Comparison of left ventricular outflow tract obstruction in interruption of the aortic arch and in coarctation of the aorta, with diagnostic, developmental, and surgical implications

A morphometric comparison of the anatomic causes of left ventricular (LV) outflow obstruction in interruption of the aortic arch and in coarctation of the aorta with ventricular septal defect (VSD), based on 30 postmortem cases of each, revealed that posterior malalignment of the conal septum with a conoventricular VSD was significantly more prevalent with interruption (93%) than with coarctation (47%) (p <0.001). The ratio of the aortic valve diameter-to-the pulmonary valve diameter, which provided a quantitative index of the degree of posterior conal septal malalignment and of the consequent LV outflow tract obstruction at and immediately below the level of the aortic valve, was significantly smaller with interruption (相似文献   

Coarctation of the aorta in early infancy. Left ventricular outflow tract obstruction and its clinical implications

In order to clarify the clinical significance of left ventricular outflow tract obstruction, 11 infants with coarctation of the aorta and ventricular septal defect were subjected to cineangiography (or autopsy) analysis. Three infants without left ventricular outflow tract obstruction were salvaged by aortoplasty using a subclavian flap or a Dacron patch with concomitant pulmonary artery banding. The remaining 8 infants died surgically or nonsurgically, and were confirmed retrospectively to have had left ventricular outflow tract obstruction. It seems worthwhile to stress that the presence of an abnormal muscle bundle in the left ventricle can be demonstrated angiographically, especially by the axial technique. From our present study, though the number of cases examined is limited, we have an impression that left ventricular outflow tract obstruction may be a determining factor with regard to the appropriateness of pulmonary artery banding in the two-stage operation for infantile coarctation associated with a large ventricular septal defect.     

Morphology of ventricular septal defect associated with coarctation of aorta.

It is now well established that the morphology of the ventricular septal defect associated with interrupted aortic arch is such as to compromise blood flow to the ascending aorta. Though there is some evidence that a similar mechanism may be operative in coarctation with ventricular septal defect, we are unaware of any necropsy study of this hypothesis. We therefore studied 25 hearts from the Heart Museum of Children's Hospital of Pittsburgh with coarctation and ventricular septal defect. Four had a malalignment defect with left ventricular outflow tract obstruction such as is seen in cases with interruption. Twenty of the remaining cases had a particular form of perimembranous defect with aortic overriding. The defects were partially closed by tricuspid valve tissue, the left ventricular outflow tract being further narrowed by various anatomical lesions. In addition, mitral valve malformations were present in 14 cases. The anatomy of the group as a whole suggests a possible reduction of aortic blood flow during fetal development. The clinical implications of these findings remain to be evaluated.     

Anatomic characteristics of ventricular septal defect associated with coarctation of the aorta

The morphologic characteristics of ventricular septal defect (VSD) and left ventricular outflow tract were studied in 45 hearts with VSD and coarctation of the aorta (C of A). Forty-one VSDs were classified into 1 of 4 categories. The first category includes 19 central muscular VSDs (43%), among which 3 hearts had the architecture of a spontaneously closed defect. The second category includes 10 perimembranous inlet defects (23%) with overlying tricuspid valve. The third category is formed by 8 perimembranous VSDs with leftward outlet septal malalignment (18%) and the fourth category includes 4 subarterial VSDs (8%) in which leftward malalignment of a deficient outlet septum results in subpulmonary localization of the defect. In addition to the main categories, 1 membranous, 1 perimembranous trabecular, 1 apical muscular and 1 doubly committed subarterial defects were identified. The left ventricular outflow tract was assessed as normal in only 2 cases. In 43 cases the left ventricular outflow tract was compromised by 1 or more of the following anomalies: anterolateral muscle bundle, anteroseptal twist, bicuspid aortic valve or a leftward malaligned outlet septum. These findings support the hypothesis of C of A being hemodynamically induced by anomalies of the left ventricular outflow tract. Previous studies have established that muscular, membranous and perimembranous inlet defects are prone to close spontaneously, in contrast to malalignment and subarterial VSDs. The present study shows that 70% of the VSDs belong to types characterized by a high incidence of spontaneous closure.     

Transposition of the great arteries and narrowing of the aortic arch. Emphasis on right ventricular characteristics

Of 279 patients with transposition of the great arteries, 14(5%) had narrowing of the aorta, including local coarctation (6), isthmus hypoplasia (6), isthmus atresia (1), and kinking of the aorta (1). There were six deaths in 10 surgically treated patients; in addition four patients died before operation. Two of the four survivors had a subpulmonary malalignment ventricular septal defect with angiocardiographic narrowing of the right ventricular outflow tract, two had evidence of redundant muscle tissue obstructing the right ventricular outflow tract. Necropsy showed the presence of anatomical right ventricular outflow tract or inflow tract obstruction or both in all 10 cases. Outflow tract obstruction was represented by anterior displacement of the infundibular septum and ventriculoinfundibular fold (in hearts with a subpulmonary malalignment ventricular septal defect) or by redundant muscle tissue; inflow tract obstruction was represented by hypoplasia of the tricuspid valve. It is concluded that the combination of transposition of the great arteries and narrowing of the aorta is always accompanied by right ventricular outflow or inflow tract obstruction or both; the right ventricular abnormalities are probably responsible for the presence of the aortic arch anomalies by reducing aortic flow during morphogenesis. Successful surgical treatment of this complex anomaly is feasible in selected cases.     

Transposition of the great arteries and narrowing of the aortic arch. Emphasis on right ventricular characteristics.

Of 279 patients with transposition of the great arteries, 14(5%) had narrowing of the aorta, including local coarctation (6), isthmus hypoplasia (6), isthmus atresia (1), and kinking of the aorta (1). There were six deaths in 10 surgically treated patients; in addition four patients died before operation. Two of the four survivors had a subpulmonary malalignment ventricular septal defect with angiocardiographic narrowing of the right ventricular outflow tract, two had evidence of redundant muscle tissue obstructing the right ventricular outflow tract. Necropsy showed the presence of anatomical right ventricular outflow tract or inflow tract obstruction or both in all 10 cases. Outflow tract obstruction was represented by anterior displacement of the infundibular septum and ventriculoinfundibular fold (in hearts with a subpulmonary malalignment ventricular septal defect) or by redundant muscle tissue; inflow tract obstruction was represented by hypoplasia of the tricuspid valve. It is concluded that the combination of transposition of the great arteries and narrowing of the aorta is always accompanied by right ventricular outflow or inflow tract obstruction or both; the right ventricular abnormalities are probably responsible for the presence of the aortic arch anomalies by reducing aortic flow during morphogenesis. Successful surgical treatment of this complex anomaly is feasible in selected cases.     

Reversed septal curvature: Association with primary pulmonary hypertension and Shone syndrome

In the normal heart the interventricular septum is curved concave to the left and functions as a component of the left ventricular wall. We have studied the hearts of five patients at autopsy, after postmortem angiography and fixation in distention, in which the septum was concave to the right ventricle. These patients ranged in age from eight months to 25 years (average, 16 years); three had primary pulmonary hypertension, and two had coarctation of the aorta and congenital aortic stenosis. Catheterization studies in the latter two patients had demonstrated subaortic stenosis. At autopsy there was no evidence of asymmetric septal hypertrophy or of abnormal septal myocardial fiber disarray. The right ventricles were markedly hypertrophied. In each, the septal curvature was reversed leading to protrusion of septal muscle into the left ventricular outflow tract with marked narrowing of the outflow tract. Since the septal configuration is determined early in life, this abnormality of ventricular topography may develop primarily or it may develop in hearts subject to early as well as long-standing right ventricular overload.     

Posterior deviation of left ventricular outflow tract septal components without ventricular septal defect.

OBJECTIVE: To describe 11 patients with narrowing of the left ventricular outflow tract caused by angular posterior deviation of both the outlet septum and the upper part of trabecular septum, which was diagnosed by cross sectional echocardiography in all and confirmed by angiocardiography in seven. RESULTS: Four patients had a subaortic systolic pressure gradient ranging from 23 to 70 mm Hg by Doppler echocardiography; cardiac catheterisation showed a significant (60 and 104 mm Hg) systolic pressure gradient in two. In four cases aortic regurgitation and two tricuspid pouches were shown by Doppler echocardiography, angiocardiography, or both. Four cases had a ridge at the angulation point on echocardiographic examination. Three patients were operated on for systolic pressure gradients of the left ventricular outflow tract and one for severe aortic regurgitation. There was proliferation of collagen-rich fibrous tissue in the subendocardial region on histopathological examination of the myectomy material. A ventricular septal defect had been diagnosed previously by contrast echocardiography in one patient; thus ventricular septal defects may close spontaneously over a period of time including fetal life. A subaortic ridge was detected in one patient at follow up. CONCLUSIONS: Deviation of the outlet and trabecular septa should be considered as a cause of ventricular outflow tract obstruction even when no ventricular septal defect is present.     

Subaortic stenosis due to antero-lateral muscular band, associated with ventricular septal defect and underdevelopment of the aortic arch. Anatomical features in three cases (author's transl)

The pathological findings in three cases with ventricular septal defect and severe coarctation or tubular hypoplasia of the aortic arch are reported. The left ventricular outflow tract was partially obstructed by muscle situated on its antero-lateral wall. The haemodynamic relationship between this type of subaortic stenosis and aortic arch underdevelopment, due to unequal partitioning of the blood flow between the ascending aorta and the pulmonary artery during foetal life, is emphasized. The normal anatomy of the left ventricular infundibulum is described and some embryological hypotheses to explain this antero-lateral muscular band are forwarded.     

Pulmonary atresia with intact ventricular septum associated with severe aortic stenosis

Pulmonary atresia with intact ventricular septum is the complete obstruction of the right ventricular outflow tract due to pulmonary valve atresia in the absence of ventricular septal defect. Pulmonary flow is dependent on the ductus arteriosus. Other morphological anomalies are also present. Aortic stenosis in association with pulmonary atresia with intact ventricular septum is extremely infrequent, and very few cases have been described.We report a 19-year-old primiparous woman in whom the fetal echocardiogram showed pulmonary atresia with intact ventricular septum. The neonate had low oxygen saturation and a systolic murmur originating in the aorta. An echocardiogram showed pulmonary atresia with intact ventricular septum and a bicuspid, thickened, stenotic aortic valve. Low cardiac output ensued and could not be controlled, and the infant died.     

The presentation of symptomatic heart disease in infancy based on 10 years' experience (1973-82). Implications for the provision of services

The diagnoses and age at presentation of 1665 infants with symptomatic heart disease, who were admitted to the Brompton Hospital, London, during the period 1973-82 were reviewed. The frequency of certain conditions had changed during the period of the study. Complete transposition of the great arteries and critical aortic stenosis had become less common, whereas the frequency of right ventricular outflow tract obstruction, and of critical pulmonary stenosis in particular, had increased. Several conditions, including coarctation of the aorta, hearts with univentricular-atrioventricular connexion, and double outlet ventricle, formed a higher proportion of cases in the present series than in the New England Regional Infant Cardiac Program study, whereas the proportion of cases of atrial septal defect, hypoplastic left heart syndrome, and pulmonary atresia with intact septum was smaller. Most of the cyanotic infants presented during the first 14 days of life at the time of ductal closure. As expected this was also true of other duct dependent circulatory disorders such as aortic coarctation and interrupted aortic arch. Acyanotic infants with potentially large left to right shunts tended to present during the second month of life, when the pulmonary vascular resistance fell. This study emphasises that most symptomatic infants with heart disease present during the first two months of life. As a consequence the provision of expert neonatal and infant medical services is an integral part of any paediatric cardiac service.     

Severe obstruction to systemic blood flow in congenitally corrected transposition (discordant atrioventricular and ventriculo-arterial connexions): an analysis of 14 patients

The more common associated cardiac anomalies in any cohort of patients with discordant atrioventricular and ventriculo-arterial connexions (congenitally corrected transposition) include ventricular septal defect, left ventricular (subpulmonary) outlet obstruction, and displacement and/or dysplasia of the morphologically tricuspid valve. There is scant mention in the literature of severe obstruction to systemic blood flow at aortic valve level or beyond in these hearts. The present study reviews those pertinent morphological-clinical and follow-up data on 14 patients with congenitally corrected transposition and aortic valve atresia (one patient); aortic arch interruption or aortic arch atresia (two patients) and coarctation of the aorta (11 patients). All but one patient (the patient with aortic valve atresia) had a perimembranous ventricular septal defect. Mild outflow tract obstruction of the morphologically left ventricle was identified in one patient. Nine patients, however, demonstrated a significant structural and functional disturbance (Ebstein-like with dysplasia) of the systemic morphologically tricuspid valve. No patient in this series was identified with isolated aortic coarctation.     

The presentation of symptomatic heart disease in infancy based on 10 years' experience (1973-82). Implications for the provision of services.

The diagnoses and age at presentation of 1665 infants with symptomatic heart disease, who were admitted to the Brompton Hospital, London, during the period 1973-82 were reviewed. The frequency of certain conditions had changed during the period of the study. Complete transposition of the great arteries and critical aortic stenosis had become less common, whereas the frequency of right ventricular outflow tract obstruction, and of critical pulmonary stenosis in particular, had increased. Several conditions, including coarctation of the aorta, hearts with univentricular-atrioventricular connexion, and double outlet ventricle, formed a higher proportion of cases in the present series than in the New England Regional Infant Cardiac Program study, whereas the proportion of cases of atrial septal defect, hypoplastic left heart syndrome, and pulmonary atresia with intact septum was smaller. Most of the cyanotic infants presented during the first 14 days of life at the time of ductal closure. As expected this was also true of other duct dependent circulatory disorders such as aortic coarctation and interrupted aortic arch. Acyanotic infants with potentially large left to right shunts tended to present during the second month of life, when the pulmonary vascular resistance fell. This study emphasises that most symptomatic infants with heart disease present during the first two months of life. As a consequence the provision of expert neonatal and infant medical services is an integral part of any paediatric cardiac service.     

Morphology of left ventricular outflow tract structures in patients with subaortic stenosis and a ventricular septal defect.

OBJECTIVE--To compare the incidence and prognosis of subaortic stenosis associated with a ventricular septal defect and to define the morphological basis of subaortic stenosis. DESIGN--Presentation and follow up data on 202 patients with subaortic stenosis seen at the Royal Liverpool Children's Hospital between 1 January 1960 and 31 December 1991 were reviewed. Survivors were traced to assess their current clinical state. Necropsy specimens of 291 patients with lesions associated with subaortic stenosis were also examined. RESULTS--In the clinical study; 65 (32.1%) of the 202 patients with subaortic stenosis had a ventricular septal defect (excluding an atrioventricular septal defect). 32 of these patients had a short segment (fibromuscular) subaortic stenosis. 33 had subaortic stenosis produced by deviation of muscular components of the outflow tracts. In 17 patients (51.5%) this was caused by posterior deviation or extension of structures into the left ventricular outflow tract, resulting in obstruction above the ventricular septal defect. In the other 16 patients (48.5%) there was over-riding of the aorta with concordant ventriculoarterial connections, (without compromise to right ventricular outflow) producing subaortic stenosis below the ventricular septal defect. Additional fibrous obstruction occurred in 39% of the patients with deviated structures. The age at presentation was lower (P < 0.01) in patients with deviated structures (median (range) 0.4 (0 to 9.2) months) than in those with short segment obstruction (median (range) 4.2 (0 to 84.9) months). The incidence of aortic arch obstruction was higher (P < 0.002) in patients with deviated structures than in those with short segment obstruction (38%). In the morphological study 35 pathological specimens showed obstructive muscular structures in the left ventricular outflow tract either above or below the ventricular septal defect. 16 had either posterior deviation of the outlet septum or extension of the right ventriculoinfundibular fold, or both of these together into the left ventricle. 19 had anterior deviation of the outlet septum into the right ventricle with overriding of the aorta (without compromise to right ventricular outflow). The earliest age at which additional fibrous obstruction was seen was 9 months. The aortic valve circumference was small in 18% of specimens. FOLLOW UP--The median (range) duration of follow up in survivors from the clinical study was 6.6 (1 to 25.7) years. 16 patients with deviated musculature (49%) and 16 with short segment fibromuscular stenosis (50%) underwent operation for subaortic stenosis. Patients with deviated structures were younger at operation than those with short segment stenosis (P < 0.005). Patients with posterior deviation or extension of structures into the left ventricular outflow tract underwent operation for subaortic stenosis more frequently (P < 0.05) than those with anterior deviation of the outlet septum and aortic override. The ventricular septal defect required surgical closure more frequently (P < 0.005) in patients with deviation (93.9%) than in those with short segment obstruction (21.9%). There was no significant difference in the mortality between patients with deviation (27%) and those with short segment obstruction (12%). CONCLUSIONS--32% of patients in the clinical study with subaortic stenosis had a ventricular septal defect. Only 51% of these had obstructive and deviated muscular structures in the left ventricular outflow tract. These patients had a significantly higher incidence of aortic arch obstruction and required surgery for subaortic stenosis at a younger age than those with short segment obstruction. The ventricular septal defect also required surgical closure more frequently in those patients with deviation. The morphological study defined the two sites of obstruction. The presence or absence and type of deviation should be clearly defined in all patients with a ventricular septal defect,     

Transposition of the great arteries: anatomic types of left ventricular outflow tract obstruction (author's transl)]

A postmortem investigation has been carried out in 48 heart specimens with transposition of the great arteries (TGA) to evaluate incidence and type of left ventricular outflow tract obstruction. The frequence of pulmonary stenosis in our series (23%) is similar to those reported from previous studies. Cases have been divided in two groups according to presence or absence of ventricular septal defect. In cases with intact ventricular septum the obstruction was determined by asymmetric hypertrophy of the septum (1 observation) and by abnormal attachement of clefted anterior mitral leaflet to the interventricular septum (2 observations). In the specimens with ventricular septal defect, 4 presented a stenosis due to malalignment of the infundibular septum, associated with valvular stenosis in 1 case and with valvular stenosis and fibrous subvalvular ring in 2. Another case presented a stenotic pulmonary valve and a parachute mitral valve. The remaining 3 showed a cleft of the anterior leaflet of the mitral valve. The high incidence of infundibular malalignment obstruction is underlined together with its association with A-P or L-position of the aorta. This peculiar relation of the great arteries could be angiocardiographically employed as indicative for the presence of this type of left outflow tract obstruction in TGA.     

Relation between aortic arch hypoplasia of variable severity and central muscular ventricular septal defects: Emphasis on associated left ventricular abnormalities

Among 25 heart specimens with a central muscular ventricular septal defect (including 3 with the architecture of a spontaneously closed defect), only 6 (24 percent) had an aortic arch of normal size and configuration. Isthmus atresia was present in one case, isthmus hypoplasia in eight cases, local coarctatlon in six and a combination of isthmus hypoplasia and local coarctation in another four. A study of the left ventricular morphologic features of the 25 hearts revealed the presence of a spectrum of additional anomalies. The aortic valve was bicuspid In 16 and mitral valve anomalies were present in 13 specimens. Mitral valve deformities present in the 13 specimens included congenital stenosis (5), deficient chordae tendineae (4), parachute valve (2), adherent valve leaflets (1) and a hypoplastic posteromedial papillary muscle (1). Furthermore, there was a large incidence of abnormal left ventricular muscular structures that could be classified into three basic types: (1) the anteroseptal “twist”: thickening and deviation of the anterobasal septum and anterior wall, resulting in a relatively posterior position of the aortic ostium (16 cases); (2) a prominent anterolateral muscle (9 cases); and (3) a posteromedial muscle (9 cases).It is concluded that a central muscular ventricular septal defect is not an isolated malformation but is commonly part of a complex developmental anomaly. This complex of associated inflow and outflow tract lesions presumably may cause reduced aortic flow during morphogenesis, resulting in aortic arch hypoplasia of various degrees of severity.     

Pulmonary atresia, "intact ventricular septum", and aortopulmonary collateral arteries.

In muscular pulmonary atresia, major aortopulmonary collateral arteries are characteristic of pulmonary atresia with ventricular septal defect and are rarely seen in pulmonary atresia with intact ventricular septum. Two unusual cases of muscular pulmonary atresia are reported, one with an intact septum and one with a perimembranous ventricular septal defect, closed in utero by aneurysmal tricuspid tissue. In both cases the pulmonary blood supply came entirely from aortopulmonary collaterals. In case 1 a collateral artery connected the left subclavian artery and hypoplastic pulmonary arteries, and several aortopulmonary collaterals arose from the descending aorta, without overlap between these two circulations. In case 2 the pulmonary trunk and arterial duct were absent and the pulmonary blood supply came entirely from collateral arteries. The right ventricle was of normal size and tripartite with a closed perimembranous ventricular septal defect, discovered only at postmortem examination. These observations suggest right ventricular outflow tract obstruction early in fetal development, with involution of the pulmonary trunk and sixth arch derivatives, and persistence of primitive aortopulmonary connections. The morphology in case 1 is at odds with the theoretical division of pulmonary atresia with intact septum and pulmonary atresia with ventricular septal defect into two separate pathological entities that occur at different stages in fetal development.     

Double outlet right ventricle {S,D,L} with subaortic ventricular septal defect and pulmonary stenosis: Report of six cases

The clinical, hemodynamic, angiocardiographic and pathologic findings are presented in an infrequent but surgically correctable type of double outlet right ventricle. This study is based on six cases, one with autopsy confirmation. In all, the viscera and atria were in situs solitus (S). A ventricular d-loop was present (D). There was l-malposition of the great arteries, the aorta being to the left of, and anterior to, the pulmonary artery (L). Hence, this anomaly may conveniently be represented as double outlet right ventricle {S,D,L}. The ventricular septal defect was subaortic because the aorta was anterior and leftward, adjacent to the ventricular septum. A bilateral conus was present beneath both the aortic and pulmonary valves, preventing any semilunar-atrioventricular fibrous continuity. The subpulmonary conus was poorly expanded, resulting in pulmonary infundibular and valvular (annular) stenosis.The clinical features were those of cyanosis, clubbing and accentuation of the second heart sound in the pulmonary area (related to aortic valve closure). There was a systolic ejection murmur along the upper left sternal border, related to pulmonary outflow tract stenosis. Selective right and left ventricular angiocardiography was diagnostic.Relatively early surgical correction is suggested to minimize the progression of pulmonary infundibular stenosis and to avoid acquired atresia. In this malformation, pulmonary outflow tract reconstruction is more difficult than in tetralogy of Fallot because of the rather posterior location of the pulmonary outflow tract, and because the right coronary artery crosses the stenotic pulmonary outflow tract in front of the pulmonary valve.     

Aortic regurgitation associated with ventricular septal defect. Echocardiographic and hemodynamic observations.

Ventricular septal defect is sometimes associated with aortic regurgitation. In this report, an echocardiogram demonstrating dramatic prolapse of the noncoronary cusp into the left ventricular outflow tract and ventricular septal defect in a patient with Down's syndrome and ventricular septal defect, confirmed by angiographic studies, is presented. The echocardiogram supports the concept of anatomic lack of support of the aortic ring due to a deficient septum and hemodynamically significant flow of blood to the right ventricle through the ventricular septal defect, resulting in trauma to aortic cusps and prolapse.