Device-induced aortic obstruction is a known rare complication following transcatheter closure of patent ductus arteriosus in extremely low-birth-weight infants. Various mechanisms have been proposed. We report the first description of late aortic obstruction due to ductal vasoconstriction on pulmonic end causing device to be gradually pushed out of aortic end in a 980-gram premature infant.

Foetal echocardiography has progressed to be able to diagnose many forms of CHD and to assess the prognosis of cardiac lesions based on their anatomy and presentation in utero. This article outlines a straightforward method for the rapid evaluation of foetus that may have congestive heart failure with or without hydrops and for the differentiation of the pre-hydropic state from normal. The presence of signs of foetal heart failure, such as cardiomegaly or valvular regurgitation, gives clues to the aetiology of hydrops. The assessment of the prognosis of hydrops foetalis can be difficult but can be aided by the use of the cardiovascular profile score. Once identified, the neurohumoral effects of foetal heart failure can be ameliorated using transplacental digoxin if the hydrops has not progressed.

Our purpose was to study serially, measurements which relate to right ventricular events, using echocardiography and Doppler in full term neonates for the first three weeks after birth. Echocardiographic and pulsed Doppler studies were performed for 21 newborns during the first 36 hours of life, and subsequent serial studies were performed in the second (n=21) and third (n=14) weeks of life. Evaluation included measurement of right isovolumic relaxation time and peak E and A velocities across the tricuspid valve. Pulmonary velocities were studied for evidence of patency of the arterial duct, and to determine acceleration in the pulmonary trunk. Mean right isovolumic relaxation time decreased significantly from 68±9 ms to 39 ± 14 ms (p<0.001) between the first and second week. Mean tricuspid peak E increased significantly between the second (48 ± 7 cm/s) and the third week of life (62 ± 8 cm/s; p<0.001). Mean peak A wave velocity did not change significantly during the time of study. The pulmonary arterial acceleration time increased from the initial measurement (68 ± 10 ms) to the second week (85 ± 9 ms; p<0.001). Changes in right isovolumic relaxation time were significantly related to the changes in the pulmonary arterial acceleration time (r=0.63, p=0.0001), suggesting a significant influence of afterload on diastolic events. These data demonstrate the normal progression of some measures of early right ventricular diastolic function.

Although noninvasive estimation of the ratio of pulmonary to systemic flow by measuring the cross-sectional areas of the pulmonary and aortic valves and the respective time integrals of the velocity of blood is widely used to determine the amount of shunting in patients with left-to-right shunts, few data exist concerning interobserver variability. We assessed such variability of shunt calculations derived from Doppler echocardiography in 10 children aged six (3.5–11) years with an isolated atrial septal defect within the oval fossa. The ratio of flows was calculated by measuring stroke volumes over the pulmonary trunk and the aorta immediately before and at an average of seven days (5–11) following surgical closure of the atrial septal defect. Two independent observers measured the cross-sectional areas of the aortic and pulmonary orifices and the velocity time integrals and calculated the size of the shunt from these data. All measurements were performed five times, and the mean of these five measurements was calculated and compared for analysis. Before surgery, observer I had measured a ratio of pulmonary to systemic flow of 3.8 (2.1–6.2) and observer II of 3.5 (1.9–5). The mean interobserver difference in terms of the size of the shunt was 1.1 (or 30%). After surgery, observer I measured a ratio of 1.3 (0.8–1.7) and observer II of 1.1 (0.7–1.6). The cross-sectional area of the pulmonary valve was measured as 3.6 (2.6–4.9) cm2 and 3.4 (2.3–5.8) cm2 respectively, with a mean interobserver difference of 0.8 cm2 or 23%. For the velocity time integrals across the pulmonary trunk, the mean interobserver difference was 13% (4–38) before and 16% (0–55) after surgery. The respective variability for the aortic velocity time integrals was 11 % (4–46) before and 9% (0.5–23) after surgery. The variability of measurements of maximal flow velocity across the pulmonary trunk was 15% (4–38) before and 18% (1–77) after surgery. The respective variability for the aortic flow velocity was 11 % (2–28) before and 9% (4–23) after surgery. A paired t-test showed no significant differences for all parameters assessed. The difficulty of reproducing measurements of the pulmonary valve is an important factor affecting interobserver variability. The magnitude of interobserver difference was significantly related to the cross-sectional area of the pulmonary trunk (r=0.84; p<0.02). The interobserver variability may be considerable, and is large enough to influence clinical decisions. Thus, we conclude that a decision regarding need for surgery cannot be based solely on calculation of the shunt derived only from Doppler measurements in patients with isolated atrial septal defects within the oval fossa.

To assess the effect of a modified Fontan operation on systemic venous blood flow and the hepatic circulation, we compared 11 patients having an atriopulmonary connection and 35 with total cavopulmonary anastomosis. The Doppler echocardiographic study of the caval venous, hepatic venous and portal venous flow was performed so as to calculate the pulsatility ratio and the variation of flow with respiration. All patients had undergone cardiac catheterization. In addition, we included specific laboratory investigations to assess function of various organs.

Significantly lower maximum velocities of flow at inspiration (0.31±0.12 rn/sec vs 0.45±0.14 m/sec) and expiration (0.23±0.09 rn/sec vs 0.32±0.11 m/sec), less pulsatility (0.43 vs 0.16) and a lower ratio of systolic to diastolic velocity (1.22 vs 1.85) were found in the patients having a cavopulmonary as compared to an atriopulmonary anastomosis. Peak velocities of hepatic venous flow during inspiration and expiration were significantly lower in those with a cavopulmonary anastornosis (p=O.OOl and p<O.OOl, respectively). In these patients, forward flow was extremely dependent on respiration, with decrease or cessation of antegrade flow during expiration in 22 patients. The velocity of portal venous flow was also significantly lower in these patients, although the pulsatility ratio did not differ significantly between the groups (0.5 ± 0.21 and 0.57 ± 0.23, respectively). The ratio of inspiratory and expiratory velocities showed no significant difference between the groups, nor was there any correlation between the pulsatility ratio of the venous vessels or the ratio of peak flow velocities during expiration and the mean systemic venous/right atrial pressure on postoperative cardiac catheterization. Hypoproteinemia was found in 8 patients after total cavopulmonary anastomosis; 9 of 10 patients with protein C deficiency belonged to this group.

The dependence of hepatic venous flow on respiration in the presence of a chronically elevated systemic venous pressure in patients after the total cavopulmonary anastornosis may influence hepatic function in the postoperative period.

We analyzed tricuspid insufficiency noninvasively using cross-sectional and continuous wave Doppler echocardiography in 49 newborn infants free of structural cardiac and pulmonary disease. In 41(83.6%) of the cases, mild insufficiency was detected using the continuous wave Doppler technique. All the infants with valvar insufficiency had normal electrocardiographic tracings and, in 25 (61%) of them, there was no audible murmur. We detected transient ductal patency in five (12.1%). The ratio of right ventricular preejection period to ventricular ejection time was in the normal ranges (below 0.05) in 39 (95.1%) of the neonates. Of the 30 cases who underwent continued surveillance over a mean period of 37.9 days, the incompetence disappeared in 15 (50%). Our findings show that, in neonates, the lack of a murmur does not eliminate the possibility of neonatal tricuspid incompetence. Furthermore, abnormalities such as postnatal transient pulmonary hypertension, fetal closure of the arterial duct and perinatal asphyxia cannot readily explain the finding of tricuspid incompetence.

Case records of 67 children who presented beyond infancy (57 male, 10 female) with congenital valvar aortic stenosis were reviewed to assess progression utilizing the Doppler derived peak gradient as the index of severity. Age at presentation ranged from 1–13 years, and mean follow-up was 67 ± 29 months (range 12–142 months). The patients were divided into three groups depending on the rate of progression. Those with the most rapid rate of progression were significantly older when aortic stenosis was detected and at the time of presentation compared to the other two groups, who progressed minimally or not at all (p < 0.002 and p < 0.01 respectively). There was no significant correlation between the progression of stenosis and other clinical or Doppler echocardiographic variables.

We evaluated 6 patients with an anomalous subaortic left brachiocephalic vein. All cases were associated with some form of congenital heart disease: 4 patients had coexistent tetralogy of Fallot, one had congenitally corrected transposition, and the other an atrial septal defect with azygos connection of the inferior caval vein. Our incidence of an anomalous subaortic left brachiocephalic vein in patients with congenital heart diseases was 1.1% (6/527). It was observed in 4 out of 117 patients with tetralogy of Fallot (3.4%). Doppler echocardiography was useful in establishing the diagnosis. Furthermore, both digital subtraction angiography and magnetic resonance imaging demonstrated well the anomalous course of the vein, and were useful in making an accurate diagnosis.

Doppler echocardiography revealed a characteristic pattern of flow in four neonates with postductal coarctation of the aorta. There was a continuous left-to-right shunt at the level of the patent arterial duct with simultaneous measurement of a large gradient across the site of coarctation with a pandiastolic gradient and prolonged pressure half-time. Infusions of prostaglandin produced no hemodynamic improvement, and urgent surgical correction was performed.

Valvar regurgitation has been commonly found by echocardiography in adults and older children with normal hearts, but there is limited knowledge in neonates. To evaluate the prevalence of valvar regurgitation and analyze risk factors, echocardiography was performed on 975 consecutive neonates (1–7 days of age) with normal hearts. Regurgitation was detected and graded with pulsed, continuous-wave and color Doppler imaging techniques. Thirty-five cases with valvar regurgitation were followed for one month. The results showed that 369 (37.8%) neonates had evidence of regurgitation in one or more valves. Tricuspid regurgitation was most commonly found and was detected in 345 (35.4%) neonates, mitral regurgitation in 51 (5.2%), pulmonic regurgitation in 25 (2.6%), and aortic regurgitation in eight (0.8%). Most of them were trivial or mild and involved just one valve. Moderate or severe regurgitation was detected only in the tricuspid valve (3%) and the mitral valve (0.1 %). Five (0.5%) neonates with severe tricuspid regurgitation had heart murmurs, and two (0.2%) of them presented with cardiorespiratory distress. The risk factors for tricuspid and mitral regurgitation included lower chronologic age, gestational age and whether the neonates had a patent arterial duct; whereas those for tricuspid regurgitation also related to lower Apgar scores, birth weight and whether the neonates had a patent oval foramen. About 90% of the tricuspid regurgitation disappeared and the remaining 10% decreased in one month. We concluded that trivial or mild valvar regurgitation is commonly found in neonates with normal hearts. Moderate or severe regurgitation is relatively rare and occurs only in the tricuspid and mitral valves with a few cases causing clinical attention.

Banding of the pulmonary trunk is an important surgical procedure for patients who have con genital cardiac malformations with unrestricted pulmonary flow. We propose a new concept for determining in such circumstances the most appropriate length of the band used to constrict the pulmonary trunk in preparation for a Fontan operation. We studied 14 patients undergoing banding of the pulmonary trunk and measured the following parameters: diameter of aorta, diameter of pulmonary trunk, length of pulmonary arterial band and maximum flow velocity across the banded segment. We calculated an index from our orig inal parameter based on the formula; length of band/(diameter of aorta diameter of pulmonary trunk). The diameter of aorta was 9.5 ± 1.4 mm, and that of the pulmonary trunk was 9.6 ± 2.3 mm. The length of the band was 16.5 ± 3.4 mm, giving a calculated index of 0.188 ± 0.038. The maximum flow velocity was 4.02 ± 0.46 m/s. No correlation was found between the length of the band and body weight, and also no correlation was found between the length of the band and maximum flow velocity. The calculated index had a negative correlation with the maximum velocity of flow across the band (y = -8.13x + 5.56, R = 0.74, p < 0.01). We believe that the proposed index is a useful guide in determining the length of a pulmonary band when preparing patients for a Fontan operation.

Aim: Subclinical valvar insufficiency, or valvitis, has recently been identified using Doppler echocardiography in cases of acute rheumatic fever with isolated arthritis or chorea. The prognosis of such patients with acute rheumatic fever and subclinical valvitis is critical when determining the duration of antibiotic prophylaxis. We aimed, therefore, prospectively to investigate the association of silent valvitis in patients having rheumatic fever in the absence of clinical evidence of cardiac involvement, and to evaluate its prognosis. Methods and Results: Between November 1998 and September 1999, we identified 26 consecutive patients with silent valvitis in presence of rheumatic fever but in the absence of clinical signs of carditis. The patients, eight female and 18 male, were aged from 6 to 16 years, with a mean of 9.9± 2.7 years. Major findings were arthritis in 16, chorea in 7, and arthritis and erythema marginatum in 1 patient. Two cases had arthralgia with equivocal arthritic signs and Doppler echocardiographic findings of pathologic mitral regurgitation. Silent pathologic mitral regurgitation was found in 12 cases, and aortic regurgitation in 2 cases. All patients with arthritic findings were treated with acetylsalicylic acid with one exception, this patient receiving both prednisone and acetylsalicylic acid. No antiinflammatory treatment was given to patients with chorea. After a mean follow-up of 4.52 months, valvar regurgitation disappeared in 4 patients, including the one with migratory arthralgia and no other major criterions. All six patients with chorea and silent carditis still have mitral insufficiency. Conclusion: Acute rheumatic fever without clinical carditis is not a benign entity. Doppler echocardiographic findings of subclinical valvar insufficiency, therefore, should be considered as carditis when seeking to establish the diagnosis of acute rheumatic fever.

Carditis is the only manifestation of acute rheumatic fever that leads to permanent disability. Hence, its diagnosis is of paramount importance. Recently, it has been reported that Doppler echocardiography has disclosed subclinical valvar regurgitation in some patients with acute rheumatic fever manifested as isolated arthritis or pure chorea. The prognosis of such patients with acute rheumatic fever and subclinical valvitis is not clear. We aimed, therefore, prospectively to investigate the potential to diagnose patients with subclinical carditis. We examined 40 patients, aged from 7 to 16 years, with Doppler evidence of mitral and aortic regurgitation, but in the absence of any pathologic murmur. The major findings satisfying the Jones criterions were arthritis in 29 patients, chorea in 10 patients, and arthritis and erythema marginatum in one patient. Of the patients, 33 had mitral regurgitation, 6 patients had combined mitral and aortic regurgitation, and one patient had aortic regurgitation. The patients were followed over a mean period of 18.1 ± 13.9 months, the valvar regurgitation disappearing in 23 (57.5%). No significant differences were observed in the resolution of the valvitis between those treated with acetylsalicylic acid, steroids, or those receiving no treatment. It is noteworthy, nonetheless, that patients treated with steroids were the fastest to recover from valvitis (p < 0.05).

Based on our study, we suggest that subclinical valvitis demonstrated by echocardiography should now be accepted as adequate evidence for the diagnosis of carditis, and become a major diagnostic criterion for acute rheumatic fever. When managing this group of patients with subclinical disease, treatment with steroids seems to have a role in promoting early resolution of the valvitis.

Aim: To assess the myocardial velocities of the mitral annulus, left ventricular lateral wall, and midseptum in healthy children, and to compare these parameters with transmitral and pulmonary venous velocities. Methods and results: We examined 72 children, half being male, who had no systemic or cardiac pathologies. Their mean age was 6.73 ± 5.10 years, with a range from 0.1 to 17.75 years, and a median age of 6.71 years. Each parameter was measured twice, at end inspiration and end expiration. The tissue velocities are similar in males and females (p > 0.05). The longitudinal velocity of the heart in early diastole has a positive correlation with age (p < 0.05; midseptum velocity r = 0.57, left ventricular lateral wall velocity r = 0.56, mitral annulus velocity r = 0.56), and the tissue velocities are not influenced by respiration (p > 0.05). The myocardial velocities of different segments of the left ventricle are not correlated with the transmitral or pulmonary venous flows (p < 0.05). When age is controlled for heart rate, age mainly affects the systolic velocity of the mitral annulus and the early diastolic velocity of the midseptum in longitudinal axis, as well as the early diastolic velocity of the midseptum in transverse axis (p > 0.05 for all, r = 0.34, 0.29, 0.30 respectively). Conclusion: This study, which has determined reference values for tissue velocities in a large healthy group of children, will now set the scene for further studies in children with heart disease.