Persistent right sinus venosus valve producing right ventricular outflow tract obstruction.

A 12-year-old girl presented with exercise intolerance. Spinnaker formation of a persistent right sinus venosus valve produced right ventricular outflow tract obstruction. The heart was otherwise normal, and surgical correction was successful.

Echocardiographic-morphologic correlations in tricuspid atresia.

OBJECTIVES: Our aim was to clarify the anatomic substrate in hearts diagnosed as having tricuspid atresia by studying autopsy specimens and comparing the findings with those in two-dimensional echocardiograms. BACKGROUND: Traditionally, tricuspid atresia was thought, and is still believed by some, to be due to an imperforate valvular membrane interposed between the floor of the blind-ending right atrium and the hypoplastic right ventricle. Others argued that the dimple, when present, pointed to the outflow tract of the left ventricle rather than to the right ventricle, making the lesion more akin to double-inlet left ventricle. METHODS: We examined 39 autopsy specimens catalogued as having tricuspid atresia. We then studied 24 two-dimensional echocardiograms from patients with a primary diagnosis of tricuspid atresia. RESULTS: Of the 39 specimens, 37 had a completely muscular floor to the right atrium (absent right atrioventricular [AV] connection). The dimple, when identified, was (except in one case) directed to the left ventricular outflow tract. Only two hearts had an imperforate tricuspid valve. Two-dimensional echocardiograms in all cases showed an echo-dense band, produced by the fibrofatty tissue of the AV groove and representing absence of the right AV connection, between the muscular floor of the morphologically right atrium and the ventricular mass. CONCLUSIONS: Tricuspid atresia is usually, but not always, due to morphologic absence of one AV connection. In most cases, the ventricular mass then comprises a dominant left ventricle together with a rudimentary and incomplete right ventricle.

Surgical significance of morphological variations in the atrial septum in atrioventricular septal defect for determination of the site of penetration of the atrioventricular conduction axis.

The morphological variation in the recognized landmarks for the atrioventricular conduction system was studied grossly in 94 hearts with atrioventricular septal defect, assessing 20 hearts with normal atrioventricular septation as a control. In all the hearts with intact atrioventricular septal structures, the tendon of Todaro demarcated the superior boundary of the triangle of Koch. In hearts with atrioventricular septal defect, however, the landmarks for the conduction axis made up a separate nodal triangle. The tendon of Todaro, along with a bridging tendon not found in the normal heart, were variably developed in hearts with atrioventricular septal defect and formed a further triangle unrelated to the axis for atrioventricular conduction. The opening of the coronary sinus was also variable in its location and size. It was the location of the inferior bridging leaflet as it crossed the ventricular septum that was the best surgical landmark to the site of penetration of the atrioventricular conduction axis.

Long-term results of survivors of surgical valvotomy for severe aortic stenosis in early infancy.

Long-term morbidity and mortality were evaluated in the 21 survivors of a cohort of 51 consecutive infants with severe aortic valve stenosis who underwent surgical treatment in the first 3 months of life during the period from 1958 to 1988. The 21 early survivors have been followed up from 3 to 27 years (median 7.5 years). There have been two late deaths: one at age 13 year from bacterial endocarditis and the other at age 14 years after dislodgment of a prosthetic valve. The calculated 10-year actuarial survival for this group is 100%, with a 15-year actuarial survival of 75% (standard error 15%). Seven repeat operations have been performed in six patients: Three had persistent stenosis and a repeat valvotomy was performed in two of them, aged 2 years and 15 years. The other underwent placement of a conduit from the left ventricle to the descending aorta at 2 years of age. Replacement of the aortic valve has been performed in four patients because of severe valvular insufficiency 13 to 27 years after the initial operation. One of these had required a repeat valvotomy at the age of 15 years. The calculated actuarial freedom from reoperation at 10 years is 90% (standard error 6%) and at 15 years, 67% (standard error 15%). Aortic insufficiency was progressive throughout the period of follow-up. No patient had more than moderate aortic insufficiency 3 to 5 years after the initial valvotomy, whereas aortic insufficiency was severe in five of the eight patients followed up for 11 or more years. Progression of aortic insufficiency and the need for reoperation were not related to the age at initial valvotomy. Survivors of surgical aortic valvotomy in early infancy have a relatively good long-term prognosis and a high freedom from reoperation in the period leading to adolescence. Aortic insufficiency in these patients is progressive, and valve replacement eventually may be required.

The role of coronary artery abnormalities in the prognosis of truncus arteriosus.

A high incidence of coronary ostial and arterial abnormalities was found in a study of 30 pathologic specimens of classic truncus arteriosus at Children's Hospital of Pittsburgh. The following were of special note: (1) left coronary ostium in a posterior and high position; (2) close relation of the left coronary ostium to the pulmonary artery segment in three-leaflet truncal valves; (3) stenosis of the coronary ostium caused by small size, slitlike shape, or the location of the ostium above or in a commissure; (4) the acute angle takeoff of the coronary artery; (5) the position of the left anterior descending artery as it courses posteriorly and close to the truncal wall, and then to the left of the interventricular septum; (6) the size and course of the conal and diagonal arteries from the right coronary artery across the right ventricular outflow area; (7) other coronary abnormalities, including a single coronary artery or ostium with branches crossing the right ventricle below the truncus, the circumflex arising from the right coronary artery and coursing behind the truncus, and the right coronary artery originating from the left anterior descending artery and vice versa. Eight heart specimens with conduit repair were reviewed, and all had injury to coronary arteries, possibly responsible for or contributing to the deaths of six of the eight patients. Coronary abnormalities, often several occurring in combination, may contribute to high operative mortality rate and may be a cause of late sudden death in truncus arteriosus. Surgical procedures should be planned with a view to protecting coronary arteries in the region of the right ventricular outflow tract below the truncus. Coronary artery obstruction (ostial or luminal) can occur and may need to be addressed as a separate issue during surgical procedures.

Morphology of ventricular septal defects in complete transposition. Surgical implications.

Postmortem examination of 62 hearts with complete transposition (concordant atrioventricular and discordant ventriculoarterial connections) and an accompanying ventricular septal defect was performed to determine the morphologic variability of ventricular septal defects and to explore the surgical implications of these defects. Particular attention was directed toward assessing alignment of the outlet septum relative to the muscular septum. Coronary arterial distributions were also evaluated, but specific patterns of distribution did not correlate with morphology of the defect. Of 49 hearts with a normally aligned outlet septum, there were 24 perimembranous, 21 muscular, and 2 doubly committed and juxtaarterial defects. Two hearts had both perimembranous and muscular defects. Twelve of the 21 muscular defects were "central," being surrounded entirely by muscle and located just below the leaflets of the pulmonary valve, and 9 were located in the inlet or apical trabecular septum. There were 13 hearts with malalignment of the outlet septum, anteriorly in 11 and posteriorly in 2. All with anterior malalignment had a subpulmonary defect that was perimembranous in 7 and muscular in 4. Both defects with posterior malalignment had a subaortic perimembranous defect. Because variations in morphology of a ventricular septal defect have a direct impact on selection of the most suitable surgical repair, specific operative approaches are discussed.

The surgical anatomy of double-outlet right ventricle with concordant atrioventricular connection and noncommitted ventricular septal defect.

In describing hearts with double-outlet right ventricle, we have had problems with how best to use the term noncommitted as applied to the ventricular septal defect. We reviewed, therefore, 63 hearts with double-outlet right ventricle in the setting of usual atrial arrangement and a concordant atrioventricular connection. From these, 18 hearts with potentially noncommitted defects were identified and studied in detail. The defect was unequivocally remote from the ventricular outflow tracts in 16 of these hearts, being perimembranous with excavation to open into the inlet of the right ventricle in 12, two of these also having straddling of an atrioventricular valve. One heart had a muscular defect situated in the inlet part of the muscular septum, whereas the defect was the ventricular component of an atrioventricular septal defect in the other three. In the remaining two hearts the defect was anatomically juxtaposed to a subarterial outlet. The pathway to the outflow tract, however, was obstructed by leaflets of a straddling valve. Our study shows, therefore, the need to distinguish between anatomic "commitment" of the defect from the problems in terms of commitment that may confront the surgeon in the operating room. Not only does the distance between the interventricular communication and one of the subarterial outflow tract need to be assessed (the anatomic commitment), but also the presence and nature of any intervening extraneous tissues (the surgical commitment) requires assessment.

Morphologic-echocardiographic correlates of Ebstein's malformation.

The cross-sectional echocardiographic findings were analysed retrospectively in 26 patients with Ebstein's malformation in the light of studies of autopsied specimens from different patients showing this lesion. The salient anatomical feature in diagnosis is the finding of the hinge point of the septal and mural leaflets of the valve within the inlet component of the right ventricle rather than at the atrioventricular junction. The other important feature is the nature of the distal attachment of the leaflets, particularly the anterosuperior one, which can either be in focal or linear fashion. The hinge point of the septal leaflet was noted echocardiographically to be displaced in 19 patients but, significantly, the leaflet was absent in the other seven. Also significant was that the hinge point of the mural leaflet at the crux had been visualized in only 15 of the patients. The anterosuperior leaflet had a distal linear attachment in 20 of the patients, with the anteroseptal commissure becoming a keyhole in six of these through which blood passed to the functional right ventricle. The valve remained a competent structure, even though closing at the junction of atrialized and functional components of the right ventricle rather than at the atrioventricular junction. Cross-sectional echocardiography is the technique of choice with which to display the salient morphological features of Ebstein's malformation.

Diagnostic echocardiographic features of the sinus venosus defect.

To establish the diagnostic criteria for a sinus venosus atrial septal defect cross sectional echocardiograms, cineangiograms, and surgical notes of all patients with this diagnosis seen at the Children's Hospital of Pittsburgh between 1986 and 1988 were reviewed. Seven patients were identified. In each the extent of the atrial septum and the nature of the junction of the superior vena cava with the atria were evaluated echocardiographically from the subcostal position. All had overriding of the superior vena cava and abnormally connected right pulmonary veins. Six patients had undergone cardiac catheterisation and cineangiography. Five patients underwent surgical repair. The operative findings were consistent with the expected morphology in all five, and these features were additionally confirmed in a specimen from the cardiopathological museum. Therefore, the basic anatomical feature of a superior sinus venosus interatrial communication is a biatrial connection of the superior vena cava. This, together with anomalous drainage of the right sided pulmonary veins, results in an interatrial communication outside the confines of the true atrial septum. Overriding of the superior vena cava across the upper rim of the oval fossa is suggested as the pathognomonic diagnostic feature that can clearly be demonstrated echocardiographically from the subcostal position. In essence the lesion is an interatrial communication rather than an atrial septal defect.

Congenitally unguarded tricuspid orifice: its differentiation from Ebstein's malformation in association with pulmonary atresia and intact ventricular septum.

Dysplasia of the leaflets is a well-known integral part of Ebstein's malformation. It is less well-recognized that occasionally the septal leaflet may be completely absent and not simply displaced from its usual annular attachment. This may make it difficult to differentiate by echocardiography between Ebstein's malformation and the rare lesion in which the tricuspid orifice is completely devoid of leaflet tissue ("congenitally unguarded orifice"). A neonatal case is described in which a partially unguarded orifice was present in association with pulmonary atresia and intact ventricular septum but misdiagnosed as Ebstein's malformation. In addition we reviewed the morphologic features of all 46 cases of pulmonary atresia and intact septum in the Cardiopathological Collection of the Children's Hospital of Pittsburgh: 17 also had Ebstein's malformation, while three had a congenitally unguarded tricuspid orifice. We found that the differential diagnosis occurred when the right ventricle was dilated rather than being a hypoplastic cavity, as was a feature of all the cases with unguarded orifice and five of those with Ebstein's malformation. The difference between the lesions is best demonstrated by examining the mural leaflet of the valve, which is absent when the orifice is unguarded but displaced in association with Ebstein's malformation. This feature should be recognizable by cross-sectional echocardiography. We have confirmed previous studies that dilatation of the chambers of the right heart indicates a very poor prognosis in cases with pulmonary atresia and an intact ventricular septum.

Coronary arteriosclerosis in pediatric heart transplant survivors: limitation of long-term survival.

Because coronary atherosclerosis after heart transplantation has been a limiting problem in long-term survival of adults, we reviewed the coronary angiograms, and autopsy data when available, from 21 of 30 children who underwent orthotopic heart transplantation and survived the perioperative period. Six patients had coronary atherosclerosis, and five of these patients died 6 months to 3 years after heart transplantation. The late deaths were sudden and unexpected. Coronary angiography demonstrated several types of lesions, including concentric narrowing, tubular segmental lesions, and abrupt obliteration of major coronary vessels. Risk factors assessed included hypertension, hyperlipidemia, cytomegalovirus infection, type of immunosuppressive regimen, number of rejection episodes, and major histocompatibility antigen mismatches. Only the frequency and duration of rejection episodes seemed to be more prevalent in the patients in whom coronary atherosclerosis developed. Despite the benefits of heart transplantation in treating children with end-stage heart disease, coronary atherosclerosis may limit long-term survival. We suggest that these children should undergo serial coronary angiography to identify those at risk for subsequent events related to coronary artery disease.

Anomalies of the left atrioventricular valve and related ventricular septal morphology in atrioventricular septal defects.

A characteristic feature of atrioventricular septal defects is a deficiency of the inlet part of the ventricular septum that results in a "scooped out" appearance. The depth of the scoop in relation to the disposition of the atrioventricular valves has been debated. To clarify the relation between the morphology of the ventricular septum and the disposition of the atrioventricular valves, we quantified these anatomic features in 151 hearts at autopsy to determine whether those features identified particular groups within the overall lesion. We found that 137 hearts had left atrioventricular valves with three leaflets. The left valve in the other 14 hearts exhibited a dual orifice, a two-leaflet or one-leaflet arrangement, or was imperforate. These anomalies could be analyzed in terms of a sequence of diminishing formation of the commissures. Also, three-leaflet valves displayed a variability in which the angular size of the mural leaflet correlated negatively with that of the inferior leaflet. In some of the hearts with a common atrioventricular orifice, the bridging leaflets did not meet over the ventricular septum, thus creating a "gap." The mural leaflet's angular size corresponded to a deficiency of the combined inferior-mural leaflet complex. Hearts with an abnormal disposition of the left atrioventricular valve had the ventricular septum "scooped" to a greater extent than those with a common orifice, although most had separate right and left atrioventricular orifices.

Abnormalities of the spleen in relation to congenital malformations of the heart: a survey of necropsy findings in children.

A series of 1042 reports of necropsies on children dying at Children's Hospital of Pittsburgh was reviewed. In each case, note was taken of the status of the spleen, the lobation of the lungs, the arrangement of the bronchi, the morphology of the atrial appendages, and the presence of any congenital malformations of the heart and great vessels and of any malformations of the abdominal organs. There was isomerism of the left atrial appendages in eight (0.77%), 13 (1.25%) showed isomerism of the right appendages, and seven (0.67%) had multiple spleens without having isomerism of the atrial appendages. Unexpectedly, a normal spleen was found in one patient with isomerism of the right appendages and also in a patient with isomerism of the left appendages. In one patient with isomeric left atrial appendages there was no spleen. The review showed that the morphology of the atrial appendages, and hence the arrangement of the atria, is not accurately predicted by the type of spleen. The arrangement of the atrial appendages is the most reliable guide to the recognised combinations of congenital cardiac malformations previously described as "splenic syndromes". Because there is no certain way of predicting all the malformations in patients with complex congenital heart disease, it is advisable to record separately for each patient the details of lobation of the lungs, the bronchial and atrial arrangement, anomalies of the heart and great vessels, the type of spleen, and any abnormal arrangement of the abdominal organs.

The morphology of the normal aortic valve as compared with the aortic valve having two leaflets.

We studied 30 normal aortic valves and 64 aortic valves with only two leaflets to examine their morphologic components. The components of the aortic valve are the leaflets, the sinuses, and the interleaflet triangles. The first part of our study shows the relationship of these component parts of the normal aortic valve to the other cardiac structures. The second part shows a wide variation in the architecture of valves having two leaflets. Having produced criteria for the number of sinuses and the number of leaflets, we discovered that previous criteria used in the definition of valves with two leaflets are inadequate when the whole valvular complex is considered. Only seven valves in the study have two leaflets supported by two sinuses. The evidence points to the other valves having started with three leaflets but becoming bicuspid during either intrauterine or postnatal life.

Cardiac transplantation in children.

Heart transplantation is becoming an accepted treatment for children with irreversible and profoundly disabling cardiomyopathy. The risk is much higher when there is underlying congenital heart disease, and even moderately elevated pulmonary vascular resistance is a contraindication to orthotopic heart transplantation. Heterotopic or heart-lung transplantation may be considered in patients with elevated pulmonary vascular resistance. In a few centers, heart transplantation is being performed as an alternative to palliative surgical procedures in children with hypoplastic left heart syndrome. Chronic immune suppression is necessary in all patients postoperatively. Cyclosporine and prednisone are the mainstays of therapy, and azathioprine is often added to the regimen. ATG is used prophylactically in the immediate postoperative period and acute rejection episodes are treated with pulses of prednisone, ATG, or OKT3. Infection continues to be a major problem, and the chronic long-term effects of both rejection and the drugs used to treat it, especially cyclosporine, are also very important. Coronary artery disease and lymphoproliferative disease are causes of death, and hypertension and decreased renal function are present in almost all survivors. The shortage of donor hearts is becoming a progressively more important problem and may affect selection criteria in the future. On the positive side, most children can return to age-appropriate activities following transplantation and they seem to tolerate their chronic illness and its attendant repeated invasive procedures surprisingly well.

Balloon pulmonary valvuloplasty in a child with Ebstein's anomaly and valvar pulmonary stenosis.

We performed a balloon pulmonary valvuloplasty in a child with Ebstein's anomaly using the standard technique. The procedure reportedly has not been performed in the face of this defect and, although difficult, was safely and successfully accomplished. In patients with Ebstein's anomaly and right ventricular outflow obstruction compounded by pulmonary valve stenosis, application of this technique may delay the need for surgical intervention.

The determination of atrial arrangement by examination of appendage morphology in 1842 heart specimens.

The morphology of the atrial appendages was examined in 1842 specimen hearts from patients with congenital lesions. The external and internal features that permitted the identification of the right and left appendages were studied in detail in one tenth of the hearts. These results were compared with a similar analysis of 25 normal hearts. This study showed that criteria for identification of right and left appendages were reliable. Application of these criteria to the overall collection identified the usual arrangement in 1776 (97%) hearts, a mirror image arrangement in eight (0.4%); left atrial isomerism in 22 (1.2%); and right atrial isomerism in 36 (1.9%). Fourteen (0.81%) had juxtaposed atrial appendages (13 with usual arrangement and one with left isomerism). This did not interfere with identification of the left and right atria on the basis of appendage morphology. In only two cases did the determination by atrial morphology produce a result that was inconsistent with the arrangement of the other thoracoabdominal organs. Further examination of the atria in these showed a mistake had been made in the initial assessment. The atrial arrangement can be accurately determined by the morphology of the atrial appendages.