Effect of intermittent positive pressure ventilation on diastolic ventricular filling patterns in premature infants.

Eleven preterm newborn infants had a Doppler echocardiographic examination of their ventricular filling patterns during intermittent positive pressure ventilation. Peak velocity during early ventricular filling (VE) and during atrial systole (VA) and diastolic velocity-time integral of early (IE) and late (IA) ventricular filling caused by atrial contraction were measured. The ratios of VE/VA and IE/IA were also calculated. Diastolic peak velocities, as well as the diastolic velocity-time integral of early and late ventricular filling measured through the mitral valve were significantly higher during inspiration. The peak of the wave representing the velocity during atrial systole (A) was always higher than the peak of the wave representing the velocity of early ventricular filling (E). As a result, the ratio of these two variables (E/A) was always less than 1, showing no significant variation from inspiration to expiration. The flow patterns into the right ventricle were the opposite of those observed through the mitral valve. During inspiration, a significant decrease (p less than 0.001) in peak and time integral velocities of both the early ventricular filling and atrial systole waves was observed. Heart rate did not vary with respiration. It is concluded that during positive pressure ventilation, inspiration decreases right ventricular filling and enhances left ventricular filling. Opposite changes are recorded during expiration. Early and late phases of diastolic filling are equally affected. These changes are different from those observed during spontaneous breathing and should be taken into account in the assessment of diastolic ventricular function in preterm infants requiring assisted ventilation.

Value of systolic time intervals in the diagnosis of large patent ductus arteriosus in fluid-restricted and mechanically ventilated preterm infants.

M-mode echocardiographic features suggesting a patent ductus arteriosus are based on two groups of indirect criteria: dilation of the left cardiac cavities and changes of systolic time intervals. The reliability of the first group of criteria has been questioned in fluid-limited, mechanically ventilated preterm infants. The sensitivity of the systolic time intervals in the same circumstances is investigated. Twenty-three patients with a large patent ductus arteriosus were selected. Review of their echocardiograms shows that the sensitivity of the various criteria (expressed as percentage of positivity) was as follows: inversion of the ratio of left ventricular preejection period to right ventricular preejection period, 91.3%; left ventricular preejection period to left ventricular ejection time over right ventricular preejection period to right ventricular ejection time less than 1,83%; left atrium dilation, 74%; shortening of left ventricular preejection period, 70%; dilation of left ventricular internal dimensions in diastole, 65%; increase in left atrium/aorta, 52%; and decrease of left ventricular preejection period to left ventricular ejection time, 48%. Three criteria involving time intervals (left ventricular preejection period to right ventricular preejection period, left ventricular preejection period, and left ventricular preejection period to left ventricular ejection time) had 100% specificity. The lowest specificity was found with criteria involving the left atrium (left atrial to aortic root ratio 75% and left atrium 63%). It is concluded that study of systolic time intervals is a reliable means of detecting preterm infants with hemodynamically significant left-to-right shunt through a patent ductus arteriosus even if the infants are mechanically ventilated and fluid restricted.

Outcome of children with atrial septal defect considered too small for surgical closure.

There are few studies providing information on the natural course of hemodynamically insignificant atrial septal defect (ASD). To review the outcome of patients with secundum ASD, we retrospectively reviewed the charts of patients who had initially not been considered for surgical closure after age 1 year, and who had either a follow-up of at least 10 years or documented closure. Thirty patients, 22 females and 8 males, fulfilled our inclusion criteria. Mean age at diagnosis was 1.3 year and mean follow-up duration was 11.5 years. Seventeen patients had spontaneous closure of the ASD at a mean age of 8.4 years. There were 7 asymptomatic patients whose ASD was still patent at the last visit (mean age 14.1 years, mean follow-up 13.2), with defect dimensions on echocardiography ranging from 1 to 6 mm. The remaining 6 patients were considered to require surgical closure on the basis of an apparent increase in size of the ASD and secondary clinical and hemodynamic manifestations. These results (1) confirm that not all secundum ASDs need to be treated surgically because they can still spontaneously close past the age of 5, and (2) suggest that in a minority of cases the size of the defect could increase.

Segmental myocardial contractility versus perfusion in Kawasaki disease with coronary arterial aneurysm.

The impact of Kawasaki-related coronary injury on the myocardium was evaluated in 13 patients with persistent coronary aneurysm after a follow-up period of 7.92+/-3.97 years (range 1.8 to 14.3). Myocardial segmental perfusion and contractility integrity were assessed by resting and exercise echocardiography and technetium-99 (Tc-99m) sestamibi scan. Eight patients (61.5%) had giant aneurysms (> or = 8 mm) and 9 had multivessel involvement; the mean diameter of the largest aneurysm was 8.6+/-2.5 mm (range 5 to 14). During the acute phase, myocardial infarction occurred in 1 patient and coronary thrombosis in another. At the latest echocardiographic evaluation, the mean aneurysm diameter was 6.8+/-2.4 mm (range 4.5 to 12), there was persistent giant aneurysms in 5 of 8 patients, and 3 of 9 patients had multivessel involvement. Coronary angiography demonstrated stenosis in 7 of 10 patients, with multiple levels in 2. At sestamibi scan, all 13 patients had perfusion anomalies at rest, whereas only 7 had detectable hypokinesia on echocardiography. With exercise, perfusion returned to near normal in 3 patients, improved in 3, remained unchanged in 4, and worsened in 3 patients. Segmental contractility similarly deteriorated in the latter 3 patients but also in 2 patients whose perfusion scan had improved with exercise. Three patients, normal at rest, developed segmental hypokinesia during exercise. When present, the location of observed changes in contractility on stress echocardiography corresponded to that of perfusion defect. In conclusion, abnormal myocardial perfusion is present long term after complicated Kawasaki disease, the worst anomalies accompanying persistent giant aneurysms. Unfavorable perfusion response was coupled with abnormal contractility; however, enhanced perfusion with exercise correlated poorly with segmental contractility response.

Echocardiographic and angiographic findings in superior-inferior cardiac ventricles.

The anatomy of superior-inferior ventricles was studied in 17 patients, aged 1 day to 22 years, using echocardiography and angiography. In all patients, the right ventricle was located superiorly and the left ventricle inferiorly. The right ventricular sinus was underdeveloped in 14 of the 17 patients. Conversely, the right ventricular outflow tract was normally developed in all 17 patients. The visceroatrial situs was solitus in all patients, and it was associated with a concordant D-loop in 9 patients and with a discordant L-loop in 8. There was a high incidence of associated transposition of the great arteries (9 patients) or double-outlet right ventricle (5). Segmental combination was unpredictable, D-loop being associated with L-position of the great arteries in 4 of 8 patients and L-loop being associated with D-position of the great arteries in 4 of 9. There were only 3 concordant ventriculoarterial connections. Frequently associated anomalies included ventricular septal defect (17 patients), atrioventricular valve malformations (17) subaortic conus (14) and pulmonary outflow tract stenosis or atresia (11). Criss-cross hearts were present in only 7 patients.

Surgical repair of superoinferior ventricles: experience with 3 patients.

Superoinferior ventricles are a rare anomaly characterized by a horizontal ventricular septum and a hypoplastic right ventricular sinus localized anterosuperiorly to the left ventricle. This anomaly frequently is accompanied by malformation of the atrioventricular valves. A large ventricular septal defect is always present, and anomalies of the ventriculoarterial relations are common. The results of surgical repair of this complex lesion have been poor. Our recent surgical experience with 3 patients, 2 of whom are well 36 and 38 months postoperatively, suggests a hopeful outcome for the repair of this complex anomaly. The surgical approach was different in each of the 3 patients, demonstrating the need for a precise echocardiographic and angiocardiographic preoperative description of the cardiac anatomy to appropriately repair the multiple variants of this complex anomaly.

Inferior vena cava stenosis in scimitar syndrome: a case report.

The scimitar syndrome, first described by Chassinat in 1836, consists aessentially of an anomalous pulmonary vein draining whole or part of the right lung into the inferior vena cava. Associated anomalies are frequent, such as hypoplasia of the right lung, dextrocardia, malformations of the right pulmonary artery and bronchial tree, and abnormal arterial supply of the right lung (the so-called sequestration). This article describes a scimitar syndrome associated with stenosis of the inferior vena cava, whose initial diagnosis was made by two-dimensional echocardiographic Doppler color flow mapping. To our knowledge this is the first description of such an unusual association.

Reverse systolic flow in dilated pulmonary arteries demonstrated by colour Doppler imaging.

OBJECTIVE: To compare patterns of reverse systolic flow in pulmonary arteries in children with dilated and normal pulmonary arteries. DESIGN: Retrospective survey. SETTING: Tertiary pediatric cardiology referral centre. PATIENTS AND METHODS: Colour Doppler images were reviewed on 45 children, aged three months to 17 years, with normal or dilated main pulmonary arteries. The patients included were unoperated, and had isolated lesions and good parasternal short axis colour Doppler images of the main pulmonary artery with no significant pulmonary insufficiency. There were 19 patients with idiopathic dilation of the pulmonary artery, seven with moderate sized atrial septal defect, seven with moderate pulmonary valve stenosis, two with pulmonary hypertension and 10 children who were normal. Reverse flow was identified by adjacent red and blue areas without aliasing. RESULTS: Reverse systolic flow extending into early diastole was seen in 27 of the 35 patients and in two of 10 normals (P = 0.003). The main pulmonary artery was substantially dilated in the patient group compared with normals. CONCLUSIONS: Significant reverse systolic flow was found in main pulmonary arteries dilated idiopathically, or from pressure or volume overload. Reverse flow may be related to excessive capacitance of the main pulmonary artery in these patients.

[Kawasaki's disease. Epidemiological aspects and cardiovascular manifestations. Apropos of 106 cases]. / La maladie de Kawasaki. Aspects épidémiologiques et manifestations cardio-vasculaires. A propos de 106 observations.

One hundred and six patients fulfilling accepted diagnostic criteria for Kawasaki disease (90 p. 100 of French-Canadian origin) were evaluated with serial electrocardiograms and echocardiograms (M mode and two-dimensional). Half of the patients (53) had cardiovascular manifestations at one time during their illness. Thirty-one had abnormal electrocardiograms: non specific ST and T wave changes inferiorly (27), prolonged QT intervals (6), first degree AV block (3). M mode echo was abnormal in 31 cases showing: slight pericardial effusion (17), flat septal movement (11), left ventricular dilatation (4), decrease of shortening fraction (2). Seven patients (6.4 p. 100) presented fusiform coronary aneurysm detected in 6 by two-dimensional echography (with angiographic confirmation) and at autopsy in another. M mode echo and electrocardiogram abnormalities were transient in the great majority of patients disappearing during an average follow-up period of 10 months. Coronary aneurysm had disappeared at the end of follow-up in two patients, regressed in three and remained identical in another. There were no relations between severity of clinical symptoms, electrocardiographic or echocardiographic (M mode) abnormalities and the development of coronary aneurysm. Serial studies with two-dimensional echo should be done in every patient with this disease for early detection and follow-up of coronary aneurysm.

[Respiratory variations in the right ventricular diameter: an echocardiographic element in the diagnosis of partial isolated pulmonary venous return anomaly]. / Variations respiratoires du diamètre ventriculaire droit: un élément échocardiographique du diagnostic de l'anomalie partielle du retour veineux pulmonaire isolée.

Isolated partial anomalous pulmonary venous drainage (PAPVD), in contrast to atrial septal defect (ASD), does not cancel out the effects of respiration on blood flow in the right ventricle. The aim of this study was to see whether this difference could contribute to the diagnosis of PAPVD without ASD on M mode echocardiography. The diastolic dimensions of the right ventricle on expiration and inspiration were compared in 4 groups of patients aged 2 to 17 years. Group 1 comprised 6 children with PAPVD without ASD; Group 2: 10 children with PAPVD and ASD; Group 3: 11 children with isolated non-restrictive ASD, and Group 4: 10 normal children. Groups 1 and 4 were comparable with a respiratory variation of RV dimension of 10 to 29%. On the other hand, in Groups 2 and 3 the percentage variation was less than 6%. The finding of isolated RV dilatation with normal respiratory variation of its internal dimension should therefore alert the operator to the possible diagnosis of PAPVD without ASD.

Endocardial fibroelastosis in L-transposition of the great arteries with Ebstein's anomaly: revisited.

Ebstein's anomaly is a congenital deformity of the tricuspid valve consisting mainly of leaflet malinsertion. Clinical presentation varies from asymptomatic patients to those with congestive heart failure secondary to significant valvular regurgitation and low right ventricular output. We report here the case of an infant with a diagnosis of corrected transposition of the great arteries and Ebstein's deformity of the left-sided tricuspid valve who developed pulmonary hypertension and endocardial fibroelastosis, two unusual associations with this lesion. We also discuss the pathophysiology of this association and related literature.

Usefulness of non-invasive indices in the monitoring of heart-transplanted pediatric patients.

Seven patients underwent cardiac transplantation at Ste-Justine Hospital between June 1984 and October 1988 (age 3-16 years). Indices derived from M-mode echocardiography--left ventricular mass (LVM), isovolumetric relaxation time (IRT), shortening fraction (SF), velocity of circumferential fiber shortening (VCF), ejection fraction (EF), LV pre-ejection period/ejection time (PEP/ET), isometric contraction time (ICT)--and electrocardiography--summation of potentials (Sum Pot)--were used as non-invasive markers of rejection (367 studies), while rejection status was monitored by serial endomyocardial biopsy (55 biopsies). Spontaneous variation of such indices, as defined by the 90% distribution interval of their variation when biopsy was unchanged, was found to be important: -20% to +28% for IRT, -30% to +28% for LVM, -30% to 32% for Sum Pot, -32% to 42% for PEP/ET, etc. Using limits of spontaneous variation such as thresholds, none of the studied indices could consistently predict the 2 biopsy-documented episodes of rejection or the 7 episodes of improvement. Among all the studied indices, IRT was the most promising, correctly predicting 1 of the episodes of rejection and almost predicting the 2nd. Remeasurement of all M-mode tracings by an unique observer, following strict rules to maximize reproducibility, did not decrease the spontaneous variation of these indices, nor did it improve their performance as predictors of changes in the rejection status. These results warrant the continuing search for other non-invasive methods for accurate monitoring of the rejection status.

[Radiologic and echographic aspect of the disease of Kawasaki: experience with 75 cases at the Hospital Sainte-Justine of Montreal]. / Aspect radiologique et échographique de la maladie de Kawasaki: experience de 75 cas à l'Hôpital Sainte-Justine de Montréal.

Seventy-five patients with Kawasaki disease were studied in Hôpital Sainte-Justine between June 1979 and December 1984. Echography (abdominal and cardiac) proved to be a useful tool in the detection of the complications of this syndrome. Coronary aneurysms were present in 9% of our patients; two-dimensional echocardiography easily identified the proximal ones. In all patients, aortography allowed us to identify the extension of the lesions more distally. The left coronary artery was affected in all patients while in five both coronary arteries were involved. In all 75 patients chest radiographs were normal. Abdominal echography permitted diagnosis of a hydrocholecyst in three patients.

Fetal central blood flow alterations in human fetuses with umbilical artery reverse diastolic flow.

Blood flow velocimetry studies in animal fetuses with reverse diastolic flow in the umbilical artery have shown marked changes in the fetal central circulation characterized by a retrograde diastolic flow in the descending aorta, and as far as the aortic arch, along with a significant forward diastolic flow in the arteries going to the brain. Documentation and the implications of this phenomenon in human fetuses, to date, have not been reported. Doppler echographic evaluations of the diastolic patterns in the umbilical artery, descending aorta, the aortic arch, and the common carotid artery were performed on 5 fetuses in whom reverse diastolic flow was observed in the umbilical artery. In all five cases reverse diastolic flow was observed not only in the umbilical artery but also in the aortic arch. In the carotid artery, however, a forward diastolic flow was always recorded. It can be concluded, by inference, that in these fetuses, the area of lowest resistance was no longer the placenta, as seen in normal conditions, but the cerebral circulation. Furthermore, in four fetuses the retrograde component of the flow profiles was more prominent in the aortic arch compared with the descending aorta, suggesting that, in diastole, blood was coming from the pulmonary artery through the ductus arteriosus.

Reversible steroid-induced hypertrophic cardiomyopathy with left ventricular outflow tract obstruction in two newborns.

Dexamethasone is now frequently used in the treatment of bronchopulmonary dysplasia. We report on two premature babies receiving dexamethasone who developed symptomatic myocardial hypertrophy and left ventricular outflow tract obstruction, as documented by M-mode echocardiography and Doppler studies. A normal heart was recorded on echocardiography before and after dexamethasone treatment. The patients had no known risk factors associated with the development of obstructive hypertrophic cardiomyopathy.

Subxiphoid two-dimensional echocardiographic diagnosis of double-chambered right ventricle.

The echocardiographic features of 14 patients with double-chambered right ventricle are presented. Diagnosis was confirmed in 13 patients at cardiac catheterization and in one at necropsy. The ultrasonic diagnosis was based on the visualization in subxiphoid short-axis view of an anomalous muscle bundle at the lower margin of the right ventricular infundibulum; this structure was present in diastole and systole and the distal portion of infundibulum was wide and free of obstruction. Associated lesions were very frequent (especially ventricular septal defects, present in 13 patients). Subxiphoid two-dimensional echocardiography is an easy, reliable, noninvasive means of assessing this disease in infants and children.

In utero sonographic diagnosis of diaphragmatic hernia with hepatic protrusion into the pericardium mimicking an intrapericardial tumour.

A case of right-sided congenital diaphragmatic hernia was detected at 33 weeks of gestation. Fetal echocardiography revealed the presence of an intrapericardial mass (3.5 x 3 cm) localized at the right of the heart and surrounded by a massive pericardial effusion. This mass had the same echogenicity as the liver, with which it shared vascular channels. The diagnosis of right diaphragmatic hernia with protrusion of hepatic tissue into the pericardial sac and secondary pericardial effusion was made and confirmed after birth. In utero diagnosis of this anomaly enabled correct assessment of perinatal risk, and optimal fetal and infant management.

Superoinferior ventricles with superior left ventricle and inferior right ventricle: a newly recognized form of congenital heart disease.

We report the first known case of supero-inferior ventricles with a superior morphologically left ventricle and an inferior morphologically right ventricle. This 2 1/2-year-old boy also had dextrocardia, double-outlet right ventricle [S,L,L], right-sided mitral atresia, left-sided tricuspid regurgitation, a large conoventricular type of ventricular septal defect, and pulmonary outflow tract stenosis. This very rare form of superoinferior ventricles appears to be due to excessive levorotation (approximately equal to 170 degrees) of discordant L-loop ventricles.