Morphometric features of ruptured congenital sinus of Valsalva aneurysm: implication for surgical treatment

Five hearts with ruptured congenital sinus of Valsalva aneurysm were studied. In 3 hearts of Caucasian patients, the sinus of Valsalva aneurysms were located in the immediate vicinity of the commissure between the noncoronary and right aortic cusps with rupture from the noncoronary sinus to the right atrium (n = 2) and from the right sinus to the right ventricle (n = 1). In 2 hearts of indigenous North Americans, the defects were sited in the immediate vicinity of the commissure between right and left aortic cusps with rupture into the right ventricle; both patients had an associated conal septal hypoplasia ventricular septal defect and aortic insufficiency. The diameters of the rupture holes at the base of the sinus of Valsalva aneurysms in the five hearts ranged from 0.4 to 1.1 cm (mean 0.7 cm). Histologic examination of longitudinal sections through the ruptured sinus of Valsalva showed 0.8 to 1.7 cm (mean 1.1 cm) wide areas in which there was lack of continuity between the aortic media and the aortic annulus. Conclusions. This study shows that the site of congenital weakness in sinus of Valsalva aneurysm in indigenous North American patients may be similar to that in Oriental patients, whereas the site tends to be different in Occidental patients. It also emphasizes the importance of patch closure rather than suture closure of ruptured sinus of Valsalva aneurysms.     

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.     

Sinus of Valsalva aneurysms: 20 years' experience

BACKGROUND: Aneurysms of sinus of Valsalva are rare. Here, we analyze retrospectively patients operated on at our center during the last 20 years. PATIENTS AND METHODS: One hundred four cases of congential aneurysm of sinus of Valsalva were operated upon between January 1977 and April 1996. Only 12 aneurysms were unruptured. The majority (76.9%) arose from the right coronary sinus. The right ventricle was the most common chamber of rupture (58.6%). Ventricular septal defect was associated in 46 patients (44.2%), of which 28 (60.9%) were supracristal. Ventricular septal defect was more common in aneurysms arising from the right coronary sinus (91.3%). Aortic incompetence was found in 45 patients (43.3%). The defect was closed through the aortic root alone in 24 patients (23.1%) and through both the aortic root and the chamber of rupture in the remaining 80 patients. Six patients underwent aortic valve repair, and 21 an aortic valve replacement. RESULTS: There were two hospital deaths (1.92%). Morbidities were few. Follow-up ranged from 1 to 20 years (mean 8.2 +/- 1.1). There was one late noncardiac death, and in the majority, the long-term follow-up was uneventful. CONCLUSION: Surgery for aneurysm of sinus of Valsalva yields gratifying results, and it should be undertaken as soon as the condition is diagnosed.     

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.     

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.     

Aortic valve prolapse and aortic regurgitation associated with subpulmonic ventricular septal defect

The natural development of aortic valve deformity was studied in 315 patients with subpulmonic ventricular septal defect. The patients with early development of aortic regurgitation had a pulmonary-to-systemic flow ratio of > 1.5, normal right ventricular pressure, and severe aortic regurgitation.     

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.     

Minimally invasive cardiac surgical techniques in the closure of ventricular septal defect: an alternative approach

BACKGROUND: Minimally invasive cardiac surgical techniques recently have been applied in the management of a variety of intracardiac lesions. METHODS: Fourteen patients (6 boys and 8 girls; age, 8.9 +/- 5.5 years; body weight, 29.0 +/- 13.5 kg) were operated on using minimally invasive cardiac surgical techniques for the closure of a ventricular septal defect (subarterial in 11 patients and perimembranous in 3 patients). The operations were performed through a left anterior minithoracotomy and were guided by video-assisted endoscopic techniques under femorofemoral cardiopulmonary bypass. The myocardium was protected by continuous coronary perfusion with hypothermic fibrillatory arrest. The right ventricular outflow tract was entered after pericardiotomy was performed. RESULTS: Closure of the defect (directly in 4 patients and by patch in 10 patients) was performed successfully in all patients. A right ventricular outflow tract obstruction and ruptured sinus of Valsalva aneurysm also were repaired in 1 patient each. The duration of cardiopulmonary bypass was 41 +/- 10 minutes (range, 28 to 100 minutes) and the total operative time was 2.2 +/- 0.8 hours (range, 1.3 to 3.5 hours). All the patients recovered rapidly from their operation and had an uneventful postoperative course. Follow-up (mean, 6.2 months; range, 6 to 9 months) was complete in all patients. There were no late deaths. Transthoracic echocardiographic examination showed no residual shunt and no aortic regurgitation in all patients. CONCLUSIONS: Our experience demonstrates that minimally invasive cardiac surgical techniques are technically feasible and an alternative option for the repair of a ventricular 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.     

Time and image

A 23-year-old man presented with progressive exercise-related dyspnea and easy fatigability. He gave a history of a murmur of aortic insufficiency since childhood. Cardiac catheterization demonstrated severe aortic insufficiency. At surgery the patient was found to have prolapse of the right coronary cusp into a significant supracristal ventricular septal defect. Repair was carried out with a Dacron patch closure of the septal defect and replacement of the valve with a 29-mm St. Jude valve. The patient recovered uneventfully and is fully active and employed 5 years later. Review of the literature documents that this is an uncommon lesion. The approaches to preoperative diagnosis include transesophageal echo. The management techniques have included ventricular septal defect closure, alone and with valvuloplasty, and septal defect closure with aortic valve replacement. The anatomic and patient characteristics guide selection of the most suitable management of these 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.     

Cardiological and general health status in preschool- and school-age children after neonatal arterial switch operation

OBJECTIVE: Cardiological and general health status 3-9 years after neonatal arterial switch operation for transposition of the great arteries should be evaluated by non-invasive methods. METHODS: A total of 77 unselected children with intact ventricular septum (75.3%) or ventricular septal defect (24.7%) without or with aortic isthmic stenosis (5.2%) were prospectively examined 3.2-9.4 years (5.4 +/- 1.6) after neonatal switch. Clinical pediatric and cardiological examination, standard and 24 h Holter electrocardiogram, M-mode, 2D-, Doppler and colour Doppler echocardiography were performed. Outcome data were compared to published normals. RESULTS: Reoperation rate was 2.6%, 96.1% were without limitation of physical activity and 98.7% without medication. Compared to normals, growth was adequate, weight and head circumference were slightly reduced. After median sternotomy, 23.4% had abnormal thoracic configuration (16.9% asymmetry, 6.5% funnel chest). ECG and Holter: 93.5% were in sinus, 6.5% in ectopic atrial or junctional rhythm. Incidence of complete right bundle branch block was 15.8% in patients with ventricular septal defect and 5.2% in those without. Ischemic ST-T changes during exercise due to coronary artery occlusion and evidence of old myocardial infarction were found in 1 patient (1.3%) each. Occasional atrial ectopy was found in 27.4%, ventricular ectopy in 15.3%: occasional in 12.5% and frequent (> 30/h) in 2.8% presenting bigemini, couplets and short runs of ventricular tachycardia at rest and during exercise. Echocardiography: Left ventricular function was normal in all. Endsystolic diameter of neoaortic valve annulus was beyond 90% confidence interval for controls in 79.2%, neoaortic root diameter in 100%. Mild aortic insufficiency was seen in 10.4%. No correlation was found between aortic insufficiency and aortic dilatation. Neoaortic stenosis was not seen, mild residual coarctation after end-to-end-anastomosis was found in 2.6%, native coarctation corrected later on in 1.3%. Supravalvular pulmonary stenosis was seen in 29.9% (19.5% trivial, 7.8% mild, 2.6% moderate), mild subvalvular pulmonary stenosis in 1.3%, pulmonary insufficiency in 2.6%. CONCLUSION: The study confirms good midterm results after neonatal arterial switch operation for transposition with or without ventricular septal defect. Long-term observation is necessary to assess rhythm, coronary artery and myocardial function as well as development of neo-aorta and pulmonary artery system.     

Replacement of aortic root with valved tubular prosthesis in a ten-year-old child with infective endocarditis--a case report

Aortic root abscess, aneurysm of sinus Valsalva, severe aortic valve insufficiency, and a fragile aortic wall caused by infective endocarditis were found in a 10-year-old child. Aortic valve replacement was intended as a preoperative strategy, but one of modifications of Cabrol had to be used urgently because of aortic root rupture between right atrial and aortic connection just after pericardiotomy.     

Unroofed coronary sinus syndrome

Unroofed coronary sinus syndrome or coronary sinus septal defect is a rare congenital cardiac anomaly. We performed corrective operations in 9 patients with such condition. Of them, seven patients had the completely unroofed coronary sinus and persistent left superior vena cava (PLSVC) directly draining into the left atrium (LA). One patient had PLSVC and the partially unroofed terminal portion of the coronary sinus (CS), or sinus ostium open into LA. Another patient had total anomalous pulmonary vein connection and the partially unroofed mid-portion of CS without PLSVC. The operative methods of the unroofed coronary sinus included: ligating PLSVC and repairing ostium primum or secundum atrial septal defects in 4 cases; creating an intra-atrial tunnel from PLSVC to the right atrium (RA) and repairing the atrial septal defect in 2 cases; reconstructing the intra-atrial septal with a patch as a baffle to guide PLSVC or sinus ostium respectively towards RA in 2 cases; enlarging the defect on the coronary sinus roof and repairing the atrial septal defect of the coronary sinus type in one case. No patient died in this group. Because unroofed coronary sinus syndrome has atypical clinical manifestation and usually complicates varied congenital anomalies, its preoperative diagnosis is very difficult in some times and may draw the reliable support from echocardiography and catheterization. The operative program and method must depend not only upon its type but also upon PLSVC presence of absence, and communication between PLSVC and RSVC.     

Clinical course of isolated ventricular septal defect: an Indian experience

To define the clinical course of ventricular septal defect, 410 consecutive patients with isolated ventricular septal defect were evaluated over a period of 13 years. Their age ranged from 12 days to 24 years at the time of first visit to the hospital. Patients with less than 2 years follow-up period were excluded. One hundred and fifty seven patients were one year of age or less. The left to right shunt size remained the same in 52.4% of cases. In 34.4% the shunt size decreased, with complete closure of ventricular septal defect in 8.8%. Closure of ventricular septal defect was observed even in patients who had initially presented with large left to right flow, and congestive heart failure in infancy. Right ventricular outflow tract obstruction developed in 8.5% of patients usually between 2 and 10 years of age. Murmur of aortic regurgitation appeared in 8.9% on follow-up. Infective endocarditis developed in 6 cases. The unfortunate complication of Eisemenger's complex was seen in 3 patients; they had not returned for follow up for a long period of time. Hence, our data show that the left to right shunt across the ventricular septal defect decreases in about one-third of patients. However, a regular follow up is essential to prevent development of Eisenmenger's complex and for early detection of other complications like aortic regurgitation and right ventricular outflow tract obstruction.     

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.     

Combined orthotopic heart and liver transplantation for genetic hemochromatosis

Penetrating chest trauma can result in multiple clinical syndromes depending on the structures involved. Tamponade, valvular regurgitation, ventricular septal defect (VSD), conduction system abnormalities, and coronary lacerations have been reported. We report a case of right ventricular free wall laceration, VSD, and coronary artery fistula involving a septal perforator.     

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.     

The management of severe subaortic stenosis, ventricular septal defect, and aortic arch obstruction in the neonate

Neonates with ventricular septal defect and aortic arch obstruction frequently have subaortic stenosis resulting from posterior deviation of the infundibular septum. Because the aortic anulus is often hypoplastic, making direct resection of the infundibular septum through the standard transaortic approach difficult, the optimal method of repair is uncertain. From September 1989 through November 1991, seven patients with ventricular septal defect, coarctation (n = 4), or interrupted aortic arch (n = 3) and severe subaortic stenosis underwent repair with use of a technique that included transatrial resection of the infundibular septum. Their ages ranged from 5 to 63 days (median 15 days) and weights from 1.3 to 5.4 kg (mean 3.1 kg). Only one patient was older than 1 month. The systolic and diastolic ratios of the diameter of the left ventricular outflow tract to that of the descending aorta were 0.53 +/- 0.09 mm (standard deviation) and 0.73 +/- 0.11, respectively. At operation, the posteriorly displaced infundibular septum was partially removed through a right atrial approach by resecting the superior margin of the ventricular septal defect up to the aortic anulus. The resulting enlarged ventricular septal defect was then closed with a patch to widen the subaortic area. In each patient the aortic arch was repaired by direct anastomosis. All patients survived operation; there was one late death from noncardiac causes 3 months after repair. The survivors remain well from 3 to 14 months after repair (mean 8 months). All are in sinus rhythm and none has a residual ventricular septal defect. One patient underwent successful balloon dilation of a residual aortic arch gradient late after repair. No patient has significant residual subaortic stenosis, although one has valvular aortic stenosis. This series suggests that in neonates with ventricular septal defect and severe subaortic stenosis resulting from posterior deviation of the infundibular septum, direct relief can be satisfactorily accomplished from a right atrial approach. This method provides effective widening of the left ventricular outflow tract and is superior to palliative techniques or conduit procedures.     

Sclerotherapy versus transection for acute variceal bleeding

A case of aortic valve replacement after 16 years from the repair of ruptured sinus valsalva aneurysm (RSVA) was reported. The patient has undergone direct closure of RSVA with VSD type I at 34 years old. At the operation, no attempt was made as to aortic valve regurgitation because of small regurgitation, Selloers 1 on aortography. At 50 years old, he developed dyspnea on exertion, to-and-fro murmur due to aortic valve regurgitation, Selloers 3. Aortic valve replacement, we confirmed the completely closure of right coronary sinus valsalva, and histopathologically observed the degenerative change of only right coronary cusp.