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.

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.

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.

Factors affecting the prognosis of Ebstein's anomaly during fetal life.

BACKGROUND: The echocardiographic criteria that have been used to evaluate severity of Ebstein's anomaly in utero are the same as those applied after birth. OBJECTIVE: The objective of this study was to establish prognostic criteria that take into account the peculiarities of the fetal hemodynamics. METHOD: The video recordings of eight fetuses with Ebstein's anomaly were retrospectively reviewed. RESULTS: The following indexes had no prognostic significance either on fetal or neonatal outcome: the ratio of functional tricuspid opening over the diameter of the annulus, the degree of displacement of the tricuspid valve opening, and the degree of tricuspid regurgitation. The index of severity (based on the surfaces of right atrium + atrialized right ventricle) and the cardiothoracic ratio had a significant impact only on neonatal survival. The smallest fossa ovalis were found in two fetuses who had hydrops. Fetuses who reached term without problems had higher left ventricular outputs. A positive linear correlation was found between the z score of the left ventricular output and the size of the fossa ovalis (r = 0.81, p < 0.05). CONCLUSION: The prognosis of Ebstein's anomaly during fetal life is not influenced by criteria described for postnatal life and may be related to factors that control the volume load of the left ventricle.

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.

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.

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.

Cardiac two-dimensional imaging and reference values for blood flow velocities in the ovine fetus.

Since the ovine model is the most commonly used for foetal haemodynamic investigation it was felt important 1) to investigate the technical difficulties involved with ultrasound fetal cardiac imaging in this species and 2) to establish normal reference values for ovine cardiac and umbilical blood flow velocity measurements. Both two-dimensional and pulsed Doppler techniques were used for this assessment in 25 unsedated ewes. All morphological features described in human features to identify the ventricular cavities could be found in the ovine fetus with the two-dimensional echocardiogram. Specific differences included a flatten thorax as visualized from the lateral position, the mesocardial position of the heart, and a large left azygos vein behind the left atrium draining blood into a dilated coronary sinus. The mean peak velocities (cm/sec) of the early diastolic wave (E) and the atrial wave (A), along with their calculated ratio, were not statistically different between the two atrio-ventricular valves (E: 30.6 +/- 6.6, 31.2 +/- 6.1; A: 43.0 +/- 8.3, 41.6 +/- 8.0; E/A: .72, .76 for mitral and tricuspid valves respectively). A significant difference was observed between the acceleration times of the blood ejected into the aorta and the pulmonary artery, with the time interval being shorter in the pulmonary artery (Aorta: 0.052 +/- 0.011; Pulmonary artery: 0.037 +/- 0.009 s). A mean pulsatility index of the umbilical artery of 0.89 was recorded. The data recorded in this study should serve as a reference base for further non-invasive studies of the ovine foetal circulatory system using the ultrasound technique.

Closure of the ductus arteriosus: determinant factor in the appearance of transient peripheral pulmonary stenosis of the neonate.

We speculated that a relationship may exist between transient peripheral pulmonary stenosis and the closure of the ductus arteriosus. Fifty preterm infants had pulmonary artery and ductal color Doppler flow velocity assessments performed before and after closure of the ductus arteriosus. No flow turbulence or increase in velocity was observed immediately after birth, although a significant discrepancy in size was observed between the main pulmonary artery and its two branches. After closure of the ductus, 15 infants had signs of transient peripheral pulmonary stenosis of the left pulmonary artery in association with a significant decrease of diameter at the origin of the same artery. In all 50 infants, no significant gradient was observed in the right pulmonary artery. We conclude that, at least in the preterm infant, transient peripheral pulmonary stenosis is not present at birth but is an acquired phenomenon closely related to closure of the ductus arteriosus.

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.

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.

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.

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.

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.

Left ventricular diastolic function during the first month of life.

UNLABELLED: In order to assess possible changes in myocardial relaxation occurring during the neonatal period, M-mode echocardiograms were recorded serially in 9 normal term infants and in another group of 10 one-month-old infants. The tracings were studied with an M-mode calculator. Although individual variations were greater in the data collected during the first 24 h, no significant difference was found in the indices of diastolic function of the left ventricle during the first 4 days of age. The following changes were observed between data recorded at 4 days and 1 month, respectively: normalized peak rate of left ventricle filling, 4.03 vs. 4.71 cm/s; diastolic peak velocity of early posterior motion of aortic root, 1.89 vs. 5.15 cm/s; peak velocity of left ventricle posterior wall motion in diastole, 3.31 vs. 3.50 cm/s; mitral valve EF slope, 59.05 vs. 84.92 mm/s; left ventricle isometric relaxation time, 43.88 vs. 28.50 ms. IN CONCLUSION: (1) greater individual variations are observed in indices of left ventricle diastolic function during the first day of life, and (2) significant increase in left ventricle compliance occurs during the first month of life. These changes should play a critical role in the clinical course of newborn with cardiopulmonary disease.

[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.

Value of respiratory variations of right ventricular dimension in the identification of small atrial septal defects (secundum type) not requiring surgery: an echocardiographic study.

In search of reliable criteria that could help differentiate insignificant atrial septal defects (ASDs) from those with a large shunt, M-mode echocardiograms of three groups of patients were studied retrospectively: group I = 10 normal children (mean age 5.7 years); group II = 10 patients (mean age 7 years) with small ASD in whom the decision was taken not to proceed to surgical closure, based on hemodynamic and angiographic criteria; and group III = 15 patients (mean age 7 years) with an "operable" shunt, who underwent corrective surgery. The results showed that right ventricular end-diastolic dimensions during expiration (RVDDE) were increased in all patients in group III but were normal in only 3 of the 10 patients in group II. A normal septal movement was found in all patients in groups I and II but also in five patients in group III. The variation in right ventricular diastolic dimension with respiration (RVDVR) was always normal in group II. However, in group III all patients but one had a small RVDVR (less than 6%). It was concluded that a normal RVDDE is very specific (100%) but not sensitive (30%), a normal septal movement is very sensitive (100%) and moderately specific (70.6%), and a normal RVDVR is both very sensitive (100%) and specific (94.4%) as a criterion for identification of small ASDs not requiring surgery.

Wall thickness, cavity dimensions, and myocardial contractility of the left ventricle in patients with simple transposition of the great arteries. A multicenter study of patients from 10 to 20 years of age.

Fifty-one patients with uncomplicated transposition of the great arteries (TGA) and normal pulmonary pressure who were 10 to 20 years old and 69 normal subjects matched for age were studied by M mode echocardiography. Left ventricular internal dimensions and posterior wall thickness showed positive correlation with the body surface area in the TGA as well as in the control groups. Values for these parameters in the TGA group were generally smaller than those in the normal population. Septal thickness of patients with TGA (5.9 +/- 1 mm) was also smaller than that in the control group (6.6 +/- 1 mm) (p less than .01). Septal motion was normal in 11 patients with TGA and paradoxical in 19 patients in this group. In the other 21 patients the septum was flat. The following systolic time intervals of the left ventricle were found for patients with TGA: preejection period (PEP) 64 +/- 11 msec, ejection time (ET) 310 +/- 37 msec, and PEP/ET 0.21 +/- 0.04. These values were significantly different from those of the right ventricle for the normal population: PEP 77 +/- 12 (p less than .01), ET 327 +/- 25 (p less than .05), and PEP/ET 0.24 +/- 0.03 (p less than .01). Shortening fraction and mean velocity of circumferential fiber shortening (49 +/- 7% and 1.6 +/- 0.3 circ/sec, respectively) were also significantly higher (p less than .01) in patients with TGA than in the control group (33 +/- 4% and 1.1 +/- 0.2 circ/sec). These data should help achieve reliable quantitative and qualitative interpretations of echocardiograms of patients with TGA.