Cierre transcatéter de la ventana aortopulmonar. ¿Vale la pena un método de cierre alternativo a la cirugía? / Transcatheter device closure of aortopulmonary window. Is there a need for an alternative strategy to surgery?

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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.

¿Será el TAVI el tratamiento de elección para la estenosis aórtica? / Will TAVI Be the standard of care in the treatment of aortic stenosis?

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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.

The problems that exist when considering the anatomic variability between the channels that permit interventricular shunting.

Although steps are being taken to produce a universally acceptable coding system for categorisation of the congenitally malformed hearts, obstacles remain in the search for consensus. One of the groups of lesions continuing to produce the greatest problems is those that permit interventricular shunting. The difficulties relate partly to the words used to describe the group itself, as those using Germanic languages describe the holes as ventricular septal defects, whereas those using Romance languages consider them to represent interventricular communications. The two terms, however, are not necessarily synonymous. Further disagreements relate to whether the lesions placed within the group should be sub-categorised on the basis of their geographical location within the ventricular mass, as opposed to the anatomic nature of their borders. In reality, attention to both the features is necessary if we are to recognise the full extent of phenotypic variability. In this review, we first review the evolution and theories of analysis naming the channels that permit interventricular shunting. We then demonstrate that embryologic techniques provide evidence that the changing morphology of the developing murine heart parallels the anatomy of the different lesions encountered in the congenitally malformed human heart. We suggest that, with attention paid to the temporal development of the normal murine heart, combined with a strict definition of the plane of separation between the right and left ventricular cavities, it will be feasible to produce a categorisation that is acceptable to all.

Cardiovascular magnetic resonance parameters associated with early transplant-free survival in children with small left hearts following conversion from a univentricular to biventricular circulation.

BACKGROUND: We sought to identify cardiovascular magnetic resonance (CMR) parameters associated with successful univentricular to biventricular conversion in patients with small left hearts. METHODS: Patients with small left heart structures and a univentricular circulation who underwent CMR prior to biventricular conversion were retrospectively identified and divided into 2 anatomic groups: 1) borderline hypoplastic left heart structures (BHLHS), and 2) right-dominant atrioventricular canal (RDAVC). The primary outcome variable was transplant-free survival with a biventricular circulation. RESULTS: In the BHLHS group (n = 22), 16 patients (73%) survived with a biventricular circulation over a median follow-up of 40 months (4-84). Survival was associated with a larger CMR left ventricular (LV) end-diastolic volume (EDV) (p = 0.001), higher LV-to-right ventricle (RV) stroke volume ratio (p < 0.001), and higher mitral-to-tricuspid inflow ratio (p = 0.04). For predicting biventricular survival, the addition of CMR threshold values to echocardiographic LV EDV improved sensitivity from 75% to 93% while maintaining specificity at 100%. In the RDAVC group (n = 10), 9 patients (90%) survived with a biventricular circulation over a median follow-up of 29 months (3-51). The minimum CMR values were a LV EDV of 22 ml/m² and a LV-to-RV stroke volume ratio of 0.19. CONCLUSIONS: In BHLHS patients, a larger LV EDV, LV-to-RV stroke volume ratio, and mitral-to-tricuspid inflow ratio were associated with successful biventricular conversion. The addition of CMR parameters to echocardiographic measurements improved the sensitivity for predicting successful conversion. In RDAVC patients, the high success rate precluded discriminant analysis, but a range of CMR parameters permitting biventricular conversion were identified.

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.

Transcatheter treatment for pulmonary artery occlusion secondary to pulmonary embolism in an infant.

We report a case of successful recanalisation of the left pulmonary artery after occlusion due to embolic thrombi in a 9-month-old infant after surgical repair of a common atrioventricular canal with tetralogy of Fallot. A transhepatic approach was used because of caval vein thrombosis. After the failure of high-pressure balloon angioplasty, the left pulmonary artery was successfully recanalised with cutting balloons, followed by stent implantation with an excellent result.

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.

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.

Echocardiographic features defining right dominant unbalanced atrioventricular septal defect: a multi-institutional Congenital Heart Surgeons' Society study.

BACKGROUND: Definition and management of right dominant unbalanced atrioventricular septal defect (AVSD) remains challenging because unbalance entails a spectrum of left heart hypoplasia. Previous work has highlighted atrioventricular valve (AVV) index as a reasonable defining echocardiographic measure. We sought to assess which additional echocardiographic features might provide further characterization. METHODS AND RESULTS: From a multi-institutional cohort of complete AVSD, 52 preoperative echocardiograms of patients with presumed right dominant unbalanced AVSD (based on AVV index) and 60 randomly selected preoperative echocardiograms from patients with presumed balanced AVSD were reviewed. Cluster analysis of echocardiographic variables was used to group patients with similar features. Discriminant function analysis was used to explore which variables differentiated these groups. Three groups were identified from the cluster analysis. Echocardiographic variables that differentiated these groups were right ventricle:left ventricle inflow angle, LV width/LV length, left AVV color diameter at smallest inflow, left AVV color diameter at annulus, right AVV overriding left atrium, and LV width. Based on procedures and outcomes, 1 group likely represented balanced patients, whereas 2 groups with similar outcomes likely represented unbalanced patients. The dominant differentiating echocardiographic variable between the 3 cluster groups was the right ventricle:LV inflow angle (partial R²=0.86), defined as the angle between the base of the right ventricle and LV free wall, using the crest of the ventricular septum as apex of the angle. CONCLUSIONS: The angle of right ventricle/LV inflow and other surrogates of inflow may be important defining echocardiographic measures of right dominant unbalanced AVSD, although confirmation is needed.

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.