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Type A aortic dissection is a very uncommon finding in children, especially among infants, where no cases seem to have been described. We report the case of an 11-month-old boy with Loeys-Dietz syndrome and asymptomatic type A aortic dissection involving the aortic root that was successfully repaired. [ABSTRACT FROM AUTHOR]

Objectives To identify determinants of adverse outcomes in patients with atrial isomerism. Methods Determinants of survival were analyzed for the group as a whole as well as separately for left and right atrial isomerism. Results There were 78 cases with right and 104 with left atrial isomerism. Nineteen patients were not offered surgery; 49 (47%) of those with left atrial isomerism and 60 (77%) with right atrial isomerism were directed to single ventricle palliation. A total of 67 patients died. Survival to 25 years was 62% (95% confidence interval [CI], 53%-69%). Independent predictors of mortality were obstructed total anomalous pulmonary venous drainage ( P < .001; hazard ratio [HR], 5.2; 95% CI, 2.7-9.7) and asplenia ( P = .008; HR, 2; 95% CI, 1.2-3.3). There was no evidence that patients born in the recent era had improved survival ( P = .47; HR, 1.1; 95% CI, 0.8-1.5). Survival was better for patients with left than right atrial isomerism: 18 years survival 74% (95% CI, 63%-82%) versus 50% (95% CI, 38%-60%). Independent predictors of mortality for patients with left atrial isomerism were dextrocardia ( P = .009; HR, 3.0; 95% CI, 1.3-6.7) and pulmonary stenosis ( P = .042; HR, 0.3; 95% CI, 0.1-0.9) and patients with right atrial isomerism, biventricular repair ( P < .001; HR, 6.0; 95% CI, 2.8-13), and obstructed total anomalous pulmonary venous drainage ( P < .001; HR, 4.2; 95% CI, 2.0-8.6). Conclusions A significant proportion of patients with isomerism still die before reaching adulthood. Only a fraction of those with obstructed pulmonary veins survive. Having biventricular repair does not confer a survival advantage to those born with right atrial isomerism. [ABSTRACT FROM AUTHOR]

Objective: Repair of partial anomalous pulmonary venous connection to superior vena cava using an internal patch has been described as a potential cause of obstruction at the systemic or pulmonary vein level and of sinus node dysfunction. Our experience with this operation was reviewed. Methods: From 1991 to 2011, 59 patients with a diagnosis of partial anomalous pulmonary venous connection to superior vena cava underwent surgical repair with intracardiac patch rerouting alone (45 patients) or with associated superior vena cava patch enlargement (14 patients). Follow-up evaluation was performed, including electrocardiogram, echocardiogram, electrocardiogram Holter monitor recording, and exercise stress test. Results: There were no early or late deaths and no reoperations at a mean follow-up of 46 ± 45 months. All patients were asymptomatic in New York Heart Association class I. Echocardiographic evaluation excluded any obstruction at the pulmonary or systemic vein level. At follow-up, 55 patients (93%) presented sinus rhythm and were free from antiarrhythmic medications, 2 patients (3%) presented atrial fibrillation, 1 patient (2%) presented atrial fibrillation and asymptomatic sinus node dysfunction, and 1 patient (2%) presented ectopic atrial rhythm. Electrocardiogram Holter recording demonstrated sinus node dysfunction in 6 of 34 patients (18%). Exercise stress test showed chronotropic incompetence in 8 of 27 patients (30%): All except 1 patient presented sinus rhythm at basal electrocardiogram, and only 4 patients had some evidence of sinus node dysfunction on electrocardiogram Holter recording. Conclusions: Intracardiac repair of partial anomalous pulmonary venous connection can be performed with good results at medium-term follow-up. The rate of sinus node dysfunction or other arrhythmias and obstruction at pulmonary or systemic vein level is comparable to other techniques. Exercise stress test evaluation is the best way to detect asymptomatic sinus node dysfunction. [ABSTRACT FROM AUTHOR]

Objective: Management of congenital mitral valve disease is challenging because of a wide morphologic spectrum, frequent associated lesions, and small patient size. We evaluated the results of a repair-oriented policy. Methods: All consecutive patients with congenital mitral valve disease who underwent surgery between 1996 and 2006 were studied retrospectively. Patients with atrioventricular canal, atrioventricular discordance, or ischemic regurgitation were excluded. Results: During this period, 71 children (median age 2.9 years, range 3 days–20.8 years) underwent surgery. All but 1 underwent primary mitral valve repair. Twenty-two (30%) were younger than 12 months. Associated cardiac lesions were present in 45 children (63%) and were addressed concurrently in 35; previous cardiac procedures had been performed in 17 patients (24%). Mitral incompetence was predominant in 60 (85%) and stenosis in 11 (15%). During a median follow-up of 47.8 months (range 2–120 months), 14 patients underwent 17 mitral reinterventions: 14 repairs and 3 replacements. After 60 months, overall survival was 94% ± 2.8%; freedoms from reoperation and prosthesis implantation were 76% ± 5.6% and 94% ± 3.6%, respectively. There were 4 deaths, and all survivors remain in New York Heart Association class I or II with moderate (6 patients) or less mitral dysfunction. Conclusion: Surgical repair of the congenital mitral valve can be successfully performed with low mortality, satisfactory valvular function at midterm follow-up, and acceptable reoperation rate while obviating risks associated with valvular prostheses. Suboptimal primary repair was significant predictor for reoperation but re-repair was often successful. [Copyright &y& Elsevier]

Background: The identification of patients with adult congenital heart disease (ACHD) who are at higher risk of death is challenging. Peak circulatory power (CircP; expressed as peak exercise oxygen uptake multiplied for peak mean arterial blood pressure) is a strong predictor of death in adults with acquired heart disease. We sought to establish the distribution and the prognostic value of peak CircP across a wide spectrum of patients with ACHD. Methods: Four hundred thirty-two consecutive patients with ACHD of varying diagnosis underwent cardiopulmonary exercise testing at a single laboratory between 1996 and 2005. Patient age was 32 ± 10 years. Results: A gradual variation in peak CircP was found across the spectrum of congenital heart defects (P < .0001 at analysis of variance). Reduced peak CircP values were associated with the presence of heart failure symptoms (P < .0001), absence of sinus rhythm (P = .010), and use of antiarrhythmic medications (P = .0013). At a follow-up of 4.4 ± 2.4 years, 23 patients (5.3%) had died. Peak CircP was a strong predictor of mortality when univariate analysis was used and the strongest independent predictor of mortality among exercise parameters. A peak CircP ≤1476 mm Hg mlO2 min−1 kg−2 was associated with a 15.4-fold increase in the 4-year risk of death. Conclusions: Peak CircP is abnormal across the spectrum of ACHD. Peak CircP appears as the strongest predictor of adverse outcome in ACHD. [Copyright &y& Elsevier]

Objective: The congenitally bicuspid aortic valve is the most common etiologic factor associated with clinically significant aortic stenosis and/or regurgitation in pediatric patients. Beyond infancy, surgical intervention typically involves valve repair with cusp thinning and commissurotomy or valve replacement, primarily with pulmonary autograft in the current era. An aortic valve repair technique using tricuspidization with cusp extension was introduced in 1999. This study compares the midterm clinical outcome in patients undergoing valve repair by tricuspidization with cusp extension with those receiving a pulmonary autograft (Ross). Methods: A retrospective study was performed on all consecutive patients with symptomatic bicuspid aortic valve disease who underwent tricuspidization with cusp extension or a Ross procedure between 1999 and 2005. In both groups, all patients were at least 1 year of age at time of the operation. Results: During this period, 21 children (median age 12.6 years, range 2.6–18 years) underwent tricuspidization with cusp extension (TCE group) and 25 children (median age 10.2 years, range 11.5 months–20.1 years) underwent the Ross procedure. Prior balloon valvuloplasty was performed in 5 (24%) of the children in the TCE group and 16 (64%) of the children in the Ross group. Prior surgical commissurotomy was performed in 4 (19%) TCE patients and in 9 (36%) Ross patients. During a median follow-up period of 36.4 months (range 2.5 months–7.4 years), 2 (10%) patients in the TCE group required valve-preserving early revision of the repair, 2 (10%) TCE patients required subsequent aortic valve replacement at 16 and 33 months, 1 (4%) Ross patient required subsequent valve repair at 5 years, and 1 (4%) Ross patient underwent cardiac transplantation at 46 months. At 36 months, the actuarial freedom from reintervention on the aortic valve or autograft was 90% in the TCE group, with 11 patients at risk, and 100% in Ross patients, with 13 patients at risk (P = .39); the freedom from moderate valve dysfunction or reintervention was 66% for TCE patients and 95% for Ross patients (P = .07). There were no deaths, and all but 1 Ross patient remain in New York Heart Association class I. Conclusions: Reintervention rates in patients undergoing tricuspidization with cusp extension or a primary Ross procedure are similar. Valve performance in the TCE group is satisfactory at midterm follow-up, but the Ross repair appears to provide greater stability of valve function. These results suggest that repair with valve tricuspidization and cusp extension provides reliable palliation of the symptomatic bicuspid aortic valve. [Copyright &y& Elsevier]

Background: Although deep hypothermic circulatory arrest has been extensively used in neonates for aortic arch surgery, the brain and other organs might be adversely affected by prolonged ischemia and deep hypothermia. Methods: Between December 1997 and January 2005, 70 consecutive neonates underwent Norwood stage I procedure for hypoplastic left heart syndrome (group A, n = 30), or aortic arch repair for interruption or coarctation with arch hypoplasia (group B, n = 40), with antegrade selective cerebral perfusion (ASCP). Mean weights were 3.0 ± 0.2 kg and 2.8 ± 0.07 kg, and mean ages were 10 ± 3.5 days and 14 ± 10.6 days in groups A and B, respectively. Only 2 patients were older than 30 days. Core body temperature was lowered to 25°C, and mean pump flow during ASCP was initiated at 10 to 20 mL/(kg · min) and adjusted to guarantee a radial/temporal artery pressure of 30 to 40 mm Hg and venous oxygen saturation of more than 70%. Hematocrit was maintained at 30%. Results: Early mortality was 17% (group A, 23%; group B, 12.5%; p = 0.19). Six late deaths occurred (3 in each group), and at 36 months, Kaplan-Meier overall survival was 64% ± 9.2% in group A and 85% ± 5.7% in group B. One patient had postoperative seizures. Age, weight, sex, prematurity, group A, and ASCP duration did not influence early mortality. Conclusions: Antegrade selective cerebral perfusion is a safe and effective procedure and might improve outcome of neonatal aortic arch surgery, minimizing neurologic impact without the need for deep hypothermia. [Copyright &y& Elsevier]

Objective: Although homograft conduits are frequently used to establish right ventricle–to–pulmonary artery continuity, the limited availability of small-size homografts is a significant constraint in pediatric cardiac surgery. We compared the performance of standard homograft conduits with that of surgically reduced bicuspid homograft conduits in patients undergoing repair of truncus arteriosus. Methods: Forty infants undergoing complete repair of truncus arteriosus with either standard homografts (n = 26) or reduced-size bicuspid homografts (n = 14) were evaluated. Results: The median downsized conduit diameter (13 mm) was similar to the standard homograft diameter (12 mm, P = .52). There were 6 early deaths and 5 late deaths, representing an overall 30-day mortality of 15% and a 5-year mortality of 25%. No deaths were directly related to homograft dysfunction. Four (29%) downsized conduits and 8 (31%) standard conduits required replacement at a median interval of 18.5 months and 42.4 months, respectively. Catheter-based interventions were required in 5 (36%) patients in the downsized group and in 3 (12%) patients in the standard group. There was no difference in freedom from surgical or catheter-based reintervention between the 2 groups (P = .42). Freedom from conduit failure (severe conduit stenosis, moderate or greater regurgitation) was 55.9% and 17.2% at 3 years in the downsized and standard groups, respectively. Conclusion: The surgically downsized homograft is an excellent option when an appropriate-sized homograft is not available and might prevent morbidity associated with the use of an oversized conduit. [Copyright &y& Elsevier]

: BackgroundCleft mitral valve without an ostium primum defect, referred to as isolated cleft mitral valve, has been the subject of many reports; yet its morphology and operability remain incompletely understood.: MethodsThe anatomic findings in 36 postmortem cases, five explanted hearts, and relevant clinical data constitute the material basis of this report. Cardiac catheterization data were available in 29 cases and two-dimensional echocardiograms in 13 cases.: ResultsTwenty cases had normally related great arteries with subpulmonary conus. Of these cases 4 (20%) had tetralogy of Fallot and 1 had tricuspid atresia. Twenty-one cases had abnormal ventriculoarterial relationships with subaortic or bilateral conus resulting in transposition in 16 (76%) and double-outlet right ventricle in 5 (24%). In the cases with normally related great arteries, the morphology of the ventricular septal defect and the mitral cleft were similar to those of the more complete forms of atrioventricular canal defects. The mitral cleft usually resulted in progressive mitral regurgitation, which can be treated by surgical closure of the cleft. In the cases with abnormal conus, the morphology of the ventricular septal defect and the mitral cleft did not resemble atrioventricular canal defects. The attachment of the cleft usually produced obstruction of the left ventricular pulmonary outflow tract. Surgical repair of the cleft cannot eliminate this obstruction.: ConclusionsThere are two different anatomic types of isolated cleft mitral valve: the canal type, and abnormal conus type. Diagnosis of the associated ventriculoarterial relationships helps to guide their surgical treatment. [Copyright &y& Elsevier]

Aortopulmonary window is rarely associated with other cardiac anomalies, such as anomalous origin of one coronary artery. This exceptional association has been reported in 15 patients, with different surgical solutions. We describe 2 patients with aortopulmonary window with anomalous origin of the right coronary artery, one of which presented with tetralogy of Fallot-pulmonary atresia. A neonatal modified Blalock-Taussig shunt and ligature of the distal pulmonary artery at the level of pulmonary bifurcation was performed in the first patient, and the second patient underwent aortopulmonary window division with anomalous coronary artery reimplantation. Both patients had an uneventful postoperative course. [ABSTRACT FROM AUTHOR]

A newborn, with SDS (S = situs viscero-atrialis solitus, D = D-loop of the ventricles, S = solitus, normally related great arteries) anomalous drainage of the right superior vena cava in the left atrium, intact atrial septum, and anomalous drainage of the right superior pulmonary veins in the right superior vena cava, underwent surgical repair at our institution. This rare cyanotic, congenital, cardiac malformation is herein described with particular regard to its anatomical, embryological, and surgical implications. [Copyright &y& Elsevier]

Atrioventricular valve duplication, also known as double orifice valve, is an excitingly rare anomaly that can involve either the mitral or more uncommonly the tricuspid valve. Herein we describe a case of a double orifice tricuspid valve that prejudiced the complete repair in a symptomatic infant with tetralogy of Fallot. [Copyright &y& Elsevier]

Abstract: Anomalous origin of coronary arteries is a rare congenital defect and usually presents as an isolated defect. In only 5% of cases, it may be associated with other cardiac anomalies. In this article, we present a case of a newborn with a very complex congenital cardiac disease accompanied with an anomalous origin of an additional coronary artery. [Copyright &y& Elsevier]

Abstract: Background: The long-term impact of transcatheter atrial septal defect (ASD) closure on right ventricular (RV) remodeling and exercise capacity is unknown. Methods: We studied with cardiopulmonary exercise testing and transthoracic echocardiography 29 adults (age 42.3±16.4 years) with hemodynamically significant ASD just before transcatheter defect closure and after 6 and >36 months from closure. Results: Compared to 6 months after closure, a further improvement of peak oxygen uptake (p <0.001) and of the slope of ventilation/carbon dioxide production (p <0.001) was observed 3 years after the procedure, so that peak oxygen uptake appeared to be within the normal range in 23/29 patients (79%). Right ventricular short-axis (p <0.05) and long-axis (p <0.05) diameters further decreased beyond the 6-month period. The long-term improvement in exercise capacity correlated with pulmonary-to-systemic flow ratio (R =0.55, p =0.003) and with percentage decrease in RV short-axis diameter (R =0.59, p =0.002), but it did not correlate with age at closure (R =0.25, p =0.46). All patients who did not achieve a normal exercise capacity after 3 years from closure had a severely depressed pre-closure peak oxygen uptake (<50% of predicted). Conclusions: Adults who undergo transcatheter ASD closure may experience a further improvement in exercise capacity in the long term. The long-term improvement in exercise capacity is associated to an improvement in cardiac form and function and is not influenced by age at closure. Even if the majority of patients may reach a normal exercise capacity after ASD closure, an abnormal exercise capacity may persist in those patients that had a peak oxygen uptake below 50% of predicted value before the procedure. [Copyright &y& Elsevier]

Abstract: Background:: Experimental studies showed that extracardiac total cavopulmonary connection provides superior hemodynamics than atriopulmonary Fontan. Methods:: We prospectively assessed the impact of conversion of atriopulmonary Fontan to extracardiac total cavopulmonary connection on exercise capacity and cardiac function in 6 consecutive patients. Results:: Six months after conversion to extracardiac total cavopulmonary connection, we observed an increase in peak oxygen uptake in all patients (p =0.01;+17%). This improvement was associated to an increase of peak O2 pulse (p =0.01;+16%), but no change in peak heart rate, arterial oxygen saturation at peak exercise, and pulmonary function. Ventricular ejection fraction did not change significantly after surgery. Conversion was associated with an improvement in heart failure symptoms as assessed by the New York Heart Association classification. Patients who had undergone additional anti-arrhythmia surgery for atrial fibrillation had no recurrence of arrhythmia at follow-up. Conclusion:: Data indicate that conversion to extracardiac total cavopulmonary connection is associated with an improvement of cardiopulmonary function and heart failure symptoms. Improved exercise capacity is due to an increase in O2 pulse and may reflect an improved cardiac stroke volume after the operation. [Copyright &y& Elsevier]

This study sought to determine if areas of late gadolinium enhancement (LGE) would be present in adults with systemic right ventricles and if LGE would be associated with markers of adverse outcomes. Using gadolinium-enhanced magnetic resonance and cardiopulmonary exercise testing, 34 adults with systemic right ventricles (23 patients with atrial repair for transposition of the great arteries and 11 with congenitally corrected transposition) were studied at a mean age of 25 years. LGE was present in 14 patients (41%). The presence of LGE was associated with older age (p = 0.037), a lower right ventricular (RV) ejection fraction (34% vs 45%, p = 0.006), higher RV wall stress (p = 0.0001), reduced peak oxygen uptake (47% vs 56%, p = 0.001), and a history of arrhythmia (p = 0.005). The RV ejection fraction was correlated with RV wall stress (r = −0.81, p <0.0001) and peak oxygen uptake (r = 0.74, p <0.0001). Twelve patients experienced worsening of their clinical conditions. This was associated with decreases in biventricular function and increases in the prevalence and number of LGE areas. In conclusion, patients with systemic right ventricles have areas of abnormal myocardium, presumably due to fibrosis, that can be seen by contrast-enhanced magnetic resonance imaging. The presence of abnormal myocardial regions is associated with RV dysfunction, poor exercise tolerance, arrhythmia, and progressive clinical deterioration. [Copyright &y& Elsevier]