Ventricular septal defect with tricuspid pouch with and without transposition. Anatomic and surgical considerations.

In a 10-year review, patients operated on for ventricular septal defect and tricuspid valve pouch were divided into two groups, because the effect of the tricuspid valve pouch is influenced by which ventricle has the higher pressure. Group I comprised patients with ventricular septal defect without transposition of the great arteries and group II, ventricular septal defect with transposition. In 72 of 392 group I patients, the septal tricuspid valve leaflet was incised to expose the edges of the hidden ventricular septal defect to accomplish proper anatomic repair. Forty-eight patients had a tricuspid valve pouch, the diagnosis being established by angiography, echocardiography, or at operation. Ages at operation ranged from 5 months to 22 years and the pulmonary-systemic flow ratio ranged from 1 to 3.4, with 16 being less than 1.5. In one patient the pouch produced a 40 mm Hg pressure gradient in the right ventricular outflow tract. At operation, through a transatrial approach, the tricuspid valve pouch was opened radially, the actual ventricular septal defect patched, and the tricuspid valve leaflet repaired. There were no deaths, no significant intraoperative or postoperative morbidity, and no tricuspid valve dysfunction. The average postoperative hospital stay was 4.8 days. In group II, six of 83 patients operated on for transposition with ventricular septal defect had significant left ventricular outflow tract obstruction from the tricuspid valve pouch. Five of six had a Mustard procedure, two requiring a left ventricular-pulmonary artery conduit, and in two of the six the ventricular septal defect was closed through the pulmonary artery. One patient had heart transplantation after a Mustard repair and tricuspid valve replacement. The sixth patient in group II had a successful arterial switch at 9 years of age, after the presence of left ventricular outflow tract obstruction was proved to be due to the pouch. The presence of a tricuspid valve pouch in group I may lead the surgeon to close false small openings produced by the pouch rather than the actual ventricular septal defect. Incising the pouch is safe and essential for proper exposure and secure closure of the true defect. In group II, the systemic right ventricular pressure can push the pouch into the left ventricular outflow tract, causing significant obstruction, and may contribute to tricuspid valve insufficiency after atrial baffle repair. Arterial switch is preferred because it returns the obstructive tricuspid valve pouch and abnormal tricuspid leaflet to the lower pressure pulmonic right ventricle.     

Surgical treatment of tricuspid regurgitation caused by L?ffler's endocarditis.

A 25-year-old man with a history of bone-marrow-transplantation for the treatment of L?ffler's endocarditis underwent surgery for massive tricuspid regurgitation with paroxysmal atrial flutter. Dense fibrosis in the right ventricular endocardium with complete obliteration of the apex was seen intraoperatively, and the right ventricular cavity was diminished. Annular dilatation of the tricuspid valve and entrapment of the posterior leaflet to the endocardial fibrosis were also seen. Annuloplication at the posterior leaflet was performed. In addition, the right atrial free wall was widely resected and the septal and inferior vena cava-tricuspid valve isthmi were cryoablated for the treatment of atrial flutter. Postoperative catheterization revealed rather high right ventricular end-diastolic pressure. However, tricuspid regurgitation disappeared with the increased cardiac output. Atrial flutter could not be induced by repetitive stimulation in the postoperative electrophysiological examination.     

A case of successful surgical treatment of straddling tricuspid valve associated with dextrocardia and VSD

A case of straddling tricuspid valve associated with dextrocardia and VSD was presented. Closure of ventricular septal defect and tricuspid valve replacement were performed on this patient. Since the straddling septal leaflet of the tricuspid valve shared a posterior papillary muscle in the left ventricle with the posterior mitral leaflet, division of this papillary muscle was thought to induce papillary muscle dysfunction of both leaflet. Hence, the chordae of straddling tricuspid leaflet was detached from the shared papillary muscle and the ventricular septal defect was closed by a large pericardial patch. Because of peculiar anatomy of the conduction system in this situation, the junctional area of the inlet septum and tricuspid annulus was avoided from stitching in VSD closure. Suture through the tricuspid septal leaflet and pericardial patch for VSD were used for tricuspid valve replacement as well. The patient showed uneventful postoperative course without any conduction disturbance including the right bundle branch block.     

A case of straddling tricuspid valve associated with VSD]

A case of straddling tricuspid valve associated with VSD was presented, who was diagnosed as VSD with pulmonary hypertension but not diagnosed as straddling in preoperative state. Two-dimensional echocardiography did not demonstrate a small right ventricle and overriding of tricuspid valve to the ventricular septum. Operative findings were as follows; the VSD was common A-V canal type and anomalous chordae of septal leaflet of tricuspid valve crossed through the VSD and attached to the contralateral side of the ventricular septum. We tried to preserve the anomalous chordae to prevent tricuspid regurgitation, so that we carried out patch closure of VSD. To avoid injury conduction system stitches were placed from upper margin of the VSD, and to keep away tricuspid regurgitation we plicated a depression of septal leaflet which caused by anomalous chordae in VSD patch closure. In postoperative state, A-V block did not arise and tricuspid regurgitation was shown a little as well as preoperative state.     

Surgical management of congenital (non-Ebstein) tricuspid valve regurgitation

Congenital tricuspid valve regurgitation (TR) is a relatively uncommon condition that includes a heterogeneous group of lesions with a unique management strategy. There are wide anatomic variations that lead to congenital TR in patients without Ebstein malformation. Possible etiologies may include primary valve abnormalities (eg, congenital absence of chordae) or other forms of tricuspid valve dysplasia as in congenitally unguarded tricuspid valve, and patients with pulmonary atresia and intact ventricular septum, which can be similar to Ebstein's valves or secondary regurgitation in association with other anomalies as in atrioventricular septal defects, right ventricular outflow tract obstructive lesions (pulmonary stenosis or atresia with ventricular septal defect [VSD]), tricuspid valve annular dilatation in association with right ventricular volume overload lesions as in congenital coronary arterial fistula with secondary right ventricular enlargement, and Uhl's anomaly. Iatrogenic etiologies in the congenital population include TR secondary to previous VSD closure (chordal or leaflet injury), pacemaker or internal cardiac defibrillator lead-induced TR, and traumatic TR (ruptured chordae). Presentation depends on the severity of the disease and may be apparent in infancy, childhood, or adulthood.     

Surgical management of Ebstein's anomaly in the adult

Ebstein's anomaly is a rare cardiac malformation that affects the tricuspid valve, right ventricle, and right atrioventricular junction. These anatomical and functional abnormalities cause important tricuspid regurgitation that results in right atrial and right ventricular dilatation and atrial and ventricular arrhythmias. Diagnosis is made by echocardiography. Operation includes tricuspid valve repair or replacement, closure of any interatrial communications, and appropriate antiarrhythmia procedures. Repair of Ebstein's anomaly eliminates right-to-left intracardiac shunting, improves exercise tolerance and functional class, and reduces supraventricular arrhythmias. In addition, quality of life and longevity are improved.     

Surgical treatment of tricuspid regurgitation caused by Löffler’s endocarditis

A 25-year-old man with a history of bone-marrow-transplantation for the treatment of Löffler’s endocarditis underwent surgery for massive tricuspid regurgitation with paroxysmal atrial flutter. Dense fibrosis in the right ventricular endocardium with complete obliteration of the apex was seen intraoperatively, and the right ventricular cavity was diminished. Annular dilatation of the tricuspid valve and entrapment of the posterior leaflet to the endocardial fibrosis were also seen. Annuloplication at the posterior leaflet was performed. In addition, the right atrial free wall was widely resected and the septal and inferior vena cava-tricuspid valve isthmi were cryoablated for the treatment of atrial flutter. Postoperative catheterization revealed rather high right ventricular end-diastolic pressure. However, tricuspid regurgitation disappeared with the increased cardiac output Atrial flutter could not be induced by repetitive stimulation in the postoperative electrophysiological examination.     

A case of successful surgical repair for congenital corrected transposition of the great arteries associated with Ebstein's malformation and diverticulum of atrialized ventricle

Congenital corrected transposition of the great arteries (C-TGA) is frequently associated with VSD and PS. Systemic atrioventricular valve regurgitation is also sometimes present in patients with C-TGA. It is though that left-sided A-V valve regurgitation is caused by the deformities of the tricuspid valve (TV) or intolerance of TV and the right ventricle (RV) against systemic pressure load. We had a patient with C-TGA associated with Ebstein's malformation. The patient suffered from congestive heart failure due to left-sided A-V valve regurgitation. In this case, the septal and posterior leaflets were displaced below the atrioventricular annulus. The edges of all valves were thickened and partially calcified. Focal agenesis was found in the septal leaflet. There was a distinct atrialized ventricle which had a thin wall and no trabeculae. It shaped like a diverticulum of 3 cm in depth toward the diaphragm. Preoperative cardiac catheterization showed that the functional RV had an adequate size (RVEDVI 162% of normal) and ejection fraction was 53%. Left-sided valve replacement was performed using SJM 31M with preservation of TV. Left-sided atrioventricular annulus was so deformed due to the diverticulum that the prosthetic valve was sutured at the ventricular wall below the atrioventricular annulus to which TV had attached. Good clinical result was obtained after surgical treatment.     

Sliding tricuspid valvuloplasty for severe tricuspid regurgitation after corrective surgery of a ventricular septal defect

We encountered a case of massive tricuspid regurgitation after corrective surgery for a ventricular septal defect. Fixation of the septal leaflet to the ventricular septum at the point where it was closed with a pericardial patch and marked annular dilatation were the lesions contributing to the severe tricuspid regurgitation. The posterior leaflet of the tricuspid valve was excised from the tricuspid annulus, slid to the adhering septal leaflet, and then reattached to a safe area of the septal leaflet to prevent conduction disturbance. The sliding repair was supported by annuloplasty with a Carpentier–Edwards ring, and a concomitant right-sided Maze procedure was conducted for atrial flutter.     

Surgical repair of a common atrium in an adult.

We report a rare successful surgical repair of a common atrium (CA) with mild tricuspid valve (TV) regurgitation due to valvular annulus enlargement in a 39-year-old man, who had a complete atrial septum defect (ASD) without the characteristic of an endocardial cushion defect. The left-to-right shunt ratio was 85 percent and the Qp/Qs was 6.7 due to the CA. Left ventriculogram revealed no evidence of typical goose-neck deformity and no mitral valve regurgitation. The operation consisted of making a new atrial septum with an autologous pericardial patch and tricuspid annuloplasty (DeVega) using extracorporeal circulation. There was no evidence of a cleft on the anterior leaflet of the mitral valve or the septal leaflet of the TV. The postoperative echocardiogram showed no residual shunt flow through a new atrial septum and no TV regurgitation, and atrioventricular (AV) dissociation did not occur. We consider this procedure to be widely applicable in consideration of the favorable results obtained after surgical treatment.     

An operative case of left ventricular-right atrial communication complicated with aortic valve stenosis and regurgitation

A 70-year-old woman suffering from aortic valve stenosis and regurgitation and tricuspid valve regurgitation was admitted to our hospital. At the operation, the right atrium and both ventricles were dilated, and a thrill was felt over the aortic root and right atrium. The aortic valve was bicuspid and heavily calcified. After aortic leaflets were resected, a defect was seen in the left ventricle. Then the right atrium was opened. The defect was located in the atrioventricular membranous septum just above the septal leaflet of the tricuspid valve. The defect was diagnosed to be a left ventricular-right atrial communication (group I according to Riemenschneider's classification). The defect was repaired directly and then the aortic valve was replaced with a prosthesis. After the operation, the heart murmur disappeared. Eleven days after the operation, the patient developed a transient complete AV block for a month.     

An unusual variation of left ventricular-right atrial communication

We herein present a 5-month-old boy with an infravalvular-type left ventricular-right atrial communication without an abnormal tricuspid valve leaflet. The thick chorda of the anterior tricuspid leaflet was inserted into the upper edge of the ventricular septal defect which was placed just under the commissure. The jet from the septal defect pushed this thick chorda and opened the commissure, which thus caused the abnormal pathway from the left ventricle to the right atrium. This mechanism of left ventricular-right atrial communication has not yet been reported elsewhere.     

Left ventricular-right atrial communication with a residual ventricular septal defect: a case report of successful VSD patch closure with tricuspid valvuloplasty using folded patch]

A 18-year-old woman with combination of left ventricular right atrial communication (LV-RA communication) and residual ventricular septal defect (VSD) was presented. She underwent the radical operation of atrial septal defect, VSD and infundibular pulmonary stenosis at the age 11 years. Surgery was performed under the diagnosis of LV-RA communication and residual VSD. Two small defect were existed at the septal leaflet of the tricuspid valve, which communicated to left ventricle. The part of patch being used as closure of VSD at the previous operation, including perforated part of the septal leaflet was resected. A folded patch was used to reinforce the residual VSD and valvuloplasty was made using the remaining intact tricuspid tissues. Post-operative clinical course was uneventful. Shunt flow from LV to RA was not demonstrated in the postoperative left ventricular angiocardiography.     

A case of two-chambered right ventricle complicating infective endocarditis and tricuspid regurgitation

A 31-year-old male of two-chambered right ventricle with ventricular septal defect, complicating infective endocarditis and tricuspid regurgitation, was presented. Two-dimensional echocardiographic study demonstrated tricuspid vegetations and a hypertrophied, anomalous muscle bundle in the right ventricle. Cardiac catheterization revealed 58 mmHg pressure gradient between inflow chamber and outflow chamber of the right ventricle. It seems that tricuspid regurgitation was resulted from infective endocarditis. He underwent resection of anomalous muscle bundle, repair of ventricular septal defect, and tricuspid valve replacement with satisfactory result. It has not been reported in Japan so far that tricuspid valve replacement was performed for the treatment of tricuspid regurgitation due to infective endocarditis in the patient with two-chambered right ventricle. In our case, cardiac catheterization was performed after subsidence of infective endocarditis. As echocardiography can detect vegetations and anomalous muscle bundle precisely, surgical intervention would be performed without cardiac catheterization in the case of infective endocarditis intractable to medical therapy.     

Extended vertical transatrial septal approach to the mitral valve

Optimal mitral valve operation requires adequate exposure without impairment of atrial physiology, namely sinus node and atrioventricular node function. We used an extended vertical transseptal atrial approach in 34 consecutive patients. The extended vertical transseptal approach combines two semicircular atrial incisions circumscribing the tricuspid and mitral annuli anteriorly and superiorly, allowing exposure of the mitral valve by deflecting the ventricular side using stay sutures. The right atrium is opened anteriorly along the atrioventricular sulcus. The atrial septum is incised vertically through the fossa ovalis. The right atriotomy is extended superiorly in the right coronary fossa between the right atrial appendage and the atrioventricular sulcus to meet the septal incision. The two joint incisions are extended onto the left atrial roof transversely. At this point, the two semicircular incisions are performed and joined, and mitral valve operation is performed. There were 18 women and 16 men. Five patients had ischemic mitral valve regurgitation, 18 had mitral valve prolapse, and 11 had rheumatic heart disease. The mitral valve was replaced in 17 patients and repaired in 17. There were no perioperative complications associated with the atriotomies, ie, no bleeding, no atrioventricular nodal dysfunction, and no sinus node dysfunction. The extended vertical transatrial septal approach provides good mitral valve exposure without inherent complications.     

Corrected transposition of the great arteries with associated tricuspid valve incompetence--report of a case of successful tricuspid valve replacement]

A 36-year-old woman with corrected transposition (C-TGA) of great arteries with associated systemic atrioventricular valve (A-V valve) regurgitation was presented. Cineangiography revealed that the right sided ventricle was morphologic left ventricle, and left sided ventricle was morphologic right ventricle. In addition, severe systemic A-V valvular regurgitation (Sellers IV) was demonstrated. Under the diagnosis of C-TGA with associated A-V valvular regurgitation, left sided atrioventricular valve replacement was performed using 29 mm SJM valve. Left sided atrioventricular valve was tricuspid valve (TV). A part of the anterior tricuspid leaflet and two pieces of papillary muscle was resected. Postoperative course was uneventful. The pathological findings indicated that valvular tissue had myxomatous degeneration.     

Complete atrioventricular canal and tetralogy of Fallot: surgical considerations

A review of 13 autopsy specimens and of 13 patients who were operated on for complete atrioventricular (AV) canal and tetralogy of Fallot between 1975 and 1985 revealed a number of anatomical details that are important in the successful repair of this combined lesion. A bridging anterior leaflet was present in 25 hearts. A septum primum was present and attached to leaflet tissue in 13. In all 26, a ventricular septal communication was present beneath the bridging anterior leaflet and extended anteriorly, but in 14 there was no ventricular septal defect underneath the posterior leaflet. Additional pathological features included the following: leaflet tissue deficiency (4 hearts), single left papillary muscle (3), accessory valve orifice (4), and left ventricular (4) or right ventricular (RV) (1) dominance. All 26 had infundibular stenosis, and 10 had hypoplastic pulmonary annuli. One had pulmonary atresia, and 6 had branch pulmonary artery stenosis. Surgical technique was modified to include incision of the septum primum in 7. Because of rightward displacement of the anterior ventricular septum and also to minimize the risk of causing subaortic stenosis, the bridging anterior leaflet was divided more toward the tricuspid orifice so as to parallel the crest of the ventricular septum. Transannular RV outflow patches were used in 10 patients, and a right ventricle-pulmonary artery conduit was placed in 1 patient. Three required repair of branch pulmonary artery stenosis. There were no hospital deaths. Three patients died late of residual AV valve regurgitation and branch pulmonary artery stenosis (2) and sepsis (1).(ABSTRACT TRUNCATED AT 250 WORDS)     

Real-time measurement of tricuspid valve annular area for annuloplasty. Experimental study

We used real-time measurement of the tricuspid valve annular area in anesthetized dogs to study the optimal size of the annular area for annuloplasty. During control conditions, the maximum tricuspid annular area appeared at the onset of ventricular systole. The minimum tricuspid annular area appeared between the ventricular isovolumic relaxation phase and the early ventricular filling phase. The maximum annular area varied in seven dogs between 2.18 and 3.10 cm2, and the minimum annular area ranged between 1.68 and 2.45 cm2. In regular sinus rhythm (heart rates 97 to 120 beats/min), the maximal decreases in tricuspid annular area during one cardiac cycle ranged from 14.3% to 23.6% of the maximum size. When the tricuspid annular area after the annuloplasty was kept larger than the minimum area that was observed during the cardiac cycle in the control study, cardiac output and right atrial pressure remained unchanged, as a result of unobstructed ventricular filling. On the other hand, when the annular area was reduced to smaller than the minimum area seen in the control study, a decrease in cardiac output and an elevation of right atrial pressure ensued. These findings suggest that the tricuspid annular area can be safely decreased by annuloplasty to the minimum area seen in the control study without causing a reduction of cardiac output or an elevation of right atrial pressure.     

Caplike double-horned double-outlet right ventricle. Report of two cases.

Two patients with a rare form of double-chambered, double-outlet right ventricle (DORV) are described. Both patients had associated pulmonary stenosis, ventricular septal defect, and a large tricuspid valve whose chordal attachments crossed the right ventricular outflow tract. The angiocardiographic, echocardiographic, and operative findings were distinctive. The right ventricle was positioned as a "cap" sitting atop the ventricular septal defect at the cephalad end of the left ventricle. The right ventricle, with its two "horns," was likened to a rider straddling a saddle. Tricuspid chordal attachments extended into each of the two right ventricular "horn." No repair was performed in the asymptomatic patient. The other repair involved closure of the tricuspid orifice and insertion of a valved extracardiac conduit from the right atrium to the pulmonary artery.     

Successful Repair of the Right Atrial Isomerism, Double Outlet Right Ventricle, Cornmon Atrioventricular Canal, Pulmonary Stenosis, and Total Anomalous Pulmonary Venous Connection

A 6-year-old boy was successfully operated on for double outlet ventricle, common atrioventricular canal with severe valvular regurgitation, right atrial Isomerism, L-loop ventricles, total anomalous pulmonary venous connection, and pulmonary stenosis with hypoplastic left pulmonary artery. The Interventricular rerouting from the left ventricle to the ascending aorta was performed with a spiral patch, the lnteratrlal switching was performed by a Mustard patch, the common atrioventricular orifice was partitioned and valve repair was performed, and an 18-mm valved conduit was inserted between the right ventricle and the pulmonary artery. Although the patient had a small residual ventricular septal defect and pulmonary stenosls, the patient Is alive and well.