Repair of the left AV valve in atrioventricular septal defect in adults

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.     

The effect of alpha-melanocyte-stimulating hormone and its analog on the growth of human melanoma cell lines with different phenotypes

Homograft aortic root replacement was done to three patients and the anterior mitral leaflet of the homograft was used with success in all cases. Case 1. A 37-year-old man had late-onset active prosthetic valve endocarditis with a fistula from the aortic annulus to the left atrium. The fistula was closed by using a homograft anterior mitral leaflet and the aortic root was replaced by a homograft with reimplantation of the coronary arteries. He is very well without evidence of recurrent endocarditis 29 months after the operation. Case 2. A 37-year-old man had early-onset active prosthetic valve endocarditis and developed the same fistula as case 1. He was treated successfully as in case 1. He is very well 4 months after the operation. Case 3. A 50-year-old woman, who had undergone aortic commissurotomy due to aortic valvular stenosis fifteen years before, deteriorated again. She had subvalvular membranous stenosis and a small aortic annulus. Konno-Soma procedure was applied to enlarge the annulus and the aortic root was replaced by a homograft. The interventricular septal incision was closed successfully with use of the anterior mitral leaflet of the homograft. Homograft aortic root replacement was an attractive procedure for prosthetic valve endocarditis or a small aortic annulus, and the homograft anterior mitral leaflet was useful for closing the fistula due to the infection and for closing the interventricular septal incision of Konno-Soma procedure.     

Size of ventricular structures influences surgical outcome in Down syndrome infants with atrioventricular septal defect

To evaluate the relation between ventricular structure size and surgical outcome in Down versus non-Down syndrome infants with an atrioventricular septal defect, we reviewed the charts and echocardiograms of 44 consecutive infants (34 with Down syndrome) who underwent atrioventricular septal defect repair. Children with Down syndrome had significantly greater aortic valve diameters, left ventricular valve areas, and left/right atrioventricular valve area ratios as well as fewer adverse outcomes than non-Down syndrome children.     

Congenitally corrected transposition with atrioventricular septal defect

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.     

Nonobstructing accessory mitral valve tissue and ventricular septal defect

A 4-month-old boy with ventricular septal defect was found to have accessory mitral valve tissue attached to the anterior leaflet of the mitral valve. Operation was successfully performed to excise the accessory mitral tissue in the left ventricular outflow tract and close the ventricular septal defect. Most previously reported cases with accessory mitral valve tissue were associated with left ventricular outflow tract obstruction. This boy had no pressure gradient across the left ventricular outflow tract. The indications for prophylactic excision of nonobstructing accessory mitral valve tissue in a patient with other forms of congenital cardiac disease are discussed.     

Double-orifice right atrioventricular valve associated with partial atrioventricular septal defect

We describe the case of a double-orifice right atrioventricular valve that was discovered at operation for an associated atrioventricular septal defect. The diagnosis and operative management are discussed. Developmental concepts illustrate the mechanism by which this defect arises, and emphasize that its great rarity remains an enigma.     

Atrioventricular valve function after single patch repair of complete atrioventricular septal defect in infancy: how early should repair be attempted?

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.     

Suspension of straddling tricuspid valve chordae into the appropriate ventricle

In 2 children with an inlet ventricular septal defect and straddling chordae tendineae of the septal leaflet of the tricuspid valve to the posteromedial papillary muscle of the mitral valve and to an accessory papillary muscle in the left ventricle, the straddling chordae were excised with a wedge of posteromedial papillary muscle and with the top segment of the accessory papillary muscle, respectively. After patch closure of the ventricular septal defect, the papillary muscle segment with its group of chordae was anchored to the right ventricular septum with resulting competence of the tricuspid valve. In contrast to the traditional repair technique, the reported modification is applicable when the straddling chordae insert into a papillary muscle of the mitral valve. In addition, various disadvantages related to the construction of a complex baffle in the inappropriate ventricle are avoided.     

Mitral valve anomalies obstructing left ventricular outflow

This paper reports on two cases of more uncommon types of subaortic stenosis. A 2-year-old boy was found with accessory mitral valve leaflet (AMVL) attaching to the anterior leaflet, ballooning into the subaortic ventricular septum associated with a discrete subaortic membrane. The obstruction was successfully relieved by removal of the AMVL and resection of the membrane. A 19-day-old newborn with accessory tissue on the mitral valve (AMVT) causing subaortic stenosis, subaortic ventricular septal defect (VSD) and patent ductus arteriosus was operated on successfully. Accessory tissue excision through the VSD, VSD patch closure and ductus ligation were performed.     

Mobilization of the left and right fibrous trigones for relief of severe left ventricular outflow obstruction

BACKGROUND: There is still no agreement about the optimal method of surgical relief of fixed subaortic stenosis, particularly the severe forms. OBJECTIVES: The purpose of this study was to describe a new technique for the relief of subaortic stenosis based on analysis of the functional anatomy of the left ventricular outflow tract and pathophysiologic features of subaortic stenosis. Methods and patients: We propose that one of the basic abnormalities in subaortic stenosis is interference with the hinge mechanism provided by the 2 fibrous trigones with progressive deposition of fibrous tissue in these angles. The technique described in this paper consists of excision of all components of the fibrous "ring," with mobilization of the left and right fibrous trigones. This results in the restoration of the normal dynamic behavior of the left ventricular outflow tract with maximal widening of the outflow tract as the result of backward displacement of the subaortic curtain and anterior leaflet of the mitral valve. This technique has been used in 57 consecutive patients who ranged in age between 5 months and 56 years (mean, 15.5 +/- 10.6 years). Gradients across the left ventricular outflow tract were between 45 and 200 mm Hg (mean, 86.7 mm Hg). Additional lesions were present in 10 patients, and 7 patients had had 8 previous operations on the left ventricular outflow tract. At operation, in addition to resection of subaortic stenosis, 3 patients had aortic valvotomy, 2 patients had homograft replacement of the aortic valve, 7 patients had patch closure of a ventricular septal defect, and 1 patient had open mitral valvotomy. RESULTS: There were 2 early deaths and 1 late sudden death during the follow-up period that ranged from 1 month to 25 years (mean, 15. 2 years). One patient experienced the development of endocarditis on the aortic valve 7 years after operation, which was successfully treated by homograft replacement. Postoperative gradients across the left ventricular outflow tract varied from no gradient to 30 mm Hg (mean, 8 mm Hg). There were no instances of recurrence of a gradient across the left ventricular outflow tract. CONCLUSION: It is concluded that mobilization of the left and right fibrous trigones results in durable relief of subaortic stenosis.     

Predisposing factors of valve regurgitation in complete atrioventricular septal defect

OBJECTIVES: We sought to determine the intrinsic risk factors of valve regurgitation in complete atrioventricular septal defect. BACKGROUND: Progression of regurgitation varies in each case, although the structure of the common atrioventricular valve itself is a predisposing factor. METHODS: In 90 consecutive patients undergoing surgical repair, we evaluated the preoperative and postoperative regurgitation, valve morphology, age at surgery and associated anomalies. A regurgitation jet with a high velocity reaching the deep left atrial wall by echocardiography was estimated as marked regurgitation. RESULTS: None of the 40 patients with Rastelli type C and an undivided inferior bridging leaflet had preoperative regurgitation in the first year of life, and 12% of them (95% confidence intervals [CI]: 0% to 28%) showed regurgitation at the age of 2. Of the remaining 50 with Rastelli type A and/or a divided inferior leaflet, regurgitation was determined in 21% (95% CI: 6% to 35%) of those 1 year old and in 49% (95% CI: 29%7 to 69%) of those 2 years old (p < 0.01). All patients underwent corrective surgery using the double-patch technique, with the "cleft" being sutured adequately. Irrespective of the valve morphology, regurgitation remained in 52% (12 of 23) of those with preoperative regurgitation, whereas regurgitation developed postoperatively in 28% (16 of 58) of those without regurgitation (p < 0.001). CONCLUSIONS: Those with Rastelli type C and an undivided inferior leaflet had a lesser degree of progression of preoperative regurgitation. However, regurgitation was likely to exist even after adequate repair once regurgitation had already advanced. Therefore, early primary repair before progression of the regurgitation may be the key to maintaining better competence of the atrioventricular valve.     

An acidic modification of the cytoplasmic domain contributes to the charge heterogeneity of the MHC class I antigens

Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital cardiac anomaly that has been classified into two types: one is a more frequent type having dysplasia of tricuspid valve (TV) with a small annulus, underdeveloped right ventricle (RV) with a hypoplastic cavity and a hypertrophic wall; the other type has severe dysplasia of TV and dilatation of RV, right atrium (RA), and right atrioventricular junction with thinning of the RV wall. We performed a morphologic study on 11 autopsied hearts with PAIVS, giving particular emphasis to the variation of morphology of the TV. We could classify these hearts into 3 groups according to the degree of right ventricular development. In the first group of 7 cases (type I), the RVs were underdeveloped. Thick leaflets, restricted valve apparatus with short chordae, and small annuli were characteristics of the TV. In the second group of 3 cases (type II), the RVs showed marked enlargement of the cavity and thinning of the wall. The TV showed redundant, dysplastic, sail-like anterior leaflets, and the downward displacement of septal leaflet and/or posterior leaflet, which are the findings frequently observed in Ebstein's malformation. The RVs were dilated and with partially unguarded tricuspid orifice. The septal leaflet of the TV was dysplastic and, in two cases, the septal leaflet showed chordal structure at the upper surface facing the RA, which is a peculiar finding that has not been described in the literature. The remaining case was a heart with a moderately developed RV (type III). The TV showed mildly dysplastic appearance and we classify this as a separate type, because we could expect the best surgical results in this type. This type had optimal size of RV and the mildest degree of dysplasia of TV. In PAIVS, the morphology of TV correlates well with the type of the right ventricular development.     

Repair of mitral valve and subaortic mycotic aneurysm in a child with endocarditis

Endocarditis requiring surgical intervention in children is uncommon. Individualized operative therapy must be fashioned to the particular pathology of each case. In this case we describe mitral anterior leaflet homograft patch augmentation valvuloplasty, subaortic homograft patch closure of a large mycotic aneurysm, and homograft aortic root replacement in a 3-year-old patient with endocarditis after remote repair of complete atrioventricular canal.     

A codeine radioimmunoassay exhibiting insignificant cross-reactivity with morphine

Viability of canine heart valve leaflet fibroblasts was assessed after varying periods of sterilization and storage in antibiotic-nutrient solution. Tissue culture and assessment of protein and collagen synthesis showed that tissue obtained under optimal conditions rarely retains viability beyond 3 weeks in antibiotic-nutrient solution and is severely impaired after 2 weeks. This casts serious doubts on viability in current clinical homograft valve practice.     

A case of congenital tricuspid regurgitation associated with atrial septal defect and peripheral pulmonary stenosis

This report describes a 5-year-old girl with congenital tricuspid regurgitation associated with an atrial septal defect and peripheral pulmonary stenosis. The girl was diagnosed with the heart murmur at birth and recently developed the cardiomegaly. Cardiac echocardiography and catheterization showed severe tricuspid regurgitation, an atrial septal defect of the secundum type and peripheral pulmonary stenosis. In the operative findings, the tricuspid annulus was dilated to 33 mm in diameter, and leaflets were attached normally to the antomic annulus. There was a large cleft of the anterior leaflet of the tricuspid valve. Suture of the cleft and annuloplasty of the tricuspid valve, suture closure of the atrial septal defect and patch dilatation of peripheral pulmonary stenosis were successfully performed. Including this case, 19 other cases with congenital tricuspid regurgitation undergoing surgery were reported to date.     

Definition of inseparably fused ventricular myocardium in thoracopagus: fetal echocardiographic utility and pathologic refinement

Correlative echocardiographic and pathological findings in a thoracopagus with conjoined hearts are reported. One twin had tricuspid atresia with discordant atrioventricular connections and concordant ventriculoarterial connections. The morphologic right ventricle was hypoplastic and there was a large muscular ventricular septal defect. The other twin had hypoplasia of the mitral valve anulus and left ventricle with double-outlet right ventricle and pulmonary valve atresia. The tricuspid valve was severely insufficient in part because of a large orifice and redundant, elongated leaflets with abnormal chordal attachments. The left ventricles of these two twins shared a perforated common "free wall" with at least two large defects allowing mixing of the circulations at that level. Not all anatomic details were established conclusively by fetal echocardiography; however, sufficient diagnostic information was obtained to support a decision not to aggressively resuscitate these twins after elective cesarean delivery at 31 weeks' gestation.     

Surgical treatment of ventricular septal defect and ruptured sinus of Valsalva associated with infective endocarditis

Two patients with ventricular septal defect of Kirklin type I and ruptured right coronary sinus of Valsalva associated with infective endocarditis were operated on. Both had bacillus vegetation clinging to the aortic and pulmonary valves and the right ventricular intimal wall around the septal defect. Aortic and pulmonary regurgitation were also found. The surgical approach included vertical incision of the right ventricular outflow tract and pulmonary trunk and transverse aortotomy. The right coronary sinus of Valsalva showed distinct aneurysmal change in one patient. The aortic valve and infected Valsalva sinus were excised in both cases, and the pulmonary valve and right ventricular wall where infection extended thoroughly débrided. The resulting defect, including the ventricular septal defect and excised right Valsalva sinus and aortic annulus, was closed with one patch, and the prosthetic valve inserted in the position of the original aortic valve using this patch as part of the annulus. Both patients had a good postoperative course and are doing well, although slight pulmonary regurgitation persists.     

Results of left atrioventricular valve reconstruction after previous correction of atrioventricular septal defects

OBJECTIVE: The objective of this study was to determine causes of severe left atrioventricular (AV) incompetence and the factors leading to the success of valve repair later after correction of atrioventricular septal defects (AVSD). METHODS: A total of 28 patients aged 5 months to 38 years (mean age 6.7 years) were operated for significant (grade II-III) left AV valve incompetence (LAVVI), two months to twenty-five years (median 1.5 years) after correction of complete (11 patients) or partial atrioventricular septal defects. Fourteen patients had initially undergone surgery during infancy. RESULTS: At reoperation a completely open or partially sutured cleft was found in 16 patients combined with dysplastic valve tissue in four cases, with a fibrotic valve in three cases, with posterior leaflet prolapse in two cases, with a double orifice valve in three cases, and a parachute valve in two cases. Partial or complete reopening of a previously sutured cleft caused by suture dehiscence was found in 12 cases combined with a fibrotic valve in five cases, with a dysplastic valve in one case and with severe deformity of valve in one case. A combination of these anomalies was observed in seven patients in both groups. Left atrioventricular valve repair including cleft closure combined with annuloplasty and other surgical procedures resulted in the disappearance or significant diminishing of LAVI in 18 patients (64%). Severe SAVI persisted in six patients, five of them exhibiting a combination of several additional left AV valve anomalies (fibrotic or dysplastic valve, parachute valve). Five of these six patients underwent successful left AV valve replacement with a mechanical prosthesis 7 days to 2 years after reoperation. The presence of additional left AV valve anomalies was the single statistically significant factor for recurrent major LAVVI after reoperation (P = 0.0106). There were two postoperative deaths in patients with mild LAVVI after surgery, and no late deaths. CONCLUSION: An open cleft is the major factor of late severe SAVVI after correction of AVSD. Although suturing the cleft in conjunction with performing annuloplasty improved valvular function in most of the cases, the presence of severe left AV valve anomalies increased the risk of recurrent LAVVI and the need for valve replacement, thus playing a major role in determining the outcome of valve reconstruction in patients after reoperation.     

The McBride operation of hallux valgus in adolescents (author''s transl)

A 23 year old previously healthy man was stabbed in the anterior chest. This resulted in a ventricular septal defect and complete atrioventricular (A-V) block. The electrocardiogram revealed complete A-V block with a QRS pattern of right bundle branch block. His bundle recordings 26 days later revealed A-V dissociation with split His potentials (P-H1 interval of 100 msec and H2-V interval of 40 msec). During the study the escape QRS shifted from right to left bundle branch block with H2 potentials still preceding each QRS interval with H2-V intervals of 40 msec. A permanent pacemaker was implanted because of persistent congestive heart failure and bradycardia due to A-V block. The patient subsequently became asymptomatic. He died suddenly 3 1/2 years later. Pathologically there were sizable openings in both the tricuspid and mitral valve substance and a ventricular septal defect involving the pars membranacea and part of the adjacent muscular septum. Serial sections of the conduction system revealed total destruction and fibrous replacement of the bifurcation and beginning of the right and left bundle branches and subtotal fibrous replacement of the branching bundle. Thus, the bifurcation of the bundle of His was totally absent at autopsy despite apparent electrophysiologic evidence of its existence 26 days after the stab wound. A possible explanation for this discrepancy is the subsequent fibrosis of the bifurcation produced by hemodynamic changes at the lower margin of the ventricular septal defect.     

Fetal atrioventricular septal defect associated with Patau and Edwards syndromes, as well as trisomy 22

The atrioventricular septal defect is usually associated with trisomy 21 and it may be observed in the heterotaxia syndromes. Atrioventricular septal defect may be associated with 8p deletion. There are reported cases of familial atrioventricular septal defect. Atrioventicular septal defect is rarely associated with other chromosomal abnormalities. We are reporting three unusual cases of atrioventricular septal defect that were associated with trisomy 13, 18 and 22. This association may be due to effect of genetic loci on the 13, 18 and 22 chromosome which could play the role in the development and fusion of endocardial cushion and atrioventricular septal defect.