Double-orifice Left Atrioventricular Valve Research Articles

Severe regurgitation of a double-orifice left atrioventricular valve in a patient with repaired atrioventricular septal defect: added value of 3D echocardiography

Severe regurgitation of a double-orifice left atrioventricular valve in a patient with repaired atrioventricular septal defect: added value of 3D echocardiography

Reoperations for left atrioventricular valve dysfunction after repair of atrioventricular septal defect

Postoperative left atrioventricular valve (LAVV) dysfunction is known to be the principal risk factor influencing outcome after repair of all types of atrioventricular septal defect (AVSD). The purpose of the present study was to identify the risk factors for reoperation and to assess the outcomes after reoperation for LAVV dysfunction. Records of 412 patients who underwent anatomical repair for different types of AVSD from January 2000 to July 2012 were reviewed. The study group (n = 60) included 13 additional patients for whom repair ± LAVV reoperation was performed in a primary institution. Outcomes, independent risk factors, reoperation and death were analysed. There were 7 early, (1.7%) and 1 late death. Forty-seven (11.4%) required 64 reoperations for LAVV dysfunction. The median delay for the first LAVV reoperation was 3.5 months (range: 5 days to 10.0 years). Unbalanced ventricles with small left ventricle [odds ratio (OR) = 4.06, 95% confidence interval (CI): 1.58-10.44, P = 0.004], double-orifice LAVV (OR = 5.04, 95% CI: 1.39-18.27, P = 0.014), prior palliative surgery (OR = 3.5, 95% CI: 1.14-10.8, P = 0.029) and discharge echocardiography documenting LAVV regurgitation grade >2 (OR = 21.96, 95% CI: 8.91-54.09, P < 0.001) were found to be independent risk factors for LAVV reoperation. Twelve-year survival and freedom from LAVV reoperation rates were, respectively, 96.1% (95% CI: 94.1-98.1) and 85.8% (95% CI: 81.3-90.3). Survival was significantly worse in patients who underwent LAVV reoperation (P < 0.001) and in those who underwent valve replacement vs valve repair (P = 0.020). After AVSD repair, LAVV dysfunction appears to be the principal factor that influences outcome. It can usually be managed by repair. Need for multiple reoperations is not uncommon. Long-term outcome in patients with repaired LAVV is favourable.

Double-Orifice Left Atrioventricular Valve in Patients With Atrioventricular Septal Defects: Surgical Strategies and Outcome

The surgical management of double orifice left atrioventricular valve (DOLAVV) in atrioventricular septal defects (AVSD) can be challenging and has important surgical implications. Between 1961 and 2009, 44 patients with DOLAVV associated with AVSD were reviewed; this constituted 6.7% of cases of AVSD. The median age at operation was 6.6 years (range 2 months-70 years). DOLAVV was associated with partial, intermediate, and complete AVSD in 28 patients (64%), 1 patient (2%), and 15 patients (34%), respectively. Forty-one patients (93%) had partial or complete closure of the zone of apposition (ZOA), and in 3 patients (7%), the ZOA was left open. The accessory orifice was found to be regurgitant in 4 patients, and in all 4 patients it was closed. Four patients had partial annuloplasties. Early mortality consisted of 1 death (2.2%). This was a patient in whom the tissue bridge was divided and severe regurgitation resulted. Median follow-up was 10.3 years (maximum, 36 years). Three adult patients required mitral valve replacement for severe mitral regurgitation (MR) at 3, 11, and 20 years, respectively. Two of these patients experienced progression of MR resulting from an unsutured ZOA. There were 3 late deaths (6%). One death occurred after mitral valve replacement and the others died of noncardiac causes. No patient had hemodynamically significant mitral stenosis. Repair of DOLAVV in AVSD can be performed with low risk, excellent late survival, and freedom from reoperation. The ZOA is typically closed unless the valve area appears small. In the majority of patients, the accessory orifice is competent and can be left alone. Late repeated repair may be difficult because of leaflet dysplasia.

Double orifice left AV valve with partial atrio-ventricular septal defect

Partial atrioventricular septal defects (PAVSD) account for three percent of all major congenital cardiac anomalies. It is surgically managed by patch closure of the primum atrial septal defect (ASD) and repair of the cleft left atrioventricular (AV) valve. Double orifice left AV valve, however, occurs in five percent of PAVSD and constitutes a management problem. We report here the echocardiographic presentation, illustrate the surgical findings and suggest technical tips for management of this rare condition.

Results of surgical repair of atrioventricular septal defect with double-orifice left atrioventricular valve

The outcome of surgical correction of atrioventricular septal defect with double-orifice left atrioventricular valve has improved in recent years but is still reported to be associated with high mortality and reoperation rates. Controversy exists about the management of the accessory orifice. We evaluated our results with correction of atrioventricular septal defect with double-orifice left atrioventricular valve. Between 1975 and 2006, 21 patients underwent correction of atrioventricular septal defect with double-orifice left atrioventricular valve. Clinical data were obtained by means of retrospectively reviewing inpatient and outpatient medical records. To evaluate the influence of double-orifice left atrioventricular valve on mortality and the need for reoperation, a comparison was made with 291 consecutive patients who, during the same period, underwent correction of atrioventricular septal defect without double-orifice left atrioventricular valve. None of the 21 patients with double-orifice left atrioventricular valve had undergone a previous operation. The accessory orifice was managed with different techniques depending on the severity of the regurgitation. There was no in-hospital mortality, and there were 3 late deaths. Seven patients required 12 reoperations, 7 for left atrioventricular valve insufficiency. Double-orifice left atrioventricular valve had no influence on mortality but was a significant predictor for reoperation compared with repair of atrioventricular septal defect without double-orifice left atrioventricular valve. At the latest follow-up, all 18 survivors were in New York Heart Association functional class capital I, Ukrainian without medication. Only 1 patient showed residual mild left atrioventricular valve insufficiency. Atrioventricular septal defect with double-orifice left atrioventricular valve can be repaired with low mortality. However, double-orifice left atrioventricular valve is a predictor for reoperation. The accessory orifice is often competent and should then be left untouched. If regurgitation of the accessory orifice is present, this is best managed with suture or patch closure.

Complex Atrioventricular Canal

Complex forms of atrioventricular (AV) canal (C) such as; AVC with left ventricular outflow tract obstruction, tetralogy of Fallot with complete AVC, double orifice left AV valve, unbalanced complete AVC, and single ventricle patients with common AVC valve require careful preoperative planning and special techniques. This review will explore these technical modifications and outcomes for repair of complex variants of AVC. Optimal results will be achieved using an individually tailored approach that is guided by careful evaluation of the preoperative studies, precise operative technique, and intraoperative assessment of the reconstructed AV valve, as well as a willingness to re-intervene should the postoperative course not proceed as anticipated.

Clinical characteristics and diagnosis of double-orifice left atrioventricular valve associated with other congenital heart disease

Four cases of double-orifice left atrioventricular (AV) valve are reported. Three of the four patients with double-orifice left AV valve had other associated congenital heart diseases, and the fourth had double-orifice left AV valve alone. A patient with associated ventricular septal defect, who presented with pulmonary congestion and hypertension, suffered from severe heart failure. However, these symptoms improved with the spontaneous closure of the ventricular septal defect. The patient with double-orifice left AV valve alone showed no cardiac symptoms. Two-dimensional and Doppler echocardiography did not detect the double-orifice left AV valve in two of the four patients. More recently, the echocardiographic technique has allowed a noninvasive and more frequent detection of this abnormality. But both of these patients had left-to-right atrial shunt and subsequent reduced transmitral flow and left ventricular volume, which may have made it difficult to detect the morphological and hemodynamic characteristics of double-orifice left AV valve. Careful and repeated echocardiographic observation of mitral configuration is required to determine the presence of double-orifice left AV valve when heart disease is associated with a left-to-right atrial shunt.

A modified 'single patch' technique for complete atrioventricular septal defect correction.

We propose a modified single-patch technique consisting in plication of the patch on the left side, which then is sutured with the free edge of the left atrioventricular valve. The proposed technique offers all the advantages of the single-patch technique and at the same time provides additional tissue for reconstructing appropriately the left atrioventricular valve in cases with leaflet tissue deficiency such as severely dysplastic valve, double orifice left atrioventricular valve. This modification augments the left atrioventricular valve tissue appropriately to the orifice size, promotes leftward displacement and improved coaptation with the mural leaflet.

Correction of Complete Atrioventricular Canal

Correction of Complete Atrioventricular Canal

Surgical results of double-orifice left atrioventricular valve associated with atrioventricular septal defects

Background. Double-orifice left atrioventricular valve (LAVV) is a rare but surgically important anomaly, which is regarded as a risk factor for surgical correction of atrioventricular septal defects (AVSDs). Methods. Of 209 consecutive patients with AVSDs, double-orifice LAVV was identified in 19 patients (9.1%, including 7 infants). Preoperative LAVV function, surgical procedures and results, incidence of postoperative LAVV dysfunction and reoperations were reviewed and compared between patients with this valve malformation (group I, n = 19) and those without it (group II, n = 190). Results. There were no operative or late deaths in group I. Preoperative LAVV function was similar in both groups. The cleft was totally closed in 77.2% of group II and 47.1% of group I ( p < 0.01). In partial AVSDs, freedom from postoperative LAVV insufficiency was 77.0% in group II versus 30.5% in group I at 5 years ( p = 0.009) and freedom from reoperation was 89.9% in group II versus 58.3% in group I at 5 years ( p = 0.012); however, there was no difference in complete AVSDs. None of the infants in group I underwent total cleft closure and 4 of them showed more than moderate LAVV insufficiency postoperatively. Conclusions. Double-orifice LAVV is a significant predictor for postoperative LAVV incompetence and reoperation in partial AVSDs, but not in complete AVSDs. Surgical procedures for the cleft should be individualized with careful intraoperative evaluation of the structure and function of this abnormal valve, especially in partial AVSDs and infants.

Echocardiographic assessment of obstructive lesions in atrioventricular septal defects

OBJECTIVESWe sought to determine the accuracy of transthoracic echocardiography (TTE) in identifying risk factors in patients with an atrioventricular septal defect (AVSD).BACKGROUNDAtrioventricular septal defect is a common lesion, and many decisions about it are based on echocardiography alone. The identification of associated left-sided inflow and outflow obstructive lesions is important, as they are responsible for mortality and morbidity.METHODSBetween 1983 to 1998, 549 patients with AVSD underwent repair. The TTE findings were correlated with surgery, angiocardiography, autopsy or postoperative TTE. Papillary muscle measurements were made in those with either a left ventricular outflow tract (LVOT) or left ventricular inflow abnormality and compared with those measurements from control subjects. Measurements of the LVOT were made in patients with an identified LVOT abnormality.RESULTSThere were 63 missed lesions, decreasing over time. Double-orifice left atrioventricular valve (DOLAVV) and nonobstructive chordae in the LVOT were more often missed. Re-operation was performed to address a missed lesion in 2 of 68 patients. Two of 55 patients died of reasons related to a missed lesion. In 67% of patients, DOLAVV was missed. Abnormal papillary muscle angles were seen with either a LVOT abnormality or DOLAVV. High insertion of the anterolateral papillary muscle was a risk factor for death or residual LVOT obstruction. Abnormal LVOT measurements were found in patients with tunnel obstruction and those with an acquired subaortic ridge.CONCLUSIONSTransthoracic echocardiography provides accurate preoperative information on AVSD.

Surgically created double-orifice left atrioventricular valve: A valve-sparing repair in selected atrioventricular septal defects

Objectives: Some features of the left atrioventricular valve (large mural leaflet, dystrophic tissue) represent a challenge for repair of atrioventricular septal defects without postoperative regurgitation. A retrospective study was conducted to evaluate the results of surgically creating a double-orifice left atrioventricular valve in such circumstances. Clinical results were analyzed according to valvular and subvalvular left atrioventricular valve measurements in pathologic specimens with atrioventricular septal defects. Methods: Among 157 patients operated on for atrioventricular septal defect since October 1989, 10 patients underwent primary repair (n = 8) or reoperation (n = 2) by this procedure. Median age at repair was 3.3 years (0.1-33 years). Anatomic types were complete (n = 3), intermediate (n = 5), and partial (n = 2). Preoperative moderate to severe left atrioventricular valve regurgitation was present in 6 patients. After the repair (two-patch technique in complete atrioventricular septal defect, cleft closed in each case), these 10 patients were found to have moderate to severe residual regurgitation not amenable to repair by annuloplasty. The top edge of the mural leaflet was anchored to the facing free edge of the cleft. Results: No hospital death or morbidity was observed. Left atrioventricular valve regurgitation was absent or trivial (8 patients) and mild (2 patients). Color-coded echocardiography did not show significant left atrioventricular valve stenosis. The mean diastolic pressure gradient across the left atrioventricular valve was 3.2 ± 1.1 mm Hg (1.4-4.5 mm Hg). At a median follow-up of 72 months (6-91 months), there was 1 late death, unrelated to left atrioventricular valve malfunction, due to pulmonary vascular obstructive disease. Left atrioventricular valve regurgitation did not increase over time, except in 1 patient in whom regurgitation recently progressed from mild to moderate. At rest, the mean diastolic pressure gradient across the left atrioventricular valve was 3.8 ± 2.9 mm Hg (1.5-11.2 mm Hg). One child had an early moderate stenosis without pulmonary hypertension. Studies on pathologic specimens (n = 34) indicated that long chordal lengths and large mural leaflet size are essential independent anatomic features to assess its feasibility. Conclusions: Surgical creation of a double-orifice left atrioventricular valve is an effective additional procedure for repair of atypical cases of atrioventricular septal defect. The operation may decrease the need for reoperation or left atrioventricular valve replacement. (J Thorac Cardiovasc Surg 2001;121:352-65)

Surgical management of complete atrioventricular septal defects: A twenty-year experience

Creation of a competent left atrioventricular valve is a cornerstone in surgical repair of complete atrioventricular septal defects. To identify risk factors for mortality and failure of left atrioventricular valve repair and to determine the impact of cleft closure on postoperative atrioventricular valve function, we retrospectively analyzed hospital records of 203 patients between January 1974 and January 1995. Overall early mortality was 7.9%. Operative mortality decreased significantly over the period of the study from 19% (4/21) before 1980 to 3% (2/67) after 1990 ( p = 0.03). Ten-year survival including operative mortality was 91.3% ±0.004% (95% confidence limit): all survivors are in New York Heart Association class I or II. Preoperative atrioventricular valve regurgitation was assessed in 203 patients by angiography or echocardiography and was trivial or mild in 103 (52%), moderate in 82 (41%), and severe in 18 (8%). Left atrioventricular valve cleft was closed in 93% (189/203) but left alone when valve leaflet tissue was inadequate and closure of the cleft might cause significant stenosis. Reoperation for severe postoperative left atrioventricular valve regurgitation was necessary in eight patients, five of whom initially did not have closure of the cleft and three of whom had cleft closure. Six patients had reoperation with annuloplasty and two patients required left atrioventricular valve replacement. Five patients survived reoperation and are currently in New York Heart Association class I or II. On most recent evaluation assessed by angiography or echocardiography (a mean of 59 months after repair), left atrioventricular valve regurgitation was trivial or mild in 137 of the 146 survivors (94%) examined; none had moderate or severe left atrioventricular valve stenosis. By multiple logistic regression analysis, strong risk factors for early death and need for reoperation included postoperative pulmonary hypertensive crisis, immediate postoperative severe left atrioventricular valve regurgitation, and double-orifice left atrioventricular valve. These results indicate that complete atrioventricular septal defects can be repaired with low mortality and good intermediate to long-term results. Routine approximation of the cleft is safe and has a low incidence of reoperation for left atrioventricular valve regurgitation. (J T HORAC C ARDIOVASC S URG 1995;110:1543-54)

Double orifice left atrioventricular valve—diagnosis and management of an unexpected lesion

AbstractDouble orifice left atrioventricular valve is an uncommon condition. This paper describes 10 consecutive patients with the anomaly encountered over 20 months. Diagnosis was assisted by cross-sectional echocardiography and supplementary color Doppler, often requiring non-standard views and a high index of suspicion. There were three anatomical variants—those in which the orifices were equal in size (three of 10 patients), those in which they were unequal and associated with an atrioventricular septal defect (four of 10 patients), and those in which they were unequal in the absence of an atrioventricular septal defect (three of 10 patients). The unequal orifice in both anatomical situations was frequently complicated by valvar regurgitation (six of seven patients). Conservative repair was possible in three of the four patients requiring surgery to the left atrioventricular valve. This condition should be thought of more often in the differential diagnosis of left atrioventricular regurgitation in childhood.

Surgical repair of complete atrioventricular canal defects in infancy: Twenty-year trends

Case histories of 301 patients with complete atrioventricular canal defect presenting to our institution in infancy between January 1972 and January 1992 were reviewed with the purpose of identifying the factors responsible for the observed improvement in perioperative mortality over this time period. A retrospective analysis of hospital records examined 46 patient-related, morphologic, procedure-related, and postoperative variables for associations with perioperative death and reoperation. Operative mortality decreased significantly over the period of the study from 25% before 1976 to 3% after 1987 (p < 0.0001). A number of the 46 variables examined showed trends over time that were similar to that for mortality. Palliative procedures decreased over time. Reoperation for most residual lesions also decreased to the degree that they were essentially eliminated in recent years. The exception to this was reoperation for postoperative left atrioventricular valve regurgitation, which also decreased but remained at 7% in recent years. Both technical and support-related procedural variables showed no trends over time, with the exception of the performance of left atrioventricular valve annuloplasty, which increased over time. Closure of the left-sided cleft was performed in 61% of the patients, with no trend over time. Annuloplasty and cleft closure were not associated with less postoperative left atrioventricular valve regurgitation, fewer reoperations, or lower mortality. Multivariate logistic regression analysis identified only earlier year of operation, the presence of double-orifice left atrioventricular valve, and postoperative residual regurgitation of the left atrioventricular valve as risk factors for death. Experience-related improvements in technical precision achieved over time best account for the reduction in the rate of reoperation for most types of residual lesions and also for the reduction in mortality. The only residual lesion that has not been essentially completely eliminated is left atrioventricular valve regurgitation, with reoperation for this lesion having been reduced in recent years, but not eliminated. Improved understanding of the structural and functional variability of the atrioventricular valve in this lesion may be necessary before postoperative dysfunction of this valve can be completely eliminated.