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Abstract: Objective: To analyse the long-term patency of coronary arteries after neonatal arterial switch operation (ASO). Methods: A retrospective study of the operative reports, follow-up and postoperative catheterisation data of 119 patients, who underwent the great arteries (TGA) repair since 1991, has been carried out. Patient population: Among the 133 survivors of the 137 ASOs performed between 1991 and 2007, 119 patients have been studied by routine control cardiac catheterisation and form the study population. Median time between repair and the coronary angiography was 2.9±1.9 years. A comparison between the eight patients (6.7% out of the entire study population), known to have postoperative coronary obstructions (group I) and the rest of the cohort with angiographic normal coronary vessels (group II) was performed by univariate analysis of variance and logistic regression models. One patient had surgical plasty of the left coronary main stem with subsequent percutaneous angioplasty, three patients had primary coronary stent implantation and four patients had no further intervention at all. In group I, all but one patient denied symptoms of chest pain and echocardiography failed to show any difference between the two groups in terms of left ventricular systolic function (ejection fraction group I 61±2% vs 62±6% of group II, p =1.0). Results: The association of coronary obstruction with complex native coronary anatomy (Yacoub type B to E) was evident at both univariate (62% of group I vs 22% of group II, p =0.04) and logistic regression (p =0.007, odds ratio (OR) 8.1) models. The type of coronary reimplantation (i.e., coronary buttons on punch vs trap-door techniques) was similar between the two groups (punch reimplantation in 25% of patients of group I vs 31% of group II, p =0.1) as was the relative position of the great vessels (aorta anterior in 100% of patients of group I vs 96% of group II; univariate, p =0.1). Conclusions: The late outcome in terms of survival and functional status after ASO is excellent. Nevertheless, the risk of a clinically silent late coronary artery obstruction of the reimplanted coronary arteries warrants a prolonged follow-up protocol involving invasive angiographic assessment. [Copyright &y& Elsevier]

Summary: The population of neonates and children with congenital heart defects presents about a 30% prevalence of associated genetic syndrome or additional extracardiac anomalies and may show an increased risk of death or major complication at cardiac surgery. Since a well-defined pattern of combined cardiac and extracardiac anomalies may be found in relation to specific genetic defects, correct understanding of the genetic issues may help improving diagnosis, surgical approach and final outcome of these patients. Hereby we review the medical and surgical issues correlated to the genetic asset in patients with congenital heart defects and genetic syndromes, including trisomy 21, deletion 22q11, Noonan/LEOPARD, Turner, Marfan and Williams syndromes. Recognition of specific surgical risk factors can lead to the preparation of specific diagnostic and perioperative protocols in order to reduce operative mortality and morbidity. [Copyright &y& Elsevier]

Three different devices were used to close secundum-type atrial septal defects in 28 patients. The "Amplatzer" is associated with an easier and shorter procedure than are the "Sideris Buttoned Device" and the Microvena "Angel Wings" devices. [ABSTRACT FROM AUTHOR]

During the early phase of the Covid-19 outbreak, the focus was to prevent infection and to care for the most fragile patients at risk for death, the elderly and the ones with severe comorbidities. Now, a major warning is spreading all over the world about the increasing occurrence of Kawasaki Disease (KD) among children living in high-risk areas, potentially linked with the coronavirus infection [[1]]. Further studies failed to prove the presence of the Coronavirus in acutely ill KD patients but, since the infection may act just as the initial trigger for an immune-mediated self-sustained disease, the virus may have disappeared long before the onset of the KD symptoms. [Extracted from the article]

Presents a late follow-up study of occlusion of the patent ductus arteriosus with the rashkind device with emphasis on long-term efficacy and risk for infections. Kaplan-Meier estimate for residual shunt; Residual leak through the prosthesis; Reappearance of the shunt after a successful occlusion.

Vascular events in patients with coarctation of the aorta have been extensively reported and account for the majority of morbidity and mortality in untreated patients. The exact mechanism for this association is not completely understood and may include acquired anomalies or congenital abnormalities of intracranial vessel. Here we report a case of intracranial internal carotid artery dissection with subsequent formation of acquired large carotid aneurysm in a child with severe systemic hypertension and coarctation of the aorta. Endovascular aneurysm exclusion was pursued and it was able to control this potentially lethal complication. This case supports the notion of acquired nature of intracranial vessel abnormalities and underscores the clinical role of interventional neuroradiology in a subset of patients with congenital heart disease. [ABSTRACT FROM AUTHOR]

Abstract: Background: We sought to determine whether migraine headache with aura (MHA) and spontaneous large right-to-left (R–L) shunt are risk factors for recurrent cerebral ischemic events in cryptogenic stroke patients with a patent foramen ovale (PFO). Methods: 140 patients with a PFO and cryptogenic stroke underwent transcatheter defect closure at our institution at a mean age of 45±13 years. We retrospectively analyzed follow-up data from the first cerebral ischemic event to the time of PFO closure. Before the procedure, all patients underwent transesophageal echocardiography (TEE); Transcranial Doppler scanning (TCD) was additionally performed on the last 59 patients. We analyzed the impact of MHA, thrombophilia, spontaneous large R–L shunt, and atrial septal aneurysm (ASA) on the risk of recurrent cerebral ischemic events. Results: 44 patients (31%) had had at least 1 recurrent event during a follow-up of 2.2±2.6 years. Patients with recurrent events were more commonly females (p =0.0001), had more often an associated thrombophilia (p =0.0077), and had a higher prevalence of spontaneously large R–L shunt both at TEE and at TCD (p <0.05). They also had more commonly a history of MHA (p =0.0009) and more frequent episodes of MHA (p =0.0048). Patients with MHA had a higher risk of recurrent events when compared to patients without (odds ratio 3.87, 95% CI 1.75 to 8.50). Thrombophilia (p =0.001) and spontaneous large R–L shunt (p =0.02) were independent predictors of recurrent stroke. Conclusions: In cryptogenic stroke patients with a PFO, a history of MHA, large spontaneous large R–L shunt, and thrombophilia are all associated with a higher risk of recurrent events. [Copyright &y& Elsevier]

We investigated the efficacy of transcatheter patent foramen ovale (PFO) closure to prevent recurrent cerebral ischemic events in patients who did and those who did not have thrombophilia. Patients who have a PFO and an associated thrombophilia may have an increased risk of cerebral ischemic events. Seventy-two consecutive patients (mean age 42 ± 13 years) underwent percutaneous PFO closure because of a cerebral stroke (51%) or a transient ischemic attack (49%). A thrombophilia was found in 20 patients (28%). A large right-to-left shunt through the PFO was found in 44 patients (61%) and an atrial septal aneurysm was found in 39 (54%). After the first event, 18 of 20 patients (90%) with a thrombophilia received oral anticoagulation. Before closure, patients who had a thrombophilia also had a higher rate of recurrences than patients who did not (p <0.0001), despite a similar follow-up (p = 0.14) and regardless of shunt entity (p = 0.59), presence of an atrial septal aneurysm (p = 0.98), and a prevalence of cardiovascular risk factors (p = 0.44). PFO closure was successful in all patients. Five patients (8%) had a residual shunt at 6 months. At 20 ± 11 months after PFO closure, 3 recurrent events occurred (4%), and the recurrence rate was similar in patients who did and those who did not have a thrombophilia (p = 0.25). Thus, in patients who have a cryptogenic stroke, the association of a PFO with a thrombophilia significantly increases the risk of recurrences. Thus, transcatheter PFO closure is effective for preventing recurrences in patients who have a thrombophilia. [Copyright &y& Elsevier]

Objective: Low birth weight or premature infants may require early surgical treatment of congenital cardiac lesions because of their poor clinical status. Even thought early repair or palliation is carried out with incremental risk factor for morbidity and mortality, it has been demonstrated to be preferable to medical management and delayed surgery. This retrospective study was undertaken to evaluate early and mid-term results in infants, weighing less than 2500 g, who underwent surgery other than patent ductus arteriosus closure. Methods: Since January 1993 to August 2002, 60 consecutive patients underwent early surgical treatment of congenital heart malformations at our institution. 27 patients were premature (born before 37 weeks of gestation). Ninety percent were severely symptomatic. Mean age at operation was 15.5 days (range 4–68 days). Mean weight was 2120 g (range 900–2500 g). Indications for surgery were: coarctation complex 11, transposition of great arteries 9, interrupted or severely hypoplastic aortic arch 9, hypoplastic left heart syndrome 7, truncus arteriosus 5, other 19. Thirty-five patients were operated on CPB, Deep Hypothermia with Circulatory Arrest was used in 9. Complete repair was achieved in 32 patients. Aortic arch reconstruction was required in 32 cases. Results: There were nine early deaths (15%): heart failure (5), multiorgan failure (3), sepsis (1). Age, weight, prematurity, type of surgery and use of cardio pulmonary by-pass did not influence early mortality. Mean intensive care unit stay and duration of mechanical ventilation were 5.8 days and 75.5 h, respectively. Postoperative neurological complications did not occur in any patient. At follow-up (mean 48 months) there were nine late deaths. Kaplan–Meier survival at 60 months was 70%. Conclusions: Surgery for congenital heart disease can be performed in low weight critically ill infants with reduced, but still acceptable early and mid-term survival. [Copyright &y& Elsevier]

Objectives: We sought to evaluate the course of cardiopulmonary function after transcatheter atrial septal defect (ASD) closure and to identify the physiopathologic mechanisms leading to this change.Background: Conflicting reports exist on cardiopulmonary functional improvement in asymptomatic adults after transcatheter closure of a secundum ASD.Methods: Thirty-two consecutive adults (13 males; age 42.6 ± 16.7 years) underwent maximal cardiopulmonary exercise testing and transthoracic echocardiography both on the day before and six months after transcatheter ASD closure. Mean pulmonary artery pressure, pulmonary to systemic flow ratio (Qp/Qs), and ASD diameter were measured before closure.Results: Peak oxygen uptake (Vo2) (p < 0.001), peak oxygen pulse (p = 0.0027), and vital capacity (p = 0.0086) improved after ASD closure, although peak heart rate did not. A significant correlation was found between peak Vo2 improvements and Qp/Qs (p = 0.0013). Left ventricular ejection fraction (LVEF) (p < 0.0001) and left ventricular end-diastolic diameter (LVEDD) (p < 0.0001) significantly increased after six months, although left ventricular end-systolic diameter did not. Right ventricular long- and short-axis dimensions decreased (both p < 0.0001). Peak Vo2 and of peak oxygen pulse improvements correlated to both LVEF (p = 0.0009 and 0.0019, respectively) and LVEDD (p < 0.0001 and 0.032, respectively) increments. The decrease of both long- and short-axis right ventricular dimensions positively correlated to both LVEF and LVEDD improvements. The improvement in LVEF correlated to Qp/Qs (p = 0.0026).Conclusions: Transcatheter ASD closure leads to a significant improvement in cardiopulmonary function within six months, via an increase in peak oxygen pulse. An increase in both left ventricular stroke volume and cardiac output due to a positive ventricular interaction is the mechanism leading to improved peak Vo2. [Copyright &y& Elsevier]

Background: The coronavirus disease 2019 (COVID-19) is currently rare in children and they seem to have a milder disease course and better prognosis than adults. However, SARS-Cov-2 pandemic has indirectly caused problems in pediatric medical assistance. In view of this we wanted to draw a picture of what happened during health emergency and analyze future prospects for restarting. Methods: We involved the Italian pediatric scientific societies institutionally collected in the Italian Federation of Associations and Scientific Societies of the Pediatric Area (FIARPED); We sent a questionnaire to all scientific societies about the pediatric care activity during the COVID-19 emergency and future perspectives for the phase of post-containment. Results: The analysis of the questionnaires showed significant decrease of:admission, outpatient visits and specialist consultancy activities during the COVID-19 emergency, primarily linked to the fear of infection. Instead it was increased the serious degree of diseases admitted. Most of scientific societies maintained the relationship with chronic patients through some form of telemedicine, reporting a strong positive opinion about this modality. Finally showed the need to give life a new approach for hospitalizations and outpatient visits through a greater use of telemedicine, educational programs on families and a more decisive role of family pediatricians. Conclusions: Our study highlighted many aspects that can be improved in pediatric care. We think that It will be necessary a new shared strategy to improve the management and continuity of care for pediatric patients, primarily developing a network of collaboration between families, family pediatrician and hospitals and by enhancing the use of new methods of telecommunications. [ABSTRACT FROM AUTHOR]

Despite recent technical advances, interventional cardiac catheterization is still challenging in neonatal age and no specific data concerning early outcome are so far published in literature. Neonatal trans-catheter cardiac interventions performed in high-volume Italian referral centers were retrospectively analyzed. Primary outcomes were procedural major adverse events, in-hospital mortality and procedural failure. Secondary outcomes were minor adverse events and need for blood transfusion. From January 2000 to December 2017, 1423 newborns (mean weight 3.0 ± 0.6 kg, range 1.0–5.8; median age 2.0 days) underwent interventional cardiac catheterization. Overall, global procedure adverse event rate and in-hospital mortality were 10.2% and 5.2%, respectively. At multi-variable analysis, primary composite outcome was significantly related to low-weight (<2.5 kg) (p < 0.01) and younger age (≤7 days) (p < 0.01) at the procedure, prematurity (p < 0.01), uni-ventricular physiology (p < 0.01), associated genetic syndromes (p < 0.01) and procedure risk category (p < 0.01). No relationship between volume of activity of any single center and procedure outcome was found. Over time, a trend toward an increased number of procedures and their complexity was recorded. Trans-catheter management of cardiac malformations with critical, duct-dependent pulmonary blood flow by arterial duct stenting or right ventricular outflow tract stenting showed the highest increase. Interventional cardiac catheterization is relatively safe and feasible in neonatal age. Peri-natal age, low weight, uni-ventricular physiology and genetic syndromes still significantly contribute to procedural morbidity and in-hospital mortality of this approach. • Interventional catheterization in newborns is an effective and safe approach. • Major adverse events rate is influenced by the complexity of the procedure. • Mortality depends on the clinical and hemodynamic characteristics of newborn. • Over time, an increase in number and complexity of procedures was recorded. [ABSTRACT FROM AUTHOR]

Background. There are few data on the mechanism of recurrent neurological events after transcatheter closure of patent foramen ovale (PFO) in cryptogenic stroke or TIA. Methods. We retrospectively reviewed PFO closure procedures for the secondary prevention of cryptogenic stroke/TIA performed between 1999 and 2014 in Bologna, Italy. Results. Written questionnaires were completed by 402 patients. Mean follow-up was 7 ± 3 years. Stroke recurred in 3.2% (0.5/100 patients-year) and TIA in 2.7% (0.4/100 patients-year). Ninety-two percent of recurrent strokes were not cryptogenic. Recurrent stroke was noncardioembolic in 69% of patients, AF related in 15% of patients, device related in 1 patient, and cryptogenic in 1 patient. AF was diagnosed after the procedure in 21 patients (5.2%). Multivariate Cox’s proportion hazard model identified age ≥ 55 years at the time of closure (OR 3.16, p=0.007) and RoPE score < 7 (OR 3.21, p=0.03) as predictors of recurrent neurological events. Conclusion. Recurrent neurological events after PFO closure are rare, usually noncryptogenic and associated with conventional vascular risk factors or AF related. Patients older than 55 years of age and those with a RoPE score < 7 are likely to get less benefit from PFO closure. After transcatheter PFO closure, lifelong strict vascular risk factor control is warranted. [ABSTRACT FROM AUTHOR]

OBJECTIVE To define the evolution of the single-ventricle (SV) heart in adult patients in terms of morbidity, mortality and quality of life. METHODS Sixty-two patients with SV physiology and aged older than 16 years were retrospectively reviewed. Three patients (5%) were in natural history, one had received a Blalock-Taussig shunt, one a Waterstone anastomosis, one a pulmonary artery banding, three a bidirectional cavopulmonary anastomosis, eight a classic Fontan procedure and 46 a total cavopulmonary connection (TCPC). The morphology of the SV was left in 48 patients (77%), right in nine (14%) and indeterminable in five (8%). Thirty-three patients underwent magnetic resonance imaging (MRI) to assess ventricular mass (VM), ventricular systolic function, pulmonary artery branch diameter and potential thrombosis of the conduit. Cardiopulmonary exercise testing (CPTE) was carried out to evaluate exercise tolerance. The quality of life was monitored with two different specific tests, the Short Form-36 (SF-36) and the congenital heart disease-TNO/AZL adult quality of life (CHD-TAAQOL). The mean follow-up time was 8.0 ± 9.1 years. RESULTS Two of the three patients in natural history underwent primary TCPC. Re-interventions were necessary in seven patients (11%). Three patients (5%) died during follow-up. Five patients (8%) underwent cardiac transplantation. Protein losing enteropathy appeared in six (10%), while the arrhythmic disorder was detected in 13 patients. On the MRI, the mean end-diastolic ventricular volume was 106 ± 448 ml/m2, the mean ejection fraction (EF) was 52.3 ± 10% and VM was 56 ± 22.1 g/m2. On CPTE, the peak of oxygen uptake (peak VO2) was moderately impaired in 92% of patients, while 4% presented a severely impaired and 4% a normal peak of VO2. No correlations were found among the peak of VO2 and the quality-of-life evaluation. CONCLUSIONS Adult patients with SV are at high risk of reoperations and need of transplant and complications. Nevertheless, in the presence of a moderately reduced peak of VO2 and a moderate reduction in the EF detected at the MRI, the results of the evaluation of daily quality of life are incredibly high. [ABSTRACT FROM AUTHOR]

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]

Adults with tetralogy of Fallot (TOF) have increased long-term mortality. The identification of patients at greater risk for death or cardiac-related morbidity is challenging. This study was conducted to assess the prognostic value of cardiopulmonary exercise testing in adults with repaired TOF. One hundred eighteen consecutive adults with repaired TOF (mean age at repair 4.8 ± 4.2 years) underwent cardiopulmonary exercise testing at a mean age of 24 ± 8 years (range 16 to 59). The degree of pulmonary regurgitation, right ventricular function, and right ventricular systolic pressure were determined by transthoracic echocardiography. After the exercise tests, patients were regularly followed up for cardiac-related events. During a mean follow-up of 5.8 ± 2.3 years (range 0.6 to 9.7), 9 patients died and 18 underwent hospitalization. Peak oxygen uptake (hazard ratio 0.974, 95% confidence interval 0.950 to 0.994), the slope of ventilation (VE) per unit of carbon dioxide production (VCO2) (hazard ratio 1.076, 95% confidence interval 1.038 to 1.115), and New York Heart Association functional class (hazard ratio 2.118, 95% confidence interval 1.344 to 3.542) were independent predictors of death or hospitalization. Patients with peak oxygen uptake ≤36% of predicted value and those with VE/VCO2 slopes >39 were at greater risk for cardiac-related death (5-year mortality 48% vs 0%, p <0.0001, and 31% vs 0%, p <0.0001, respectively). In conclusion, the measurement of peak oxygen uptake and VE/VCO2 slope in adults with repaired TOF can be prognostically important and could become a powerful tool to rationalize decisions regarding the prevention of premature sudden death and the need for reintervention. [Copyright &y& Elsevier]

Abstract: Background: Patients with atrial repair for transposition of the great arteries and patients with congenitally corrected transposition have a right ventricle (RV) in the systemic position and they may develop RV dysfunction and exercise intolerance with advancing age. No data is available on the effect of carvedilol in patients with dysfunctional systemic RV. Methods: We studied with cardiovascular magnetic resonance (CMR), cardiopulmonary exercise testing, and standard 12-leads electrocardiogram, 8 adults (median age 26 years, range 18–31) with chronic stable heart failure and systemic RV dysfunction (6 patients with atrial repair and 2 patients with congenitally corrected transposition). Assessment was done before and after 12 months of carvedilol administration. The initial dose was 3.125 mg twice daily, and the target dose was 25 mg twice a day. Results: Carvedilol administration was safe and the target dose was achieved in 5/8 (62%) patients. Right ventricular end-diastolic (119±31 vs. 112±28 ml/m2, p =0.01) and end-systolic volumes decreased (79±17 vs. 65±14 ml/m2, p =0.006), and RV ejection fraction improved (34±6 vs. 42±7%, p =0.004). Left ventricular ejection fraction increased (44±8 vs. 49±9%, p =0.01), suggesting a positive biventricular remodelling. Peak oxygen uptake did not change with carvedilol (26.8±5.3 vs. 27.3±5.7 ml O2/Kg/min, p =0.58), whereas exercise duration increased (13.4±2.6 vs. 17.3±3.1 min, p =0.008). Conclusions: In this small cohort, carvedilol administration was safe and it was associated with positive RV remodelling as well as improved exercise duration. [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]

Abstract: Background: Patients with repaired tetralogy of Fallot (ToF) featuring severe pulmonary regurgitation (PR) and/or right ventricular (RV) dysfunction have reduced exercise tolerance. Aims: To assess the impact of PR and of RV function on the ability to recover from exercise in ToF patients. Methods: 61 consecutive patients aged 23.1±12.1 years underwent maximal cardiopulmonary exercise test (CPX), transthoracic echocardiography and magnetic resonance imaging. This data was compared to those of 153 matched healthy subjects. Results: 19 patients (31%) had severe PR. RV dysfunction was noted in 19 patients (31%). Nine patients (15%) had both severe PR and RV dysfunction. Patients had lower peak oxygen uptake (VO2), VO2 slope, carbon dioxide production (VCO2) slope and O2 pulse slope (p <0.0001), especially those with severe PR and RV dysfunction (p <0.0001). Heart rate slope was similar between groups. No patient with severe PR and RV dysfunction had a predicted peak VO2 >40%. CPX had a high sensitivity and specificity to identify patients with severe PR and RV dysfunction. Conclusions: In ToF patients, severe PR and RV dysfunction lead to delayed recovery from exercise. CPX can identify patients with severe PR and RV dysfunction and may be useful to guide the pulmonary valve replacement decision-making process. [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]

ABSTRACT. Posterior fossa malformations, hemangio-mas, arterial anomalies, coarctation of the aorta, and car-diac defects and eye abnormalities (PHACE) is a rare congenital anomaly with a broad spectrum of clinical manifestations with a striking female predominance. We describe an infant with PHACE anomaly and aortic co-arctation who underwent cardiac catheterization to clearly define the complex anatomy of the aortic lesion before surgical repair. Review of the literature docu-ments a highly significant association between ipsilat-eral hemangiomas and cerebrovascular and aortic arch anomalies. We conclude that cardiac catheterization is required to define the complex anatomy of aortic lesions and should be performed in all patients with PHACE and evident aortic involvement to plan appropriate and safe surgical repair. Pediatrics 2004;113:412--415; PHACE, aor-tic arch, hemangioma, aortic coarctation, cardiac cathe-terization. [ABSTRACT FROM AUTHOR]