Repair of double orifice left av valve (dolavv) with endocardial cushion defect in adult

Abstract Double orifice left atrioventricular valve (DOLAVV) or double orifice mitral valve (DOMV) is a rare congenital cardiac anomaly manifesting either as an isolated lesion (mitral stenosis or mitral insufficiency) or in association with other congenital cardiac defects. Signs of mitral valve disease are usually present along with the symptoms of associated coexistent congenital heart diseases. Mitral insufficiency due to annular dilatation is seen when DOLAVV is associated with endocardial cushion defects. Surgical intervention like mitral valve repair or replacement is required in 50% of patients and yields good results. We report a case of a 56-year-old lady who successfully underwent surgical correction of DOLAVV with partial atrioventricular canal defect.

Factors associated with moderate or severe left atrioventricular valve regurgitation within 30 days of repair of complete atrioventricular septal defect / Fatores associados à insuficiência da valva atrioventricular esquerda nos primeiros 30 dias após correção de defeito de septo atrioventricular total

AbstractIntroduction:Left atrioventricular valve regurgitation is the most concerning residual lesion after surgical correction of atrioventricular septal defects.Objective:To determine factors associated with moderate or severe left atrioventricular valve regurgitation within 30 days of surgical repair of complete atrioventricular septal defect.Methods:We assessed the results of 53 consecutive patients 3 years-old and younger presenting with complete atrioventricular septal defect that were operated on at our practice between 2002 and 2010. The following variables were considered: age, weight, absence of Down syndrome, grade of preoperative atrioventricular valve regurgitation, abnormalities on the left atrioventricular valve and the use of annuloplasty. Median age was 6.7 months; median weight was 5.3 Kg; 86.8% had Down syndrome. At the time of preoperative evaluation, there were 26 cases with moderate or severe left atrioventricular valve regurgitation (49.1%). Abnormalities on the left atrioventricular valve were found in 11.3%; annuloplasty was performed in 34% of the patients.Results:At the time of postoperative evaluation, there were 21 cases with moderate or severe left atrioventricular valve regurgitation (39.6%). After performing a multivariate analysis, the only significant factor associated with moderate or severe left atrioventricular valve regurgitation was the absence of Down syndrome (P=0.03).Conclusion:Absence of Down syndrome was associated with moderate or severe postoperative left atrioventricular valve regurgitation after surgical repair of complete atrioventricular septal defect at our practice.

ResumoIntrodução:A insuficiência da valva atrioventricular esquerda é a lesão residual mais preocupante após o tratamento cirúrgico do defeito de septo atrioventricular.Objetivo:Determinar fatores associados à insuficiência da valva atrioventricular esquerda de grau moderado ou importante nos primeiros 30 dias após correção de defeito de defeito de septo atrioventricular total.Métodos:Avaliamos os resultados em 53 pacientes consecutivos menores de 3 anos com defeito de septo atrioventricular total, operados em nosso serviço entre 2002 e 2010. Avaliamos as seguintes variáveis: idade, peso, ausência de síndrome de Down, grau de insuficiência da valva atrioventricular esquerda antes da correção, anormalidades na valva atrioventricular e uso de anuloplastia. A mediana da idade foi de 6,7 meses e a do peso de 5,3 Kg; 86,8% tinham síndrome de Down. Antes da operação, 26 apresentavam insuficiência da valva atrioventricular esquerda pelo menos moderada (49,1%). Anormalidades na valva atroventricular foram encontradas em 11,3% dos casos; anuloplastia foi realizada em 34% dos pacientes.Resultados:Após a correção, houve 21 casos com insuficiência moderada ou grave da valva atrioventricular esquerda (39,6%). Após realização de análise multivariada, o único fator associado com esses graus de insuficiência foi a ausência da síndrome de Down (P=0,03).Conclusão:Ausência de síndrome de Down esteve associada com insuficiência moderada ou grave da valva atrioventricular esquerda após correção cirúrgica de defeito de septo atrioventricular total em nosso serviço.

Factors associated with moderate or severe left atrioventricular valve regurgitation within 30 days of repair of incomplete atrioventricular septal defect / Fatores associados à insuficiência moderada ou grave da valva atrioventricular esquerda nos primeiros 30 dias de correção de defeito de septo atrioventricular incompleto

Abstract Introduction: Left atrioventricular valve regurgitation is the most concerning residual lesion after surgical correction of atrioventricular septal defect. Objective: To determine factors associated with moderate or greater left atrioventricular valve regurgitation within 30 days of surgical repair of incomplete atrioventricular septal defect. Methods: We assessed the results of 51 consecutive patients 14 years-old and younger presenting with incomplete atrioventricular septal defect that were operated on at our practice between 2002 and 2010. The following variables were considered: age, weight, absence of Down syndrome, grade of preoperative left atrioventricular valve regurgitation, abnormalities on the left atrioventricular valve and the use of annuloplasty. The median age was 4.1 years; the median weight was 13.4 Kg; 37.2% had Down syndrome. At the time of preoperative evaluation, there were 23 cases with moderate or greater left atrioventricular valve regurgitation (45.1%). Abnormalities on the left atrioventricular valve were found in 17.6%; annuloplasty was performed in 21.6%. Results: At the time of postoperative evaluation, there were 12 cases with moderate or greater left atrioventricular valve regurgitation (23.5%). The variation between pre- and postoperative grades of left atrioventricular valve regurgitation of patients with atrioventricular valve malformation did not reach significance (P=0.26), unlike patients without such abnormalities (P=0.016). During univariate analysis, only absence of Down syndrome was statistically significant (P=0.02). However, after a multivariate analysis, none of the factors reached significance. Conclusion: None of the factors studied was determinant of a moderate or greater left atrioventricular valve regurgitation within the first 30 days of repair of incomplete atrioventricular septal defect in the sample. Patients without abnormalities on the left atrioventricular valve benefit ...

Resumo Introdução: A insuficiência da valva atrioventricular esquerda é a lesão residual mais preocupante após o tratamento cirúrgico do defeito de septo atrioventricular. Objetivo: Determinar fatores associados à insuficiência da valva atrioventricular esquerda de grau moderado ou importante nos primeiros 30 dias após correção de defeito de defeito de septo atrioventricular. Métodos: Avaliamos os resultados em 51 pacientes consecutivos menores de 14 anos com defeito de septo atrioventricular incompleto, operados em nosso serviço entre 2002 e 2010. Avaliamos as seguintes variáveis: idade, peso, ausência de síndrome de Down, grau de insuficiência da valva atrioventricular esquerda antes da correção, anormalidades na valva atrioventricular e uso de anuloplastia. A mediana da idade foi de 4,1 anos e a do peso de 13,4 Kg; 37,2% tinham síndrome de Down; antes da operação, 23 apresentavam insuficiência da valva atrioventricular esquerda pelo menos moderada (45,1%); anormalidades na valva atrioventricular foram encontradas em 17,6% dos casos; anuloplastia foi realizada em 21,6% dos pacientes. Resultados: Após a correção cirúrgica, 12 casos apresentaram insuficiência da valva atrioventricular esquerda pelo menos moderada (23,5%). A variância entre os graus de insuficiência da valva atrioventricular esquerda pré e pós-operatória nos pacientes com anormalidades na valva atrioventricular não teve significância estatística (P=0,26), ao contrário daqueles sem tais anormalidades (P=0,016). Pela análise univariada, apenas a ausência de síndrome de Down teve significância estatística (P=0,02). Porém, após análise multivariada, nenhum dos fatores teve significância. Conclusão: Nenhum dos fatores estudados foi determinante de insuficiência da valva atrioventricular esquerda de grau moderado ou importante nos primeiros 30 dias após a correção de defeito de septo atrioventricular incompleto na população avaliada. Pacientes sem anormalidades na valva atrioventricular ...

Ultrasound diagnosis of fetal partial and intermediate atrioventricular septal defect / 中华围产医学杂志

Objective To investigate the clinical value of fetal echocardiography in prenatal diagnosis of partial and intermediate atrioventricular septal defect (AVSD). MethodsUltrasound images of11casesof partialandintermediateAVSDconfirmedbyautopsy orpostnatal echocardiography were retrospectively analyzed and the characteristics were summarized. Results Fetal echocardiography of seven cases of partial AVSD and four cases of intermediate AVSD at fourchamber view showed ostium primum atrial septal defect and the mitral and tricuspid valves attaching at the same level on the interventricular septum. Atrio-ventricular valves took on a straight, bar-liked appearance when closed and constituted a “T” shape with interventricular septum. One partial AVSD case showed left ventricle-right atrium canal. All intermediate AVSD cases showed upper ventricular septal defect. There were four cases associated with mitral reflux, one associated with mitral and tricuspid reflux and one with bilateral radius absence. Conclusions “T” shape image is of great clinical value in prenatal diagnosis of fetal partial and intermediate AVSD. The coronary sinus may lead to false positive diagnosis.

Biventricular refair for endocardial cushion defects with double outlet right ventricle / 中华胸心血管外科杂志

ObjectiveDouble-outlet right ventricle,which often associated with total anomalous pulmonary venous connection and complete endocardial cushion defects,has been considered a risk factor for biventricular repair procedure.To reviewed cases treated by biventricular repair for endocardial cushion defects with double outlet right ventricle.MethodsFrom July to November of 2009,6 patients (3 males and 3 females) aged from 7 to 24 (16.17±5.98) years and with endocardial cushion defects and double outlet right ventricle underwent operation of biventricular repair The duration of follow-up ranged from 10 days to 2 years(median,16 months).Endocardial cushion defects were repaired with a 2-patch technique.The artificial vascular patch was implanted to connect the ventricular septal defects and the aorta for draining the blood stream from the left ventricle to the aorta.The other patch was used to repair the ostium primum atrial septal defects.Right ventricular outflow tract obstructions was released and reconstructed by transplanting a bovine pericardium patch.If the size of pulmonary valve annulus was far more below the normal,a transannular pericardial patch was used.Rastelli procedure with a valved conduit between the right ventricle and the pulmonary artery would be performed if the obstruction in the right ventricular outfolw tract was severe.ResultsOne death occurred 2 days after the operation,resulting in a mortality rate of 16.6%.The case,a boy of 7 years old,had a mirror-image dextrocardia,complete endocardial cushion defect ( C type),anomalous pulmonary venous drainage and single atrium.In this case,the operation lasted for 8 hours,acute renal failure happened next day to the operation,the effect of CRRT was unsatisfied,and eventually cardiac arrest occurred as a result of hyperkalemia.The remaining cases had survived.Follow-up examinations showed that the systolic pressure gradients across the pulmonary valves decreased,with a range of 14 to 40 mm Hg,(23.9 ± 11.03) mm Hg.Mild mitral and tricuspid regurgiration were identified in 4 cases and moderate mitral regurgitation was identified in one case.The cardiac function in all patients was classified as NYHA class Ⅰ/Ⅱ,Conclusion Endocardial cushion defects with double outlet right ventricle can be corrected by means of biventricular repair procedure.The procedure was associated with a low mortality,The interim life quality of patients may be improved.The longterm outcomes should be further studied.

Results for repair of 28 cases of endocardial cushion defects combined with double-orifice mitral valve / 中华胸心血管外科杂志

Objective To review the surgical results of 28 cases of endecardial cushion defect combined with double-ori-rice mitral valve. Methods Of 860 consecutive patients with endocardial cushion defect, double-orifice mitral valve was iden-tiffed in 28 patients (3.26% ) form October 1996 to November 2007. Intracardiac deformities were corrected simultaneously during the operation. Preoperative mitral valve function, surgical procedures and incidence of pestoperative mitral valve dys-function were reviewed and compared between patients with total endecardial cushion defect ( group Ⅰ, n = 11 ) and partial en-docardial cushion defect ( group Ⅱ, n = 17). Results There were 4 operative deaths in group Ⅰ caused by severe pulmonary inflammation in 3 cases and low cardiac output in 1 case. Two cases were identified as severe mitral valve dysfunction or steao-sis in reoperaticu or autopsy. There was no later death. Mitral valve function is not eatisfactory in group Ⅱ as compared with group Ⅰ in 3 to 89 months of follow-up( averaging 33 months). Conclusion Double-orifice mitral valve is a high risk factor for operative death in total endocardial cushion defect and the longer term results of partial endocardial cushion defect combined with double-orifice mitral valve is not satisfactory.

Reestablishment the roof of coronary sinus in treating unroofed coronary sinus syndrome with endocardial cushion defect and persistent left superior vena cava / 中华胸心血管外科杂志

Objective To summarize the surgical procedures of unroofed coronary sinus syndrome with endocardial cushion defect and persistent left superior vena cava.Methods From 2000 to 2008,753 cases of endocardial cushion defect underwent surgical repair.Among them 15 cages(2.0%)were associated with persistent left superior vena cava and unroofed coronary sinus syndrome.The drainage of the persistent left superior vena cava and coronary vein were corrected simultaneously during the operation by reestablishing the roof of the coronary sinus.The persistent left superior vena cava were drained to right atrium by intra-atrial tunnel with pericardial patch or left atrium folding in 14 cases and it was ligated in the remain one.Most of the coronary veins were drained into right atrium after the correction of cardiac defect.Results One patient died of severe pulmonary infection early after operation.Echocardiogram indicated that the velocity of left superior vena cava increased in the site of the roof of left atrium in one patient after operation.No obstruction of vena cava and pulmonary vein and remnant shunt between the atriums were found in the other 14 cases.There were no death and other complications during3 months to 6 years of follow-up.Conclusion The surgical procedures of unroofed coronary sinus syndrome with endocardial cushion defect and persistent left superior vena cava is difficult and good result can be expected by reestablishing the roof of the coronary sinus.

Case Report of Partial Endocardial Cushion Defect with Mild Pulmonary Hypertension in Old Age / 대한흉부외과학회지

The partial endocardial cushion defect including ostium primum atrial septal defect and anterior mitral leaflet cleft, presents less significant clinical symptoms than complete endocardial cushion defect. But, as mitral insufficiency develops, cardiomegaly, congestive heart failure, pulmonary arterial hypertension appear. So, partial endocardial cushion defect has poor prognosis and is rarely seen in elderly patients. A 67 years old woman admitted at our hospital for operative treatment with partial endocardial cushion defect. She had increased pulmonary pressure of 45/22 mmHg, mean 32 mmHg. She had repair of ostium primum defect with patch, and the mitral valve was treated with valve replacement. Because advanced atrioventricular block developed postoperatively, she received permanent pacemaker.

Case Report of Partial Endocardial Cushion Defect with Mild Pulmonary Hypertension in Old Age / 대한흉부외과학회지

The partial endocardial cushion defect including ostium primum atrial septal defect and anterior mitral leaflet cleft, presents less significant clinical symptoms than complete endocardial cushion defect. But, as mitral insufficiency develops, cardiomegaly, congestive heart failure, pulmonary arterial hypertension appear. So, partial endocardial cushion defect has poor prognosis and is rarely seen in elderly patients. A 67 years old woman admitted at our hospital for operative treatment with partial endocardial cushion defect. She had increased pulmonary pressure of 45/22 mmHg, mean 32 mmHg. She had repair of ostium primum defect with patch, and the mitral valve was treated with valve replacement. Because advanced atrioventricular block developed postoperatively, she received permanent pacemaker.