Common Atrioventricular Valve Research Articles

Factors associated with marked reduction in mortality for Fontan operations in patients with single ventricle

The Fontan operation was originally employed for patients with tricuspid atresia, but its application has been extended to those with a variety of complex defects in which there is functionally only a single ventricular chamber. The outcome of 225 modified Fontan operations carried out between 1984 and 1990 at the Children's Hospital, Boston, for patients with defects other than tricuspid atresia was reviewed. Overall 30 patients (13.3%) had failure of this operation (death or takedown). Results improved significantly during the period of the study, with failure rates of 6.5% (2 of 31) and 3.4% (2 of 59) in the last 2 years of the review. Multivariate analysis showed that pulmonary artery distortion, pulmonary artery pressure, age less than 3 years, use of a nonoxygenated glucose K+ cardioplegic solution, and cardiopulmonary bypass time greater than 180 minutes were associated with worse outcome, while "favorable" atrioventricular valve anatomy (non-left atrioventricular valve stenosis/atresia or common atrioventricular valve) and age greater than 9 years were associated with improved outcome. Excluding cardiopulmonary bypass time from the multivariate analysis, the technique of atrial partitioning for patients with left atrioventricular valve atresia/stenosis became the most important variable, followed by the others noted in the initial multivariate analysis except for age greater than 9 years. In the last 2 years of the study 31.1% of patients were less than 3 years of age. During the period of the study there was no significant decline in preoperative risk factors. These results show that modified Fontan operations can be carried out with a high likelihood of success in properly selected patients with complex defects in whom there is functionally a single ventricle.

Echocardiographic evaluation of atrioventricular orifice anatomy in children with atrioventricular septal defect

In atrioventricular (AV) septal defect, the common AV valve can have a common orifice or can be divided by bridging leaflet tissue into two separate orifices. To determine the accuracy of a two-dimensional echocardiographic technique devised specifically for evaluation of the number of AV valve orifices, all 69 children undergoing surgical repair of AV septal defect from April 1987 to August 1999 were examined prospectively. The presence of bridging leaflet tissue and the number of AV valve orifices were determined with use of a subcostal imaging plane. From a standard subcostal four-chamber view, the plane of sound was rotated 30 ° to 45 ° clockwise until the AV valve was seen en face. The plane of sound was then tilted from a superior to an inferior direction so that cross-sectional views of the AV valve were examined from the inferior margin of the atrial septum to the superior margin of the ventricular septum.Of the 69 patients, 6 (9%) were excluded because the appropriate subcostal images were not obtained (in 3 because of obesity and in 3 as a result of operator failure). The remaining 63 children, ranging in age from 1 day to 13.5 years and in weight from 1 to 55 kg, constituted the study group. Echocardiographic results were compared with surgical observations in 62 patients and with autopsy findings in 1 patient.With the two-dimensional echocardiographic technique, 32 of 33 patients with a common orifice and 28 of 30 patients with two separate AV valve orifices were correctly identified. By chi-square analysis, the echocardiographic technique allowed correct identification of a common orifice valve with 94% sensitivity and 97% specificity. For correct identification of two separate orifices, the echocardiographic technique had 97% sensitivity and 94% specificity. The positive predictive value of the echocardiographic technique was 97% for a common orifices and 93% for two separate orifices. Thus, in patients with AV septal defect, the presence of bridging leaflet tissue and the number of AV valve orifices can be accurately determined with use of a subcostal two-dimensional imaging plane.

Prevalence at birth, “natural” risk and survival with atrioventricular septal defect

SummaryPrevalence of atrioventricular septal defect in liveborn infants at birth was calculated in Bohemia (population of 6.3 million). Of 664,208 liveborn infants, 4,409 had a congenital malformation of the heart and in 126 of them (0.19/1,000 live births, 2.9% of all heart defects) this was found to be an atrioventricular septal defect. The ratio between those with a common valvar orifice (complete form) versus those with separate right and left atrioventricular valvar orifices (incomplete form) was 1.14:1. The ratio of girls to boys was 1.17:1. Children born in March, July and October were more likely to have the defect (0.26/1,000 live births) than those born in May (0.10/ 1,000 live births). The defect was the eighth most frequent critical congenital cardiac malformation. Eight in each 100,000 liveborn children, and 3.56% of all patients with congenital cardiac disease, are at high risk of dying due to deficient atrioventricular septation, which in 82.5% resulted in the presence of a common atrioventricular valve (complete form). Unconditional death rate was studied in 1,008 liveborn children who died with a cardiac malformation before the age of 15 before the advent of cardiac surgery. Of these, 64 (6.35%) died with an atrioventricular septal defect which was complete in 5.95% and incomplete in 0.40%. All but one of 60 children with the complete form (common atrioventricular valve) died in the first year of life. The last child died between the age of 2 and 5 years. The estimate for natural survival was calculated using our own data on the prevalence and age distribution of mortality. Of all the liveborn children with atrioventricular septal defect, 91% are expected to survive the first month, 81% (78−85) the first trimester, 63% (56−69) six months, and 49% (35−54) the first year. The survival curve remains stable thereafter.

Corrected Transposition of the Great Arteries, Common Atrioventricular Valve, and Dextrocardia

Corrected transposition of the great arteries with a common atrioventricular valve is a rare congenital anomaly. In the presence of two well-defined, atrioventricular valves, cross-sectional echocardiography is the method of choice for the diagnosis of this cardiac defect. The presence of a common atrioventricular valve can be a limitation of the method. Angiocardiography provides additional information for the diagnosis. (ECHOCARDIOGRAPHY, Volume 8, September 1991)

Bidirectional cavopulmonary anastomosis using vertical vein for right isomerism, pulmonary atresia, and TAPVR

An 8-year-old girl who was diagnosed as having right atrial isomerism, pulmonary atresia, butterfly-shaped juxtaductal pulmonary arterial stenosis, total anomalous pulmonary venous return of the supracardiac type, regurgitant common atrioventricular valve, and univentricular heart of the right ventricular type underwent bilateral, bidirectional cavopulmonary anastomosis using a vertical vein and anastomosis between the common pulmonary venous trunk and atrium. The surgical procedure in this patient is described.

Inferior vena cava-pulmonary artery extracardiac conduit: A new form of right heart bypass

From November 1988 to May 1989, four patients underwent total right heart bypass by means of bidirectional cavopulmonary anastomosis and interposition of an extracardiac conduit from the inferior vena cava to the pulmonary artery. All of them had an uneventful postoperative course, and there have been no early or late deaths. We propose this technique as an alternative surgical option in candidates for a Fontan procedure with (1) hypoplasia or atresia of the left atrioventricular valve, (2) common atrioventricular valve, (3) anomalies of systemic and pulmonary venous return, or (4) auricular juxtaposition.

Aortopulmonary shunt with replacement of a common atrioventricular valve in a patient with double inlet ventricle

Replacement of a common atrioventricular valve with a St. Jude medical prosthesis, together with construction of a central aortopulmonary shunt using a polytetrafluoroethylene graft, were performed on a patient with double inlet ventricle complicated by severe regurgitation across an atrioventricular valve and hypoplastic pulmonary arteries. No complications were noted during a postoperative follow-up of 58 months; indeed, a considerable increase was noted in postoperative exercise capacity. This simple surgical procedure should be considered as a mode of treatment in patients with this combination of lesions.

Common atrioventricular valve guarding double inlet atrioventricular connexion: natural history and surgical results in 76 cases

We describe 76 patients in whom a double inlet atrioventricular connexion was guarded by a common atrioventricular valve. The atriums were connected to a dominant right ventricle in 42, to a left ventricle in 29 and to a solitary indeterminate ventricle in 5. The most common atrial arrangement was isomerism of the right atrial appendages, seen in 46% ( 35 76 ). Of these, there was associated anomalous pulmonary venous connexion in 72% and absence of the spleen in 63%. Double outlet right ventricle was the most frequent ventriculoarterial connexion, present in 37 cases. Abnormal atrioventricular valvar function could already be detected in 46% of cases at the time of initial presentation. A decrease of function was observed in 18 patients, in 14 of them 2 weeks to 16 years (mean 5.7 years) after palliative surgery. The majority of the palliative procedures were constructions of shunts, since 75% ( 57 76 ) of the patients showed obstruction to flow of pulmonary blood. Patients with double inlet ventricle guarded by a common atrioventricular valve have a high early mortality (37% died in the neonatal period). Management should be designed to protect the integrity of the pulmonary vascular bed. Definitive repair with a Fontan type procedure is the most suitable final approach.

Echocardiographic spectrum of double inlet ventricle: Evaluation of the interventricular communication

To determine the spectrum and associated anomalies of double inlet ventricle (single ventricle), echocardiographic data of 50 patients with double inlet ventricle were reviewed and compared with the data obtained by cardiac catheterization, cardiac surgery and autopsy. Standard echocardiographic planes were used to determine the cardiac anatomy and the size of the interventricular communication. Double inlet by way of two perforate valves was found in 44 patients. In 42 of the 44 patients the dominant ventricular morphology was of the left ventricular type (double inlet left ventricle); in 13 of these 42 patients stenosis of one atrioventricular (AV) valve was found. Double inlet right ventricle and double inlet indeterminate ventricle were each found in one patient. Double inlet by way of a common AV valve was found in six patients, all of whom had atrial isomerism. The diagnosis of double inlet ventricle was accurate by two-dimensional echocardiography in all 44 patients. A restrictive interventricular communication was shown in 13 patients and a nonrestrictive communication in 17 patients by cardiac catheterization. Patients with a restrictive interventricular communication had a significantly smaller interventricular communication area normalized by the body surface area (mean +/- SD 1.21 +/- 0.53 cm2/m2) than did those with a nonrestrictive interventricular communication (2.33 +/- 0.71 cm2/m2) (p less than 0.01). Infants with an aortic anomaly had a significantly smaller interventricular communication area (1.35 +/- 0.65 cm2/m2) than did those without an aortic anomaly (2.57 +/- 0.76 cm2/m2) (p less than 0.05). Echocardiography provides an accurate noninvasive diagnosis in patients with double inlet ventricle, offering reliable information about the restrictive interventricular communication.

Truncus solitarius pulmonalis

The case of a female neonate with truncus solitarius pulmonalis (TSP), a rare congenital heart anomaly, is reported. She died four days after birth. A single right ventricle with common atrioventricular (AV) valve was associated with (Collet & Edwards type II) truncus arteriosus communis, and appeared to have a single coronary artery with an abnormally high take-off near the origin of the right subclavian artery. However, the vessel that appeared to be the single coronary artery was in fact an extremely hypoplastic ascending aorta associated with aortic atresia and agenesis of the left ventricle and mitral valve.

Septation and Fontan repair of univentricular atrioventricular connection

From April 1986 to September 1988, 12 patients with double- or common-inlet left ventricle and left anterior rudimentary right ventricle underwent septation while 17 patients with double- or common-inlet left or right ventricle underwent the Fontan operation. In the septation group, three patients who had pressure gradients ranging from 10 to 85 mm Hg between the left ventricle and the aorta underwent enlargement of the outlet foramen, and all survived. One of the 12 patients had a common atrioventricular valve that was repaired by separating the atrioventricular valve by the procedure used for atrioventricular septal defect. One had complete heart block before septation and the other one had it after separation. One 17-year-old woman, who had the smallest left ventricle (168% of normal), died in the hospital (mortality, 8.3%). In the Fontan group, one patient who died in the hospital (5.9%) had high pulmonary resistance of 4.4 U/m2, and one late death (5.9%) occurred in a patient who had complete heart block and a high mean pulmonary arterial pressure of 20 mm Hg. Because of suprasystemic pressure in the pulmonary ventricle, two patients had immediate takedown of the septation repair and substitution of the Fontan repair. Although right atrial pressure was almost equal in both groups after operation, the cardiac index was significantly higher in the septation group than in the Fontan repair group (p less than 0.01). These data suggest that patients who are candidates for either septation or Fontan repair might fare better with septation.

共通房室弁逆流を伴う複雑心奇形に対する外科治療 共通房室弁修復法の検討

共通房室弁逆流を伴う複雑心奇形に対する外科治療 共通房室弁修復法の検討

Cross sectional echocardiographic anatomy of common atrioventricular valve in atrial isomerism.

The morphology of the common atrioventricular valve and its relation to the ventricles was assessed by cross sectional echocardiography in 17 patients with atrial isomerism--four with left isomerism and 13 with right isomerism. In 13 patients the atrioventricular valve consisted of four leaflets: a large anterosuperior leaflet, two lateral leaflets, and a posteroinferior leaflet. The remaining four patients had two anterosuperior leaflets. Five patients had hearts with a double inlet right ventricle and all of them had a rudimentary left ventricle. All the commissures were attached to the right ventricle. Five patients showed severe right ventricular dominance. The atrioventricular morphology in these five hearts differed from that in the patients with the usual atrial arrangement, concordant atrioventricular connection, and hypoplasia of the left ventricle. Only the anterosuperior leaflet bridged the rudimentary left ventricle, and the posteroinferior leaflet was tethered to the ventricular septal crest. Six patients had a biventricular atrioventricular connection and two of them showed mild right ventricular dominance. The atrioventricular morphology in these hearts was the same as that seen in the patients with usual atrial arrangement and a concordant atrioventricular connection. The remaining patient showed dominance of the left ventricle. Only the anterosuperior leaflet bridged the rudimentary right ventricle in this heart.

Intraoperative prebypass and postbypass epicardial color flow imaging in the repair of atrioventricular septal defects

The use of intraoperative epicardial Doppler echocardiography with color flow imaging both before and after cardiopulmonary bypass to assist repair of congenital heart defects in infants and small children has not been reported. To demonstrate its simplicity and utility, we obtained immediately prebypass and postbypass examinations from 18 consecutive patients undergoing repair of atrioventricular septal defects between March 1987 and March 1988 (aged newborn to 3 years, smallest 2.4 kg, 11 patients less than 1 year old). Direct application of a sterile transducer to the epicardial surface eliminates any restriction imposed by the transesophageal approach, and the routine use of color flow imaging enables the operating surgeon to directly evaluate intracardiac anatomy and flows in a variety of orientations. Average examination time was 3.95 +/- 1.96 minutes. Prebypass studies disclosed that a short-axis inspection through the common atrioventricular valve orifice produced a unique visualization of the dynamic commitments of atrioventricular valve tissue throughout systole and diastole that was helpful in planning valve allocation during repair. In addition, echocardiography demonstrated features not previously appreciated in seven of 18 patients (39%). In all, image quality and resolution were vastly superior to preoperative chest wall studies. Postbypass studies revealed significant residual interventricular shunts in two of 18 patients (11%). Views obtained from various orientations directed specific and efficient repair immediately so that all patients left the operating room with documented, surgically acceptable results. Comparison of ventricular function between prebypass and postbypass studies enabled appropriate application of pharmacologic agents in the operating room if necessary. All patients survived their operation. There have been two late deaths, and 16 patients are alive and doing well (follow-up: 9 to 21 months). These experiences indicate that intraoperative epicardial Doppler color flow imaging (1) can be easily learned and applied by the surgeon, (2) enhances the repair of atrioventricular septal defect by providing unique spatial, anatomic, and flow information in the beating heart at the time of repair, (3) increases confidence of a surgically acceptable repair before the patients leave the operating room, (4) guides specific surgical or anesthetic adjustments to optimize results, and (5) works as a valuable aid that may help reduce poor results in the repair of complex congenital cardiac lesions.

Annuloplastic reconstruction for common atrioventricular valvular regurgitation in right isomerism

Two patients who had common atrioventriculac valvular regurgitation associated with right isomerism, univentricular heart of the right ventricular type, transposition of the great arteries, pulmonary stenosis, and both systemic and pulmonary venous anomalies underwent common atrioventricular valvular annuloplasty. They also underwent bilateral, bidirectional cavopulmonary shunt. A Carpentier's ring was used in patient 1 and a polytetrafluoroethylene tube was used in patient 2 to reduce the diameter of the common atrioventricular annuli. Postoperative catheterization confirmed complete elimination of regurgitation of the common atrioventricular valve in both patients.

The significance of tricuspid regurgitation in hypoplastic left-heart syndrome

Palliation of hypoplastic left-heart syndrome involves use of the morphologic right ventricle as the systemic ventricle and the tricuspid valve (in cases of mitral atresia/stenosis) or the common atrioventricular valve (in cases of malaligned atrioventricular canal) as the systemic atrioventricular valve. To determine the relationship between tricuspid or common atrioventricular valve function and the ultimate outcome of palliative surgery, 100 patients with hypoplastic left-heart syndrome were evaluated preoperatively by Doppler echocardiography to determine the degree of tricuspid regurgitation. These patients were then followed serially to assess changes with time in the functional status of the tricuspid or common atrioventricular valve and to determine the correlation of tricuspid or common atrioventricular valve regurgitation with survival. We discovered that tricuspid or common atrioventricular valve regurgitation is common in hypoplastic left-heart syndrome. Thirty-seven percent of the patients had mild, 13% had moderate, and 3% had severe tricuspid or common atrioventricular valve regurgitation on their preoperative Doppler echocardiograms. Throughout the first 2 postoperative years most patients had no significant change in the degree of tricuspid or common atrioventricular valve regurgitation when findings were compared to those of the preoperative echocardiogram. Patients with moderate or severe tricuspid or common atrioventricular valve regurgitation preoperatively had a significant reduction in their survival when contrasted with patients with no or mild atrioventricular valve regurgitation. We therefore conclude that tricuspid or common atrioventricular valve competence is a significant factor in long-term survival after palliative surgery for hypoplastic left-heart syndrome. This function, however, appears to be unaffected by palliation and remains relatively constant over the first 2 postoperative years.

Corrective Repair of Complete Atrioventricular Canal Defects and Major Associated Cardiac Anomalies

Twenty-nine patients with balanced forms of complete atrioventricular (AV) canal defects and tetralogy of Fallot in 12, double-outlet right ventricle (DORV) in 10, and additional anomalies associated with atrial isomerism in 7 underwent corrective repair. Age ranged from 39 days to 24 years (median, 3.7 years). Repair included reconstruction of the common AV valve in 25 patients and prosthetic valve replacement in 4. Construction of an intraventricular tunnel connecting the left ventricle with the aorta was accomplished in 23 of 25 patients with ventriculoarterial concordant or double-outlet connections. In 2 patients with situs inversus and associated DORV and in 4 patients in the isomerism group who had ventriculoarterial discordant connections, repair included intraatrial venous diversion by a modified Senning procedure in 2, a modified Mustard venous switch in 1, and a tailored atrial baffle in 3. A valved extracardiac conduit was required for repair in 4 patients. Partial anomalous systemic venous connection was repaired in 7 patients and total anomalous pulmonary venous connection, in 5. There was 1 hospital death (3%) and 3 late deaths (11%). There was 1 (3.6%) reoperation to replace an incompetent right (systemic) AV valve 17 months postoperatively, and each of the 25 surviving patients was in New York Heart Association Functional Class I (80%) or II (20%) at follow-up.

The modified Fontan operation for asplenia and polysplenia syndromes

From 1975 through 1986, 49 patients with asplenia (23 patients) or polysplenia (26 patients) syndromes underwent a modified Fontan operation. All patients had anomalous systemic venous return, and 43 (88%) had anomalous pulmonary venous return. The atrioventricular valve anatomy varied: 36 patients had a common atrial chamber and common atrioventricular valve, eight had mitral valve atresia, and five had severe mitral valve hypoplasia. Redirection of systemic venous return was accomplished by (1) atrial baffle (29 patients), (2) intraatrial conduit (19 patients), and (3) extraatrial conduit (one patient). There were 21 (43%) hospital deaths overall. However, since 1985, six of 22 patients (27%) have died. Increased mortality was seen in patients requiring atrioventricular valve repair or replacement (8/11, 73%) and in patients with asplenia (65%). Lower mortality was seen in patients with polysplenia (24%) and those receiving an intraatrial conduit (26%), although this decrease also represents the more recent experience. We conclude: (1) The Fontan operation for patients with asplenia or polysplenia syndromes has resulted in a significant (although recently declining) mortality; (2) mortality is higher in patients with asplenia; (3) patients with atrioventricular valve insufficiency requiring repair or replacement are at higher risk; and (4) intraatrial conduits seem to offer promise for successfully accomplishing this repair.

Clinical implications of atrial isomerism.

Right atrial isomerism or left atrial isomerism is frequently diagnosed as situs ambiguous without further discrimination of the specific morbid anatomy. Thirty six cases of right atrial isomerism and seven cases of left atrial isomerism were collected from the records and pathological museum at the National Taiwan University Hospital. There was a necropsy report for 18 cases. In all patients one or more of the following conditions was met: (a) isomeric bronchial anatomy, (b) echocardiographic and angiocardiographic evidence of isomerism, and (c) surgical or necropsy evidence of abnormal atrial anatomy. An anomalous pulmonary venous connection was present in 55% of patients with right atrial isomerism; in left atrial isomerism one case (14%) had a partial anomalous pulmonary venous connection. Forty per cent of cases of anomalous pulmonary venous connection with right atrial isomerism had obstruction. Six (86%) of seven cases with left atrial isomerism had an ambiguous biventricular atrioventricular connection. In contrast, univentricular atrioventricular connection (26 of 36, 72%) was significantly more common in right atrial isomerism. A common atrioventricular valve was the most frequent mode of connection in both forms. Two discrete atrioventricular valves were significantly more common in left atrial isomerism. Atrioventricular valve regurgitation was detected in 14 cases. Double outlet right ventricle was the most common type of ventriculoarterial connection. The most commonly cited causes of death after either palliative or definitive operation were undetected anomalous pulmonary venous connection, pulmonary venous stricture, and uncorrected atrioventricular valve or aortic regurgitation complicated by abnormal coagulation. Although the prognosis is poor, successful operation depends on knowledge of the precise anatomical arrangement associated with atrial isomerism.

Modified Fontan procedure: atrial baffle and systemic venous to pulmonary artery anastomotic techniques.

Two technical modifications to the modified Fontan procedure are presented. Systemic venous to pulmonary artery continuity is achieved by superior vena cava (SVC) division and end to side anastomosis to the right pulmonary artery (RPA), particularly following a right Blalock shunt, or by RPA division and anastomosis to the SVC, particularly in the presence of RPA stenosis. Intra-atrial partitioning is achieved by a systemic venous baffle rather than a pulmonary venous baffle. This is particularly useful in the presence of left atrioventricular valve atresia, but may be a preferable technique with double-inlet single ventricle or single ventricle with common AV valve. These techniques were applied successfully to 10 of 12 children with various forms of single ventricle, including 5 with left AV valve atresia or stenosis.