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1.
P Lang  W I Norwood 《Circulation》1983,68(1):104-108
Ten patients with hypoplastic left heart syndrome underwent cardiac catheterization to assess the effectiveness of palliative surgery designed to prepare them for a modified Fontan procedure. The objectives of palliation were to establish unobstructed systemic blood flow, normalize pulmonary blood flow and pressure, and relieve pulmonary venous obstruction. In the first four patients, systemic blood flow from the right ventricle was established by means of a conduit from either the right ventricular free wall or the proximal main pulmonary artery to the thoracic aorta. Pulmonary blood flow was limited by pulmonary artery banding and ligation of the ductus arteriosus in three patients and by a Blalock-Taussig shunt in one. Conduit obstruction of systemic blood flow developed in two of these infants, and pulmonary vascular abnormalities precluded reparative surgery in the other two. In the other six patients, systemic blood flow was established by direct anastomosis of the proximal main pulmonary artery to the ascending aorta and aortic arch. The pulmonary vasculature was protected by providing pulmonary flow through a central or Blalock-Taussig shunt. The interatrial communication was enlarged by atrial septectomy or balloon atrial septotomy. One infant had progressive tricuspid regurgitation necessitating valve replacement. One, in whom balloon atrial septotomy had been performed, developed an obstructive interatrial communication necessitating late atrial septectomy. These six patients were candidates for physiologic correction. To date, three patients have undergone a modified Fontan procedure; two are clinically well at 9 and 12 months after surgery. Three patients await this procedure.  相似文献   

2.
Thirteen patients with left atrioventricular (AV) valve atresia and a normal aortic root were studied to evaluate the status of the interatrial communication with advancing age. Six patients had cardiac catheterization within the first 2 weeks of age; of these, five had repeat studies before 7 months of age. The seven other patients initially underwent catheterization after 2 weeks of age. In the group with catheterization before 2 weeks of age, the mean left atrial pressure was 7.8 +/- 5.5 mm Hg and the left atrial-right atrial mean pressure gradient was 1.7 +/- 2.4 mm Hg. In the combined group of patients with catheterization after 2 weeks of age, the mean left atrial pressure was 25.9 +/- 5.6 mm Hg and the mean left atrial-right atrial pressure gradient was 21.1 +/- 5.1 mm Hg. Seven of the 13 patients have survived and have now reached a median age of 6.5 years. Balloon atrial septostomy was adequate for long-term survival in one patient; all of the others have required surgical atrial septectomy. Progressive obstruction of the foramen ovale is part of the natural history of left AV valve atresia, and obstruction develops despite the absence of a left atrial-right atrial gradient during newborn study. Balloon atrial septostomy is recommended during the neonatal period in all patients with left AV valve atresia, even in the absence of an interatrial gradient. Because early surgical atrial septectomy is usually necessary for long-term survival, these patients should have serial noninvasive evaluation of the patency of the interatrial communication.  相似文献   

3.
Prospective echocardiographic diagnosis of absence of the left atrioventricular connexion, with the right atrium connected to a morphologic left ventricle through a bileaflet morphologically mitral valve, was made in six infants. The rudimentary right ventricle was left-sided in all patients, and separated from the left atrium by sulcus tissue. The ventriculoarterial connexions were discordant. Associated defects included subpulmonary stenosis (2 patients), pulmonary atresia (1 patient), and a patent duct (4 patients). All patients developed early left atrial hypertension due to a restrictive interatrial septum, and required transcatheter septostomy (5 patients), or surgical septectomy (3 patients). One patient who had a severely restrictive ventricular septal defect died following cardiac catheterization. In three others the ventricular septal defect has become progressively restrictive on serial catheterization. Successful intermediate term palliation has been performed in two patients using a bidirectional Glenn anastomosis, together with enlargement of the ventricular septal defect and a Damus-Kay-Stansel procedure in one. It is possible to distinguish this malformation from "mitral atresia" using cross-sectional echocardiography. The long-term outlook is influenced by early relief of left atrial hypertension. Balloon atrial septostomy alone is usually inadequate, and either blade septostomy or surgical septectomy are required. Serial cardiac catheterization is mandatory for planning definitive palliation.  相似文献   

4.
Interatrial shunt flow profiles in 36 normal term infants were examined serially by colour flow and pulsed Doppler echocardiographic techniques from within an hour of birth to four or five days after birth. Shunt flow across the foramen ovale was detected in 33 normal infants (92%) within an hour of birth (mean 40 minutes). The occurrence of interatrial shunting decreased with age, but a shunt signal was still detected in 17 infants (47%) on the fourth or fifth day of life, by then the ductus arteriosus had already closed in all the normal infants. The direction of interatrial shunt flow was predominantly left-to-right, but in 64% there was a coexistent small right-to-left shunt in diastole within an hour of birth; by four to five days it was found in 19%. In the six patients with persistent fetal circulation the direction of the interatrial shunt flow was predominantly right-to-left with biphasic peaks in diastole and systole at the early stage of the disease, and the period of right-to-left shunt flow during each cardiac cycle was significantly longer than that in normal infants examined within 1 hour of birth. In all patients the ductus closed before the foramen ovale. At the time of ductal closure in all patients with persistent fetal circulation right-to-left shunt flow was seen during diastole and its period was still prolonged. These findings suggest that interatrial shunting, predominantly left-to-right, is common in normal newborn infants. Evaluation of the characteristics of the interatrial shunt by Doppler echocardiography may be useful for predicting the progress of or improvement in neonates with persistent fetal circulation.  相似文献   

5.
Among 74 survivors of the Fontan type of operation abnormal cardiac signs were detected in 46 (62%) at postoperative examination. The findings were analysed in relation to the state of the cardiovascular system of these patients. Cyanosis was present in 10 (13.5%) patients. The causes of cyanosis included residual interatrial shunt (six patients), acquired pulmonary arteriovenous fistulas (three patients) and acquired systemic-to-pulmonary vein communication (one patient). Signs of chronic fluid retention were detected in six (8%) patients. In four of them the fluid retention was related to conduit obstruction and in the remaining two it was secondary to severe subaortic stenosis in one and atrioventricular valvar regurgitation in the other. Organic heart murmurs were heard in 29 (39%) patients. The aetiologies of these murmurs were multiple. They included aortic valve regurgitation (eight patients), subaortic stenosis (seven patients), atrioventricular valvar regurgitation (five patients), pulmonary valve regurgitation (five patients), residual Blalock-Taussig shunt (two patients), residual ventricular septal defect (two patients), residual communication in the main pulmonary artery which had been ligated but not divided (one patient), and left ventricular to right atrial shunting (one patient). Cardiac rhythm disturbances of varying aetiology were noted in 23 (31.1%) patients. Sixteen (21%) had supraventricular arrhythmias and seven (9.5%) had conduction abnormalities. The present review suggests that among survivors of the Fontan type of operation abnormal cardiac signs are indicators of residua or sequelae or both of the native cardiovascular anomalies or surgical procedures.  相似文献   

6.
Interatrial shunt flow profiles in 36 normal term infants were examined serially by colour flow and pulsed Doppler echocardiographic techniques from within an hour of birth to four or five days after birth. Shunt flow across the foramen ovale was detected in 33 normal infants (92%) within an hour of birth (mean 40 minutes). The occurrence of interatrial shunting decreased with age, but a shunt signal was still detected in 17 infants (47%) on the fourth or fifth day of life, by then the ductus arteriosus had already closed in all the normal infants. The direction of interatrial shunt flow was predominantly left-to-right, but in 64% there was a coexistent small right-to-left shunt in diastole within an hour of birth; by four to five days it was found in 19%. In the six patients with persistent fetal circulation the direction of the interatrial shunt flow was predominantly right-to-left with biphasic peaks in diastole and systole at the early stage of the disease, and the period of right-to-left shunt flow during each cardiac cycle was significantly longer than that in normal infants examined within 1 hour of birth. In all patients the ductus closed before the foramen ovale. At the time of ductal closure in all patients with persistent fetal circulation right-to-left shunt flow was seen during diastole and its period was still prolonged. These findings suggest that interatrial shunting, predominantly left-to-right, is common in normal newborn infants. Evaluation of the characteristics of the interatrial shunt by Doppler echocardiography may be useful for predicting the progress of or improvement in neonates with persistent fetal circulation.  相似文献   

7.
Among 74 survivors of the Fontan type of operation abnormal cardiac signs were detected in 46 (62%) at postoperative examination. The findings were analysed in relation to the state of the cardiovascular system of these patients. Cyanosis was present in 10 (13.5%) patients. The causes of cyanosis included residual interatrial shunt (six patients), acquired pulmonary arteriovenous fistulas (three patients) and acquired systemic-to-pulmonary vein communication (one patient). Signs of chronic fluid retention were detected in six (8%) patients. In four of them the fluid retention was related to conduit obstruction and in the remaining two it was secondary to severe subaortic stenosis in one and atrioventricular valvar regurgitation in the other. Organic heart murmurs were heard in 29 (39%) patients. The aetiologies of these murmurs were multiple. They included aortic valve regurgitation (eight patients), subaortic stenosis (seven patients), atrioventricular valvar regurgitation (five patients), pulmonary valve regurgitation (five patients), residual Blalock-Taussig shunt (two patients), residual ventricular septal defect (two patients), residual communication in the main pulmonary artery which had been ligated but not divided (one patient), and left ventricular to right atrial shunting (one patient). Cardiac rhythm disturbances of varying aetiology were noted in 23 (31.1%) patients. Sixteen (21%) had supraventricular arrhythmias and seven (9.5%) had conduction abnormalities. The present review suggests that among survivors of the Fontan type of operation abnormal cardiac signs are indicators of residua or sequelae or both of the native cardiovascular anomalies or surgical procedures.  相似文献   

8.
The interatrial septum (IAS) was studied by subxiphoid two-dimensional echocardiography (S2DE) in 88 infants under 12 months of age who weighed 1.2-9.1 kg. The IAS was adequately displayed in 87 of 88 patients. The morphology, presence and localization of defects in the IAS were evaluated by S2DE and retrospectively related to the findings at cardiac catheterization. In seven patients with no interatrial communication at cardiac catheterization, the IAS was straight, with an area of central thinning corresponding to the veil-like cover of the septum primum over the foramen ovale. The morphology of the IAS with a stretched, patent foramen ovale (56 patients) indicated the coexistence of a right or left ventricular volume or pressure overload, and was readily distinguishable from the IAS with a secundum type communication (13 patients). In patients with a stretched, patent foramen ovale and left ventricular overload lesions, the IAS was a nearly homogenous, curvilinear structure bowing into the right atrium, with a small area of septal dropout at the superior rim of the septum primum. In the presence of right ventricular overload lesions, the central defect of the foramen ovale was associated with a redundant flap valve of the septum primum billowing into the left atrium. In secundum type communications, the centrally located defect represented a deficiency rather than a redundancy of the septum primum. Balloon atrial septostomy (BAS) in 17 patients produced a secundum-type defect bordered by the flail remnants of the torn septum primum. Blalock-Hanlon septectomy (two patients) resulted in a large, posterior, sinus venosus-type communication which incorporated the preexisting BAS. Ostium primum defects (seven patients) were distinguished from the secundum lesions by their eccentric position in the IAS adjacent to the atrioventricular ring.  相似文献   

9.
J L Bass  J M Berry  S Einzig 《Circulation》1986,74(2):315-322
We used pulsed Doppler ultrasound to determine the flow dynamics of the aorta and patent ductus arteriosus (PDA) in 21 infants (ages 1 to 16 days) with PDA and aortic atresia (n = 15) or aortic stenosis (n = 6). The flow within the PDA was recorded in 19, and was right-to-left during systole in all. There was a diastolic left-to-right PDA shunt in 11 patients with aortic atresia and in three with aortic stenosis, and the shunt was associated with large (3 to 11 mm in diameter) interatrial communications. In two patients with aortic atresia and three with aortic stenosis, however, the diastolic PDA shunt was from right to left, and the interatrial communications were small (0 to 2 mm in diameter). The right-to-left diastolic PDA shunting may be best explained by the relative pulmonary (high with left-sided inflow obstruction and a small interatrial communication) and systemic resistances. All patients with aortic atresia and three with aortic stenosis had retrograde systolic flow in the transverse aortic arch, probably resulting from inadequate left ventricular output. Antegrade diastolic flow in the transverse aortic arch toward the PDA was observed in all infants with aortic atresia and a left-to-right PDA shunt. Ascending aortic flow was recorded in 11 patients with aortic atresia, and was retrograde during diastole in each, the result of coronary perfusion. Application of pulsed Doppler ultrasound can lead to a better understanding of the hemodynamics and physiology of patients with congenital cardiovascular disease.  相似文献   

10.
Three patients with normal hearts and no pulmonary abnormality had neonatal tricuspid regurgitation causing cardiorespiratory distress and cyanosis. The signs of tricuspid regurgitation resolved over a few weeks. In the acute phase echocardiography showed gross dilatation of the right atrium and ventricle. The interatrial septum bulged into the left atrium during the whole cardiac cycle. Doppler echocardiography showed clinically significant tricuspid regurgitation, a right to left shunt through the foramen ovale, reduced flow through the pulmonary valve, and in two patients ductal flow into the pulmonary artery. In one patient tricuspid regurgitation was so great that it impeded the opening of the pulmonary valve and produced functional "atresia" of the pulmonary valve. The presence of regurgitant blood flow through the pulmonary valve showed that the "atresia" was functional rather than organic. Doppler echocardiographic study is useful in distinguishing functional neonatal tricuspid regurgitation from structural abnormality of the tricuspid valve.  相似文献   

11.
Three patients with normal hearts and no pulmonary abnormality had neonatal tricuspid regurgitation causing cardiorespiratory distress and cyanosis. The signs of tricuspid regurgitation resolved over a few weeks. In the acute phase echocardiography showed gross dilatation of the right atrium and ventricle. The interatrial septum bulged into the left atrium during the whole cardiac cycle. Doppler echocardiography showed clinically significant tricuspid regurgitation, a right to left shunt through the foramen ovale, reduced flow through the pulmonary valve, and in two patients ductal flow into the pulmonary artery. In one patient tricuspid regurgitation was so great that it impeded the opening of the pulmonary valve and produced functional "atresia" of the pulmonary valve. The presence of regurgitant blood flow through the pulmonary valve showed that the "atresia" was functional rather than organic. Doppler echocardiographic study is useful in distinguishing functional neonatal tricuspid regurgitation from structural abnormality of the tricuspid valve.  相似文献   

12.
Patients with left atrioventricular (AV) valve atresia or stenosis were studied retrospectively to determine the incidence of early and late failures of procedures to enlarge an interatrial communication. The 61 patients underwent 80 procedures: 5 balloon atrial septostomies, 12 blade atrial septostomies and 63 surgical septectomies. No balloon septostomy provided adequate long-term palliation. Of 12 blade septostomies, 4 resulted in gradients across the atrial septum of 5 to 8 mm Hg and 8 in gradients 3 mm Hg or less. Results from blade septostomy were unrelated to underlying diagnosis, age, gradient before the procedure, number of previous procedures, pulmonary blood flow or size of the postprocedure defect by balloon sizing, but were related to size of the postoperative defect estimated by echocardiography. Among 8 patients with gradients of 3 mm Hg or less after blade septostomy, 7 were followed 9 +/- 7 months and showed no evidence of restenosis. Of 63 surgical septectomies, 11 (17.5%) were inadequate, and in at least 7 cases the failure was due to restenosis of the defect as documented by serial catheterizations or echocardiograms. Outcome after surgical septectomy was unrelated to underlying diagnosis, age or number of previous procedures, but was related to size of the defect created. Our results reveal improved results in terms of residual gradient for blade septostomy compared with previous studies and the need to follow these patients carefully, even those undergoing surgical septectomy.  相似文献   

13.
In this review, the role of transcatheter methods in the management of cyanotic congenital heart defects is discussed. In patients with interventricular right-to-left shunting secondary to pulmonary outflow tract obstruction (most commonly tetralogy of Fallot), balloon dilatation may be an effective palliative procedure in a substantial proportion of patients, obviating the need for a palliative shunt. We would recommend this if the patient's size or cardiac anatomy makes that patient an unsuitable candidate for safe total surgical correction. Infundibular myectomy with atherectomy catheter in tetralogy of Fallot patients may become a useful adjunct in the management of these infants. Cyanotic children with interatrial right-to-left shunt secondary to severe valvar pulmonary stenosis respond to balloon pulmonary valvuloplasty in a manner similar to that seen with isolated pulmonary valve stenosis. In these patients, balloon valvuloplasty is the treatment of choice and may be corrective in most cases. In patients with a narrowed Blalock-Taussig shunt, balloon angioplasty may improve pulmonary oligemia and systemic arterial hypoxemia and may obviate the need for a second systemic-to-pulmonary artery shunt. Balloon angioplasty is recommended if the patient's cardiac defect is not amenable to surgical correction at a low risk either because of the size of the patient or because of the complexity of the cyanotic heart defect. In patients with pulmonary valve atresia, initial opening of the atretic pulmonary valve by either laser or surgery with subsequent balloon dilatation is potentially beneficial in reducing the total number of surgical procedures that these children are likely to require. However, further clinical trials are needed prior to their general use.  相似文献   

14.
Massive aneurysmal dilatation of the foramen ovale was diagnosed angiocardiographically in a patient with tricuspid atresia. The angiographic findings are distinct for this condition, and the pathogenesis appears to be related to a restrictive atrial communication in the patient with obligatory right to left shunting at atrial level. The anatomic potential for atrial restriction in the patient with tricuspid or pulmonary atresia, intact ventricular septum and diminutive right ventricle necessitates balloon atrial septostomy at the initial diagnostic cardiac catheterization. In the patient with aneurysmal dilatation of the foramen ovale, satisfactory decompression may be achieved by Blalock-Hanlon atrial septectomy, open atrial septectomy or, possibly, balloon septostomy.  相似文献   

15.
We present the case of a 26 years-old woman with pulmonary atresia and interventricular septal defect. Classical Blalock-Taussig and Waterston-Cooley shunts were performed in infancy. When she was 14 years old related progressive cyanosis and dyspnea and angiography showed severe stenosis of the Blalock-Taussig. Cardiac catheterization performed 12 years later demonstrated complete obstruction of the shunt. A new surgical palliation was precluded by hemorrhagic complications. Two self-expandable stents were successfully deployed in Blalock-Taussig anastomosis: previously, a balloon-expandable stent was implanted in right pulmonary artery. Oxygen saturation increased from 68% to 89% after stents implantation. Twelve months later clinical and laboratory tests are still improved, and Doppler confirmed patency of the shunt.  相似文献   

16.
In a 1 year period, 56 neonates with a clinical diagnosis of ductus arteriosus had Doppler echocardiographic confirmation of the ductus; 33 (59%) had additional, turbulent left to right flow at the atrial level through a valve-incompetent foramen ovale. Normalized left atrial dimensions in the group with the atrial shunt were significantly larger than when there was a competent foramen ovale; when the ductus closed and left atrial enlargement receded, the atrial shunt disappeared. However, several infants with large left chambers had no interatrial shunting. When the atrial shunt was present, there were up to three flow pulses, corresponding to atrial systole, ventricular systole and ventricular diastole, but these were frequently fused into two pulses or even one pulse per cycle. The atrial septal morphology provided supporting clues: general bowing of the septum or a localized bulge in the region of the foramen ovale indicated relatively high left atrial pressure, and frequently a slitlike dropout could be seen at the superior edge of the foramen. During the study, three additional neonates with a ductus arteriosus were found to have a secundum atrial septal defect with a typical echographic image, "matchhead" appearance of the septal rim of the defect, but the Doppler flow patterns were indistinguishable from those of a valve-incompetent foramen ovale. The hemodynamic effects of the interatrial shunt, from either cause, seemed slight during the hospital course, but the presence of a valve-incompetent foramen ovale indicated a relatively large ductal shunt. Quantification of the ductal shunt, however, continues to rely primarily on measurement of the left atrial and ventricular size.  相似文献   

17.
Four patients with complex cardiac lesions, who underwent successfully a Fontan type of operation are presented, each case representing a particular problem in surgical management. The first exhibited displacement of a trileaflet straddling and overriding left atrioventricular valve in addition to tricuspid atresia, while the second one had unilateral lung perfusion. In the third case, a stenosis had developed near the origin of the right pulmonary artery, and pulmonary vascular resistance could not be determined prior to the definite palliation procedure, whereas the fourth patient presented with stenosis of the left atrioventricular valve. Surgery was planned after detailed evaluation of the morphologic and hemodynamic features by means of echocardiography and cardiac catheterization. Our results illustrate the common tendency to extend the limits for modified Fontan procedures, which also includes revision of the original criteria for selection.  相似文献   

18.
In aortic atresia, coronary perfusion normally occurs through retrograde blood flow in the ascending aorta. We report on two patients with antegrade flow in the ascending aorta despite aortic atresia. In one patient with hypoplastic left heart syndrome (aortic atresia, severe mitral stenosis), an intact interatrial septum/premature closure of the foramen ovale was found. While no other way of left atrial or ventricular decompression was found, echocardiography, angiography and the post-mortem examination showed left ventricular to coronary sinusoids as the sole pathway for systemic oxygenation. In a second patient with complex congenital heart disease, including aortic atresia, antegrade flow in the ascending aorta was through a left coronary fistula with shunt flow originating from the pulmonary trunk. This report describes systemic perfusion depending on retrograde coronary flow due to coronary-cameral (sinusoids) and coronary arterio-venous fistulas leading to the phenomenon of antegrade blood flow in the ascending aorta despite aortic atresia.  相似文献   

19.
In postpartum persistent right-to-left shunt at the atrial level, the valve of the foramen ovale fails to close. As a thin valve-flap the septum primum is pushed to the left during the phases of right atrial pressure predominance and closes to the septum secundum, when left atrial pressure exceeds right atrial pressure. Thus, it performs a marked movement during the cardiac cycle, reflecting the interatrial pressure-flow dynamics. With use of M-mode echocardiography, this movement pattern was studied in 24 patients: 13 with cyanotic heart disease (age 2 days to 21 years) and 11 newborns with persistent transatrial right-to-left shunt due to noncardiac disease. Cardiac defects were confirmed by cardiac catheterization and cineangiocardiography. Interatrial right-to-left shunts were proved by M-mode and 2-dimensional contrast echocardiography. The comparison of the M-mode echocardiographic findings in our patient groups with normal atrial septal movement studied in 20 healthy infants and children revealed considerable differences. The characteristic movement of the valve of the foramen ovale also was compared with results obtained by cineangiography and 2-dimensional echocardiography. Analysis of interatrial blood pressure difference provided a pathophysiologic explanation of the septum primum movement in transatrial right-to-left shunt.  相似文献   

20.
The opening of the tricuspid valve and the onset of right ventricular filling precede the opening of the mitral valve and the onset of flow in the normal adult. Sixty-five studies of atrioventricular flow with range-gated pulsed Doppler echocardiography, performed on 32 normal neonates, consistently demonstrated the reverse sequence. Further investigation showed that at the time of mitral valve opening, while the tricuspid valve was still closed, the valve of the foramen ovale began to bow posteriorly into the left atrium and remained posteriorly bowed for most of diastole. The magnitude of posterior bowing varied among the neonates but, concomitant with the more prominent grades of posterior bowing, right to left shunting across the foramen ovale was demonstrated on color flow mapping. Ultrasound studies in the normal fetus also revealed earlier opening of the mitral valve, bowing of the valve of the foramen ovale into the left atrium and right to left shunting across the foramen ovale. These findings indicate that in the normal immature heart isovolumic ventricular relaxation is completed earlier on the left than on the right side and that left ventricular compliance appears to be greater than right ventricular compliance. The relation of left and right ventricular compliance in the adult is different from that in the normal immature heart. Whereas systemic and pulmonary vascular resistance and pressure levels change rapidly in the newborn period, ventricular compliance matures over a longer period of time. As a result of the differential maturity, for a variable period of time in the normal neonate, a left to right ductal shunt coexists with a right to left atrial shunt.  相似文献   

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