Common Atrioventricular Orifice Research Articles

59. Early and late results of routine leaflet augmentation for complete atrio-ventricular septal defect repair

Complete AVSD (CAVSD) is characterized by the presence of a common atrio-ventricular (AV) orifice, an inter-atrial communication, and a ventricular septal defect (VSD). Results of surgical correction of atrio-ventricular septal defects (AVSDs) have improved over the last decades; however, the need for reoperation for left atrio-ventricular valve regurgitation, after primary AVSD repair remains a major concern. The aim of our study is to assess the outcome of the routine leaflet augmentation technique in CAVSD repair. A retrospective database and chart review analysis of all patients who underwent AV canal repair at king Abdul-Aziz Cardiac Center during period from 1999 to September 2014 was conducted. Demographic data, associated anomalies, operative data, ICU and hospital course were reviewed. Early outcomes were reviewed for postoperative complications (Chylothorax, complete AV block, Arrhythmias, early mortality) and late outcomes were reviewed for Left AV valve regurgitation requiring for re-intervention and late mortality. Two hundred and sixty patients underwent leaflet augmentation technique to repair complete AVSD, between January 1999 and September 2014. The mean age was (131.5 months), and mean weight (6.06 kg). A variety of concomitant procedures were performed at the time of repair of the CAVSD, including a total of 49 patients (18.8%) who underwent PDA ligation. Repair of TV (Right AV valve) was performed in 11 patients (4.2%), 9 patients (3.46%) required RVOTO resection, in 5 patients (1.92%), PA plasty was done and 2 patients (0.76%) required ECMO after CAVSD repair. Regarding reoperations, a total of 17 patients (of 260) required reoperation after initial CAVSD repair. The most common indication for reoperation was left AV valve regurgitation in 16 patients (6%) in the follow up period up to 15 years. One patient (0.38%) required diaphragmatic plication. The overall mortality was 3 patients (1.1%). Leaflet augmentation for the repair of the complete AVSD, represent a good surgical alternative technique, allows for good exposure, good LAVV reconstruction and close to anatomical repair and results in reduced incidence of late Left atrio-ventricular valve regurgitation.

症例 前方および後方房室伝導系の共存した,大血管転位・共同房室口症の2剖検例

症例 前方および後方房室伝導系の共存した,大血管転位・共同房室口症の2剖検例

Spectrum of congenital heart defects in Croatia

The aim of our study was to investigate the incidence of congenital defects in children born in Croatia during a period of 5 years, its association with extracardiac malformations, its treatment, and outcome. Medical information about the patients was obtained from 14 paediatric cardiology centres that cover the whole country. Diagnosis was made by clinical findings, electrocardiography, chest X-ray, echocardiography, catheterisation, or autopsy. Between October 1, 2002 and October 1, 2007, there were 205,051 live births in Croatia, 1,480 of which were patients diagnosed with congenital heart disease, accounting for 0.72% of the live-born children. The distribution was made up of 34.6% children with ventricular septal defect, 15.9% with atrial septal defect, 9.8% with patency of arterial duct, 4.9% with pulmonary valvar stenosis, 3.3% with tetralogy of Fallot, 3.3% with transposed great arteries, 3.3% with aortic stenosis, 3.2% with aortic coarctation, 4.3% with atrioventricular septal defect and common atrioventricular orifice, 2.3% with hypoplastic left heart syndrome, and 8.3% other with severe defects. The average age in the time of diagnoses is 70.41 days (SD, 188.13), with low average time of diagnoses of severe heart defects, 9.6 days (SD, 32.52). Among patients, 14.5% had chromosomal defects, syndromes, and/or other congenital major anomalies. During the study, 57 patients died because of cardiac anomalies or other related problems, 24 who died were operated. The rates of specific cardiac defects and association with extracardiac malformations are generally comparable with those reported in similar studies. In spite of all problems, mortality rate of 3.85% is low but could be improved.

Complete Atrio-Ventricular Septal Defect and Wolf-Parkinson-White Syndrome

A full term female infant was born after 41 weeks of gestation, with a birth weight of 3815g and normal Apgar scores (9-10- 10). Immediately after birth tachycardia was noted. An electrocardiogram (ECG) showed a heart rate of 300 beats per minute (bpm) and narrow QRS complexes. The newborn was clinically stable. She was admitted to the neonatal intensive care unit and an intravenous line was inserted for treatment with Adenosine. While preparing for Adenosine infusion her heart rate spontaneously decreased. Her ECG at presentation with normal heart rate is shown in figure1a. On auscultation, a soft systolic murmur was heard. Echocardiography was performed and demonstrated a complete atrio-ventricular septal defect (AVSD) with a large primum defect, large ventricular septal defect, common atrio-ventricular orifice (AV-valve), and balanced size of the right and left ventricles. At around two hours of age the infant started to get frequent, short episodes of supraventricular tachycardia alternating with sinuous rhythm (figure 1b). Prophylactic treatment against recurrent tachy-arrythmia with Digoxin was started (full digitalisation within the first 24 hours and then with a maintenance dose of 5 ?g/kg, B.I.D). The infant was observed for several days and continued to be free of symptoms. She was discharged to her home at five days of age. She was seen five days later and was found to be clinically stable with no tachy-arrhythmia. Her ECG then is shown in figure 2.

Solitary trunk from the right ventricle with a cleft mitral valve simulating the trifoliate left valve of an atrioventricular septal defect with common atrioventricular junction

We report a case of a true cleft in the anterior leaflet of the mitral valve. The cleft, however, is directed toward the ventricular septum, and the left ventricular papillary muscles have the same arrangement as seen in the setting of a common atrioventricular orifice. The atrioventricular septum is intact. This appearance reflects the presence, in this patient, of right ventricular origin of a solitary arterial trunk, so that there was no outflow tract within the left ventricle to interpose between the mitral valve and the septum.

Successful Fontan procedure for asplenia with pulmonary atresia and major aortopulmonary collateral arteries

Asplenia with a single ventricle and major aortopulmonary collateral arteries (MAPCAs) is a rare congenital cardiac anomaly. In this patient group achievement of the Fontan procedure depends on ample rehabilitation of the pulmonary arteries to secure sufficient pulmonary vascular beds and the prevention of progressive pulmonary vascular obstructive disease. Here we report a successful 1-stage bilateral unifocalization of the pulmonary blood supply followed by a staged Fontan procedure in a patient with a univentricular heart and MAPCAs. Clinical Summary The patient was given a diagnosis of asplenia, univentricular heart (right ventricular type), common atrioventricular orifice, pulmonary atresia, bilateral superior vena cavas, total anomalous pulmonary venous connection (intracardiac type), and MAPCAs on the basis of echocardiography and cardiac catheterization. His neonatal aortography showed a small central pulmonary artery (3 mm in diameter) and bilateral multiple MAPCAs (Figure 1, A). The patient’s arterial oxygen saturation (Sa O2) was around 80%, and his pulmonary/systemic blood flow ratio (Qp/Qs) was 1.6 with stable hemodynamics. Subsequent cardiac catheterization at 6 month of age revealed a good-sized central pulmonary artery (Figure 1, B) and 5 large MAPCAs. His SaO2 was approximately 87%, and his Qp/Qs had increased to 3.7. At 1 year of age, he underwent a primary complete unifocalization of his bilateral pulmonary arteries, total anomalous pulmonary venous connection repair for progressive pulmonary venous obstruction (Figure 2), and a left modified Blalock-Taussig shunt (polytetrafluoroethylene [PTFE],* 5 mm). The postoperative course was uneventful except for transient bilateral phrenic nerve palsy. He subsequently did well, while becoming increasingly cyanotic. A catheterization at 2 years of age revealed peripheral pulmonary stenosis, low pulmonary vascular resistance (1.2 Wood units), and normal pulmonary blood flow (Qp/Qs 1.0). The patient then underwent a bilateral bidirectional cavopulmonary shunt and take down of his left modified Blalock-Taussig shunt without cardiopulmonary bypass. This postoperative course was also uneventful, with an SaO2 of 85% and superior vena caval pressure of 10 mm Hg. Eight months after the bilateral bidirectional cavopulmonary shunt, a cardiac catheterization was performed, demonstrating good-shaped pulmonary arteries (Figure 3), low pulmonary vascular resistance (1.4 R*U), low pulmonary artery pressure (mean, 10 mm Hg), and low pulmonary blood flow (Qp/Qs 0.8). Two weeks before definitive surgical intervention, he underwent balloon angioplasty for peripheral pulmonary stenoses and coil embolization of aortopulmonary collateral arteries. At 3 years of age, a fenestrated modified Fontan procedure was performed. An 18-mm PTFE tube graft with a 4-mm PTFE tube graft anastomosed in end-to-side fashion was used for the extracardiac total cavopulmonary connection. The 4-mm PTFE tube graft was anastomosed to the common atrium as a fenestration. The patient was hemodynamically stable intraoperatively, with superior vena caval and inferior vena caval pressures of 11 mm Hg, a common atrial pressure of 4 mm Hg, and an SaO2 of 94%. The patient returned to the cardiovascular intensive care unit in stable condition and was extubated 3 hours after the operation. His postoperative course was uneventful. He was transferred to the general ward on the first postoperative day and discharged home 13 days later without any complications. Three months after the Fontan procedure, another cardiac catheterization was performed, demonstrating a spontaneously occluded tube graft fenestration, an Sa O2 of 96%, low pulmonary artery pressure (mean, 12 mm Hg), and excellent Fontan circulation.

Correction of Complete Atrioventricular Canal

Correction of Complete Atrioventricular Canal

Relationship between scooping of the ventricular septum, morphology of the inferior bridging leaflet and electrocardiographic findings in atrioventricular septal defect with common valvar orifice

AbstractWe described previously that the degree of deficiency of the ventricular septum (scooping) affects electrocardiographic findings in atrioventricular septal defect with separate right and left atrioventricular orifices. The aim of this study was to clarify the clinicomorphological relationships of the degree of scooping, the precise morphology of the atrioventricular valve, and electrocardiographic findings in hearts having a common atrioventricular orifice. In 63 patients with usual atrial arrangement, the outlet, scoop and inlet dimensions of the ventricular septum were measured by biplanar cineangiography and were compared with the body surface area. The scoop/outlet ratio and inlet/outlet ratio were also compared with the body surface area, atrioventricular valvar morphology, electrocardiographic findings, and severity of regurgitation of the atrioventricular valve. The results showed that the three individual dimensions of the septum correlated significantly with the body surface area, even though the scoop/outlet ratio and inlet/outlet ratio did not. A correlation was found between the scoop/outlet ratio and the degree of left axis deviation on the electrocardiogram (r=0.74, p<0.001). In 16 patients with division of the inferior bridging leaflet, there was a lower scoop/outlet ratio than in the remaining 37 with an undivided bridging leaflet (p<0.01). Of 24 patients with counterclockwise rotation in the horizontal loop of the vectorcardiogram, 22 had an undivided inferior bridging leaflet (p<0.01). The morphology of the superior bridging leaflet, in contrast, was not associated with the scoop/outlet ratio, and neither was the severity of valvar regurgitation. We conclude that the degree of scooping of the ventricular septum is related not only to electrocardiographic findings, but also to the morphology of the inferior bridging leaflet in atrioventricular septal defect with common atrioventricular orifice.

Left atrioventricular valve incompetence after repair of common atrioventricular canal defects

Reoperation rate for residual atrioventricular (AV) valve regurgitation after repair of common atrioventricular canal defect (AVCD) is currently between 5% and 10%. This retrospective analysis evaluates the impact of AV valve anatomy, age at repair, and surgical technique on postoperative AV valve incompetence. Between January 1982 and July 1994, 205 patients with common AVCD underwent surgical repair at our institution. A complete form with common atrioventricular orifice (CCAVCD) was present in 81 patients. One hundred twenty-four had separate AV valves and orifices; 22 of these had a restrictive ventricular septal defect component. Preoperative moderate to severe AV valve regurgitation (AVVR) was present in 74 (36%), with a lower incidence in the CCAVCD group (20/81, 25%) versus the transitional (8/22, 36%) or the partial forms (46/102, 45%) (p = 0.02). The incidence was 12.5% (3/24) for CCAVCD patients less than 4 months of age (p = 0.02). Repair of the left AV valve was performed according to the trifoliate approach in most cases (142/205, 70%). Follow-up was 98% complete. By Kaplan-Meier analysis, survival at 12.3 years was 97.8% +/- 1% in partial AVCD, 95.4% +/- 4% in transitional AVCD, and 73.2% +/- 5% in CCAVCD (median follow-up, 60 months). Freedom from reoperation at 12.3 years was 93.5% +/- 2% for partial AVCD, 76.9 +/- 9% for the transitional form, and 68.3% +/- 5% for CCAVCD: Postoperative moderate to severe AVVR occurred in 42 patients (21%), with lower incidence for CCAVCD (10/81, 12.5%) versus transitional AVCD (8/22, 36%) and partial AVCD (24/102, 24%) (p = 0.02). Postoperative moderate AVVR was found in only 1 patient with CCAVCD less than 4 months of age (p < 0.01). Nine patients (5%) underwent reoperation for residual postoperative AVVR Valve repair was performed in all with no operative death. By Cox proportional risk multivariate analysis, preoperative AVVR and double orifice "mitral" valve were associated with increased risk of postoperative left AVVR (p < 0.01), whereas a bifoliate approach appeared to reduce the risk of this event in partial AVCD (p = 0.03). Postoperative AVVR is related to the type of anatomy of the AV valve, to the age at repair, and to the surgical technique employed. Residual AVVR can still be corrected with conservative techniques at low mortality rates. Early repair of common AVCD is associated with a lower incidence of preoperative and postoperative AVVR in CCAVCD and seems to prevent progression of annular dilation and preoperative AVVR in partial AVCD.

Double outlet right atrium and complete atrioventricular septal defect with abnormal findings of the biopsied lung

SummaryWe describe a rare type of atrioventricular septal defect with common atrioventricular orifice and malaligned atrial septum, the latter feature resulting in severe obstruction of the left atrial egress. Excision of the atrial septum and banding of the pulmonary trunk was performed at the age of thirteen months. Lung biopsy demonstrated marked medial hypertrophy with severe intimal fibrosis in the small pulmonary arteries and mild thickening of the pulmonary veins, considered contraindications for corrective surgery.

Biventricular repair of cardiac isomerism with common atrioventricular canal with the aid of an endocardial cushion prosthesis

Between 1983 and 1990, six patients with atrial isomerism associated with common atrioventricular canal and double-outlet right ventricle were operated on with the aid of an endocardial cushion prosthesis. This prosthesis, which has been used for the repair of common atrioventricular canal since 1975, is composed of a vertical Dacron patch and "wings" constructed from the patient's own pericardium, which are attached to both sides of the patch to compensate for the defective atrioventricular valve tissue. The ventricular portion of the patch was extended anteriorly to construct the route for blood flow from the left ventricle to the aorta. Intraventricular repair was performed through the common atrioventricular orifice in all patients and also through an additional right ventriculotomy in one patient. The complex systemic and pulmonary venous return was separated by use of the atrial part of the endocardial cushion prosthesis together with additional prosthetic materials. All patients survived the operation and were followed up from 1 to 9 years. All of them are doing well, although one patient has a significant interventricular residual shunt. Repair of atrial isomerism with common atrioventricular canal and double-outlet right ventricle with an endocardial cushion prosthesis was performed with satisfactory immediate and intermediate-term postoperative results.

The use of intraoperative epicardial echocardiography with color flow imaging during the repair of complete atrioventricular septal defects

The use of intraoperative echocardiography with Doppler color flow imaging has acquired increased popularity for early assessment of efficacy of repair of congenital heart defects soon after discontinuance of cardiopulmonary bypass. This technology has been found useful also for evaluating the anatomy of the lesion prior to repair. Furthermore, with the use of color flow mapping, additional anomalies, such as patent arterial duct or multiple ventricular septal defects, which might have been overlooked during the initial examination, can be easily diagnosed. Moreover, intraoperative echocardiography provides the quickest and most sensitive method for a surgeon to look for residual atrioventricular valves regurgitation and/or residual atrial or ventricular septal defects as well as for depressed ventricular contractility. Previous studies1–4have discussed specifically the applicability of intraoperative color flow imaging during the repair of atrioventricular septal defect with a common atrioventricular orifice using both epicardial and transesophageal methodology. Routine intraoperative echocardiography with Doppler color flow imaging has been used since March 1987 at Duke University Medical Center during repair of congenital heart defects. Our aim was to confirm the preoperative dignosis and to assess the quality of repair soon after discontinuance of cardiopulmonary bypass. Our overall experience now includes 612 patients of whom 239 or 39% were less than one year of age at the time of repair. This indicates our tendency to undertake early correction regardless of the age of patients and of the severity of the lesion.

Management of the left atrioventricular valve in 95 patients with atrioventricular septal defects and a common atrioventricular orifice—a ten year review

SummaryDuring a 10 year interval, between January 1, 1981 and January 1, 1991, primary repair of atrioventricular septal defect with a common atrioventricular orifice was performed in 95 patients younger than 1 year. Patients with atrioventricular septal defect having two atrioventricular orifices and a small ventricular septal defect, and those with severe hypoplasia of the left ventricle, were not included in the present study. According to the presence of associated anomalies, patients were divided into those with simple forms (72%) and those with complex forms (28%). These included parachute deformity of the left atrioventricular valve (10 cases), multiple muscular ventricular septal defects (5 cases), low-lying infundibular stenosis of the right ventricle (4 cases), obstruction of the left ventricular outflow tract (3 cases), tetralogy ofFallot (2 cases) and hypoplasia of the left ventricle (2 cases).

Significance of left ventricular wall stress in infants with atrioventricular septal defect, common atrioventricular orifice and regurgitation across the left atrioventricular valve

SummaryThe outcome of primary repair in young infants having atrioventricular septal defect with a common atrioventricular orifice and regurgitation across the left atrioventricular valve is not yet satisfactory. We studied the significance of the characteristics of left ventricular volume and mass and the predicted wall stress for the outcome of repair in 13 infants with this lesion. Three patients died of left heart failure after operation, although neither residual shunting at ventricular level nor regurgitation across the left atrioventricular valve was present. End-diastolic volume and ejection fraction of the left ventricle were 228 ±66% and 0.65 ±0.06 of normal, respectively, with no difference between the survivors and non-survivors. End-diastolic thickness of the posterior ventricular wall, determined by echocardiogram, was within normal range for body size in all patients.

Technique of management of the left atrioventricular valve in the repair of atrioventricular septal defect with a common atrioventricular orifice

SummaryDespite improvement in the early outcome for patients undergoing complete repair of atrioventricular septal defects with a common atrioventricular orifice, the incidence of reoperation for failure of the repair of the left atrioventricular valve remains high. The surgical techniques employed in our most recent experience, including routine suture of the septal commissure and liberal use of valvar annuloplasty, were successful in achieving a very satisfactory competence of left atrioventricular valve with a iow incidence of reoperation for failure of the repair.

Technique of repair of atrioventricular septal defect with a common atrioventricular orifice

SummaryWe advocate primary repair of atrioventricular septal defect with a common atrioventricular orifice in symptomatic children presenting with intractable congestive heart failure. The single patch technique has been utilized as our procedure of choice. A low operative mortality and a low incidence of residual ventricular septal defect and regurgitation of the left atrioventricular valve requiring reoperation has been achieved. This report emphasizes the technical details of the repair. Careful delineation of anatomic detail is considered to be of the utmost importance. Precise suture placement, tailoring the patch, and reconstruction of the left atrioventricular valve are essential to achieve complete closure of the atrial and ventricular septal defects and functional integrity of the left atrioventricular valve.

Atrioventricular septal defect and the left atrioventricular valve at reoperation

SummaryBetween 1968 and December 1990, 29 patients underwent 33 reoperations for dysfunction of the left atrioventricular valve after correction ofatrioventricular septal defect. Repair of the valve was possible in all 10 patients who, initially, had a common atrioventricular orifice, using straightforward procedures such as closure of the septal commissure and annuloplasties according to Wooler. Additional valvar abnormalities (double orifice; fenestration of leaflets; malpositioned or malformed papillary muscles; and additional clefts) were rare in this group of patients. In contrast, these anomalies were frequent in the 19 patients who, initially, had separate right and left atrioventricular valves.

Atrioventricular septal defect in fetal life—a clinicopathological correlation

SummaryIn a series of over 7,000 pregnancies referred for fetal echocardiography, atrioventricular septal defects were detected in 103 fetuses, (17% of 594 anomalies). A large proportion (42%) also had isomerism of the atrial appendages, abnormalities in arrangement and morphology of the thoracoabdominal organs, and a high incidence of associated cardiac malformations. When this was associated with abnormalities of rhythm, the fetus often showed signs of intrauterine congestive heart failure; hydrops, pleural effusion and ascites. Those with trisomic karyotypes (predominantly trisomy 21) were associated with usual atrial arrangement and concordant connections of the cardiac segments. Of the 103 fetuses, 53 were examined at autopsy. Extracardiac abnormalities were documented, and detailed morphologic examination and measurements of the heart were made. The ratios of inlet to outlet septal disproportion were not significantly different from those found in postnatal hearts. Again, as in postnatal life, those hearts with common atrioventricular orifice had a greater degree of ‘scooping’ of the ventricular septum compared to those with separate right and left atrioventricular valves. There were no significant differences in these findings in relation to the karyotype of the fetus. There was an increased incidence of ventricular dominance, and also of coarctation or hypoplasia of the aortic arch, in fetal hearts when compared to autopsied hearts obtained in postnatal life. In summary, the distinguishing morphological characteristics of atrioventricular septal defect are present from 16 weeks of gestation.

Double-inlet ventricle presenting in infancy: I. Survival without definitive repair

Survival before definitive operations was studied in 191 infants with double-inlet ventricle presenting before 1 year of age (1973 to 1988, median follow-up 8.5 years). The morphologic spectrum was broad, with a great prevalence of associated lesions. The actuarial survival rate before definitive repair was 57% at 1 year, 43% at 5 years, and 42% at 10 years, worse than prior reports because of the younger age at entry into our series. Analysis of univariate risk factors established that right atrial isomerism (18% of the group, relative risk 2.9), common atrioventricular orifice (42%, 2.0), pulmonary atresia (20%, 3.4), obstruction of the systemic outflow tract (18%, 2.5), and extracardiac anomalous pulmonary venous connection (13%, 3.1) were strongly associated with poorer survival. Pulmonary stenosis (40%, 0.35), balanced pulmonary blood flow (9%, 0.40), and presentation at an older age (3%, 0.42 to 0.18) were beneficial (p less than 0.05 to 0.0001). Multivariate analysis allowed the creation of patient-specific curves for prediction of survival for different anatomic and physiologic variants of double-inlet ventricle. A simple additive index was then derived from the multivariate Cox coefficients to enable stratification of risk for these morphologic subgroups of patients and so assist in the making of clinical decisions in infancy.

Imperforate Ebstein's malformation in atrioventricular septal defect

SummaryAnatomic data are presented of a female abortus with the unusual combination of Ebstein's malformation and atrioventricular septal defect with right isomerism of the atrial appendages, left-hand ventricular topology and discordant ventriculoarterial connection. The common atrioventricular orifice was guarded by an effectively common valve, the right half being guarded by a well-formed three-leaflet valve, but the posterior and left lateral wall of the orifice being deficient of valvar leaflet tissue. The right ventricular inlet was a blind chamber, an imperforate dimple being seen which was surrounded by valvar remnants. The apical trabecular and outlet components of the right ventricle communicated with the left ventricle through an outlet ventricular septal defect in front of the anterosuperior leaflet of the atrioventricular valve, giving the potential clinically for the lesion to be misdiagnosed as double inlet left ventricle.