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Over the past 2 decades, several categorizations have been proposed for the abnormalities of the aortic root. These schemes have mostly been devoid of input from specialists of congenital cardiac disease. The aim of this review is to provide a classification, from the perspective of these specialists, based on an understanding of normal and abnormal morphogenesis and anatomy, with emphasis placed on the features of clinical and surgical relevance. We contend that the description of the congenitally malformed aortic root is simplified when approached in a fashion that recognizes the normal root to be made up of 3 leaflets, supported by their own sinuses, with the sinuses themselves separated by the interleaflet triangles. The malformed root, usually found in the setting of 3 sinuses, can also be found with 2 sinuses, and very rarely with 4 sinuses. This permits description of trisinuate, bisinuate, and quadrisinuate variants, respectively. This feature then provides the basis for classification of the anatomical and functional number of leaflets present. By offering standardized terms and definitions, we submit that our classification will be suitable for those working in all cardiac specialties, whether pediatric or adult. It is of equal value in the settings of acquired or congenital cardiac disease. Our recommendations will serve to amend and/or add to the existing International Paediatric and Congenital Cardiac Code, along with the Eleventh iteration of the International Classification of Diseases provided by the World Health Organization. [ABSTRACT FROM AUTHOR]

In the setting of transposition, recognition of the variability in the morphology of the outflow tracts and the arterial trunks is essential for surgical repair. Presence of a unifying system for classification would minimize the risk of misunderstanding. We examined an archive of 142 unrepaired hearts with transposition, defined as the combination of concordant atrioventricular and discordant ventriculoarterial connections, combined with the clinical records of 727 patients undergoing the arterial switch procedures. In the setting of usual atrial arrangement, we found the aortic root in leftward or posterior location in up to 5% of our cohorts, making the common term "d-transposition" ambiguous. Variability in the relationship of the trunks was commoner in the setting of deficient ventricular septation, especially when the trunks were side-by-side (14% vs 5.5% when the ventricular septum was intact). Bilateral infundibulums were present in 14% of cases, and bilaterally deficient infundibulums in 3.5%. Both of these findings were more common with deficient ventricular septation. Fibrous continuity between the tricuspid and pulmonary valves was not always seen with perimembranous defects, particularly when there were bilateral infundibulums. Fibrous continuity between the leaflets of the tricuspid and mitral valves, in contrast, proved a unifying characteristic of the perimembranous defect. The combination of concordant atrioventricular and discordant ventriculoarterial connections is best described simply as "transposition," with precision provided when details are given of atrial arrangement and the relationships of the arterial trunks. [ABSTRACT FROM AUTHOR]

The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past 9 years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases. The scheme to categorize a ventricular septal defect uses both its location and the structures along its borders, thereby bridging the two most popular and disparate classification approaches and providing a common language for describing each phenotype. Although the first-order terms are based on the geographic categories of central perimembranous, inlet, trabecular muscular, and outlet defects, inlet and outlet defects are further characterized by descriptors that incorporate the borders of the defect, namely the perimembranous, muscular, and juxta-arterial types. The Society recognizes that it is equally valid to classify these defects by geography or borders, so the emphasis in this system is on the second-order terms that incorporate both geography and borders to describe each phenotype. The unified terminology should help the medical community describe with better precision all types of ventricular septal defects. [ABSTRACT FROM AUTHOR]

Objective The study objective was to evaluate the outcomes of the cavopulmonary shunt after the Norwood procedure with a particular focus on age, weight, and whether surgery was planned or expedited by clinical findings. Methods We studied 297 consecutive patients with hypoplastic left heart syndrome undergoing the cavopulmonary shunt operation between 2002 and 2014. All patients underwent the Norwood procedure with a right ventricle to pulmonary artery conduit. Unplanned surgery was any situation in which surgery was expedited because of refractory desaturation, angiographic findings, or failure to discharge. Results There were 222 planned and 75 unplanned procedures. Planned surgery was performed at median of 5.4 (4.6-6.3) months compared with 3.9 (3.3-5.0) months for the unplanned group ( P < .001). Early mortality was 1.3% in the planned group compared with 5.3% in the unplanned group ( P = .07). At 5 and 10 years, survival was significantly worse in the unplanned group (73% vs 85%, P = .03). On multivariable analysis, atrioventricular valve regurgitation, the need for atrioventricular valve repair, and impaired ventricular function (graded composite variable) were the predominant risk factors (hazard ratio [HR], 7.49), with an incremental risk within these 3 variables. The planned versus unplanned nature of the surgery was less significant (HR, 1.75) because it was eclipsed by ventricular function and tricuspid intervention. Additional factors were aortic atresia/mitral atresia subgroup (HR, 2.07) and somatic growth (z-score weight) at the time of surgery (HR, 0.60 for z-score change from −2.54 to −1.16). Age and weight at surgery were not significant risk factors for survival. Interstage interventions on the aortic arch or right ventricle to pulmonary artery conduit did not adversely influence outcomes. Conclusions The ventricular function, atrioventricular valve regurgitation, and need for tricuspid intervention were the primary risk factors for survival after the cavopulmonary shunt operation. Although unplanned surgery is associated with poor outcomes, this is related to the primary risk factors and not the timing or age of the patient. Somatic growth also has a significant influence on survival. Thus, an earlier cavopulmonary shunt operation is safe in infants who are thriving, but those with poor weight gain are at higher risk. [ABSTRACT FROM AUTHOR]

Objectives: Our purpose was to investigate a new approach to bicommissural repair of regurgitant aortic valves. Background: Repair of regurgitant aortic valves is not widely accepted, but interest is increasing, particularly for bicuspid valves. We hypothesize that a systematic, segmental approach to morphology and corresponding morphology-directed repair will improve decision making and success. Methods: From December 2001 to July 2007, a systematic surgical approach to valve analysis and bicommissural repair was applied prospectively to 63 consecutive patients with pure aortic valve regurgitation, mean age 40 ± 12 years. Cusp, commissure, and root morphologies were analyzed sequentially by direct inspection. Each abnormality was corrected by corresponding morphology-directed repair procedures. Retrospectively, 2 echocardiographic indexes—of tissue pliability (change in systolic to diastolic area) and coaptation deficiency (conjoint and reference cusp heights vs. “annulus” diameter)—were developed to evaluate repairability. Results: Forty-two (67%) valves were repaired and 21 (33%) replaced. Regurgitation was related primarily to cusp (prolapse, restriction) and commissure (splaying) morphology; root pathology was less important. Morphology-directed repair included cusp maneuvers in all, commissural maneuvers in 71%, and root procedures in 33%. Restriction and cusp tissue deficiency limited repairability. Echocardiography reflected this in greater tissue pliability of successfully repaired valves compared with replaced ones (conjoint cusp 61 ± 16% vs. 34 ± 17%; reference cusp 65 ± 16% vs. 42 ± 16%; p = 0.0001) and less coaptation deficiency (1.06 ± 0.24 for repaired and 1.27 ± 0.19 for replaced valves; p = 0.002). Conclusions: Systematic segmental analysis of morphology and a logical morphology-directed surgical approach facilitate aortic valve repair. Initial application of this paradigm suggests sufficient mobile cusp tissue is a key determinant of repairability. [Copyright &y& Elsevier]

: BackgroundThe aim of this study was to evaluate the impact of weight less than 5 kg at operation on mortality and morbidity in patients with atrioventricular septal defect (AVSDc) undergoing total correction.: MethodsBetween January 1990 and December 2002, 190 consecutive patients with AVSDc underwent total biventricular correction. They were divided into two groups: group I (n = 64 patients weighing < 5 kg) and group II (n = 126 patients weighing > 5 kg). Associated major cardiac malformations were found in 49 (25.8%) patients. Associated left atrioventricular valve (LAVV) malformations were found in 35 (18.4%) patients. The mean follow-up time was 4.1 ± 2.9 years (range 2 months–10.7 years).: ResultsThe in-hospital mortality in group I was 7.8% (5 patients) versus 8.7% (11 patients) in group II (p = 0.95). Major associated cardiac malformations (p < 0.001) and pulmonary hypertension (p = 0.006) were found to be strong predictors for poor postoperative survival. At discharge the mean LAVVR grade in group I was 1.45 ± 1.2 versus 1.2 ± 1 in group II (p = 0.13). The actuarial overall survival rates at 1, 3, 5, and 7 years were 96.5%, 92.5%, 91.5%, and 89% respectively and the actuarial overall reoperation free survival rates at 1, 3, 5, and 7 years were 95%, 87%, 84%, and 73%. Twenty-three patients underwent reoperation due to severe left atrioventricular valve regurgitation (LAVVR). Strong predictors for overall reoperation free survival were the operation year before 1995 (p < 0.001), postoperative LAVVR greater than or equal to 2 (p = 0.006), major associated cardiac malformations (p = 0.00034), associated LAVV malformations (p = 0.0044), and non or partial LAVV cleft closure (p = 0.012). The actuarial survival rates between patients weighing less than 5 kg versus patients weighing more than 5 kg were similar (p = 0.51); instead the overall reoperation free survival was significantly lower in patients weighing less than 5 kg (p = 0.022) according to the log-rank test. Weight less than 5 kg (p = 0.023, β = −0.6) was one of the predictors for reoperation due to severe LAVVR in this series.: ConclusionsWe may conclude that in the current era repair of AVSDc can be carried out successfully in patients less than 5 kg, however, weight less than 5 kg at initial complete repair seems to be a predictor for late reoperation due to LAVVR. Suture separation at the cleft site or between the leaflets of the newly created mitral valve and the patch remain the main causes of postoperative LAVVR in patients weighing less than 5 kg. [Copyright &y& Elsevier]

Background. The aims of this study were to determine early and midterm survival and freedom from reoperation, and to identify the predictors for poor postoperative outcome in children undergoing mitral valve (MV) repair owing to congenital malformations of the mitral valve.Methods. Between January 1990 and February 2001, 94 consecutive children with congenital MV disease underwent valve repair. The mean age was 5.2 ± 3.3 years (range 20 days to 15 years). Twenty-five (26.6%) children were less than 1 year old. Isolated MV disease was found in 21 (22.4%) patients. MV stenosis was the predominant lesion in 21 (22.4%) patients with a mean left atrial to left ventricle diastolic peak gradient of 24.5 ± 9.2 mm Hg. MV regurgitation was the predominant pathophysiology in 73 (77.6%) patients with a mean regurgitation grade of 3.3 ± 0.7.Results. The hospital mortality was 8.5% (8 of 94). Three patients required permanent pacemaker implantation owing to complete atrioventricular block. Two patients underwent mediastinal exploration for significant bleeding. Postoperatively the echocardiography color Doppler study demonstrated a significantly lower mean end diastolic left atrium to left ventricle gradient 8.7 ± 2.2 mm Hg (p < 0.001) in patients with MV stenosis and a mean regurgitation grade of 0.9 ± 0.6 (p < 0.001) in patients with MV regurgitation. Actuarial survival and actuarial reoperation-free survival were 89.2% and 76.3%, respectively. Multivariate analysis demonstrated that age less than 1 year (p = 0.035), hammock MV (p = 0.0093), cardiothoracic ratio greater than 0.6 (p < 0.0001), and associated cardiac anomalies (p = 0.003) were strong predictors for poor overall freedom from reoperation and midterm survival.Conclusions. Mitral valve repair for congenital mitral valve disease yields acceptable early and midterm mortality and reoperation rates. Strong predictors for poor overall freedom from reoperation and midterm survival were age less than 1 year, hammock MV, cardiothoracic ratio greater than 0.6, and associated cardiac anomalies. [Copyright &y& Elsevier]