Color Doppler detection of multiple ventricular septal defects

Combined two-dimensional and Doppler echocardiography has a high sensitivity and specificity for detection of isolated perimembranous ventricular septal defects. However, muscular or multiple ventricular septal defects may be difficult to diagnose with noninvasive methods, particularly in older children, necessitating angiography for accurate diagnosis. Detection of single and multiple ventricular septal defects with two-dimensional color flow mapping was compared with detection by standard two-dimensional imaging and Doppler. Both techniques were compared with four-chamber left ventricular angiography. Fifty-one patients (age 3 months to 25 years, mean 5.6 years) were studied. Eighteen had solitary ventricular septal defects, 18 had multiple ventricular septal defects, and 15 patients with intact ventricular septum served as a control group. At least one ventricular septal defect was detected by color Doppler and two-dimensional/Doppler methods in all patients with ventricular septal defect proved by angiography with no false positives. In the detection of multiple ventricular septal defects, the sensitivity of color Doppler was 72% and that of two-dimensional/Doppler was 38% (100% specificity in both). Color Doppler failed to identify multiple ventricular septal defects in five patients (two weighing less than 4 kg and three with reduced pulmonary blood flow). However, no large additional muscular defects were missed by imaging and color Doppler. Color Doppler is useful for the detection of ventricular septal defects and has higher sensitivity than two-dimensional/Doppler for multiple ventricular septal defects. The contribution of color Doppler appears to be in the detection of additional small muscular ventricular septal defects.     

国产室间隔缺损封堵器与Amplatzer封堵器的疗效比较

目的比较国产室间隔缺损封堵器与Amplatzer封堵器在经导管室间隔缺损封堵术中的疗效。方法41例患儿采用Amplatzer封堵器,76例患儿采用国产封堵器进行室间隔缺损封堵术,对比两组的疗效、并发症和费用等情况。结果国产组75例封堵成功（成功率99%）,进口组40例封堵成功（成功率98%）。两组患儿术前各项临床指标、手术和X线曝光时间、封堵成功率及住院天数,术后各项并发症发生率比较差异无统计学意义,两组患儿治疗前后左室舒张末径、收缩末径及C/T值减少程度无明显差异,治疗总费用进口组明显高于国产组。结论国产室间隔缺损封堵器与Amplatzer封堵器相比较其疗效、并发症发生率无显著差异,治疗费用低,临床应用前景广泛。     

Doppler assessment of interventricular pressure gradient across isolated ventricular septal defect

Continuous wave Doppler ultrasound was used to estimate the pressure gradient between the right and left ventricle for assessment of pulmonary arterial systolic pressure in 30 patients with isolated ventricular septal defect and for subsequent comparison with similar data obtained on cardiac catheterization. The age of the patients ranged from 8 months to 45 years (6.8 +/- 8.6 years). No patient had right or left ventricular outflow tract obstruction. Doppler measurements were done within 24 h of cardiac catheterization. Pressure gradient across ventricular septal defect on cardiac catheterization ranged from 7 to 95 mmHg (48 +/- 24 mmHg) and that on Doppler assessment ranged from 8 to 78 mmHg (42 +/- 20 mmHg). Doppler measurements of interventricular pressure gradient correlated well with those obtained on cardiac catheterization (r = 0.90, p less than 0.001). Correlation was better in patients with pressure gradient across ventricular septal defect less than 75 mmHg (r = 0.96). Correlation was poor in three of five patients with very small ventricular septal defects (interventricular pressure gradient greater than 75 mmHg) because the jet used was not ideal. Thus continuous wave Doppler ultrasound is an accurate noninvasive means of measuring pressure gradient across ventricular septal defect, which is a useful parameter for assessment of pulmonary artery systolic pressure in patients with isolated ventricular septal defect without right and left ventricular outflow tract obstruction.     

急性心肌梗死并发室间隔穿孔六例介入治疗

目的 评价Amplatzer肌部室间隔封堵器封堵室间隔穿孔(VSR)和同期经皮腔内冠状动脉成形术(PTCA)及冠脉支架术联合介入方法治疗急性心肌梗死(AMI)并发VSR的可行性及安全性.方法 6例患者术前均经超声心动图检查诊断为AMI并发心尖部VSR,出现心原性休克后均予主动脉球囊反搏(IABP)、机械通气支持.于3周后行室间隔封堵术,同期完成PTCA及支架术.结果 6例子Amplatzer肌部室间隔封堵器成功封堵VSR,2例有少量残余漏;同期冠状动脉造影3例患者为前降支中段及回旋支局限性狭窄,分别予植入1～2枚支架,另外3例患者为多支多段狭窄,未予以植入支架;3例并行支架的患者存活出院.结论 应用Amplatzer肌部室间隔封堵器封堵VSR同期行PTCA及冠状动脉支架术治疗AMI并发VSR是安全可行的.     

Superior QRS axis in ventricular septal defect

The relation between the superior orientation of the QRS axis and the anatomical site of the defect in the ventricular septum in patients with a ventricular septal defect was studied. Of 1031 patients with a ventricular septal defect, 64 (6.2%) had a superior axis on their electrocardiogram. In 59% of these patients the defect was in the inlet portion of the ventricular septum or affected this area. None of the cases was classified as an isolated ventricular septal defect of the persistent atrioventricular canal type. While there is an association between a superior axis and perimembranous inlet ventricular septal defect, a superior QRS axis does not characterise a ventricular septal defect as being of the atrioventricular canal type.     

Ventricular septal motion and left ventriclular dimensions during abnormal ventricular activation.

To determine the effect of abnormal ventricular activation on ventricular septal motion, left ventricular endocardial motion and left ventricular dimensions, 12 patients with normal motion were studied with echocardiography during incremental pacing of the right ventricular apex, outflow and inflow regions. Three types of abnormal ventricular septal motion were seen: The type I pattern was characterized by an early rapid preejection posterior ventricular septal motion followed by another posterior systolic motion that lasted throughout ejection, both of which were associated with septal thickening. In the type II pattern an early rapid preejection posterior ventricular septal motion was followed by an anterior ejection motion; the latter was not accompanied by septal thickening. The type III pattern consisted of an early preejection posterior ventricular septal motion followed by a mid and late systolic posterior motion: the latter motion extended through diastole. During right ventricular apical pacing, 8 of 11 patients showed a type 1 pattern, 1 a type II pattern and 2 a normal septal motion. During right ventricular outflow pacing,seven of nine patients showed a type II pattern, one a type III pattern and one a type I pattern. During right ventricular inflow pacing, eight of nine patients showed a type II pattern and one a type III pattern. At faster pacing rates patterns of types I and III changed to a type II pattern (five patients). End-diastolic dimensions decreased significantly during incremental right ventricular pacing when compared with those during sinus rhythm. End-systolic dimensions decreased significantly only during right ventricular apical and outflow pacing at maximal rates. In the seven patients who had pacing from all three sites, the decrease in left ventricular dimensions did not significantly differ when the three pacing sites were compared. These findings suggest that (1) abnormal ventricular septal motion during right ventricular pacing (induced left bundle branch block patterns) is dependent on the sequence of ventricular activation; (2) ventricular septal motion during right ventricular outflow and inflow pacing is similar to that seen in spontaneous left bundle branch block, whereas the pattern of septal motion during right ventricular apical pacing is different from that of spontaneous left bundle branch block; and (3) changes in left ventricular dimension are dependent on ventricular pacing rate but independent of pacing site.     

Evaluation of aortic valve prolapse in ventricular septal defect

Angiographic criteria for the recognition of aortic valve prolapse in isolated ventricular septal defect were based on the degree of aortic cusp deformity and the presence or absence of aortic regurgitation. Ninety eight consecutive patients with isolated perimembranous or infundibular ventricular septal defects who were catheterised and had aortography performed were reviewed. They included five with postoperative ventricular septal defects and three with additional mild right ventricular outflow tract obstruction. Eighteen were found to have aortic valve prolapse. Although eight of the 18 were noted to have aortic regurgitation angiographically, only three had an early diastolic murmur. Only eight of the 18 patients had cross sectional echocardiographic findings suggestive of prolapse. All of these had at least moderate prolapse angiographically. Cross sectional echocardiography was found to be insensitive in diagnosing mild degrees of aortic valve prolapse. A trend towards a decreasing left to right shunt was noted as the degree of aortic valve prolapse increased. Spontaneous decrease in the size of a ventricular septal defect may be due to unrecognised aortic valve prolapse without clinical evidence of aortic regurgitation.     

Superior QRS axis in ventricular septal defect.

The relation between the superior orientation of the QRS axis and the anatomical site of the defect in the ventricular septum in patients with a ventricular septal defect was studied. Of 1031 patients with a ventricular septal defect, 64 (6.2%) had a superior axis on their electrocardiogram. In 59% of these patients the defect was in the inlet portion of the ventricular septum or affected this area. None of the cases was classified as an isolated ventricular septal defect of the persistent atrioventricular canal type. While there is an association between a superior axis and perimembranous inlet ventricular septal defect, a superior QRS axis does not characterise a ventricular septal defect as being of the atrioventricular canal type.     

Transcatheter occlusion of ventricular septal defect

The patent ductus arteriosus occlusion device (Rashkind-USCI) was employed to occlude a residual ventricular septal defect after Fontan procedure in a patient with hypoplastic right ventricle, ventricular septal defect, and pulmonary stenosis. There was significant right-to-left shunting across the ventricular septal defect, with cyanosis exacerbated by exercise. After placement of the 17 mm occlusion device, the right-to-left shunt was markedly diminished, and the cyanosis resolved. Occlusion of ventricular septal defects in patients with complex congenital heart disease may be performed with the patent ductus arteriosus occluder in selected instances.     

Abnormal right ventricular size and ventricular septal motion after atrial septal defect closure: etiology and functional significance.

Postoperative echocardiogram often demonstrate persistent right ventricular dilatation and paradoxic ventricular septal motion after repair of an atrial septal defect. To determine the prevalence, causes and significance of these echocardiographic abnormalities, 31 patients were studied with catheterization and echocardiography before and after repair of an atrial septal defect. Before operation, every patient manifested right ventricular dilatation, and all but one had abnormal septal motion. After operation, right ventricular dilatation was noted in 24 (77%) and abnormal septal motion in 21 (68%) patients despite the absence of residual left to right shunting in 30 (97%). These echocardiographic abnormalities could be correlated with age at operation and length of postoperative follow-up study but did not correlate with the degree of preoperative right ventricular enlargement or with shunt size or right ventricular pressure before or after operation. There was no associated functional deficit as demonstrated by the normal maximal oxygen consumption in all 13 patients who underwent treadmill exercise testing 5 to 38 months after operation; these patients included 9 with persistent right ventricular enlargement and abnormal septal motion.     

Traumatic ventricular septal defect.

A 26 year old man was admitted to hospital following a traffic accident. He had been sitting in the back of a car without wearing a seat belt. He suffered crush injuries on the anterior chest wall, trunk, and legs. On admission he was awake and cooperative, but restless, and obviously in severe pain. Radiography of the skull, facial bones, chest, spine, pelvis, and legs revealed a shaft fracture of the left femur and tibia and fracture of the 7th and 8th right ribs. The patient was transferred to the University Hospital of Zurich for further assessment and surgical repair of the lower limb fractures three days later. Because of worsening clinical condition with onset of partial respiratory insufficiency and new loud systolic murmur at the left sternal edge, a transthoracic echocardiography was performed, which showed an apical ventricular septal defect. Surgery was performed immediately. The ventricular septal defect was successfully repaired using a Teflon felt patch and interrupted sutures with pledgets, and sealed with glue. At six months' follow up the patient was doing well. Ventricular septal defects after blunt chest trauma occur either because of heart compression between sternum and the spine or because of myocardial infarction. In the present case the ventricular septal defect appeared three days after the accident, probably secondary to a post-traumatic myocardial infarction. Patients with blunt chest trauma and suspicion of cardiac contusion should be monitored carefully.     

Echocardiographic features of supracristal ventricular septal defect with prolapsed aortic valve leaflet.

Anatomically diagnostic echocardiographic features of a supracristal ventricular septal defect with prolapsed right coronary aortic leaflet are described in four children aged 2 to 10 years. Both single crystal M mode as well as 80 ° phased array sector scan techniques were used. The echographic features in the M mode scan from the aorta to the left ventricle in three of four patients included (1) the position of the ventricular septal defect as a clear space between the interrupted septal echoes below the aortic root, and (2) the prolapsed right coronary aortic leaflet as anomalous linear echoes in the right ventricular outflow tract. Angiographic, intraoperative and echocardiographic contrast studies were used to establish the diagnosis.On sector scanning using the long axis view, the supracristal ventricular septal defect was recognized as a clear space between the top of the ventricular septum and the anterior segment of the aortic root in three of four patients. The right coronary aortic leaflet was seen to prolapse into the right ventricular outflow tract through this defect, and its motion could be clearly followed during systole and diastole. It is concluded that echocardiography provides anatomic diagnosis of this lesion. Furthermore, the severity and progression of this lesion can be assessed by quantitation of the left ventricular size and performance.     

Echocardiographic evaluation of ventricular septal aneurysms.

The spontaneous closure of ventricular septal defects is frequently associated with septal aneurysm formation. In this paper we discuss the M-mode and two-dimensional echocardiographic findings in nine children with aneurysms of the ventricular septum in association with ventricular septal defects. In all patients the diagnosis was confirmed by angiography. The ventricular septal aneurysms were detected by both M-mode and two-dimensional echocardiography. With M-mode echocardiography, septal aneurysms could be recognized by a pattern of multiple systolic echoes within the right ventricle. With two-dimensional echocardiography, the protrusion of the septal aneurysm into the right ventricle could be seen from several views and the location and the relative size of the aneurysm assessed. Echocardiographic techniques useful in the detection of ventricular septal aneurysms are discussed and examples presented.     

Transcatheter closure of postinfarction ventricular septal defects using Amplatzer devices

We carried out transcatheter procedures to close postinfarction ventricular septal defects (PIVSDs) in 19 patients: two had recanalization after surgical closure, and 17 had a primary PIVSD. In three of the latter patients, who had acute PIVSDs, the procedure was carried out in the first 3 weeks after infarction; in the 13 patients with subacute PIVSD, it was carried out 3.5-12 weeks after infarction. There was another procedure in one patient with chronic PIVSD. In total, 22 procedures were completed: 17 using an Amplatzer atrial septal occluder, two using an Amplatzer postinfarction ventricular septal defect occluder, and two using an Amplatzer muscular ventricular septal defect occluder. The procedure was successful in 14 patients: in 11 with subacute PIVSD, one with chronic PIVSD, and two with postsurgical PIVSD. Transcatheter closure of PIVSDs using an Amplatzer atrial septal occluder is probably the treatment of choice in patients undergoing surgery more than 3.5 weeks after myocardial infarction and in those with recanalization after previous surgical closure.     

应用国产封堵器治疗嵴内型室间隔缺损与膜周型室间隔缺损的对比研究

目的:探讨国产封堵器介入治疗室间隔缺损(VSD)的适应证,并比较其治疗嵴内型VSD和膜周型VSD的差别。方法:VSD患者共32例,其中嵴内型VSD组6例,膜周型VSD组26例,比较2组的手术步骤及方法,总的手术时间及建轨时间,封堵成功率,术后残余分流及并发症发生情况。结果:嵴内型组总手术时间120~170min,平均147min,其中建立轨道时间28~87min,平均47min;膜周型组总手术时间70~280min,平均105min,其中建立轨道时间15~110min,平均34min。2组比较,嵴内型组的总手术时间及建立轨道时间均明显长于膜周型组(均P<0.01);两者封堵成功率均为92%以上,差异无统计学意义(P>0.05);2组患者术中均出现一过性心律失常,改变导管位置或撤离导管后均立即消失,膜周型组1例患者术后第4天发生脑栓塞,经脑血管介入溶栓治疗症状缓解出院,2例术后12h内发生完全性左束支传导阻滞,均经激素治疗后3d内恢复正常。术后24h复查超声心动图,嵴内型组无残余分流,膜周型组1例存在少量残余分流。结论:应用国产封堵器介入治疗嵴内型VSD能达到和膜周型VSD封堵同样的疗效,另外,应用国产封堵器除其明显的价格优势外,对于嵴内型VSD,国产偏心型封堵器较进口Amplatzer封堵伞更具优势。     

Transcatheter closure of muscular ventricular septal defect using the Amplatzer devices

TECHNIQUE: From November 1997 to June 2002, percutaneous transcatheter closure of muscular ventricular septal defects was attempted in seven patients. Four patients had single and three had multiple defects. Surgical closure was performed in two patients in an attempt to close a perimembranous defect, leaving behind a large apical muscular defect, which was successfully closed using a device in one patient, whilst the second patient succumbed to septicemia/endocarditis 3 weeks after failure of device implantation. One patient had previous pulmonary artery banding and in another intraoperative placement of two Clamshell devices followed by additional transcatheter closure using Gianturco coils in two different sessions was performed. RESULTS: Transcatheter closure of ventricular septal defect was successful in six patients. Three patients underwent closure of their ventricular septal defects using the Amplatzer Septal Occluder and in the remaining three patients. Amplatzer Muscular Ventricular Septal Defect Occluder was used. The median age was 15 months (range 14 months to 10 years) and the median weight was 7.8 kg (range 7-16 kg). The devices were deployed antegradely in all patients. Immediate complete closure was obtained in three patients while two patients had small and one had a large residual following the procedure. The later was due to another multiple muscular septal defect. Transient heart block occurred in one patient during the procedure and another patient developed heart block on day 3 post-procedure. The latter required temporary pacemaker. During the follow up, one patient who had residual multiple muscular defects underwent a successful redo transcatheter closure using two Amplatzer Muscular Ventricular Septal Occluder devices. CONCLUSIONS: We conclude that transcatheter closure of muscular ventricular septal defect using Amplatzer devices is feasible and effective.     

Colour flow imaging in the diagnosis of multiple ventricular septal defects

Thirty one patients with multiple ventricular septal defects were studied by cross sectional echocardiography, conventional pulsed and continuous wave Doppler, colour flow imaging, and left ventriculography to determine the relative diagnostic benefits and pitfalls of each technique. The patients studied had a wide range of congenital heart defects with 19 patients having isolated multiple ventricular septal defects, three with associated tetralogy of Fallot, five with double outlet right ventricle, three with complete transposition and ventricular septal defect, and one with a complete atrioventricular septal defect. In 23 patients the defects were inspected at operation. Cross sectional imaging with integrated pulsed and continuous wave Doppler correctly identified multiple defects in only 12 (39%) patients. In contrast, colour flow imaging was accurate in 24 (77%) patients and left ventriculography in 20 (65%) patients. When patients were subdivided on the basis of relative peak systolic ventricular pressures into restrictive defects (18 patients) and non-restrictive defects (13 patients) the diagnostic value of colour flow imaging was different for each group. Colour flow mapping correctly identified multiple ventricular septal defects in 16/18 (89%) patients with restrictive defects but only 8/13 (62%) with non-restrictive defects. The comparative diagnostic accuracy of left ventriculography was 15/18 (83%) in the restrictive group and 5/13 (38%) in the non-restrictive group. Colour flow imaging was the single investigative technique with the greatest diagnostic accuracy in the diagnosis of multiple ventricular septal defects. It failed to be consistently accurate in defined subgroups with non-restrictive defects as did left ventriculography. The greatest overall diagnostic accuracy in this series was obtained when both colour flow imaging and ventriculography techniques were used in combination in a complementary fashion.     

Colour flow imaging in the diagnosis of multiple ventricular septal defects.

Thirty one patients with multiple ventricular septal defects were studied by cross sectional echocardiography, conventional pulsed and continuous wave Doppler, colour flow imaging, and left ventriculography to determine the relative diagnostic benefits and pitfalls of each technique. The patients studied had a wide range of congenital heart defects with 19 patients having isolated multiple ventricular septal defects, three with associated tetralogy of Fallot, five with double outlet right ventricle, three with complete transposition and ventricular septal defect, and one with a complete atrioventricular septal defect. In 23 patients the defects were inspected at operation. Cross sectional imaging with integrated pulsed and continuous wave Doppler correctly identified multiple defects in only 12 (39%) patients. In contrast, colour flow imaging was accurate in 24 (77%) patients and left ventriculography in 20 (65%) patients. When patients were subdivided on the basis of relative peak systolic ventricular pressures into restrictive defects (18 patients) and non-restrictive defects (13 patients) the diagnostic value of colour flow imaging was different for each group. Colour flow mapping correctly identified multiple ventricular septal defects in 16/18 (89%) patients with restrictive defects but only 8/13 (62%) with non-restrictive defects. The comparative diagnostic accuracy of left ventriculography was 15/18 (83%) in the restrictive group and 5/13 (38%) in the non-restrictive group. Colour flow imaging was the single investigative technique with the greatest diagnostic accuracy in the diagnosis of multiple ventricular septal defects. It failed to be consistently accurate in defined subgroups with non-restrictive defects as did left ventriculography. The greatest overall diagnostic accuracy in this series was obtained when both colour flow imaging and ventriculography techniques were used in combination in a complementary fashion.     

Evaluation of aortic valve prolapse in ventricular septal defect.

Angiographic criteria for the recognition of aortic valve prolapse in isolated ventricular septal defect were based on the degree of aortic cusp deformity and the presence or absence of aortic regurgitation. Ninety eight consecutive patients with isolated perimembranous or infundibular ventricular septal defects who were catheterised and had aortography performed were reviewed. They included five with postoperative ventricular septal defects and three with additional mild right ventricular outflow tract obstruction. Eighteen were found to have aortic valve prolapse. Although eight of the 18 were noted to have aortic regurgitation angiographically, only three had an early diastolic murmur. Only eight of the 18 patients had cross sectional echocardiographic findings suggestive of prolapse. All of these had at least moderate prolapse angiographically. Cross sectional echocardiography was found to be insensitive in diagnosing mild degrees of aortic valve prolapse. A trend towards a decreasing left to right shunt was noted as the degree of aortic valve prolapse increased. Spontaneous decrease in the size of a ventricular septal defect may be due to unrecognised aortic valve prolapse without clinical evidence of aortic regurgitation.     

Congenital heart malformations associated with disproportionate ventricular septal thickening.

Asymmetric septal hypertrophy, or ASH, is a genetically determined myocardial disorder that is transmitted as an autosomal dominant trait. ASH is characterized by a disproportionately thickened ventricular septum that contains numerous hypertrophied, bizarrely-shaped and disorganized cardiac muscle cells. Disproportionate hypertrophy of the ventricular septum has also been observed in association with certain congenital cardiac malformations. To determine whether such congenital cardiac malformations are part of the disease spectrum of genetically determined ASH, cardiac pathologic observations were made in eight patients with disproportionate septal thickening (ventricular septal to posterobasal left ventricular free wall thickness ratios of 1.5 to 2.5) and the following three categories of associated lesions: 1) parachute deformity of the mitral valve (occurring either as an isolated lesion or with ventricular septal defect, coarctation of the aorta, supravalvular ring of the left atrium, or double outlet right ventricle); 2) complete interruption of the aortic arch; and 3) ventricular septal defect. The arrangement of cardiac muscle cells in the disproportionately thickened ventricular septum was normal in six of the eight patients; in the other two patients (one with parachute deformity of the mitral valve and one with ventricular septal defect) numerous bundles of hypertrophied cardiac muscle cells were interlaced in a disorganized fashion among more normally arranged bundles of cells. First degree relatives of six of the eight patients were studied by echocardiography and found to have normal ventricular wall thicknesses and septal-free wall ratios. It is concluded that disproportionate ventricular septal thickening may occur in patients with a variety of congenital heart malformations, but that such a finding is not necessarily a manifestation of the disease spectrum of genetically determined ASH.