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Background: The aim of this study is to identify echocardiographic predictors of transient left ventricle dysfunction after pulmonary valve balloon dilatation (PVBD), in neonates with pulmonary valve stenosis (PVS) and atresia with intact septum (PAIVS) at birth. Methods: The study includes patients admitted at the Bambino Gesù Children Hospital from January 2012 to January 2017. Clinical, echocardiographic and cardiac catheterization data before and after PVBD were retrospectively analyzed. Results: Twenty-nine infants were included in the study (21 male and eight female). The median age was 5.8 ± 7.1 days. Eight patients developed transient LV dysfunction (three PAIVS and five PVS) and comparing data before and after the procedure, there was no difference in right ventricle geometrical and functional parameters except for evidence of at least moderate pulmonary valve regurgitation after PVBD. Conclusion: Moderate to severe degree pulmonary valve regurgitation was significant associated to LV dysfunction (p < 0.05) in PVS and PAIVS patients. [ABSTRACT FROM AUTHOR]

Posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta, and cardiac 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 coarctation who underwent cardiac catheterization to clearly define the complex anatomy of the aortic lesion before surgical repair. Review of the literature documents a highly significant association between ipsilateral 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, aortic arch, hemangioma, aortic coarctation, cardiac catheterization., Infantile hemangiomas are common vascular lesions, occurring in 8% of all newborns, and are usually solitary (90%). However, segmental hemangiomas, which may occur in unusual locations in deep tissue, occur [...]

Objective.--To investigate the overall occurrence of congenital heart disease in 103 pedigrees with a proband affected with atrioventricular canal. Design.--Family study of patient series. Setting.--Department of Pediatric Cardiology, Bambino Gesu Hospital, Rome, Italy. Participants.--One hundred three consecutive patients with atrioventricular canal and normal karyotype and their first- and second-degree relatives. Interventions.--None. Measurements/Main Results.--In 12 pedigrees (11.7%), one or more relatives had concordant or discordant congenital heart disease. Congenital heart disease occurred in four (1.9%) of the 206 parents of probands, in four (3.6%) of the 111 siblings, and in five (0.8%) of the 644 uncles or aunts. None of the grandparents had congenital heart disease. Atrioventricular canal affected several family members in five pedigrees. Conclusions.--Familial aggregation of atrioventricular canal in several pedigrees requires careful collection of family histories, accurate cardiologic evaluation of the first-and second-degree relatives of the probands, and fetal echocardiography in all pregnant women in at-risk families. (AJDC. 1993;147:1295-1297), Children of parents with atrioventricular canal (AVC) may have an increased risk of having AVC, especially if the affected parent is the mother. AVC is a birth defect involving a gap between the heart septa. AVC may be an inherited condition, possibly associated with a major gene. Researchers studied families of 103 patients with AVC between the ages of 3 months and 28 years to determine the occurrence of congenital heart disease. Of 317 parents and siblings, 2.5% had a congenital heart disease. Three of the four parents and two of the four siblings with congenital heart disease had AVC. Almost 12% of the 103 patients with AVC had at least one relative with congenital heart disease. In five of the families affected by congenital heart disease, most of the family members had AVC. Molecular analysis of affected family members has not revealed the genetic involvement. Close relatives of patients with AVC should be evaluated for heart defects and receive prenatal counseling on their children's risk of AVC.

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]

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]

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: 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]

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]

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]

Single chamber rate responsive pacing offers many potential advantages over the more complex dual chamber atrial tracking pacing mode in children, and the preservation of atrioventricular synchrony could be unnecessary in selected groups of pediatric patients. Twenty-two pediatric patients (age range 9 months to 12 years; mean 6.5 years) had implantation of ventricular rate responsive (VVIR) pacemakers over a 2-year period. All patients had chronic third-degree atrioventricular block, and a normal ventricular function at rest. During the follow-up each patient underwent a 24-hour Holter monitoring, and ten performed a graded treadmill test in both ventricular fixed rate (VVI) and rate responsive (VVIR) pacing mode. Paced ventricular rates were found to be normal for age in all 22 patients; maximum rate did not reach the higher programmed rate during daily activities in any patient. Comparing the mean paced ventricular rate to the mean rates of blocked P waves, six patients showed a difference of more than 20 beats/min, which induced the pacemaker parameters to be reprogrammed. In all patients a significant correlation was found between variations of paced ventricular rate and variations of spontaneous blocked atrial rhythm (P < 0.05); this correlation persisted in the subsequent Holter controls in the ten patients with longer follow-up. Exercise tolerance resulted normal in the ten patients who performed a treadmill test either in VVIR or VVI mode, with increased maximal heart rates and maximal systolic blood pressure in VVIR mode (P < 0.0013). Rate responsive ventricular pacemakers seem to adequately respond to the physiological needs of daily life of this selected group of children requiring permanent pacing. [ABSTRACT FROM AUTHOR]