Atrial septal defect with tricuspid valve regurgitation and heart failure in early childhood: report of a case with successful surgical management consisting of special patch closure technique and use of polydioxanone suture for the tricuspid annuloplasty

A management of surgery for infant having a small left ventricular cavity associated with atrial septal defect and tricuspid valve regurgitation was presented. A right upper part of the defect was remained in open during a patch closure of ASD and this portion was temporarily sutured by prolene stayed extracardialy through Waterston's groove. During 20 minutes after weaning from the cardiopulmonary bypass, left heart failure did not appear and then it was completely closed. DeVega's method was employed for the tricuspid valve regurgitation using a absorbale Polydioxanone suture, because of a growth of the sutured annulus. Postoperative course was uneventful and trivial tricuspid valve regurgitation was recognized in angiocardiographic studies performed in 2 weeks and 6 months after operation.     

Pulsatile total cavopulmonary shunt for hypoplastic right heart syndrome with abnormal systemic venous return--a case report

A pulsatile total cavopulmonary shunt was successfully performed on a 5-year-old girl with hypoplastic right heart syndrome associated with abnormal systemic venous return; at the same time, modified mitral valve replacement was performed for mitral regurgitation. The right atrium, tricuspid valve and right ventricle were all extremely dimunitive. The diameter of the tricuspid valve was 50% of normal and the volume of the right ventricle was 8.6% of normal. In addition, there were severe subpumonary stenosis, a restrictive ventricular septal defect (VSD) and an atrial septal defect (ASD). The bilateral superior venae cavae (SVCs) and the hepatic vein drained to the left atrium, and the inferior vena cava was infrahepatically interrupted with a hemiazygos connection to the left superior vena cava. At the operation, each SVC was anastomosed end-to-side to each branch of the pulmonary artery (PA). The restrictive ventricular septal defect and stenotic subpulmonary lesion were left. The diameter of the ASD was reduced from 12 mm to 7 mm. The main PA was neither divided nor banded. The pulsatile blood flow from the left heart to the PA was regurated by a native restrictive VSD and stenotic subpulmonary lesion, and that from the right heart via the ASD was limited by reducing the size of the ASD. These described anatomic arrangements produced adequate antegrade pulsatile flow in the PA, which might prevent the development of pulmonary arteriovenous fistulae and, besides permit transfer of drainage of the hepatic vein from the left to the right atrium via the ASD in future.     

Tetralogy of Fallot in adults--107 cases

Clinical and haemodynamic profile of 107 adult patients above the age of 15 years with TOF was analysed. Cardiac catherization and selective cine-angiography were performed in all cases. Infundibular pulmonary stenosis, mal-alignment type of ventricular septal defect, mitral-aortic fibrous continuity and equal systolic pressures in both the ventricles and aorta were considered mandatory for the diagnosis of Tetralogy of Fallot. Aortic regurgitation was seen in 26 cases (24%), tricuspid regurgitation in 22 cases (21%), absent pulmonary valve in 3 cases (3%), branch pulmonary artery stenosis in 9 case (8.4%), major aortopulmonary collaterals in 15 cases (14%), right atrial pressure was more than 10 mmHg in 10 cases (11%) and right ventricular end diastolic pressure more than 9 mmHg in 73 cases (68%). The left ventricular end diastolic pressure was above 13 mmHg in 58 cases (54%).     

Case of MPO-ANCA positive interstitial pneumonitis and necrotizing, crescentic glomerulonephritis

A 56-year-old female who was diagnosed incomplete endocardial cushion defect, underwent closure of ostium primum defect and repair of mitral cleft 2 years ago. Mitral and tricuspid ring annuloplasty was done 9 months after the first operation because of an increment of mitral and tricuspid valve regurgitation. The Third operation, mitral valve replacement by mechanical valve and tricuspid valve replacement by bioprosthetic valve, was performed because of the gradually increased mitral stenosis and tricuspid regurgitation. Post-operative course was uneventful. Resected anterior cusp of mitral and tricuspid valve revealed hypertrophy and shortness macroscopically, and revealed fibrosis and calcification histopathologically. It was considered that remarkable regurgitation was due to secondary change of both valves. We concluded that valve replacement should be performed for case of secondary change of atrioventricular valve in adult patient.     

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.     

A case report of incomplete endocardial cushion defect with mitral stenosis in an elderly patient

The following paper describes a mitral valve replacement (SJM 27 mm), the patch closure (EPTFE) of an ostium primum atrial septal defect and tricuspid annuloplasty (De Vega's method) in a 64-year-old female patient with an incomplete endocardial cushion defect and mitral stenosis. Surgery revealed thickened, mitral valve leaflets and the presence of a cleft, findings similar to those observed in case of rheumatic degeneration. Investigation of patient hemodynamics confirmed a diagnosis of Lutembacher syndrome and a lower with left ventricle volume. After surgery, the volume of left ventricle increased and the patients clinical course was uneventful.     

Traumatic tricuspid valve regurgitation and cerebral emboli

We report a case of traumatic tricuspid valve regurgitation and late presentation with transient ischemic attacks caused by the presence of a right-to-left shunt through a small atrial septal defect.     

Prevalence of heart disease in school children in rural Kenya using colour-flow echocardiography

In a survey using colour-flow echocardiography and a portable generator, of one thousand one hundred fifteen children, three had clinical and echocardiographic evidence of rheumatic heart disease giving a prevalence rate of 27/1000. Sixty nine (6.2%) of the children examined had trivial mitral regurgitation (TMR). Of these, three had associated trivial aortic regurgitation. Isolated trivial aortic regurgitation was not seen. Four children had isolated mild-moderate regurgitation of the pulmonary valve. Congenital heart disease (CHD) was found in two children-one with secundum atrial septal defect and one with a ventricular septal defect and pulmonary stenosis, giving a prevalence of 1.8/1000. One child had a bicuspid aortic valve and two persistent left superior vena cava. It is feasible to carry out an echocardiographic survey using a portable generator in schools where electricity is not available. The prevalence of rheumatic heart disease (RHD) is higher than previously found in Kenya. The prevalence of TMR is surprisingly high. This was associated with familial clustering reminiscent of RHD. The importance of these findings must await the results of further investigation.     

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.     

Mimics of Ebstein's anomaly

The purpose of this study was to determine the most discriminating clinical and echocardiographic features that are most helpful in correctly identifying Ebstein's anomaly of the tricuspid valve from other causes of tricuspid regurgitation. Ebstein's anomaly is an uncommon malformation of the tricuspid valve with diagnostic echocardiographic features. Other cardiac disorders associated with tricuspid valve regurgitation and predominate right-sided heart chamber enlargement can be misdiagnosed as Ebstein's anomaly. All patients who were referred to our institution between 1982 and 1995 with the diagnosis of Ebstein's anomaly but were found to have other abnormalities of the tricuspid value or right ventricle were identified. Their clinical, echocardiographic, and surgical records were reviewed retrospectively. Twenty-two patients (12 males and 10 females), aged 7 to 68 years (mean 33 years), were referred to our institution with the diagnosis of Ebstein's anomaly but were found to have another abnormality that mimicked clinical and diagnostic features of Ebstein's anomaly. The most common initial symptom was exercise intolerance (13 [59%] patients) followed by atrial arrhythmia (seven [32%] patients). Two patients had cyanosis. Three patients had paroxysmal and six had chronic atrial fibrillation/flutter. Cardiomegaly on chest x-ray film was noted in 18 (82%) patients. Referral diagnosis of Ebstein's anomaly had been made by echocardiography (12 patients), cardiac catheterization (four patients), both techniques (five patients), and echocardiography and magnetic resonance imaging (one patient). All 22 patients had predominate right atrial and right ventricular enlargement, and 18 (82%) of 22 patients also had right ventricular dysfunction. However, Ebstein's anomaly was confidently ruled out with repeat comprehensive echocardiography at our institution by establishing (1) absence of significant apical displacement of the septal tricuspid valve leaflet (> or = 8 mm/m2) and (2) lack of a redundant, elongated, anterior tricuspid valve leaflet in all 22 patients (100%). All had significant tricuspid regurgitation caused by tricuspid valve dysplasia (nine patients), tricuspid valve prolapse (four patients), trauma (four patients), right ventricular dysplasia (three patients), endocarditis (one patient), and annular dilation caused by free pulmonary regurgitation (one patient). In all 15 patients who subsequently underwent surgery (tricuspid valve repair [seven patients] or replacement [eight patients]), the absence of Ebstein's anomaly was confirmed. Echocardiographic absence of the characteristic degree of displacement of the septal leaflet of the tricuspid valve (> or = 8 mm/m2) and the presence of a nonelongated, nonredundant anterior tricuspid valve leaflet consistently excluded the diagnosis of Ebstein's anomaly. Under such circumstances, other anomalies of the tricuspid valve or right ventricle were consistently identified. Recognition of the mimics of Ebstein's anomaly had important surgical implications.     

Assessment of the role and reliability of sonographic post-prandial flow response in grading Crohn''s disease activity

CONTEXT: The incidence of infective endocarditis after surgical repair of congenital heart defects is unknown. OBJECTIVE: To determine the long-term incidence of endocarditis after repair of any of 12 congenital heart defects in childhood. DESIGN: Population-based registry started in 1982. SETTING: State of Oregon. PARTICIPANTS: All Oregon residents who underwent surgical repair for 1 of 12 major congenital defects at the age of 18 years or younger from 1958 to the present. MAIN OUTCOME MEASURE: Diagnosis of infective endocarditis confirmed by hospital or autopsy records. RESULTS: Follow-up data were obtained from 88% of this cohort of 3860 individuals through 1993. At 25 years after surgery, the cumulative incidence of infective endocarditis was 1.3% for tetralogy of Fallot, 2.7% for isolated ventricular septal defect, 3.5% for coarctation of the aorta, 13.3% for valvular aortic stenosis, and 2.8% for primum atrial septal defect. In the cohorts with shorter follow-up, at 20 years after surgery the cumulative incidence was 4.0% for dextrotransposition of the great arteries; at 10 years, the cumulative incidence was 1.1% for complete atrioventricular septal defect, 5.3% for pulmonary atresia with an intact ventricular septum, and 6.4% for pulmonary atresia with ventricular septal defect. No children with secundum atrial septal defect, patent ductus arteriosus, or pulmonic stenosis have had infective endocarditis after surgery. CONCLUSION: The continuing incidence of endocarditis after surgery for congenital heart defect, particularly valvular aortic stenosis, merits education about endocarditis prophylaxis for children and adults with repaired congenital heart defects.     

A modified repair technique for tricuspid incompetence in Ebstein's anomaly

OBJECTIVE: A modified technique for tricuspid valve repair in Ebstein's anomaly restructures the valve mechanism at the level of the true tricuspid anulus by using the most mobile leaflet for valve closure without plication of the atrialized chamber. Midterm results of this therapeutic approach for patients with Ebstein's anomaly and tricuspid valve incompetence are reported. METHODS: Between October 1988 and April 1997, the incompetent tricuspid valve was repaired with our technique in 19 patients (12 female, 7 male; 2 to 54 years, mean 21 years). The indication for operation was congestive heart failure of various degrees in all patients. Tricuspid incompetence was grade II in two patients, grade III in 14, and grade IV in three. Associated congenital malformations were simultaneously repaired (interatrial communication in 18, ventricular septal defect in two, pulmonary stenosis in two, mitral valve prolapse in one). Follow-up ranged between 10 and 103 months (median 28 months) and was complete for all patients. RESULTS: There were no operative deaths. One patient with active endocarditis and pulmonary abscess died 2 months after the operation of recurrent sepsis; there were no late deaths. During follow-up, New York Heart Association functional class improved from 2.8 before the operation to 1.9 without recurrent cyanosis, and tricuspid incompetence decreased from a mean grade of 3.1 to one of 0.9, without any echocardiographic deterioration of the tricuspid valve function or right ventricular dilation. CONCLUSIONS: Our technique allows tricuspid valve repair in patients with Ebstein's anomaly, even in cases usually reserved for primary valve replacement, without late functional deterioration.     

Studying electric field effects on embryonic myocytes

OBJECTIVE: Little attention has been paid to the occurrence of aortic regurgitation after complete repair in patients with pulmonary atresia and ventricular septal defect or tetralogy of Fallot. To highlight the development of aortic regurgitation or aortic root dilation severe enough to necessitate aortic valve replacement with or without aortic aneurysmorrhaphy or aortic root replacement, we retrospectively reviewed the records of patients who underwent aortic valve operation at our institution subsequent to repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot. METHODS: We searched the Mayo Clinic database for patients with pulmonary atresia and ventricular septal defect or tetralogy of Fallot who subsequently had aortic valve or aortic root operations. The degree of aortic regurgitation before operation was noted. Aortic sinus and root dimensions were measured. RESULTS: Sixteen patients underwent complete repair at a median age of 17 years, followed by an aortic operation a median of 13.5 years later. All 16 patients had dilated aortic sinuses at the time of the aortic valve operation. These 16 patients had aortic valve replacement: 11 with mechanical prostheses and 5 with bioprostheses. Five of the 16 also had reduction of aortic dilation by lateral aneurysmorrhaphy, and 1 had graft replacement of the ascending aorta. Five patients had associated conditions (evidence of valvular damage, recurrent ventricular septal defect, or history of endocarditis) discovered at the aortic valve operation that have been reported to be related to the development of aortic regurgitation. The remaining 11 patients had progressive aortic regurgitation despite complete, uncomplicated repair. CONCLUSIONS: Progressive aortic regurgitation and aortic root dilation can occur despite complete repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot.     

Monocusp homograft VSD patch in atrioventricular canal with tetralogy of Fallot

A monocusp aortic homograft was used to compensate for deficient right atrioventricular valve tissue during repair of complete atrioventricular canal defect with tetralogy of Fallot. The homograft was used to produce a comma-shaped ventricular septal defect patch together with the septal leaflet of the right atrioventricular valve, thus committing native leaflet tissue to left atrioventricular valve reconstruction. One year postoperatively the child is in New York Heart Association class I with no tricuspid regurgitation.     

Congenital malformations of the mitral valve in children. Pathology and surgical treatment

A systematic study of congenital mitral valve malformations was undertaken on a surgical basis in an attempt to develop techniques of valvular reconstruction adapted to the various lesions. Forty-seven children between the ages of 4 months and 12 years (average 6 years, 4 months) have been operated upon between January, 1970, and March, 1976. Valvular lesions were classified into four group: Group I, mitral insufficiency owing to valvular lesions: Group II mitral insufficiency with subvalvular lesions; Group III, mitral insufficiency and stenosis; Group IV, stenosis. Associated lesions (ventricular or atrial septal defects, coarctation, or aortic valve stenosis) were present in 31 patients (65 per cent) and were corrected during the same operation. Valve reconsruction was possible in 38 patients whereas valve replacement was necessary in 9 patients. In the valve repair group there were three operative deaths (8 per cent), no late deaths, one reoperation for residual ventricular septal defect, and one myocardial infarction. In the valve replacement group of 9 patients, there were three operative deaths, three late deaths, and one case of repeated embolization. Thirty-one of 38 patients in the valve repair group were classified into Functional Class I after the operation (86 per cent), 2 were in Class II, and one in Class III. Minimal or moderate regurgitation and cardiomegaly persisted in the majority of the patients. Pulmonary artery pressure significantly decreased, however, as demonstrated by postoperative catheterization in 17 patients.     

Insertion of prosthetic tricuspid valve two years after the tricuspid valvulectomy

A case of a 14-year-old boy who underwent a prosthetic tricuspid valve insertion two years after the tricuspid valvulectomy due to intractable right-sided active endocarditis is presented. At the initial operation, the tricuspid valve was thoroughly resected because of marked destruction and attachment of vegetations, and a concomitant ventricular septal defect was directly closed. When a prosthetic valve was placed in the tricuspid portion, a semicircular Dacron patch was attached to the right side of the interventricular septum in a fashion of up-chord and down-arc to cover the bundle of His. The straight edge of the patch was located near the AV node and was not sutured to the tricuspid valve annulus. A 31 mm of CarboMedics prosthetic valve was sutured to the tricuspid valve annulus and to the free edge of the patch at the position near the AV node to prevent AV block. Postoperative course was uneventful and ECG showed regular sinus rhythm.     

Balloon pulmonary valvuloplasty for pulmonary valve stenosis with atrial septal defect

We performed successful balloon pulmonary valvuloplasty (BPV) in 6 patients with pulmonary valve stenosis and atrial septal defect (ASD) accompanied by atrial left-to-right shunt without an increase of shunting immediately after BPV. It suggests that such patients should be treated by BPV initially and the need for ASD repair can be assessed during long-term follow-up.     

Haemodynamics after Mustard's operation for transposition of the great arteries

Cardiac catheterization data from 54 investigations after Mustard's procedure were examined to study the influence of the operation on pressure events in the atria, great veins, and pulmonary circulation. Systemic venous atrial pressure tracings were characterized by a rapid, sharp 'y' descent. Pressure gradients between the venae cavae and systemic venous atrium were invariable, whether or not vena caval pathway obstruction was present, the 'y' trough and 'a' wave gradients being greater than the mean gradient. Pulmonary venous atrial pressure tracings were not different from normal except when tricuspid regurgitation was present. It is suggested that the baffle effectively reduces the size and compliance of the systemic venous atrium, but influences the pulmonary venous atrium to a lesser degree. The systolic pressure gradient from the left ventricle to pulmonary artery was decreased postoperatively, suggesting that it may be flow-related; the greatest changes were seen in the group with preoperative ventricular septal defect. The ratio of pulmonary: systemic vascular resistance did not change significantly after operation, and it is suggested that both the pre- and postoperative values were higher than normal. Examination of the left ventricular or pulmonary arterial mean pressure postoperatively should raise the suspicion of a complication, e.g. pulmonary venous obstruction or tricuspid regurgitation.     

Determinants of successful balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum

OBJECTIVES: This study reviewed our experience with percutaneous balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum and defined the anatomic and hemodynamic characteristics of infants in whom this procedure is successful and provides definitive therapy. BACKGROUND: Unlike children with valvular pulmonary stenosis, the follow-up of infants with critical pulmonary stenosis undergoing percutaneous balloon valvotomy is limited. METHODS: Between December 1987 and August 1992, percutaneous balloon valvotomy was attempted in 12 infants with critical pulmonary stenosis (n = 10) or pulmonary atresia with intact ventricular septum (n = 2). Two outcome groups were identified: Group A patients are acyanotic, have mild residual pulmonary stenosis and have not required operation; Group B patients have required operation. RESULTS: Of the 12 infants, 11 had a successful balloon valvotomy procedure. Group A patients (n = 7) have a residual gradient of 22 +/- 18.7 mm Hg (mean +/- SD) at follow-up of 3.2 years (range 1.2 to 5.0). In Group B (n = 5), operation was required for inability to cross the pulmonary valve (n = 1) or persistent severe hypoxemia for > or = 2 weeks after valvotomy (n = 4). Significant differences (p < or = 0.01) between the two groups (Group A vs. Group B) were identified in pulmonary valve annulus (Z value) 8.1 mm (-1.1) versus 5.5 mm (-3.4); tricuspid valve annulus (Z value) 14.0 mm (0.8) versus 8.8 mm (-1.8); right ventricular volume 65 versus 29 ml/m2; and Lewis index 10.9 versus 8.9. CONCLUSIONS: Percutaneous balloon valvotomy is effective and likely to provide definitive therapy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum who have a tricuspid valve annulus > 11 mm, pulmonary valve annulus > or = 7 mm and right ventricular volume > 30 ml/m2.     

Systemic collateral and pulmonary artery stenosis in patients with congenital pulmonary valve atresia and ventricular septal defect

Angiograms of 30 patients with congenital pulmonary valve atresia, ventricular septal defect, and large systemic-pulmonary collateral arteries (SPCAs) were evaluated. All had aortography, 28 had SPCA arteriography, and 26 had right ventriculography. Seventeen (65%) of 26 patients had a right ventricular infundibulum, 23 (77%) had a pulmonary artery confluence, and five of the nine patients without a right ventricular infundibulum had a confluence. Sixty-six SPCAs of aortic origin were seen; 28 (42%) had narrowing and 21 patients (70%) had one or more narrowed SPCAs. Five patients had collaterals from internal mammary, subclavian or innominate arteries. Fourteen (47%) had hilar pulmonary artery stenosis. Of these 14 patients mild peripheral stenosis was demonstrated in five. Right aortic arch was present in 15 patients (50%). Complete angiographic delineation of pulmonary vasculature is an essential procedure for preoperative detection of pulmonary and SPCA stenoses in these patients.