years old underwent assessment of longitudinal myocardial deformation in the basal septal segment of the left ventricle (LV) using two-dimensional speckle tracking echocardiography. Parameters of afterload and preload, which may influence deformation, were determined from cardiac magnetic resonance imaging. BMI was determined from the child’s height and weight at the time of echocardiogram. Greater pediatric BMI was associated with greater longitudinal myocardial deformation or strain in the basal septal segment of the LV (β = 1.6, p

WOS: 000324638500019, PubMed ID: 23591803, This study investigated cardiac function in 65 fetuses of mildly preeclamptic mothers and 55 fetuses of healthy mothers at 26-40 weeks of gestation. Fetuses with intrauterine growth restriction were excluded. Cardiac functions were evaluated by M-mode, pulsed-wave, and tissue Doppler echocardiography. The two groups were similar in terms of maternal age, gravidity, parity, and gestational age. Peak systolic aortic and pulmonary artery velocities were significantly lower in the fetuses of the preeclamptic mothers than in the fetuses of the healthy mothers. The two groups did not differ significantly in terms of shortening fraction or with regard to mitral or tricuspid annular plane systolic excursion. Pulsed-wave Doppler-derived E/A ratios in the mitral and tricuspid valves were similar in the two groups. The deceleration time of early mitral inflow was prolonged in the fetuses of the preeclamptic mothers. The Ea, Aa, and Ea/Aa ratios in the interventricular septum, left ventricle posterior wall, and right ventricle free wall were lower in the preeclampsia group than in the control group. The E/Ea ratio was higher in the preeclampsia group than in the control group. The isovolumic relaxation time and the right and left myocardial performance indices were higher in the fetuses of the preeclamptic mothers than in the fetuses of the healthy mothers. An increased ductus venosus pulsatility index (PI) and a decreased middle cerebral artery (MCA) PI were found in the fetuses of the preeclamptic mothers. All the fetuses were asymptomatic. The results suggest that the increase in fetal cardiac afterload in mild preeclampsia may have caused early subclinical changes in fetal systolic and diastolic cardiac function. In addition, the decrease in MCA-PI may have been caused by redistribution of fetal cardiac output in favor of the left ventricle, secondary to increased placental vascular resistance.

The objective of this study was to investigate mechanisms of exercise-induced pulmonary hypertension in patients with congenital cardiac septal defects. This was a randomized, placebo controlled, crossover drug trial in a single national pediatric cardiology centre that performs congenital cardiac defect surgery. There were 14 patients with cardiac septal defects and known exercise-induced pulmonary hypertension. The intervention consisted of 50 mg oral sildenafil versus placebo. Measurements included supine bicycle exercise echocardiography and oxygen uptake. The outcome measure was right-ventricular systolic pressure as estimated by Doppler tracings of tricuspid regurgitant jet as well as systolic and diastolic longitudinal myocardial velocities by color tissue Doppler echocardiography. Sildenafil did not change exercise right-ventricular systolic pressure during exercise; however, decreased systemic systolic pressure was seen. Enhanced biventricular longitudinal function with sildenafil compared with placebo was indicated by greater tissue Doppler velocities and displacement measurements during exercise. Finally, a less steep increase of right-ventricular pressure during exercise was associated with greater left-ventricular diastolic myocardial tissue Doppler velocity. Exercise-induced pulmonary hypertension in cardiac septal defects does not seem to have a pulmonary vasoconstrictive component, but it may be related to left-ventricular filling pressure. Furthermore, sildenafil improved biventricular systolic performance in this patient group, possibly related to decreased systemic afterload.

This prospective observational study conducted in a neonatal intensive care unit aimed to evaluate echocardiographic changes provoked by anemia and transfusion of packed red blood cells (pRBCs) in premature infants. In this study, 32 anemic premature infants had serial echocardiographic assessment of left ventricular (LV) systolic performance, LV preload, and afterload immediately before, within 48 h, and up to 120 h after the transfusion of pRBCs. Pretransfusional evaluations also were compared with similar assessments of 71 nonanemic inpatient premature infants analogous for sex, gestational age at birth, and postnatal age. Left ventricular systolic performance was estimated from fractional shortening, LV output, and LV myocardial performance index (LVMPI). The LV preload was estimated from the LV end-diastolic dimension and the ratio of left atrium-to-aortic root dimension (LA/Ao ratio). The LV afterload was estimated from end-systolic wall stress. The LVMPI was found to decrease with increasing corrected gestational age in both the nonanemic (R = 0.173; p = 0.03) and anemic (R = 0.460; p = 0.007) infants. The LVMPI was the only index that changed after transfusion of pRBCs, decreasing in the younger anemic infants (p = 0.011) and increasing in the older anemic infants (p = 0.012). Finally, a significant inverse relationship between pre- and posttransfusional LVMPI values (R = 0.730; p < 0.001) was noted. The LVMPI may allow for identification of preterm infants more likely to be helped by transfusion of pRBCs.

We report three patients with partial atrioventricular septal defect who presented in the neonatal period with excessive left to right shunting and progressive pulmonary hypertension. Successful biventricular repair was accomplished despite the fact that left ventricular area and left sided structures did not meet the criteria established previously for biventricular management in children with critical aortic stenosis or complete atrioventricular septal defect. Indication for biventricular management was based on the fact that none of our patients had morphological mitral or aortic stenosis and that the hypoplastic left ventricle proved capable to maintain the systemic circulation following closure of the arterial duct. Fenestrated closure of the atrial septum and an individualized approach regarding the closure of the cleft in the left sided atrioventricular valve appear to be important surgical options to reduce postoperative left atrial pressure and to allow the ventricles to adapt to the new loading conditions.

A female born with aortic atresia, large ventricular septal defect, normal mitral valve, and left ventricle is well at 21 years of age following biventricular repair. She had palliative surgery at 15 days and closure of ventricular septal defect with placement of a valved conduit from the left ventricular apex to descending aorta at 15 months. Conduit was replaced at 34 months and at 10 and 21.5 years of age.

Patients with intraatrial baffle procedure for transposition of the great arteries (TGA) have diastolic dysfunction, decreased exercise capacity, stroke volume response and elevated systemic vascular resistance (SVR) during exercise. Angiotensin-converting enzyme (ACE) inhibitors improve exercise capacity in adults with congestive heart failure by improving diastolic function and decreasing SVR. We tested the hypothesis that ACE inhibitors decrease SVR and improve exercise capacity in patients after intraatrial baffle procedure for TGA. We studied the effects of enalapril in nine patients with TGA s/p intraatrial switch (mean age, 13.8 +/- 3 years) 7 to 21 years (mean, 12 +/- 4 years) after intraatrial baffle procedure. Enalapril (0.5 mg/kg/day, maximum dosage 20 mg bid) was administered for 12 months. Patients exercised using a cycle ergometer ramp protocol (0.25 W/kg/min) before enalapril (baseline), 1 month, 6 months, and 12 months after treatment initiation. Heart rate, blood pressure, cardiac output, respiratory rate, minute ventilation, oxygen consumption (VO2), total exercise time, work, and power were measured. SVR, cardiac index, and stroke volume index (SVI) were calculated. Two-tailed paired Student's t-test was used to compare data to those of normal control patients and the patients' baseline data. Patients had lower resting heart rate, cardiac index, maximum heart rate, cardiac index (CI), SVI, VO2, exercise time, work, and power and higher maximal SVR at baseline compared to normal control patients. There was no significant difference in total exercise time, work, power, VO2 (rest/peak), SVR, SVI, and CI after 12 months of therapy compared to patients' baseline values. We conclude that short-term (1 year) use of enalapril does not improve exercise performance in patients with TGA in whom the intraatrial baffle procedure has been performed.

Pulsus alternans was observed in a child with left ventricular dysfunction secondary to systemic hypertension. The Echo-Doppler of supravalvular aorta and a simultaneous electrocardiographic trace (ECG) clearly demonstrated the mechanism of an apparent abrupt halving of pulse frequency compared with the ECG, similar to an electromechanical dissociation 2: 1. Besides the extreme pulsus alternans, a P wave and a PP cycle length change were also observed. Both mechanical and electrical alternans disappeared with afterload reduction by nitroprusside treatment.

In adult patients with severe chronic mitral regurgitation, many predictors for estimating postoperative left ventricular systolic function have been proposed. However, none has been defined in children. We analyzed the relationship between such predictors and postoperative left ventricular fractional shortening in children with isolated congenital mitral regurgitation. Eight patients with isolated congenital mitral regurgitation were examined before and after surgery with echocardiography. Fractional shortening, left ventricular end-diastolic and end-systolic dimension indices, and left ventricular end-systolic wall stress/left ventricular end-systolic dimension index in the preoperative status were poorly correlated with postoperative left ventricular fractional shortening. Predictive wall stress, which was calculated from preoperative data of end-diastolic dimension, end-diastolic wall thickness, and diastolic blood pressure, correlated well with postoperative left ventricular fractional shortening (r = -0.90, p = 0.0024). It is important to consider not only myocardial contractility but also postoperative afterload for estimating postoperative left ventricular performance in chronic severe mitral regurgitation.

Cases of hypoplastic left heart syndrome (HLHS) were studied angiographically in 18 patients and pathologically in 22 patients. They were divided into three subgroups according to the morphological features of the left heart: mitral atresia with aortic atresia (MA/AA), mitral stenosis with aortic atresia (MS/AA) and mitral stenosis with severe aortic stenosis (MS/AS). Patients with MS/AA had a significantly lower right ventricular end-diastolic volume index and more hypokinesis of the right ventricular posterior wall than those with MA/AA. MS/AA not only increased the thickness of the left ventricular posterior wall and interventricular septum but also increased endocardial thickness compared with MA/AA. Myocardial histology revealed more frequent abnormal findings such as myocardial necrosis, calcification and interstitial fibrosis in the mitral stenosis groups (i.e., MS/AA and MS/AS) than in MA/AA. Right ventricular function appeared to be greatly influenced by left heart structure. The presence of larger left ventricular muscle bulk and frequent myocardial damage seen in MS/AA seems disadvantageous to right ventricular end-diastolic volume and right ventricular wall motion.

Atrioventricular valve regurgitation (AVVR) is a clinically important element of the common atrioventricular canal defect. Cardiac preload and afterload increase from prenatal to postnatal life. These hemodynamic changes may increase the degree of regurgitation and affect management and prognosis. We sought to investigate the frequency of change in degree of AVVR from fetal to postnatal life in this patient population. Subjects who underwent both fetal and postnatal echocardiography within 4 weeks of life between January 2008 and September 2010 were included in the study. Degree of AVVR was assessed by color Doppler imaging and scored as 0 (no regurgitation), 1 (hemodynamically insignificant regurgitation), and 2 (hemodynamically important regurgitation). Forty-nine subjects were included. Mean gestational age at fetal echocardiogram was 34 ± 2.8 weeks; age at postnatal echocardiogram was a median of

The physiologic impact of aortic valve stenosis is most directly reflected by an increased workload placed on the ventricle. In the pediatric population the morphology of aortic stenosis varies considerably. Fluid dynamic principles suggest that different morphologies may require the ventricle to accelerate blood to different maximal velocities for constant cardiac outputs and valve areas, resulting in different ventricular workloads. This study examined this important concept in in vitro models designed to isolate the effect of valve geometry on distal velocity, pressure gradients, and proximal work. Four stenotic valve morphologies were examined using a variable-voltage pump system. For constant orifice areas and flows, markedly different workloads were required by the pump, and this difference was reflected in direct measurements of pressure gradient and Doppler predictions of gradient. These fundamental fluid dynamic studies isolate the relationship between flow, work, and stenotic valve morphology. Different orifice geometries affect the value of the coefficient of contraction, which is reflected in different maximum velocity values for stenosis with constant anatomic areas and flows. The proximal pumping chamber must generate different levels of force to achieve these different velocities, and this variability is reflected in the clinically measured pressure gradient.

To determine whether diastolic ventricular interdependence mechanisms would act in the presence of an open pericardial sac, as during cardiac surgery, moderate acute right ventricle afterload increases were applied to eight dogs with the chest and pericardium open while left ventricular filling dynamics were being assessed by Doppler echocardiography. Dogs were studied under basal conditions and after acute banding of the main pulmonary artery tightened to produce a 100% increase in right ventricular systolic pressure. With banding, the left ventricular filling velocity ratio (E/A), as assessed by Doppler echocardiography of mitral inflow, changed from a baseline value of 1.32 +/- 0.05 to 1.16 +/- 0.03 (p0.02), suggesting a restrictive pattern to early left ventricular filling, which is differed to that during the second half of diastole. Isovolumic relaxation time, measured as the time interval between aortic valve closure and mitral valve opening, assessed by M-mode echocardiography of both valves, was prolonged, though not significantly, from 63.3 +/- 2.5 ms to 69.4 +/- 2.9 ms, by banding of the pulmonary artery. E wave deceleration time, a filling variable influenced by chamber pressure/volume relations, was shortened by pulmonary artery banding, changing from 75.1 +/- 1.7 ms to 68.0 +/- 1.8 ms (p0.01). It was concluded that pressure loads applied to the right ventricle restricted early left ventricular filling. Prolonged relaxation and altered pressure-volume chamber relations were the diastolic interdependence mechanisms involved that proved to be acting even under open pericardium conditions.

We report the use of nesiritide in two infants following cardiac surgery. Both infants had increased intracardiac filling pressures postoperatively, despite traditional afterload reduction and diuretics. Both infants demonstrated clinical improvement with nesiritide therapy. There is currently limited data available describing the use of nesiritide in infants.

To determine the correlation between electrocardiographic (ECG) findings and anatomy utilizing echocardiography in children with pressure- or volume-loaded left ventricles, we analyzed the preoperative ECG tracings of 19 patients who underwent surgery for significant aortic stenosis and 12 patients who underwent cardiac catheterization or surgery for clinically significant ventricular septal defects. We then compared them with a group of 21 normal controls. The left ventricular muscle mass in these patients was calculated from echocardiograms using the simplified cubed formula. Posterior and septal wall thickness and cavity size were significantly greater in the aortic stenosis group than in the normal group. Only cavity size was significantly greater in the ventricular septal defect group than in the normal group. Eighteen aortic stenosis patients (95%) and ten ventricular septal defect patients (83%) had a left ventricular muscle mass greater than 2 standard deviations above the mean for the normal group. Significant differences were found in the voltages of SV1 + RV6 and in the voltage of RV6 alone between normals, aortic stenosis patients, and ventricular septal defect patients regardless of age. Using conventional ECG criteria for left ventricular hypertrophy, the highest sensitivity in aortic stenosis patients (67%) and ventricular septal defect patients (60%) was modest. The likelihood ratio for a positive test in either group was the best for SV1 + RV698th centile for age; RV698th centile for age was the best single measurement. No correlation was found between voltage and any measurable hemodynamic or anatomic data. Conventional pediatric ECG criteria for left ventricular hypertrophy have only modest sensitivity regardless of whether the heart is under pressure or volume load. Because left ventricular muscle mass can be precisely determined by echocardiography, these ECG criteria should be applied cautiously.

Two-dimensional (2D) speckle tracking (2DST) is a new technique independent of ventricular geometry but not independent of preload and afterload. Using 2DST, this study aimed to investigate differences in right ventricular (RV) function and intraventricular dyssynchrony in patients with hypoplastic left heart syndrome (HLHS) before and after preload-reducing stage 2 palliation. For 31 HLHS patients, this study compared global longitudinal strain (S) and strain rate (SR) as well as regional peak systolic longitudinal S, SR, and velocity (V) in six RV segments on echocardiograms before and after stage 2 surgery. Intraventricular dyssynchrony was assessed by calculating the standard deviation of the intervals from the beginning of systole to peak S, SR, and V. Global S (-16.7 ± 5.0 vs -15.6 ± 5.5%) and global SR (-1.2 ± 0.3 vs -1.2 ± 0.3 s(-1)) did not change after surgery. After surgery, V decreased in the mid lateral segment (2.3 ± 1.3 vs 1.7 ± 0.9 cm/s; p = 0.01) and the basal lateral segment (3.6 ± 1.1 vs 2.8 ± 1.0 cm/s; p = 0.001), whereas S was lower in both of these segments (-19.9% ± 6.0% vs -17.4% ± 6.3%; p = 0.01 and 20.0 ± 5.1 vs 15.8 ± 7.1%; p = 0.002, respectively). Segmental SR and dyssynchrony did not change. Decreased V and S in the RV free wall could be explained by reduced preload of the systemic RV after stage 2 palliation.

The evaluation of left ventricular systolic performance in infants undergoing extracorporeal membrane oxygenation (ECMO) using traditional ejection-phase indices is hampered by significant alterations in preload and afterload. Therefore, a load-independent index, which relates heart-rate-corrected mean velocity of circumferential fiber shortening (VCFc) to afterload, measured as end-systolic wall stress (ESS), was used to assess left ventricular function in 18 term neonates undergoing ECMO. The mean age at the onset of ECMO was 75.5 h and the duration of therapy was 171 +/- 106 h. Left ventricular performance was highest before the onset of ECMO (VCFc = 1.65 +/- 0.49 circ/s) and decreased toward normal during (1.38 +/- 0.33 circ/s) and following ECMO (1.29 +/- 0.16 circ/s). Initially, nine of 17 (53%) patients had enhanced performance for the degree of afterload but in only 16 of 48 (33%) studies during ECMO and none following ECMO was VCFc elevated beyond the normal range predicted for ESS. These changes in left ventricular performance may be the result of variations in exogenous, as well as endogenous, catecholamines rather than intrinsic alterations in myocardial contractility. It is concluded that the VCFc/ESS relation permits a meaningful assessment of ventricular performance in critically ill neonates undergoing ECMO.

Left ventricular (LV) function is impaired by increased afterload in neonates with severe coarctation of the aorta, which may result in endocardial fibroelastosis. Repair of the coarctation usually solves the problem, with LV function normalizing after a few weeks. This report describes a patient who underwent successful repair of critical coarctation with normalization of LV function despite signs of endocardial fibroelastosis but with persisting elevation of cardiac troponin T. Cardiac catheterization showed the rare coincidence of anomalous origin of left coronary artery from the right pulmonary artery (ALCAPA) and coronary sinus orifice atresia with left superior vena cava.

In 19 human fetal and newborn hearts with atrioventricular septal defect (AVSD), not associated with other anomalies, the two ventricles were studied morphometrically. A total of 17 different parameters were studied: nine in the right ventricle and eight in the left. In the right ventricle we analyzed ventricular wall thickness, length of right ventricular inflow and outflow tracts, and volume of right ventricular inflow and outflow tracts. The data for these parameters were compared with the patterns of normality published previously, and the volumetric data were compared with patterns of normality published previously by us. The ventricular inflow tract was shorter than the outflow tract, the difference being especially significant in the left ventricle. The length of the diaphragmatic wall of the heart in both the right and left ventricle was equal to the sum of the length of the inflow tract and the thickness of the ventricular wall at the apex.

A seventeen-year-old male was referred with fatigue on effort and cyanosis. On examination, he was cyanotic and had a systolic murmur at the left sternal border. Echocardiography showed normal situs, a large subaortic ventricular septal defect, tricuspid atresia, pulmonary artery stenosis, and a normal right ventricle. (Figs. 1 and 2). Both arteries arose exclusively from the left ventricle. The pulmonary artery was positioned posterior and to the left, and the aorta was positioned anterior and to the right. Cardiac catheterization revealed a pulmonary artery pressure of 41/18 mmHg (mean, 30) and arterial saturation of 91%. The patient was not believed to be suitable for single ventricle pallation.

Patients with transposition of the great arteries often show poor mixing for different reasons, even after adequate balloon atrial septostomy. We present a patient with such a lesion whose clinical status improved dramatically after phentolamine was applied. We believe this improvement is due to reduction in afterload caused by the alpha(2) blocker and also possibly as a response to a presumptive effect of the drug on the diastolic function of the right ventricle, allowing more left-to-right shunt across the atrial septal defect. Both phenomena can improve cardiac output in such a situation.

In neonates with coarctation of the aorta, left ventricular function may be impaired due to an increased afterload and an associated endocardial fibroelastosis. Repair of the coarctation usually results in improvement in left ventricular function over the postoperative weeks. We report a patient in whom, despite successful repair of coarctation, left ventricular performance continued to deteriorate. Investigation revealed the unusual coincidental finding of anomalous left coronary artery from the right pulmonary artery.

We report a case of an infant with complex congenital heart disease who was placed on captopril for afterload reduction following cardiac surgery and subsequently developed pulmonary infiltrates with eosinophilia. The patient was readmitted with symptoms of rhinorrhea, poor feeding, and decreased activity level. She was found to have diffuse pulmonary infiltrates on chest radiograph and a marked peripheral eosinophilia without leukocytosis. After discontinuing captopril and starting systemic steroids, her symptomatology rapidly improved, and her eosinophilia and radiographic abnormalities both resolved.

We evaluated the aortic outflow tract (AOT) and coronary artery dimensions in pediatric patients with unicommissural aortic valves. A retrospective review of an echocardiographic database identified 37 patients with unicommissural aortic valves. A total of 115 echocardiograms were reviewed, and the right coronary artery (RCA), left main coronary artery (LM), left anterior descending coronary artery aortic valve annulus, aortic root, sinotubular junction (STJ), and ascending aorta were measured and z scores determined. The aortic stenosis peak gradient and the amount of aortic regurgitation (AR) were also measured. The RCA diameter (z score, 1.85 +/- 1.8, p = 0.03) and LM diameter (z score, 1.74 +/- 1.47, p = 0.04) are significantly dilated, as are all the AOT measurements: aortic annulus (2.02 +/- 1.9, p = 0.02), aortic root (2.25 +/- 1.9, p = 0.02), STJ (2.22 +/- 1.74, p = 0.01), and ascending aorta (4.38 +/- 2.03, p < 0.001). Longitudinal follow-up showed that there was no significant variation over time in any variable. The AOT measurements were significantly correlated with each other. A trend was found in which an increasing amount of AR gave an increase in AOT measurements. The aortic gradient was not significantly associated with any measurement. Our study population demonstrated significant dilatation of the RCA and LM as well as the AOT. The dilatation of the AOT structures is likely caused by the same mechanism that accounts for the AOT dilatation in patients with bicommissural aortic valves. Dilatation of the coronary arteries may represent an intrinsic abnormality in the vessel wall. Further studies are needed to define possible changes.

The cardiovascular system of the fetus is physiologically different than the adult, mature system. Unique characteristics of the myocardium and specific channels of blood flow differentitate the physiology of the fetus from the newborn. Conditions of increased preload and afterload in the fetus, such as sacrococcygeal teratoma and twin-twin transfusion syndrome, result in unique and complex pathophysiological states. Echocardiography has improved our understanding of human fetal cadiovasvular physiology in the normal and diseased states, and has expanded our capability to more effectively treat these disease processes.

An infant is presented who at birth met criteria consistent with hypoplastic left heart syndrome. He was followed clinically and by 11 weeks of age demonstrated substantial growth of the left ventricle. He underwent successful repair of coarctation of the aorta and continues to do well with moderate aortic stenosis. The difficulties of predicting left ventricular growth and function are discussed, and management options are reviewed.

Despite providing a physiological correction, measurements of contractility using the midwall stress-velocity relationship still show evidence of an unexplained hypercontractile state in some children. We investigated if by using midwall shortening indexes, the known overestimation of contractility at low afterload could be prevented. In 12 piglets (5 or 6 weeks old), afterload was manipulated by balloon occlusion of the descending aorta and infusion of sodium nitroprusside up to 5 mg/kg/min, and left ventricular function was measured using multiple variables. The regression line between the echocardiographically derived midwall velocity of circumferential fiber shortening and end systolic wall stress differed from the regression line of the endocardial stress-velocity relationship. Although the midwall regression line was almost horizontal (or afterload independent) for end systolic wall stress values of more than 30 g/cm2, the slope was still steeper below a certain point of afterload. The increased midwall velocity of fiber shortening at low afterload is comparable to the endocardial stress-velocity relationship and could account for the pseudo-hypercontractile state found in some children.

A cyanotic, tachypneic newborn was diagnosed to have double-outlet right ventricle of the Taussig-Bing type. Cardiac failure did not respond to medical treatment or surgical palliation. Postmortem examination revealed two ventricular septal defects (VSDs), one a malalignment VSD in the membranous septum and adjacent tissue and the other in the anterosuperior part of the muscular septum. The D-malposed aortic root emerged mainly from the right ventricle, with aortic-mitral continuity. The larger posterolateral pulmonary root arose almost entirely from the right ventricle, confluent with the muscular VSD, and unrelated to the mitral valve. Its right ventricular aspect was obstructed by hypertrophied infundibulum. This unique malformation of the heart functioned as a double-outlet right ventricle of Taussig-Bing type. In addition, however, the malformation had elements of tetralogy of Fallot because of the malaligned VSD and hypertrophied conal musculature (although pulmonary flow was excessive), and also of complete transposition of the great arteries because of the arrangements of the two VSDs, which favored aortic flow from right ventricle and pulmonary blood flow from the left ventricle. Thus, a single heart presented similarities to three anatomic and functional entities.

In 810 schoolchildren (aged 5-14 years) the prevalence of a grade 1-3/6 vibratory innocent heart murmur (VIHM) is 41%. Restricted to a grade 2 or 3 VIHM, the prevalence is 14%, decreasing from 21% in the age-class 5-6 years to 8% for children 13-14 years of age. The prevalence of a grade 3 VIHM is 1%. Together with a matched control, 84 children with a grade 2 or 3 VIHM underwent further cardiologic examination including electrocardiography, phonocardiography, and Doppler echocardiography. A positive correlation was found between the presence of a VIHM and higher left ventricular voltages on the ECG, but within the normal range; lower heart rate; smaller diameter of the ascending aorta (AAO); and higher blood flow velocity and higher maximal acceleration of the blood flow in the LVOT and the AAO. In 40% of the children with a VIHM, a systolic aortic valve vibration was seen with a frequencyor = 100 Hz and an amplitudeor = 1 mm, whereas this type of vibration was present in only one case control. No significant difference was found concerning the prevalence of false tendons in the left ventricle, systolic and diastolic diameter of the left ventricle, systolic time intervals, and shortening fraction of the left ventricle. The VIHM is strongly associated with a smaller AAO, with higher velocity and acceleration of the blood flow in the LVOT and AAO, and with a vibratory phenomenon of the aortic valve, pointing towards the LVOT-aortic valve region as the site of origin of the VIHM.

Eighteen of 25 survivors of aortic valvotomy in infancy were reinvestigated by cross-sectional echocardiography a mean of 7.5 (2.3-13.4) years after surgery. They had been operated at a median age of 38 (5-330) days. At the follow-up examination the gradient across the aortic valve was 41 +/- 19 (15-85) mmHg and the ejection fraction was 0.73 +/- 0.10 (0.48-0.84). Left ventricular (LV) end-diastolic volume was 66 +/- 17 (33-191) ml/m2. LV mass was 96 +/- 36 (44-204) g/m2 and the LV mass volume index (LVMVI) (mass divided by end-diastolic volume) was 1.43 +/- 0.4 (0.9-2.28). Eleven of 18 patients had an abnormally high mass volume index compared with 95 age-matched controls with structurally normal hearts. The correlation between the residual pressure gradient across the aortic valve and mass volume index yielded an r value of 0.75 (p less than 0.0004). One patient had been reoperated and underwent resection of a subaortic stenosis 4 years after the initial operation. Four patients with a resting gradient of more than 50 mmHg and one with grade 4 aortic regurgitation are scheduled for further surgical treatment. We conclude that, although LV function was normal in most patients who underwent aortic valvotomy in infancy, LV mass remains elevated in a significant number of patients, who may remain at risk of developing subendocardial ischemia.

Twenty-three children with diabetes mellitus, their ages ranging from 0.2-9.8 years, but with no sign of diabetic microvascular disease were investigated by M-mode and Doppler echocardiography, along with a comparable group of control subjects. In the diabetics, the fractional shortening and the mean velocity of fractional shortening were 14 and 18% higher, respectively, whereas the left ventricular end-systolic wall stress, an indicator of left ventricular afterload, was markedly reduced (22%). Assuming an unchanged preload in the two groups, this indicates a reduced afterload in these children. Systolic and diastolic time intervals, heart rate, and blood pressure were similar in diabetics and controls. Doppler-derived transmitral left ventricular filling indices were also similar. Thus, in these diabetic children no signs of left ventricular diastolic abnormality were detected. The state of hypercontractility of the left ventricle is considered to be due to a reduced afterload in early insulin-dependent diabetes.

Echocardiographic evidence of systolic aortic regurgitation following a Damus-Kaye-Stansel procedure for palliation of complex double-outlet right ventricle is presented. This procedure directs left ventricular output to the aorta through a proximal main pulmonary artery-aortic anastomosis and utilizes a valved conduit between the right ventricle and distal pulmonary artery. Postoperative Doppler and color flow echocardiographic findings revealed systolic and diastolic regurgitation from the native aorta to the right ventricle. Aortic valve closure at the time of the original Damus-Kaye-Stansel procedure would eliminate regurgitant flow and circumvent subsequent closure of this valve due to increased systolic aortic regurgitation.

The purpose of this study was to analyze potential “risk-factors” for late left ventricular dysfunction after surgical correction of Fallot's tetralogy (FT). As the ejection-phase indices cannot distinguish abnormalities of contractility from altered loading conditions, the slope values of the end-systolic pressure-length and stress-shortening relationships were analyzed by increasing afterload. Thirty-two patients were studied after surgical correction of FT in infancy. The age at investigation was 19.2±5.6 years, total correction had been performed at the age of 7.7±3.3 years. In 20 patients a one-stage operation was performed, and in 12 patients a two-stage correction. The control group consisted of 30 healthy volunteers, aged 18–30 years. The following potential risk factors for left ventricular dysfunction were evaluated: one-stage vs. two-stage correction, age at total correction, preoperative systemic oxygen saturation, preoperative hematocrit, occurrence of hypoxic spells, preoperative ratio of left-to-right ventricular peak systolic pressure, and preoperative ratio of left-to-right ventricular end-diastolic volume. In most patients the baseline data for end-systolic wall stress lay outside the normal range, indicating abnormal loading conditions. Thus, analysis of load-independent indices of the contractile state seems to be mandatory in these patients. Our data show that the severity of preoperative hypoxemia is an important risk factor for late dysfunction of the left ventricle (p

In a one-day-old male newborn with severe heart failure, the skin of the upper right thorax was pink, whereas the remaining areas were cyanotic. No peripheral pulses were palpable and the blood pressure could not be measured. On cardiac catheterization, systolic and diastolic pressures were elevated in the left ventricle (137/4/12 mmHg), but in the descending aorta, reached via a patent ductus arteriosus, the pressure was only 55/45 mmHg. O2 saturation was 97% in the left ventricle and 67% in the descending aorta. Angiocardiography showed an extreme obstruction of the ascending aorta and the aortic arch. The infant died on the second day. Postmortem examination revealed a wall-adherent calcified thrombus that totally occluded the lumen. No etiologic explanation could be obtained from the histologic examination, anamnestic data, or clinical findings.

M-mode echocardiograms were correlated with cardiac anatomy in six newborn infants who had autopsy-confirmed mitral-aortic atresia. Echoes from the mitral valve or the aortic valve were not identified. An echo resembling that from the ventricular septum was recorded posterior to the tricuspid valve in all six infants. This echo was separated from the posterior ventricular wall echo by a space that varied in width from 3 to 8 mm. In two infants, the echo was continuous with that from the posterior aspect of the aortic root area and in all six infants displayed an anterior systolic motion pattern. Autopsy demonstrated that in every case the left ventricle was minute and was located superiorly in the wall of the right ventricle; in life, the transducer beam would not have traversed the left ventricle. The right ventricle was enlarged in every case and had prominent trabeculations of the septal band that created large anatomic spaces within the right ventricle.

Hearts with one underdeveloped and one dominant ventricle form a spectrum of anomalies extending from the heart with two clearly adequate chambers to those with a true single ventricle. An angiographic concept of grouping such hearts is presented. 129 patients with unequal ventricles underwent catheterization and cineangiography between 1974 and 1983. The age at first catheterization ranged from one day to 24 years (mean 3.9 years). Male-female ratio was 2:1. Five groups of hearts (with their relative frequencies in the spectrum) were established: (1) dominant left ventricle (53%); (2) dominant right ventricle (20%), each with normally related chambers; (3) dominant left ventricle (20%); (4) dominant right ventricle (3%), each with ventricular inversion; (5) true single ventricle (5%). The incidence of atrial anatomy, venous return, intracardiac connections and associated lesions within each group was assessed. From the standpoint of deranged physiology as well as surgical implications there are more similarities than differences among these hearts. The fact that one ventricle will not generate an adequate stroke volume after repair is overwhelmingly more important than most other considerations. For the diagnostic and surgical approach, we believe that the system offers many advantages.

The effect of tolazoline was assessed in 29 hypoxic neonates. Tolazoline was given in a bolus starting at 1 mg/kg and repeated or infused for 5-134 hours. A "good clinical response," defined as a rise in PaO2 of more than 20 mm Hg, was obtained in 23 (79%), 20 of this group were weaned from the respirator, and three died. Six infants did not respond initially and four died. Failure to respond to tolazoline or to be weaned from the ventilator was usually associated with severe additional pathology. Urine output (greater than 1 ml/kg/h) was adequate in most neonates during therapy. In those with preexisting oliguria (less than 1 ml/kg/h), output improved during therapy. Blood pressure monitoring showed a fall in blood pressure in 19 patients during tolazoline administration, but true hypotension only occurred in four; in seven there was no fall and in three there was a rise in blood pressure. Echocardiography was performed prior to therapy in 19 patients and repeated in 12 patients after 24 h. Additional "tracking" was performed at 10 min, 1 h, and 4 h in seven patients. Prior to therapy, right ventricular dysfunction was demonstrated by abnormal right ventricular systolic time intervals (RVSTIs) in 17 of the patients tested. A rapid improvement was evident during therapy especially with "tracking." Left ventricular dysfunction, assessed by left ventricular systolic time intervals (LVSTIs), ejection fraction (EF), shortening fraction (SF), and velocity of circumferential fiber shortening (VCF), was also evident prior to therapy and improved, though more gradually than the RVSTIs.(ABSTRACT TRUNCATED AT 250 WORDS)

In 19 consecutive children with aortic valve stenosis, the left ventricle was entered retrogradely with a Gensini catheter guided by a tip-deflector guidewire which could produce any desirable degree of curvature at its tip. In all patients, the aortic valve was retrogradely traversed for measurement of the pressure gradient (mean gradient = 53 mmHg, mean valve area = 0.36 cm2), and left ventriculography. No complications were encountered. This technique is safe, simple, and effective in retrograde catheterization of the left ventricle in children with aortic stenosis.