Successful Management of Severe Tricuspid Regurgitation Associated with Hydrops Fetalis in a Case of Dysplastic Tricuspid Valve

We report a case with hydrops fetalis and severe tricuspid regurgitation due to dysplastic tricuspid valve, diagnosed in utero and followed after birth. The patient was successfully managed on the basis of useful echocardiographic informations.     

Noninvasive evaluation of systolic pressures of pulmonary artery and right ventricle using contrast-enhanced doppler echocardiography: Comparative study using sonicated albumin or glucose solution

Evaluation of pulmonary arterial pressure is essential for the diagnosis and management of patients with congenital heart disease; it is usually done by cardiac catheterization. An alternative, noninvasive method may be clinically more useful. The purpose of this study was to assess the usefulness of the noninvasive determination of systolic pressure of the pulmonary artery and right ventricle by contrast-enhanced Doppler echocardiography. We selected 30 pediatric patients (28 with trivial or nonsignificant tricuspid regurgitant Doppler signals and 2 with significant tricuspid regurgitant Doppler signals) aged 2 months to 21 years. The flow velocity of tricuspid regurgitation was measured with continuous-wave Doppler of the right ventricular inflow view or left parasternal or apical four-chamber view before and after injection of two types of contrast medium (hand-agitated 5% glucose or sonicated albumin). The systolic pressure of the pulmonary artery was assessed as the estimated right ventricular systolic pressure (albumin method) minus the peak pressure gradient across the pulmonary valve (nonenhanced Doppler method). After injection of hand-agitated 5% glucose and sonicated albumin, trivial tricuspid regurgitation signals were enhanced in 25 of 28 patients (89%). In two patients, spectral envelopes were well defined enough to obtain the peak systolic velocity of the tricuspid regurgitation jet without contrast medium injection. Peak velocity was not altered by injection of contrast medium in these patients. There was significant correlation between the estimation by contrast-enhanced Doppler using hand-agitated 5% glucose and the cardiac catheterization measurement of the transtricuspid pressure gradient (r= 0.88). The transtricuspid pressure gradients obtained by continuous-wave Doppler during sonicated albumin enhancement corresponded closely to those measured by cardiac catheterization (r = 0.95). Pulmonary arterial and right ventricular systolic pressures measured by Doppler using sonicated albumin and those obtained by cardiac catheterization were highly correlated (right ventricle, r = 0.96; pulmonary artery, r = 0.95). In conclusion, this technique may be a valuable noninvasive method for determining accurate right ventricular and pulmonary arterial systolic pressures.     

Isolated Tricuspid Valve Endocarditis in a Child with a Normal Heart

Infective endocarditis localized in the tricuspid valve was observed in a previously completely healthy 10-yeardd male. The pathogen was identified as Staphylococcus uureus. Twodimensional and Doppler echocardiography revealed rupture of the chordae tendineae and prolapse of the tricuspid septal leaflet with tricuspid regurgitation. Isolated infective tricuspid valve endocarditis is mostly confined to drug addicts, and, to out knowledge, the literature contains only four cases of this disease in children without heart disease. The present case is considered to represent an extremely rare condition.     

Transient Neonatal Cyanosis: Unusual Presentation of Right-Sided Cardiac Masses

We report a case of a neonate with multiple cardiac masses, cyanosis, and a heart murmur. Arterial desaturation was the result of right-to-left shunting at the foramen ovale level caused by tricuspid regurgitation. Three right-sided cardiac masses were detected by echocardiography. By 2 weeks of age the patient had complete resolution of his cyanosis and improved tricuspid regurgitation following the normal decrease in pulmonary vascular resistance. At 2 years of age, he has no cardiovascular symptoms and the masses are calcified and have no hemodynamic consequences.     

The Abnormal Contralateral Atrioventricular Valve in Mitral and Tricuspid Atresia in Neonates: An Echocardiographic Study

Abnormalities of the mitral valve (MV) or the tricuspid valve (TV) morphology and/or function in patients with functional single ventricle may result in early morbidity and death. The purpose of this study was to determine the incidence of contralateral atrioventricular valve (AVV) pathologies in mitral valve atresia (MA) and tricuspid valve atresia (TA). We retrospectively reviewed the echocardiographic data of 50 neonates with MV and 20 with TA. Appearance of the papillary muscles, chordae tendinae, and valve leaflets was assessed. AVV regurgitation was semiquantitated by color-flow Doppler and the AVV annulus diameter was measured and indexed to body surface area. MV abnormalities were found in 9 of 20 (45%) of patients with TA. The MV was myxomatous in 9 patients, the leaflets were redundant in 5 patients, and prolapsing occurred in 4 patients. Mild regurgitation was found in 2 patients. In 18 of 20 (90%) patients MV annulus size was larger than 95% of predicted normal values. TV abnormalities were found in 12 of 50 (24%) patients with MA. The TV was myxomatous in 4 patients, prolapsing in 2, and redundant in 3, and moderate TV regurgitation was found in 3 patients. In 29 of 50 (58%) patients TV annulus size was larger than 95% of predicted normal values. Contralateral AVV abnormalities in tricuspid and mitral valve atresia are common and should be assessed carefully before surgical procedures.     

Contribution of Doppler Atrioventricular Flow Waves to Ventricular Filling in the Human Fetus

The normal fetal flow velocity profile across the atrioventricular valves is characterized by an early peak (E), which is related to preload and to active ventricular muscle relaxation, and a higher late peak (A), which is caused by the atrial contraction and also influenced by ventricular compliance. The purpose of this study was to determine how these two elements of ventricular filling change during gestation in both ventricles. A total of 485 normal fetuses from 17 weeks to term were examined by Doppler echocardiography. We measured E and A peak velocities and E/A ratio for both mitral and tricuspid valves. Simple regression analysis was applied to assess possible correlation between Doppler variables and gestational age. Moreover, E and A peak velocities were compared using paired Student's t-test. With the advance of gestation a significant linear increase in the E wave and E/A ratio was found for both mitral and tricuspid valves. The A wave shows little change throughout pregnancy. We found significantly higher Doppler velocities for the tricuspid valve than for the mitral valve. The relationship between the E/A ratios for the two valves and gestational age diverge slightly, with higher values for the mitral E/A ratio. This study shows that the A wave velocity remains constant throughout gestation, suggesting little or no change in ventricular compliance. The E wave is mainly responsible for the change in E/A ratio for both atrioventricular valves during gestation. These findings suggest progressive enhancement of relaxation and elastic recoil, an increase in preload, or both, throughout gestation, rather than a change in myocardial compliance as an explanation for the observed increase in the E/A ratio.     

Diagnostic and Perinatal Management of Fetal Extrasystole

Fifty fetuses referred to the Polish Mother's Memorial Hospital for fetal echocardiography between January 1, 1991 and June 1, 1995 were evaluated. The mean fetal gestational age at the time of diagnosis of arrhythmia was 34.1 weeks, and the mean gestational age at the time of delivery was 38.7 weeks. Checkup echocardiographic examinations were performed every 10–14 days, for a mean 2.4 studies per fetus. In most cases (48/50, 96%), premature atrial contractions were present during the first echocardiography examination. The fetal heart study was normal in 30 cases; in 7 (14%) there was tricuspid valve regurgitation, in 7 (14%) an atrial septal aneurysm, in 4 congenital heart defects, in 1 myocardial hypertrophy, and in 1 disproportion in the four-chamber view. Of the 50 fetuses, 43 underwent regular echocardiographic monitoring alone; in 7 cases, based on the presence of additional echocardiographic findings, pharmacotherapy was applied (digoxin, verapamil, or both). Three neonates died after delivery owing to malformations in two cases (one critical aortic stenosis, one spina bifida plus hygroma colli) and due to myocarditis in one case. In six of seven newborns treated in utero, myocarditis was diagnosed after birth (including the one with neonatal demise). Most of the newborns were in good condition after birth, their mean Apgar score being 8.6 and the mean birth weight 3259 g. We concluded that most extrasystoles represent an isolated anomaly, not affecting the fetal condition. Their presence should not influence the obstetric care and may require only echocardiographic monitoring. In most of our cases the premature contractions subsided after birth, although sometimes they preceded fetal supraventricular tachycardia or appeared after congenital myocarditis.     

Morphologic Analysis of Common Atrioventricular Valves in Patients with Right Atrial Isomerism

The objective of the study was to examine the relation between the morphology of the common atrioventricular valve and regurgitation of the valve in patients with right atrial isomerism. We examined seven consecutive patients with documented right atrial isomerism who subsequently underwent postmortem examination during a 10-year period. The degree of regurgitation and the diameters of the common valve were evaluated via cineangiography, and the site of regurgitation was evaluated by echocardiography. The morphology of the common atrioventricular valve was assessed further at autopsy. Cineangiography revealed valve diameters ranging from 14.8 to 27.8 mm (mean 20.9 mm). Valvar regurgitation was revealed within 2 months of birth in all patients. Regurgitation abruptly worsened in three patients after placement of a Blalock–Taussig shunt or a central shunt and postintubation. Autopsies revealed that the common atrioventricular valve consisted of four leaflets in five patients, and three leaflets in two. The anterior leaflets were large and protruding in all patients, and the lateral leaflets were thickened in six. All patients had a mass consisting of the left lateral leaflets and chordae with direct attachment of the chordae to the ventricular muscle (the right lateral leaflet was attached to the ventricular muscle and immobile in one patient). The lateral leaflets clung to the ventricular wall and exhibited poor movement in six patients. Leaflets with poor mobility corresponded to the regurgitant valvar site as assessed by echocardiography in six patients; and the regurgitation in three patients with acute deterioration occurred at the valvar side with poor mobility. It is concluded that the common atrioventricular valve in patients with right atrial isomerism has morphologic characteristics that may be associated with valvar regurgitation and malignant potential for abrupt deterioration after replacement of systemic–pulmonary shunting.     

Valve Repair in Children with Congenital Mitral Lesions: Late Clinical Results

Mitral valve repair may be performed without ring support with advantages related to results and complications. The objective of this study was to analyze the long-term clinical results following surgical repair and reconstruction without the use of rings in cases of congenital mitral lesions in children less than 12 years of age. Twenty-one patients who had undergone surgery during the period from 1975 to 1998 were evaluated. The mean age was 4.6 +/- 3.4 years. Females represented 47.6% of the total. Mitral regurgitation was present in 57.1% (12 patients), stenosis in 28.6% (6 patients), and the mixed lesion group represented 14.3% (3 patients). Perfusion time was 43.1 +/- 9.5 minutes and ischemic time 29.4 +/- 10.5 minutes. Follow-up time was 41.5 +/- 53.6 months for the regurgitation group, 46.3 +/- 32.0 months for the stenosis group, and 39.41 +/- 37.51 months for the mixed lesion group. Echocardiographical follow-up time was 37.17 +/- 39.51 months for the regurgitation group, 42.61 +/- 30.59 months for the stenosis group, and 39.41 +/- 37.51 months for the mixed lesion group. Operative mortality was 9.5% (two cases). There were no late deaths. In the regurgitation group, 10 patients (83.3%) were asymptomatic (p = 0.004). In the echocardiographical follow-up, most of the patients had minimal regurgitation. In the clinical follow-up of the stenosis group all patients were in functional class I (NYHA). The mean transvalvular gradient measured by echocardiography was from 8 to 12 mmHg with a mean gradient of 10.7 mmHg. In the mixed lesion group there was one reoperation at postoperative month 43. There were no cases of endocarditis or thromboembolism. Mitral valve repair in congenital lesions is associated with good late results. The majority of cases in the regurgitation group remain asymptomatic and do not require reoperation. Rings or annular support are not necessary in such cases. Satisfactory repair is more difficult to achieve in cases of mitral stenosis due to valvular abnormalities and the seriousness of the associated lesions.     

Bakterielle Endokarditis der Trikuspidalklappe bei einem 17 Jahre alten Mädchen ohne angeborenen Herzfehler oder andere Risikofaktoren

Background.

Infective endocarditis of the tricuspid valve is rare, occuring usually either in patients with congenital heart disease or with risk factors such as central lines, intravenous drug abuse or transvenous pacemaker leads. Since the symptoms of tricuspid valve endocarditis are unspecific, the diagnosis is often delayed.

Case report.

We present the case of tricuspid valve endocarditis occuring in a 17 years old girl without any risk faktors. The patient presented initially with unspecific symptoms like malaise, weightloss and intermittend fever as well as with a pleuropneumonia. The diagnosis was established by positive blood cultures (staphylococcus aureus) and echocardiographic identification of vegetations. Unequivocal demonstration of these alterations however required transesophageal echocardiography. Even though the vegetations were quite large and had destructed the posterior tricuspid leaflet, it was possible to reconstruct the valve with the remaining two leaflets.

Conclusions.

Children with severe pneumonia and a positive blood culture for staphylococcus aureus should always be evaluated for tricuspid valve endocarditis. If echocardiography reveals suspicious findings transesophageal echocardiography should be performed immediately.     

The Histology of the Lung in Neonates with Tricuspid Valve Disease and Gross Cardiomegaly Due to Severe Regurgitation

Lung autopsies from four neonates with Ebstein's anomaly or tricuspid valve dysplasia and gross cardiomegaly were examined. The mean cardiothoracic ratio was 92 ± 0.5%. The degrees of pulmonary hypoplasia and pulmonary artery muscularization were assessed and were compared with those in lungs from four controls and four patients with diaphragmatic hernias. Lung and body weight ratios and radial alveolar counts, which reflect pulmonary hypoplasia and immaturity, were significantly decreased only in patients with diaphragmatic hernia. The thickness of the medial muscle layer in small pulmonary arteries was greater in patients with diaphragmatic hernia; however, in patients with tricuspid valve disease, it was relatively small. Abnormal vascular muscle extension was seen only in patients with diaphragmatic hernia. In the cases of tricuspid valve disease, although cardiomegaly and lung compression were severe, lung hypoplasia and immaturity were not, and neither abnormal medial thickening nor extension were found. Our results suggest that, at least in full-term infants with tricuspid valve disease, surgical relief of lung compression may improve respiratory function, even if the cardiomegaly is severe.     

Balloon Dilatation of the Pulmonary Valve in a Patient with Pulmonary Atresia and Intact Ventricular Septum Using a Commercially Available Radiofrequency Catheter

Using a commercially available 5F deflectable radiofrequency catheter, we have succeeded in percutaneous valvotomy of an imperforate pulmonary valve and consecutive balloon dilatation in a baby with pulmonary atresia and intact ventricular septum. After the procedure, right ventricular systolic pressure fell from 125 mmHg to 65 mmHg, and right ventriculography demonstrated anterograde blood flow into the pulmonary arteries. There were no major complications. Doppler echocardiography at 1 year after the procedure demonstrated a pressure gradient across the pulmonary valve of 20 mmHg with mild pulmonary and tricuspid regurgitations.     

Utility of Intraoperative Transesophageal Echocardiography in the Assessment of Residual Cardiac Defects

To investigate the accuracy of immediate postbypass transesophageal echocardiography in the assessment of residual cardiac defects, we compared intraoperative transesophageal echocardiograms with intra/postoperative data in 86 patients, aged 4 days to 30.7 years (median = 1.4 years), at risk for a total of 174 postoperative lesions: right (n= 55) or left (n= 26) ventricular outflow tract obstruction, ventricular septal defect (n= 65), aortic (n= 12) or mitral regurgitation (n= 8), or mitral stenosis (n= 8). Accuracy of intraoperative transesophageal echocardiography was evaluated based on comparison with (1) immediate post-bypass left (n= 4) or right (n= 9) ventricular outflow tract pressure gradients by pullback in the operating room, (2) direct surgical inspection of residual ventricular septal defects (n= 3), (3) pulmonary artery oxygen saturation (n= 49), (4) right ventricular outflow tract pullback gradient (n= 24), and (5) transthoracic echocardiogram (n= 51) performed within 40 days of surgery. The results indicate that intraoperative transesophageal echocardiography agreed with intra/postoperative data in 87% of patients at risk for right ventricular outflow tract obstruction, 96% at risk for left ventricular outflow tract obstruction, 97% at risk for ventricular septal defect, and 100% at risk for aortic regurgitation, mitral regurgitation, or mitral stenosis. Significant residual lesions led to immediate surgical revision in 11 cases: 3 ventricular septal defects, 6 right and 2 left ventricular outflow tract obstructions. Of these, intraoperative transesophageal echocardiography confirmed and quantified suspected residual lesions in 7 and identified unsuspected lesions in 4 cases. Immediate postbypass transesophageal echocardiography proved reliable for assessing residual ventricular septal defect, mitral stenosis, and mitral or aortic regurgitation. Although accurate for assessment of the left and right ventricular outflow tracts in most patients, transesophageal echocardiography may not reliably reflect the severity of obstruction in all cases.     

Prediction of Postoperative Left Ventricular Pump Function in Congenital Mitral Regurgitation

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.     

Validation of the Proximal Isovelocity Surface Area Method for Assessing Mitral Regurgitation in Children

The proximal isovelocity surface area (PISA) method for calculating volume flow through the regurgitant orifice has attracted significant attention. A number of in vitro studies and clinical studies in adults suggest that the method is accurate. However, when applying the method to children it must be noted that the absolute regurgitation volume is small, and the range of body sizes is wide. This study investigated the accuracy of the PISA method for quantitative assessment of the severity of mitral regurgitation in children. Twenty children aged 7 months to 12 years (average 4.7 years) with mitral regurgitation but without interventricular shunt or aortic stenosis were selected for this study. Underlying cardiac diseases included atrioventricular septal defects in nine, isolated mitral regurgitation in five, and association with other heart defects in six. The PISA radius (r) and the duration of regurgitation (T) were measured on color M-mode recordings, with the M line passing through the center of the PISA. Assuming that the PISA is a hemisphere, maximal regurgitant flow rate (MFR: ml/s) was calculated as MFR = 2π×~ r ²×~ V (r= maximal radius, V= aliasing velocity), and regurgitant stroke volume (RSVpisa) as RSVpisa = 2π×~ MSR ×~ V×~ T (MSR = mean square of the PISA radius during regurgitation). As a validating standard, total stroke volume (TSV) using two-dimensional echocardiography determined by the area–length volumetry method and forward stroke volume (FSV) by the pulsed Doppler method were measured, and regurgitant stroke volume (RSV_D: RSV_D= TSV − FSV) and regurgitant fraction (RF: RF = RSV_D/TSV) were calculated. A linear correlation was found between MFR, RSVpisa, and RSV_D (X) (MFR = 4.2X + 54.0, r= 0.84. RSVpisa = 1.0X + 9.8, r= 0.90), and both RSVpisa and MFR divided by body surface area (BSA: m²) revealed a significant correlation with regurgitant fraction (X) by nonlinear regression analysis (RSVpisa/BSA = 26.2 ×~ X/(1 − X) + 16.8, r= 0.85. MFR/BSA = 121.8 ×~ X/(1 − X) + 92.2, r= 0.79). It is concluded that maximal regurgitant flow rate, regurgitant stroke volume, and regurgitant fraction can be accurately predicted in children using the PISA method by Doppler echocardiography.     

Congenital complete heart block in the fetus: hemodynamic features, antenatal treatment, and outcome in six cases

Congenital heart block (CHB) can result in intrauterine cardiac failure leading to fetal or neonatal loss. To establish perinatal hemodynamic factors which might predict adverse outcome, six fetuses with CHB diagnosed between 20 and 30 gestational weeks were examined by echocardiography at 2-week intervals. Neonatal morbidity and outcome in infancy are detailed. The fetuses showed a significant decrease in ventricular rate (VR) with advancing gestation (60 ± 7 vs 51 ± 4 beats/min, p= 0.03). Cardiac decompensation defined as hydrops or pericardial effusion was associated with VR of lower than 55 beats/min in two fetuses. Three mothers had a therapeutic trial with a sympathomimetic and digoxin. Salbutamol increased VR 10% in one of three fetuses treated. Digoxin decreased pericardial effusion in one hydropic fetus with autoimmune myocarditis. In this fetus, poor left ventricular fractional shortening (LVFS) was accompanied with high umbilical artery resistance index (RI). High amniotic fluid erythropoietin indicated severe hypoxia preceding death. Pacemaker was indicated in all the newborns. At the age of 2 weeks all the surviving infants had tricuspid regurgitation and a shunt through foramen ovale due to asynchronized atrioventricular contraction. During the 12-month follow-up two of five surviving infants had no symptoms. One had symptomatic neonatal lupus. Two infants had patent ductus arteriosus, one with dilated cardiomyopathy. In conclusion, poor fetal outcome was associated with low VR, low LVFS, and high RI. Despite early pacing, morbidity was high in infancy due to cardiomyopathy and associated heart defects. Regular echocardiographic monitoring during pregnancy and after delivery is required in order to optimize care and timing of any interventions.     

Cardiovascular Effects of Acute Bronchiolitis

ABSTRACT. Twenty-one children with normal hearts were studied during acute bronchiolitis. Doppler echocardiography showed tricuspid valve regurgitation in 11 patients, many of whom had evidence of raised pulmonary artery systolic pressure. Serial studies in those with severe infection showed that tricuspid regurgitation disappears with clinical improvement.     

Cardiovascular effects of acute bronchiolitis

Twenty-one children with normal hearts were studied during acute bronchiolitis. Doppler echocardiography showed tricuspid valve regurgitation in 11 patients, many of whom had evidence of raised pulmonary artery systolic pressure. Serial studies in those with severe infection showed that tricuspid regurgitation disappears with clinical improvement.     

Congenital Malformations of the Tricuspid Valve in Siblings

The occurrence of familial heart disease in association with tricuspid atresia is rare. The first reported instance of tricuspid atresia and Ebstein's anomaly in siblings is presented. The presence of these two distinct pathologic variants of tricuspid valve malformations in siblings suggests that these malformations result from a common abnormality occurring during the development of the inlet portion of the right ventricle.     

Serial Pulsed Doppler Assessment of Pulmonary Artery Pressure in Very Low Birth-Weight Infants

We assessed pulmonary artery pressure (PAP) during the early neonatal period in very low birth-weight (VLBW) infants using serial echocardiographic measurements of the ratio of the pulmonary artery acceleration time to the right ventricular ejection time corrected by heart rate [AT:RVET(c)]. Eighty-four VLBW infants weighing less than 1500 g were examined using serial color Doppler echocardiography from 3 hours to day 7 after birth. The AT:RVET(c) of infants born after 30 weeks of gestation showed a rapid, significant increase during the early neonatal period, whereas those of the groups born at less than 30 weeks showed no significant increase before day 14. At 24 hours after birth, the AT:RVET(c) values of VLBW infants did not correlate well with either the ratio of the right preejection period to the right ventricular ejection time on M-mode echocardiography or the pressure gradient between the right ventricle and the right atrium, as estimated by tricuspid regurgitation on pulsed Doppler echocardiography. The AT:RVET(c) value for the chronic lung disease (CLD) group did not differ significantly from that for the oxygen-dependent group at any assessment point. During the early neonatal period, the AT:RVET(c) of VLBW infants, as calculated by pulsed Doppler echocardiography, differed with their gestational age and did not appear to correlate well with PAP. Our data also suggest that AT:RVET(c) values may not be a good predictor of the subsequent occurrence of CLD in VLBW infants.