Repair of Double Orifice Left AV Valve (DOLAVV) with Endocardial Cushion Defect in Adult.

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.

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.

Early Complete Atrioventricular Canal Repair Yields Outcomes Equivalent to Late Repair.

BACKGROUND: Repair of complete atrioventricular canal early in infancy has traditionally carried greater morbidity and mortality than repair performed later. However, an individualized anatomy-based repair may give young infants outcomes that are equivalent to older patients. METHODS: We retrospectively reviewed 139 patients who underwent complete atrioventricular canal repair from January 2005 to December 2012. An individualized approach was used: 2-patch repair was performed in 98 patients for large ventricular septal defects and a modified single-patch ("Australian technique") was used in 41 for "shallow" ventricular septal defects. RESULTS: The average age was 25.5 ± 3.9 weeks, 50% were boys, and 78% had trisomy 21. Mean follow-up was 5.1 ± 0.2 years, with 100% completeness of data. There were 3 in-hospital deaths (2.1%) and 1 late death (0.7%). A permanent pacemaker was required in 2 patients (1.4%). The rate for left atrioventricular valve reoperation was 8% at a mean of 211 ± 238 days after the original repair (range, 6 to 682 days). Compared with patients aged older than 3 months, the 39 patients (28%) who were younger than 3 months had similar perioperative courses and rate of reoperation. Compared with patients with an Australian repair, the 98 patients (71%) with a 2-patch repair were more likely to have trisomy 21 and had slightly increased cardiopulmonary bypass and cross-clamp times but similar outcomes. Multivariate analysis showed postoperative left atrioventricular valve regurgitation greater than 2 and left ventricular outflow tract obstruction were significant risk factors for reoperation on the left atrioventricular valve (both p < 0.05). CONCLUSIONS: Repair of complete atrioventricular canal using an individualized surgical approach yields reoperation and early mortality rates similar for younger infants compared with older infants, obviating the need to delay operation in symptomatic patients.

Biventricular repair in heterotaxy patients.

BACKGROUND: Heterotaxy patients' hearts may or may not be suitable for biventricular repair depending on anatomy. Even in the subset that are amenable to surgical septation, cardiac anatomy may present multiple difficulties in achieving a satisfactory repair. However, it is also well known that heterotaxy patients are not ideal candidates for univentricular repair. METHODS: From 2007 until 2012, a total of 20 patients (11 male) with heterotaxy syndrome underwent biventricular repair (left atrial isomerism: 10 and right atrial isomerism: 10) in our center. Their median age at surgery was 40 (range: 3-108) months. Ten patients had dextrocardia. Eleven patients presented with bilateral superior vena cava, three with inferior vena cava (IVC) draining into left atrium, and six with IVC interruption with azygos or hemiazygos continuation. Anomalous pulmonary venous drainage was present in eight patients. One had a common atrium. Atrioventricular septal defect (AVSD) occurred in nine (complete AVSD in seven) patients. Eight patients had double outlet right ventricle (DORV), one had d-transposition of great arteries (d-TGA), and two had congenitally corrected transposition of the great arteries (CC-TGA). Prior palliative procedures included pulmonary artery banding in three patients and left modified Blalock-Taussig shunt in one patient. Complex intra-atrial baffle constructions were required in all patients to direct pulmonary and systemic venous inflow to the appropriate ventricle. Complete AVSDs were corrected using two-patch technique. Intraventricular tunnel repair was done for DORV. Combined atrial and arterial switch was required to rectify abnormal connections in a child with congenitally corrected transposition with normal pulmonary valve, while a Rastelli + Senning was needed in two children with CC-TGA with pulmonary atresia (n = 1) and double outlet of the right ventricle (n = 1). RESULTS: Major early postoperative complications included intestinal gangrene in four patients for which they underwent bowel resection. Two of these patients could not be salvaged. One patient required coiling of aortopulmonary collateral for early postoperative pulmonary hemorrhage. Two patients needed a tracheostomy for prolonged mechanical ventilatory support. Five patients had a pacemaker implanted for complete heart block. There were no instances of atrial baffle stenosis. Median follow-up was 27 (range: 2-46) months. There was one late death secondary to pneumonia. CONCLUSIONS: Satisfactory survival outcomes can be achieved in heterotaxy patients who undergo hemodynamically acceptable biventricular repair. Borderline ventricular hypoplasia and trivial atrioventricular valve regurgitation should not be considered as discouraging factors in anatomically suitable heterotaxy patients as it is possible to adopt a two-stage repair in such patients to achieve biventricular repair at a later stage. Anticipating a higher incidence of conduction problems and gut malrotation preemptively can help reduce the morbidity.

Unique combination of atrioventricular septal defect with cor triatriatum and complete vascular ring.

Atrioventricular septal defect can present with one or more associated anomalies. Cor triatriatum (subdivided left atrium) and vascular rings are among the less frequent. We describe a two-month-old patient with these three cardiovascular anomalies. This case highlights the importance of exhaustive preoperative evaluation in order to achieve successful surgical correction in one stage.

Feasibility and early effectiveness of a custom, hand-made systemic atrioventricular valve using porcine extracellular matrix (CorMatrix) in a 4-month-old infant.

A need persists for a prosthetic, systemic atrioventricular valve replacement in the pediatric population that can be customized to a wide range of annular sizes, has a low risk of thrombosis, possesses optimal hemodynamic performance, and has the potential to remodel and grow with the patient. We describe a technique for successful systemic atrioventricular valve replacement in a 4-month-old infant by use of a handmade, bileaflet systemic atrioventricular prosthesis constructed from porcine extracellular matrix.

Coronary sinus obstruction after atrioventricular canal defect repair.

The coronary sinus can become obstructed with any instrumentation at or near the ostium such as in atrioventricular canal defect repairs. This complication may lead to a wide range of consequences including dyspnea, angina, myocardial infarction, and sudden death. The following report illustrates the importance of careful perioperative echocardiographic evaluation of the coronary sinus in procedures that may affect the sinus and its ostium.

Association between anemia and packed cell transfusion and outcomes of ventricular septal defect and atrioventricular canal repair in children.

Data on the prevalence and impact of anemia and packed red blood cell (PRBC) transfusions in children with congenital heart disease are limited. Our objectives were to determine the prevalence of anemia and its impact and the impact of PRBC transfusion in the initial 5 days after surgical repair on postoperative outcomes in infants with ventricular septal defect (VSD) and atrioventricular (AV) canal. Retrospective chart review of infants (1 year old) (n = 195) with AV canal or VSD who underwent surgical repair at Children's Hospital of Michigan during a 10-year period. Statistical analyses (SPSS 17.0) included Chi square and Student t test as well as regression analysis with significance set at p = 0.05. Preoperative anemia was diagnosed in 45 of 195 (23%) children. Anemic infants had VSD more frequently (80%), significantly shorter bypass and cross-clamp durations, and higher red cell distribution widths. Postoperative outcomes and PRBC transfusions were similar in the groups. On regression analysis, AV canal was associated with a significantly lower (odds ratio 0.21; 95% confidence interval 0.07-0.68, p = 0.009) risk of anemia. Infants who received a PRBC transfusion (n = 42) had significantly lower birth weights as well as weights at surgery and longer postoperative durations of pressor use, ventilation, oxygen supplementation, and length of stay than those who did not (n = 153) receive transfusions. PRBC transfusion was independently associated with longer postoperative length of stay, oxygen, pressor use, and ventilator duration. Approximately 23% of infants with AV canal or VSD are anemic. PRBC transfusions in the initial 5 postoperative days, but not anemia, are independently associated with adverse postoperative outcomes. Further studies to evaluate conservative transfusion strategies in this population are needed.

Two-ventricle repairs in the unbalanced atrioventricular canal defect spectrum with midterm follow-up.

OBJECTIVES: Unbalanced atrioventricular (AV) canal defects include a hypoplastic ventricle (HV) and AV valve (HAVV) precluding complete 2-ventricle repairs (2VRs). Catch-up growth would solve this problem and was induced by increasing HAVV flow. The objectives were to assess reliability of HV and HAVV growth and provide 5- to 15-year 2VR follow-up. METHODS: From 1990 to 2005, 23 consecutive infants (13 females and 10 males) with echo-diagnosed unbalanced AV canal defects (n = 20) or subsets (n = 3) underwent 2VRs. HV volumes (18 left and 5 right) and HAVV sizes estimated from biplane echoes and z values (standard deviation from expected) were determined. Hypoplasia was defined by a z value of less than -2.0. Three operative approaches were used: (1) Staged repairs (n = 9) had complete AVV repairs with partial atrial septal defect and ventricular septal defect closures, which increased HAVV flow and maintained stability. The septal defects were closed later. (2) An asymmetric valve partition (n = 8) was used to increase HAVV size. (3) For moderate hypoplasia, HAVV flow was increased and ASDs/VSDs were left for stability (n = 6). Follow-up at 5 to 19 years was done locally. RESULTS: Staged repairs began at 20 to 328 days (average, 129 days) and were completed 5 to 145 days later (average, 101 days). Midterm survival was 87% (20/23) after 1 central nervous system bleed after trial weaning from extracorporeal membrane oxygenation and 2 later deaths from hyperkalemia. Reoperations for AVV regurgitation (n = 3), AVV stenosis (n = 1), and mitral valve replacement (n = 1) were satisfactory. On follow-up, all hypoplastic structures (HV and HAVV) had grown to normal size. Two patients "doing well" were lost to follow-up. Survivors have satisfactory 2VRs, with 15 of 18 taking no cardiac failure medications. CONCLUSIONS: Reliable HV/HAVV catch-up growth was induced, and all midterm 2VRs were satisfactory.

Biventricular conversion after single-ventricle palliation in unbalanced atrioventricular canal defects.

BACKGROUND: Management of unbalanced common atrioventricular canal (UCAVC) defect by a single-ventricle (SV) approach frequently results in poor outcomes, especially in trisomy 21 patients. In this report we describe our results with conversion to biventricular circulation in UCAVC patients with SV palliation. METHODS: Retrospective review of patients with UCAVC undergoing biventricular conversion from prior SV palliation between 2003 and 2011 was conducted. Mortality and freedom from reinterventions were analyzed using nonparametric methods. RESULTS: Sixteen children with UCAVC (8 patients [50%] were left dominant) and prior SV palliation underwent conversion to biventricular circulation between 2003 and 2011. Median follow-up was 18 months (range, 3 to 94 months). Surgical indications included worsening cyanosis, severe atrioventricular valve regurgitation, or failing bidirectional Glenn or Fontan physiology. All patients had either unequal distribution of the common atrioventricular valve of greater than 60% or one hypoplastic ventricle. By magnetic resonance imaging or computed tomography, 8 patients with right dominant atrioventricular canal had a median left ventricular end-diastolic volume of 32 mL/m(2) (range, 22 to 35 mL/m(2)). Eight patients with a left dominant atrioventricular canal had a median right ventricular end-diastolic volume of 42 mL/m(2) (range, 26 to 64 mL/m(2)). Eleven patients (69%) had trisomy 21, and 3 patients (19%) had heterotaxy. Stages of palliation included stage I in 2 patients, bidirectional Glenn in 10 patients, hemi-Fontan in 2 patients, and Fontan in 2 patients. There was 1 (6%) operative (right ventricle dominant) and 1 (6%) late death (left ventricle dominant). Eight patients required reinterventions, 3 (19%) surgical and 6 (38%) catheter-based. On follow-up, all had improvement in cyanosis and symptoms. CONCLUSIONS: Biventricular conversion from failing SV palliation in UCAVC can be accomplished with an acceptable early and late morbidity and mortality, although need for reintervention was not uncommon.

[Clinical and morphological parallels in mitral valve disorders in infants with atrioventricular defect].

Mitral valves tissue samplings from children with complete (13 patients) and partial (6 patients) atrioventricular defects at the age of from I month to 3 years old were examined. The biopsy material was received during the repeat surgical operation on mitral valve, performed due to residual mitral valve regurgitation grade 3-4 at the period of time from 2 days to 1 year after radical defect correction. On histological examination the areas of myxomatous tissue degeneration occupying more than 50% of mitral valves surface were found in 6 (32%) of the 19 patients. There were dispersed star-shaped cells, architectonics disturbances, deposition of acid mucopolysaccharides and increased content of matrix metalloproteinase 13 in such areas of myxomatous degeneration. The sizes of these areas correlated with mitral valve regurgitation grade. After the radical correction of atrioventricular defect the sutures on the folds and fibrous ring of the mitral valve "cut through" reliably more often in patients with wider areas of myxomatous degeneration, which indicates poor prognosis. According to the ultrastructural classification the majority of mitral valve cells regarded as fibroblasts; there also were found cells with the signs of myogenic differentiation--myofibroblasts and isolated hystiocytes. According to the immunohistochemistry assay the cells phenotype regarded as fibroblastic and endothelial differentiation; in some patients there were found cells of smooth muscle origin.

Cierre de comunicaciones interauriculares en adultos vía percutánea con dispositivo amplatzer. Primera serie reportada en Honduras / Percutaneous closure of atrial septal defects in adults with Amplatzer device. frst serie reported in Honduras

Antecedentes: La Comunicación Interauricular es una de las cardiopatías congénitas más frecuentes. En Honduras en pacientes con indicación de cierre, el método tradicional es la cirugía, otra opción es a través de dispositivos insertados por procedimiento de intervencionismo cardíaco. Pacientes y Métodos: Estudio descriptivo en el cual se incluyeron 27 pacientes con diagnóstico de comunicación interauricular tipo Ostium Secundum con cortocircuito intracardíaco (Qp/Qs) mayor de 1.5: 1, con adecuado tamaño de los bordes, únicas y sin hipertensión arterial pulmonar severa. Resultados: De 27 pacientes, en 24 (90%) se obtuvo éxito en cerrar la comunicación interauricular con dispositivos Amplatzer, sin encontrar complicaciones a corto ni a largo plazo. Conclusión: El cierre de comunicación Interauricular tipo Ostium Secundum a través de dispositivos Amplatzer es un procedimiento seguro y con alta tasa de éxito.

A technique for repair of atrioventricular canal defect in dextrocardia.

Congenital heart disease with dextrocardia, situs solitus, is not uncommonly seen in paediatric cardiac surgery. An approach through the right atrium for correction of associated cardiac anomalies is needed in most of these cases. We present a technique for operating on this subset of patients wherein the heart can be displaced into the left pleural space allowing for surgery in an anatomical orientation that is familiar to the surgeon and emphasize the precautions to be taken following surgery.

Surgical repair of a complete endocardial cushion defect in a dog.

OBJECTIVE: To describe surgical repair of a complete endocardial cushion defect (ECD) in a dog. STUDY DESIGN: Clinical report. ANIMAL: A 5-month-old, 9.2 kg male Shetland sheepdog. METHODS: Echocardiographic examination revealed an ostium primum atrial septal defect (ASD), an inlet ventricular septal defect (VSD), mitral regurgitation (MR) and tricuspid regurgitation (TR), and a complete ECD was diagnosed. Surgical correction was performed using cardiopulmonary bypass (CPB) via right atriotomy. A polytetrafluoroethylene (PTFE) patch was secured along the margin of the inlet VSD using simple continuous suture, then the cleft in the septal mitral leaflet was sutured. Similarly, the cleft in the septal tricuspid leaflets was sutured. To complete inlet VSD closure, the VSD patch was secured to these sutured leaflets by simple continuous suture. Another PTFE patch was used to close the ostium primum ASD. RESULT: After surgery, MR, TR, and interventricular shunting were decreased. The dog was alive 6 years and 5 months after the surgery with no evidence of an interventricular shunt, TR, or other clinical signs. CONCLUSIONS: Complete ECD in a dog was corrected using a 2-patch technique under CPB.

[Clinical analysis of unroofed coronary sinus syndrome with atrioventricular canal defect].

OBJECTIVE: To analyze symptoms, associated anomalies, diagnostic approach, and surgical procedures in patients with unroofed coronary sinus syndrome and atrioventricular canal defect. METHODS: The clinical data of 20 patients with unroofed coronary sinus syndrome from September 1999 to October 2007 were reviewed retrospectively. There were 10 male and 10 female patients. The age ranged from 6 months to 38 years old, with a mean of (11.4 +/- 11.0) years old. The body weight ranged from 6.7 to 73.0 kg, with a mean of (28.4 +/- 21.3) kg. There were 18 cases of partial atrioventricular canal defect, 2 cases of complete atrioventricular canal defect, and 12 cases of common atrium. The initial diagnosis of unroofed coronary sinus syndrome was made by the surgeon at repair of a partial or complete atrioventricular canal defect in 20 patients. Complex unroofed coronary sinus with left superior vena cava (LSVC) directly draining into the left atrium was found in 11 cases, 1 case of LSVC was ligated, 10 cases were reconstructed the intraatrial baffle or the intracardiac tunnel to drain LSVC to right atrium. The other 9 patients with simple unroofed coronary sinus were repaired with other procedures. The associated cardiac lesions were corrected concomitantly. RESULTS: Death occurred in 1 patient with complex congenital cardiac disease due to pulmonary infection. In the 14 early survivors, who had been followed up from 4 months to 3 years, there was no death and severe complications. CONCLUSIONS: When associated with a partial or complete atrioventricular canal defect, LSVC and a common atrium, unroofed coronary sinus syndrome should be considered as a possible additional finding. Repair according to the type of unroofed coronary sinus syndrome is effective.

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

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Quantification of AS and AR.

Trans-esophageal echocardiography (TEE) is routinely used in valvular surgery in most institutions. The popularity of TEE stems from the fact that it can supplement or confirm information gained from other methods of evaluation or make completely independent diagnoses. Quantitative and qualitative assessment permits informed decisions regarding surgical intervention, type of intervention, correction of inadequate surgical repair and re-operation for complications. This review summarizes the various methods for quantification of aortic regurgitation and stenosis on TEE. The application of Doppler echo (pulsed wave, continuous wave and color) with two-dimensional echo allows the complete evaluation of AV lesions.