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1.
Cryothermia, a new technique for definitive treatment of the pre-excitation syndrome, is described in two patients. The first patient presented with a normal P-R interval with a delta wave and reciprocating tachycardia. Preoperative electrophysiologic study suggested a free-wall atrioventricular connection on the left posterior atrioventricular (A-V) groove. At surgery, epicardial mapping confirmed the site of pre-excitation on the posterior left ventricular (LV) wall. An electrogram arising from the accessory pathway (AP) was recorded at the site of earliest ventricular activation. Interatrial delay combined with an apparently long accessory pathway to the ventricle caused the P-R interval to appear normal. Local pressure abolished pre-excitation. The site of early ventricular activation was cooled to -60 degrees C with a specially designed cryoprobe. All evidence of pre-excitation and arrhythmias disappeared. The second patient presented with a refractory reciprocating tachycardia and was found to have an AP in the septum capable of only retrograde conduction. Retrograde conduction was abolished by applying a temperature of 0 degrees C to the anulus at this site during tachycardia. Conduction over the AP and reciprocating tachycardia returned with rewarming. Ablation of the AP was obtained by applying a temperature of -60 degrees C for 90 seconds on two occasions to the same area. The His bundle was not injured.  相似文献   

2.
In two patients with WPW syndrome Type A suffering from syncopes and dizziness intermittent high degree A-V block was observed. The analysis of the surface Ecg revealed in the first case a complete A-V block within the normal conduction system at the level of the A-V node. In the second case there was a constant left bundle branch block with intermittent block in the right fascicle (intermittent trifascicular block). In both cases the preexcitation syndromes could be best explained by accessory tracts bypassing the normal nodal system left side. One-to-one conduction through the bypass occurred only at a distinct range of cycle lengths, at lower frequencies the accessory tracts were refractory and a IInd or IIIrd degree A-V block occurred. However, outside this frequency zone some P waves were conducted through the accessory tracts without changes in cycle lengths. The findings support the thesis of at least two functionally different atrioventricular pathways in patients with preexcitation syndrome.  相似文献   

3.
OBJECTIVE: The mouse with trisomy 16 (Ts16) is held to be a genetic model for humans with Down's syndrome (Ts21). Both trisomies are associated with atrioventricular septal defects, but the precise morphology in the mouse remains unclear. We have therefore characterised cardiac morphology in the mouse with Ts16. METHODS: Ts16 fetuses, from a Rb(11.16)2H/Rb(16.17)7Bnr x C57BL/6J cross, were collected on gestational days 17 or 18 (full term = 19 days) and studied using scanning electron microscopy and serial sections. RESULTS: The hearts showed a spectrum of deficient atrioventricular septation which we categorised into two types. In one, a common atrioventricular junction was separated into right and left orifices by a tongue of tissue joining two valvar leaflets that bridged the ventricular septum to varying extent. In the other, a common atrioventricular junction was connected exclusively to the left ventricle. All hearts had ostium primum atrial and ventricular septal defects, together with abnormal ventriculo-arterial connections. No heart had the typical morphology seen in the human with Down's syndrome, namely a balanced common atrioventricular junction, guarded by a common valve, with the aorta connected exclusively to the left ventricle. CONCLUSIONS: The cardiac defects seen in Ts16 mice show marked differences from the typical anatomy in human Ts21, suggesting more complex mechanisms of cardiac dysmorphogenesis in Ts16. The mouse model will prove valuable in elucidating the mechanism of normal expansion of the atrioventricular junctions, and help in charting the precise steps involved in atrial and ventricular septation.  相似文献   

4.
Paroxysmal atrial tachycardia with atrioventricular block usually indicates potentially dangerous overdigitalization, and serious heart disease is almost universally present. In this report, we describe a patient with a structurally normal heart who manifested spontaneously intra-atrial reentrant tachycardia with Wenckebach atrioventricular block in the absence of medications. In this patient, the longest atrial paced cycle length that induced atrioventricular nodal block was 390 ms, and the atrial cycle length during tachycardia ranged from 360 to 400 ms. The electrophysiologic study in our patient demonstrated that second-degree atrioventricular block during atrial tachycardia may occur in patients without structural heart diseases or taking any medication.  相似文献   

5.
In double outlet left ventricle,both the pulmonary artery and the aortaarisefromthemorphologicleft ventricle. This paper presents the anatomic and cineangiocardiographic features of five patients who had this condition proved at surgery or autopsy. The condition of the first patient was incorrectly diagnosed as transposition of the great arteries with pulmonary stenosis and ventricular septal defect; the anatomic features were correctly interpreted at operation in 1966 and appropriate repair was made, but the patient died postoperatively. The condition of the other four patients was correctly diagnosed. The second patient had Ebstein's malformation of the tricuspid valve and hypoplastic right ventricle in addition to double outlet left ventricle; her condition was not considered operable. Two patients, one with visceroatrial situs solitus, concordant d-loop and dextrocardia, were surgically treated with good long-term results. The fifth patient died 2 years postoperatively and is the first patient reported to have double outlet left ventricle with visceroatrial situs inversus, a discordant d-loop and levocardia. The segmental approach to the classification and diagnosis of connection disorders is discussed and the radiologic criteria by which double outlet left ventricle may be diagnosed considered in detail. The importance of using the radiologic projection (usually left anterior oblique) that profiles the ventricular septum is emphasized because the diagnosis can be established only by delineating the relations of the origins of the great arteries to the ventricular septum. The surgical significance of other anatomic features, including the number, size and position of ventricular septal defects and conal malformations, is also discussed.  相似文献   

6.
We describe 4 cases of congenitally corrected transposition associated with atrioventricular septal defect, diagnosed by echocardiography and angiocardiography. Two had usual atrial arrangement and two had mirror imaged atrial arrangement . All cases were associated with subpulmonary valvar stenosis. All patients presented with cyanosis and were in sinus rhythm. Atrioventricular septal defect with common atrioventricular junction was easily diagnosed on the basis of a common atrioventricular valve permitting interatrial and interventricular communications. All patients had balanced right and left ventricles. The echocardiographic recognition of the ventricles was based on the presence of the moderator band within the morphologically right ventricle, the characteristics of the apical septal trabeculations, and the shape of the ventricles. Angiocardiographic recognition of the ventricles was achieved on the basis of right and left ventriculography. In one case with usual atrial arrangement, we recorded two His bundle potentials, one anteriorly and another posteriorly. Atrial stimulation revealed blocked atrioventricular conduction at the level of the posterior bundle, and normal atrioventricular conduction through the anterior bundle. In both cases with atrial mirror-imagery, only a posterior His bundle potential was found, with normal atrioventricular conduction revealed by atrial stimulation The clinical course with this combination depends on the other lesions present in addition to the common atrioventricular valve. Our electrophysiological studies show that the conduction system in presence of a common atrioventricular valve is as expected for congenitally corrected transposition with two atrioventricular valves.  相似文献   

7.
A 20-hear-old patient with mitral valve prolapse and minimal mitral regurgitation associated with intermittent marked sinus bradycardia and sinus arrhythmia, and atrioventricular block, proximal to the bundle of His, varying from first-degree to high-grade, is described. Both the murmur and the atrioventricular block had been documented since the age of eight years, and probably since the first year of life, and has shown no subsequent progression. The patient's symptoms of chest pain and severe lightheadedness and near syncope have been shown by telemetry electrocardiogrphic monitoring to be unrelated to changes in cardiac rhythm.  相似文献   

8.
OBJECTIVE: To describe our experience in prenatal diagnosis and perinatal management of congenital atrioventricular heart block, as well as pacemaker treatment in the neonate. MATERIAL AND METHODS: A total of 13 fetuses are included. The diagnosis of atrioventricular dissociation was established by Doppler heart rate sample in the right atrium to show the atrial activity while the sample in the Aorta reflected the ventricular heart rate. Gestational age at diagnosis, ventricular heart rates, autoimmune maternal pathology, maternal blood tests for autoantibodies antiRo+, congenital structural heart disease, fetal hydrops, maternal medical treatment, perinatal results and pacemaker neonatal implantation are described. RESULTS: Gestational age at diagnosis ranged between 22 and 32 (mean 27.6) weeks. Ventricular heart rates ranged between 32 to 80 (mean 54) beats/min. AntiRo+ antibodies were detected in 5 mothers, and clinical systemic lupus erythematosus was found in only one. Four had congenital heart disease (2 ventricular inversion and corrected TGA, 1 complete atrio-ventricular canal and 1 tricuspid atresia). Signs of heart failure and hydrops were detected in 9 fetuses. Treatment with beta-metasona and ritodrine was administered to 7 mothers when the ventricular heart rate dropped below 60 beats/min. Intrauterine fetal death occurred in 3 fetuses with structural congenital heart disease and hydrops. Delivery was performed by cesarean section in 8 preterm fetuses (one them a twins), 3 spontaneous deliveries at term and 3 stillbirth. Postnatal pacemaker implantation was carried out in 9 newborns (3 cases with unicameral temporal right ventricle electrode and 6 cases with permanent bicameral electrodes implanted through the subclavian vein and DDD pacemaker). Follow-up of the bicameral pacemaker group was satisfactory. CONCLUSION: Persistent fetal bradycardia is the first sign to diagnose prenatal complete atrioventricular heart block. Echocardiography asses fetal haemodynamic status and may detect signs of fetal deterioration. Hydrops and further drop in the ventricular heart rate warrant urgent cesarean section and pacemaker management of the newborn.  相似文献   

9.
A patient with Wolff-Parkinson-White syndrome type B developed 2:1 atrioventricular block resulting from the association of persistent right bundle-branch block with tachycardia-dependent (phase 3) left bundle-branch block. Electrophysiological studies disclosed the coexistence of a tachycardia-dependent (phase 3) block in the accessory pathway. This conduction disturbance was exposed, not by carotid sinus massage as in previous studies, but by pacing-induced prolongation of the interval between two consecutively conducted atrial impulses. Furthermore, the surface electrocardiogram showed, at different times, ventricular complexes resulting from: (1) exclusive atrioventricular conduction through the normal pathway without bundle-branch block; (2) predominant, or exclusive, atrioventricular conduction through a right-sided accessory pathway; (3) exclusive atrioventricular conduction through the normal pathway with right bundle-branch block; (4) exclusive conduction through the normal pathway, with left bundle-branch block; (5) fusion between (1) and (2); and finally, (6) fusion between (2) and (3) However, QRS complexes resulting from simultaneously occurring Wolff-Parkinson-White syndrome type B and left bundle-branch block could not be identified. Future electrophysiological investigations should re-evaluate the criteria used to diffrentiate between true and false patterns of Wolff-Parkinson-White syndrome type B coexisting with left bundle-branch block.  相似文献   

10.
Radiofrequency catheter ablation (RF-CA) has been widely used to cure paroxysmal supraventricular tachycardia (PSVT). However, its use has never been reported in familial PSVT caused by an accessory atrioventricular pathway (AP), which is known as one of the typical familial cardiovascular diseases. Two cases of using RF-CA for familial PSVT due to APs are presented, in a brother and sister, supporting a potential genetic role in the developmental failure to lose the atrioventricular connection during fetal life. The sister, a 24-year-old woman, had intermittent episodes of palpitation accompanied by chest pain for 2 years. An electrophysiologic study (EPS) confirmed her clinical tachycardia was atrioventricular reentrant tachycardia (AVRT) due to a left lateral concealed AP, which was subsequently successfully ablated with RF-CA. The brother, a 22-year-old man, had a 5-year history of paroxysmal palpitation. A resting electrocardiogram showed a right bundle branch block and left axis deviation with a delta wave. During his EPS, AVRT was reproducibly induced and a manifest AP was localized and then ablated at the left posteroseptal site, resulting in disappearance of the delta wave. PSVT, however, recurred 1 month later and during a repeat EPS the tachycardia was proved to be AVRT due to a right anterior concealed AP. The right anterior AP was successfully ablated with RF-CA. Both patients remained asymptomatic for more than 3 years following the successful ablation procedures.  相似文献   

11.
OBJECTIVE: To describe our initial experience with transcatheter radiofrequency ablation, a useful new treatment for supraventricular tachyarrhythmias related to the presence of an accessory atrioventricular (AV) pathway or dual atrioventricular nodal pathways. PATIENTS AND METHODS: One hundred and ten patients, including 77 with accessory pathways, 32 with dual atrioventricular (AV) nodal pathways and one with both, underwent electrophysiological studies and were treated with transcatheter radiofrequency ablation in a large metropolitan teaching hospital. RESULTS: Ninety-five patients (86%) were without evidence of accessory pathway conduction or inducible supraventricular tachycardia and were free of symptoms after a mean follow-up of 13 months (range, 3.0-51 months). Sixty-six of 79 accessory pathways (83.5%) were ablated including 42 of 46 left-sided (91%), 14 of 21 posteroseptal (66%), six of seven anteroseptal (86%), three of four right-sided and one of one midseptal pathways. Thirty-one patients with AV nodal reentry were successfully treated by ablation of either the slow (12 patients) or fast (19 patients) conducting AV nodal pathway. There was a progressive improvement in the success rate of the first procedure from 17% to 64% with the use of large-tip catheters and from 64% to 91% when a purpose-built radiofrequency generator was employed. Complications occurred in nine patients: cardiac tamponade (two patients); mild mitral regurgitation (four); subclavian vein thrombosis (one); transient cerebral ischaemic attack (one); and non-thrombocytic purpuric rash (one). These occurred predominantly during the early experience and were without long-term sequelae. Late in our experience, one patient developed complete atrioventricular block requiring permanent pacemaker implantation. CONCLUSIONS: In this institution, radiofrequency catheter ablation has been a safe and effective treatment strategy for patients with life-threatening or highly symptomatic supraventricular arrhythmias.  相似文献   

12.
The membranous portion of the interventricular septum represents the final phase of the ventricular growth. It is situated between the orifice of the coronary sinus and the supraventricular crest; immediately below the right aortic semilunar valves and not the coronary. Four cases of aneurism of the membranous portion of the interventricular septum are presented; in two, the diagnosis was made by angiocardiography study and in the rest it was made with the findings of an autopsy. All were of the female sex. Two patients presented a systolic murmur in the low mesocardia; three had heart failure, two of which were secondary to an arteriovenous short circuit through an interventricular communication, and the other due to alternations in the automatism and in the atrioventricular circulation. One case had W-P-W, type A and during its evolution presented paroxysms of atrial fibrillation and flutter, variable degrees of atrioventricular block with Stokes-Adams syndrome and ventricular fibrillation. In one case an obstruction at the level of the outflow tract of the right ventricle was suspected through phonocardiographic studies, and was confirmed subsequently with hemodynamic study. This same case presented a protosystolic aortic snap, at 0.13-0.14 sec. of the q wave of the electrocardiogram, described as of value for the diagnosis of this malformation. In two cases the angiocardiographic study showed the presence of the aneurism in the membranous portion of the interventricular septum, in one, it was visualized in the posterioanterior projection and in another in the lateral. One of the specimens had the aneurism adhered to the tricuspid septal valve, and also a fissure which communicated the left ventricle with the right atrium. In the other, the aneurismal sac was located below the septal valve of the tricuspid, producing a distortion in the anatomical architecture of the atrioventricular orifice.  相似文献   

13.
A 15-year-old female with Ebstein's anomaly was referred to hospital for radiofrequency (RF) current catheter ablation of her refractory paroxysmal supraventricular tachycardia (PSVT) after tricuspid valve replacement. A surface ECG showed ventricular preexcitation of type B Wolff-Parkinson-White (WPW) syndrome. In a baseline electrophysiological study, two types of PSVT with left and right bundle branch block (LBBB and RBBB) configurations were induced. The LBBB type was antidromic and the RBBB type was orthodromic atrioventricular reciprocating tachycardia (AVRT) with a right posterolateral accessory pathway. RF current was successfully delivered at the posterolateral site above the prosthetic valve (V-delta interval = -30 msec). The patient has been free from arrhythmias during a follow-up period of 9 months. RF current ablation seems to be useful for AVRT patients with corrected Ebstein's anomaly.  相似文献   

14.
The results are reported of a study of 83 necropsied hearts with atresia of the right atrioventricular orifice. It is emphasised that right atrial or atrioventricular orificial atresia is a better term to describe this anomaly than "tricuspid atresia". Use of the latter term can be confusing when the morphologically tricuspid valve is located beneath the left atrium. It is accepted that the definition employed may include cases in which the mitral valve may be atretic, blocking normal exit from the right atrium, but it is argued that such cases would present clinically as "tricuspid atresia" and therefore are correctly designated as right atrial orificial atresia. The results show that the majority of hearts with right atrial orificial atresia have the ventricular morphology of primitive ventricle, most with, but a few without an outlet chamber. However, in a minority of hearts an imperforate membrane interposes between the right atrium and a formed but hypoplastic right ventricle. In two of the hearts, the imperforate membrane showed features of Ebstein's malformation. The hearts could be further subdivided according to the ventriculoarterial connection. Most had normally connected arteries (66 of 83), and all but 2 also had normal relations between the arteries; in these 2 hearts there was "anatomically corrected malposition". Twelve hearts showed transposition, one had double-outlet outlet chamber, and another persistent truncus arteriosus. The remaining 3 hearts, all without outlet chamber, had by definition a double outlet connection. A segmental approach provides the best way of classifying this anomaly, and an embryological explanation is offered for the variations in anatomy observed.  相似文献   

15.
Since June 1987, 10 of 19 consecutive patients with atrioventricular and ventriculoarterial discordance (average age 4 +/- 2 years) had undergone a double switch operation with the morphologically left ventricle used as a systemic ventricle. There were two combinations of procedures. Atrial switch combined with arterial switch was used in two patients who had a normal pulmonary valve. Atrial switch combined with ventriculoarterial switch by Rastelli's procedure was used in eight patients with pulmonary stenosis or atresia and a large ventricular septal defect. One early death and two late deaths have occurred in a postoperative follow-up period of up to 4 years. Subsequent problems were mainly related to the results of atrial switch procedures in patients who had a small atrium because of low pulmonary flow, especially in patients with apicocaval juxtaposition. Our experience suggested that the double switch operation would open a new era of definitive surgical treatment in half of the patients with atrioventricular and ventriculoarterial discordance.  相似文献   

16.
HISTORY AND CLINICAL FINDINGS: A 28-year-old woman was admitted after syncope which had been preceded by several flulike episodes. There was no history of any other serious disease. Physical examination was unremarkable. Heart sounds were regular and normal, there were no murmurs. INVESTIGATIONS: White cell count was 9400/microliter, with a normal differential count. Erythrocyte sedimentation rate and C-reactive protein were also normal. Virus serology revealed no abnormality. The electrocardiogram (ECG) showed complete (third degree) atrioventricular (AV) block with an idioventricular rhythm of 38 beats/min and right bundle branch block pattern. TREATMENT AND COURSE: A temporary transvenous pacemaker was inserted on the first hospital day. As myocarditis was suspected a right ventricular endomyocardial biopsy was obtained. Histological and immunohistological examinations demonstrated no unequivocal findings. But molecular-biological tests revealed. Coxsackie-B3 virus genome. The pacemaker was removed on the 6th day, when the ECG had shown intermittent second degree AV block. Regular sinus rhythm with a PR interval of 0.18 s was recorded on day 12, and 24-hour ECG monitoring for several days until her discharge on the 18th day confirmed this rhythm throughout. CONCLUSION: In aetiologically undetermined disease molecular-biological techniques can be indispensable for the exact diagnosis and may be decisive for administering specific treatment.  相似文献   

17.
BACKGROUND: Though repair of complete atrioventricular septal defect in infancy has become routine at most centers, it is not unusual for very young infants to be managed medically because of concerns about the fragility of the atrioventricular valve tissue. METHODS: Since July 1992, seventy-two infants have undergone primary repair of complete atrioventricular septal defects at a median age of 3.9 months (40% < 3 months). A single-patch technique was used in all patients. The cleft was closed completely in 61 patients and partially (n = 10) or not at all (n = 1) in select patients at risk for valve stenosis. Left atrioventricular valve annuloplasty was performed in 18 patients. On the basis of transesophageal echocardiographic findings, 10 patients were returned to bypass for revision of the valve repair. RESULTS: There was one early death in a patient with single left papillary muscle, no early reoperations, and no new permanent arrhythmias. Only three patients had moderate left atrioventricular valve regurgitation at discharge. During a median follow-up of 24 months, there was one late death and five reoperations for left atrioventricular valve regurgitation (n = 2) and/or systemic outflow obstruction (n = 4). Follow-up left atrioventricular valve regurgitation was moderate in three patients, mild in 14, and none/trace in 54. Age had no relation to postoperative atrioventricular valve regurgitation, death, or reoperation. CONCLUSIONS: Despite concerns about fragility of valve tissue in very young patients, excellent results can be achieved with meticulous techniques. From neonates to older infants, age at repair does not influence outcome or valve function.  相似文献   

18.
Aortic outflow tract obstruction can complicate the clinical course and surgical management of patients with heterotaxy syndromes, but its anatomic basis has not been described in detail. In 20 postmortem cases with asplenia (n = 4) or polysplenia (n = 16), the anatomic causes of aortic outflow tract obstruction were absence of the subaortic conus in association with (1) narrowing of the subaortic outflow tract between the conal septum anteriorly and the common atrioventricular (AV) valve posteriorly in six (30%) patients; (2) aortic valvar atresia in four (25%), three with asplenia and one with polysplenia; (3) redundant AV valve leaflets in four (20%); (4) excessive AV valve fibrous tissue in four (20%); (5) marked hypoplasia of the mitral valve and left ventricle in two (10%); and (6) aneurysm of membranous septum in one (5%). One patient belonged to group (1) and (4). Aortic outflow tract obstruction was much more common with polysplenia (28%) than with asplenia (4%) (p < 0.001).  相似文献   

19.
BACKGROUND: This study examined the septal cleft and septal commissure of the left atrioventricular (AV) valve, which are two different anatomical structures. METHODS: We presented 36 cases of adult partial atrioventricular septal defect. A distinction was made between patients based on the anatomy of the anterior leaflet of the left AV valve. The left AV valve appeared to be normal or to have minimal radial openings from the free edge of the anterior leaflet of the left AV valve in 10 patients (28%). There was a septal commissure structure in 8 (22%), and a septal cleft structure in 18 (50%) patients. In the commissure type anatomy, leaflet coaptation was usually adequate and no or mild degree of left AV regurgitation existed preoperatively. Cleft type structure usually was associated with some degree of left AV regurgitation. Attempts were made to close the septal clefts and leave the septal commissures unsutured during the repair of the partial AV septal defects. RESULTS: We have not found any increase of left AV regurgitation in patients with commissures during the follow-up period. Closure of the cleft successfully eliminated regurgitation. Long-term results for septal cleft and septal commissure after repair of partial AV septal defect were excellent with survival of 100% and freedom from reoperation of 100% at mean 6.5 years. CONCLUSIONS: Septal cleft and septal commissure should be considered two different structures. Repairing procedures for left AV valve abnormalities associated with partial AV septal defect should only be done in patients who have cleft type of leaflet structure.  相似文献   

20.
Radiofrequency transcatheter ablation of ventricular tachycardia in the setting of a prior myocardial infarction is typically performed with application of energy to the left ventricular endocardium. In this article, two cases are described in which successful radiofrequency transcatheter ablation of ventricular tachycardia occurred with energy delivery to the right ventricular septum after failed ablation attempts from the left ventricle. Both patients had tachycardias with a left bundle branch block morphology and markedly presystolic activity recorded from the right ventricular septum. Right ventricular septal activation mapping during ventricular tachycardia should be performed in patients with left bundle branch block tachycardia morphology and coronary artery disease to maximize efficacy of the catheter ablation procedure.  相似文献   

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