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Objectives To evaluate early results, learning-curve changes, and mid-term outcome of arterial duct (AD) stenting in congenital heart disease with duct-dependent pulmonary circulation (CHD-DPC) in a high-volume, tertiary referral center. Background In spite of wide acceptance as cost-effective alternative to surgical palliation, AD stenting indications, results and mid-term outcome still largely depend on small series, experiences in particular subsets of patients or multicenter series with different approaches and interventional philosophy. Methods Between April 2003 and December 2013, 119 patients underwent AD stenting as lower-risk palliation of CHD-DPC at our Institution. Procedural and mid-term follow-up data of these patients are reported. Results The procedure was successfully completed in 93.3% of cases, with a complication rate and in-hospital mortality of 17.6% and 3.6%, respectively. No patient underwent rescue surgical shunt but elective Blalock-Taussig shunt was needed in 15 patients (13.5%). Over time, favorable trends toward higher feasibility and efficacy in complex ductal anatomy as well as lower procedural risk were recorded. Presurgical cardiac catheterization (n = 36) showed significant and balanced pulmonary artery (PA) growth (Nakata Index +113 ± 101%; left PA z-score +87 ± 52%; right PA z-score +97 ± 53%, P

OBJECTIVE: Children undergoing Ross operation were expected to have longer autograft, but shorter homograft durability compared with adults. In order to define the outcome in the second decade after Ross operation in children, a nationwide review of 23 years of experience was undertaken. METHODS: 305 children underwent Ross operation in 11 paediatric units between 1990 and 2012. Age at surgery was 9.4±5.7 years, indication aortic stenosis in 103 patients, regurgitation in 109 and mixed lesion in 93. 116 (38%) patients had prior procedures. Root replacement was performed in 201 patients, inclusion cylinder in 14, subcoronary grafting in 17 and Ross-Konno in 73. RESULTS: There were 10 (3.3%) hospital and 12 late deaths (median follow-up 8.7 years). Survival was 93±2% and 89±3% and freedom from any reoperation was 76±3% and 67±6% at 10 and 15 years. 34 children had autograft 37 reoperations (25 replacement, 12 repair): three required transplantation after reoperation. Freedom from autograft reoperation was 86±3% and 75±6% at 10 and 15 years. 32 children had right heart redo procedures, and only 25 (78%) conduit replacements (15-year freedom from replacement, 89±4%). Prior operation (p=0.031), subcoronary implant (p=0.025) and concomitant surgical procedure (p=0.004) were risk factors for left heart reoperation, while infant age (p=0.015) was for right heart. The majority (87%) of late survivors were in NYHA class I, 68% free from medication and six women had pregnancies. CONCLUSIONS: Despite low hospital risk and satisfactory late survival, paediatric Ross operation bears substantial valve-related morbidity in the first two decades. Contrary to expectation, autograft reoperation is more common than homograft.

We report the case of a 15 years old girl with Williams-Beuren syndrome and atrial mixoma.

Background Evaluation of the impact of prosthesis-patient mismatch (PPM) on long-term outcome and quality of life (QOL) in elderly patients who underwent implantation of small size bileaflet prostheses for aortic stenosis. Methods Between September 1988 and September 2006, 377 patients aged greater than 70 years underwent aortic valve replacement with a small size bileaflet prosthesis (17, 19, and 21 mm) in one Institution. The study population's survivors (345 patients) were divided into three groups according to the indexed effective orifice area (EOAI): Group A included patients with EOAI less than 0.60 cm 2 /m 2 ; group B included patients with EOAI ranging between 0.61 and 0.84 cm 2 /m 2 ; and group C included patients with EOAI 0.85 cm 2 /m 2 or greater. Cumulative and comparative analyses of long-term outcomes and of left ventricular mass regression were performed. The QOL was evaluated with the 36-Item Short Form Health Survey (SF-36) questionnaire. Results Overall hospital mortality was 8.5% (32 patients). Group A included 33 patients (9.6%), group B 175 (50.7%), and group C 137 (39.7%). Actuarial survival was 88.8% ± 0.016 at 1 year, 82.1% ± 0.022 at 5 years, and 76.7% ± 0.032 at 10 years. No difference emerged among the three groups. A significant reduction in left ventricular mass was observed in all groups and in all patient subsets of prosthetic size. The scores obtained in the SF-36 test were similar in the three groups and significantly higher than those of the general population ( p Conclusions Incidence of severe PPM is low after aortic valve replacement. Presence of severe or moderate PPM, did not influence long-term outcome, left ventricular mass regression and QOL in a population of septuagenarians.

Background. Superimposed acute right ventricular dysfunction in the setting of preexisting pulmonary hypertension is a nearly fatal complication after heart transplantation. The optimal treatment modality remains a matter of debate. Recently, sildenafil citrate, a nonselective pulmonary vasodilator, has gained popularity in the treatment of pulmonary hypertension in transplant candidates. Methods. Herein we have presented a series of 13 patients in whom sildenafil was used to treat right ventricular dysfunction and pulmonary hypertension as detected by transesophageal echocardiography and Swan-Ganz right heart catheterization after heart transplant. Their characteristics were mean age 49 11.4 years; 38.4% with previous cardiac procedures, 30.8% status I, basal pulmonary vascular resistance index 10.4 4.6 WoodU, mean transpulmonary gradient 18.7 5.4 mmHg. In addition to conventional inodilator support, we administered 1 to 3 mg per kilogram of sildenafil. Complete hemodynamic measurements were obtained before and after the institution of the therapy and at 1-month follow-up. Results. Within the first 72 hours, acute right ventricular dysfunction resolved in all cases without untoward side effects or significant systemic impact. Sildenafil significantly decreased the transpulmonary gradient and pulmonary vascular resistance index relative to baseline values; 5.6 1.82 versus 10.4 4.6 WU, (P .05), 13.5 3.4 mm Hg versus 18.7 5.4 mm Hg (P .05), respectively. Improved indices of right ventricular function were observed on echocardiographic monitoring. After 1 month, sildenafil treatment was discontinued. Conclusion. Management of acute right ventricular dysfunction in heart transplant recipients with pulmonary hypertension using sildenafil proved safe and effective.

The aim of this study was to determine whether changes in prognosis and quality of life (QOL) after aortic valve replacement (AVR) in octogenarians differ depending on the choice of mechanical (MP) or tissue (BP) valves.Between July 1992 and September 2006, 160 consecutive octogenarians underwent AVR with (18.8%) or without concomitant coronary artery bypass grafting. At follow-up (mean 3.4 +/- 2.8 years, 552 patient-years, 98.3% complete), 121 were still alive and answered the Medical Outcomes Study Short-Form 36 Health Survey (SF-36) QOL questionnaire.Group BP had 62 patients. Group MP had 98 patients. Preoperative risk factors were comparable except group BP was older. Global hospital mortality was 8.8%. There were 21 late deaths, 61.9% of which were not valve- or anticoagulation-related. A significant difference emerged in 1-, 3-, 5- and 8-year actuarial survival rates (BP: 86.4% +/- 0.04%, 76.9% +/- 0.06%, 58.1% +/- 0.1%, 46.5% +/- 0.14%, respectively, vs MP: 91.3% +/- 0.03%, 88.6% +/- 0.03%, 81.6% +/- 0.05%, 70% +/- 0.67%; p = 0.025) but not in terms of 8-year freedom from valve-related complications (82.6% +/- 0.1% vs 87% +/- 0.053%, p = 0.55). One anticoagulant-related hemorrhage occurred in group MP; one stroke occurred in group BP. Survivors had significant improvement in New York Heart Association functional class compared with preoperatively (1.1 vs 2.8, p0.001) Mean QOL scores were satisfactory and substantially comparable between the two groups; in seven domains, scores were higher than those of the age- and sex-matched general Italian population.Long-term survival after AVR in selected octogenarians was similar to that of the general elderly population. The device type exerted no influence on QOL.

Congenital heart disease (CHD), the most common cardiac malformation in the fetal and neonatal period, represents a heterogeneous group of defects with little known cause. The aim of this study was to investigate the incidence rate (IR) of CHD without spontaneous resolution in the first year of life, the age at diagnosis, the most common type of CHD, and the IR changes in severe and nonsevere CHD during the study period.From January 1997 to December 2002, 4559 live births with CHD (diagnosed until December 2004) were enrolled in the Campania Region. Number of live-born children by month was derived from national registries.About 70% of CHD patients were diagnosed within the first year of life. The overall mean regional IR of CHD was 11.1 (confidence interval 10.8-11.4) patients per 1000 live-born children. The overall mean IR of CHD was significantly different in the five provinces of the Campania Region, but the annual IR remained stable in each of them throughout the study period. The relative risk in live-born children and residents in the areas with an elevated IR of CHD was higher for nonsevere CHD, but of low significance for severe CHD. At least one-third of CHD patients had atrial septal defects.We described the highest IR of CHD from one of the largest samples used up to now for this purpose (410 181 live-born children), but we think that this value could be higher (poor surveillance in some areas). Moreover, this study increases the awareness that CHD patients with a few symptoms are frequently underdiagnosed in areas with inadequate health services and that by an improved availability of pediatric echocardiographic assesments seem warranted. Eight years of surveillance and an increasing attention of both pediatricians and cardiologists to mild CHD in some provinces allowed us to demonstrate that, despite previous reports, the most common CHD in our region was atrial septal defect.

Bilateral arterial ducts serving nonconfluent pulmonary arteries is a very rare pattern of pulmonary blood flow in congenital heart disease with pulmonary atresia. In this setting, neonatal ductal closure might result in abrupt pulmonary hypoperfusion and life-threatening systemic hypoxia, thereby indicating emergent surgical palliation or repair. However, percutaneous arterial duct stenting might be an alternative to surgery, especially in high-risk patients. This article reports on two critical neonates with complex heart disease and discontinuous pulmonary arteries dependent on bilateral arterial ducts who successfully underwent transcatheter ductal stenting as first-step palliation toward lower-risk corrective surgery.

Objective: This study aimed to describe the features and identify the predictors of ascending aorta dilatation in patients with congenital bicuspid aortic valve (BAV). Methods: In 280 adult patients with isolated BAV undergoing echocardiography, multivariate logistic regression models, including clinical and echocardiographic variables, were developed to predict dilatation (aortic ratio exceeding 1.1) at both ascending and root level. Factors predicting aneurysm with surgical indication were also investigated. Classification tree models were used to identify factors influencing the probability of having a small aorta, normal aortic dimensions, a dilated ascending aorta or a dilated root (aortic phenotypes). Results: Aortic dilatation was present in 83.2% patients, prevailing at the mid-ascending tract in 83.7% of them. Surgical indication criteria were reached in 43.2% patients. A small aortic root was found in 16 patients (5.7%), thereafter excluded from multivariate models predicting dilatation. Age (maximal risk at 50—60 years: OR = 13.7; reference category: 60 (OR = 2.6, p = 0.022) and severe regurgitation (OR = 3.9, p = 0.011) were determinants of root involvement, while stenosis (moderate; OR = 0.3, p < 0.001) was a protective factor. Aortic stenosis (any degree, OR = 2.4) and hypertension (OR = 4.3) were the most significant predictors of mid-ascending aneurysm reaching surgical indication. Classification analysis showed that increasing age significantly increased the prevalence of ascending dilation phenotype, stenosis increased the prevalence of small aorta phenotype, and male gender of root dilation phenotype. Once excluding patients with small aortas from the analysis, a positive correlation was observed between degree of stenosis and mid-ascending size (p = 0.016). Conclusions: BAV patients constitute an importantly heterogeneous population in terms of risk and features of aortic disease. The most common condition is an ectasia of the mid-ascending tract, with unaffected or mildly involved root. If further confirmed, this could suggest that surgical approach may spare the root in most BAV patients. Mid-ascending dilatation is proportional to stenosis severity, suggesting a post-stenotic causative mechanism. Root dilatation is rarer, mostly observed in younger men, and unrelated to the presence and severity of stenosis. The two different aortic dilatation phenotypes (mid-ascending and root) may be subtended by different pathogeneses. # 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

ObjectivesThis study sought to evaluate the impact of arterial duct (AD) stenting in promoting catch-up growth of hypoplastic pulmonary artery (PA) tree in congenital heart disease with duct-dependent pulmonary circulation (CHD-DPC).BackgroundSignificant and balanced PA growth following AD stenting has already been consistently reported in the literature. However, no data are so far available about the role of this approach in severe PA hypoplasia, which significantly impacts the risk of surgical repair.MethodsPre-surgical angiographic PA evaluation was performed in 45 patients with confluent PAs submitted to neonatal AD stenting as palliation of CHD-DPC. PA growth was evaluated as Nakata Index and McGoon ratio as well as individual PA z-score changes, both in the whole population and according to the original vessel size (Nakata Index 100 mm2/m2, Group II [n = 30]).ResultsControl angiography was performed 7.5 ± 6.5 months (median 6 months) after duct stenting, showing significant and balanced PA growth. The Nakata Index increased from 143 ± 73 mm2/m2 to 270 ± 88 mm2/m2 (124 ± 118%, p < 0.0001); left PA z-score from −0.7 ± 1.7 to 1.0 ± 1.4; right PA z-score from −0.6 ± 1.3 to 1.2 ± 1.3 (p < 0.0001 for both comparisons). Group I showed a greater increase of global PA growth (Nakata Index increase 227 ± 141% vs. 72 ± 57%, p < 0.001) as compared with Group II. Final PA size did not significantly differ between the groups (246 ± 105 mm2/m2 vs. 282 ± 78 mm2/m2, p = NS).ConclusionsPercutaneous AD stenting is highly effective in promoting a significant and balanced catch-up growth of diminutive PAs, being therefore advisable in this subset of patients as a reliable alternative to surgical palliation.

Right chamber dilatation and right-to-left volumetric unbalance are well-known cardiac consequences of atrial septal defect (ASD) shunt, accounting for most of its long-term complications. Thus, cardiac volumetric unloading is a major aim of ASD closure. Different from surgery, transcatheter option might be considered as an "unbiased" tool to evaluate the cardiac geometric remodeling following ASD closure.Extent and time-course of cardiac geometric changes were assessed by echocardiography 24 h, 1 and 6 months after percutaneous closure of large ASD (mean diameter 17+/-6 mm, QP/QS 2.2+/-0.9) in 42 asymptomatic patients (age 22+/-18 years).Transcatheter closure was accomplished using the Amplatzer Septal Occluder device (mean 23+/-7 mm, median 24 mm), achieving a complete occlusion in all patients at the 6-month follow-up control. After ASD closure, right atrial (RA) volume reduced from 45+/-24 to 28+/-12 ml (-37.8%, p0.001), while left atrial (LA) volume did not significantly change. Inlet and infundibulum right ventricular (RV) end-diastolic diameters reduced by 23+/-2% and 23+/-3%, respectively (p0.001 for both measurements), although with a different time-course of changes. Finally, transverse left ventricular (LV) end-diastolic diameter increased from 39+/-7 to 44+/-5 mm (+11.4%, p0.01). These geometric changes resulted in an RV/LV diameter ratio decrease by 34+/-3% (p0.001). Nearly 90% of cardiac remodeling ensued within 1 month from shunt disappearance (50% within 24 h).Percutaneous ASD closure results in early and striking cardiac geometric changes that almost completely revert the right-to-left volumetric unbalance. Most of this geometric remodeling ensues within a few weeks from ASD closure.

Arterial tortuosity syndrome is a connective tissue disorder characterized by elongation and tortuosity of large elastic arteries, potentially resulting in multiple vascular stenoses. This paper reports a novel hybrid strategy to treat 'complex' bilateral peripheral pulmonary artery stenoses consisting of transcatheter stent implantation via midline sternotomy and reductive angioplasty of the proximal pulmonary arteries.

Congenital sinus of Valsalva aneurysm is a rare cardiac malformation that usually becomes symptomatic as a consequence of intracardiac or extracardiac rupture. It is difficult to suspect in association with complex cardiac defects and its rupture may be misdiagnosed as progressive aortic regurgitation. This case report refers to a patient with tricuspid atresia submitted to Fontan procedure five years previously, in whom a sinus of Valsalva aneurysm rupture into the accessory ventricular chamber caused rapidly progressive heart failure. The malformation was suspected by echocardiography and treated by percutaneous implantation of an Amplatzer duct occluder, with immediate improvement of the patient's clinical and functional status.

Double right aortic arch with mirror-image branching is a very uncommon vascular anomaly as a cause of symptomatic dysphagia in neonatal age [1,2]. It results from persistence of the right fourth embryonic aortic arch and involution of the left fourth arch between the subclavian artery and the dorsal descending aorta. When the ductus arteriosus or its “remnant” (ligamentum arteriosus) originates from the descending aorta, a complete vascular ring is formed. A 10 day-old neonate underwent cardiac evaluation due to the suspicion of vascular ring causing swallowing difficulty and habitual regurgitation. Clinical examination and electrocardiography did not show any significant finding. Echocardiography imaged just a right aortic arch with mirror-image branching without any intra-cardiac abnormality. However, the unusual location of a small patent ductus arteriosus, arising very distal from the midthoracic aorta, made us to suspect a vascular compression of the oesophagus. Simultaneous oesophagography and aortic angiography confirmed the oesophageal compression due to the Kommerell's diverticulum that gave rise to a left-sided ductus (Figs. 1 and 2). Surgical exploration found that the

Sudden death is a possible occurrence for newborns younger than 1 year with severe aortic coarctation (CoA) before surgical correction. Basic research and animal experiments have shown electrophysiologic changes during mechanical ventricular pressure overload. The current study aimed to evaluate the effect of severe CoA on the heterogeneity of ventricular repolarization by examining corrected QT and JT interval dispersion (respectively, QTc-D and JTc-D) and electrocardiographic parameters of spatial heterogeneity of ventricular repolarization in newborns with no associated congenital cardiac malformations. The study enrolled 30 isolated severe CoA neonates (age, 45 ± 15 days; 17 males) with normal size and wall thickness of the left ventricle before surgical correction and 30 age- and sex-matched healthy newborns used as control subjects. Heart rate, QRS duration, maximum and minimum QT and JT intervals, and QTc-D and JTc-D measurements were performed. The healthy control group did not significantly differ from the CoA group in terms of heart rate, weight, height, and echocardiographic parameters. Compared with the healthy control group, the CoA group presented significantly increased values of QTc-D (109.7 ± 43.4 vs. 23 ± 15 ms; P = 0.03) and JTc-D (99.1 ± 43.3 vs. 65.8 ± 24.1 ms; P = 0.04). A statistically significant correlation was found between the Doppler peak pressure gradient across the coarctation site and the values of QTc-D (r = 0.48; P = 0.03) and JTc-D (r = 0.42; P = 0.04). Our study showed significantly increased QTc-D and JTc-D in isolated CoA newborns with normal left ventricular geometry. © Springer Science+Business Media, LLC 2011.

High prevalence of somatic mutations in the cardiac transcription factor genes NKX2.5 and GATA4 have been reported in the affected cardiovascular tissue of patients with isolated cardiac septal defects, suggesting a role of somatic mutations in the pathogenesis of these congenital heart defects (CHDs). However, all somatic mutations have been identified in DNA extracted from an archive of formalin-fixed cardiac tissues. In the present study, to address the hypothesis that somatic mutations are important in isolated CHDs, we analyzed the GATA4 and NKX2.5 genes in the fresh-frozen pathologic cardiac tissue specimen and corresponding non-diseased tissue obtained from a series of 62 CHD patients, including 35 patients with cardiac septal defects and 27 with other cardiac anomalies. We identified one variant and two common polymorphisms in the NKX2.5 gene, and six variants and two common polymorphisms in the GATA4 gene. All identified variants were seen in both the fresh-frozen pathologic cardiac tissue and the corresponding non-diseased tissue, which indicates that they all were constitutional variants. The present study has identified NKX2.5 and GATA4 constitutional variants in our CHD cohort, but was unable to replicate the previously published findings of high prevalence of somatically derived sequence mutations in patients with cardiac septal defects using fresh-frozen cardiac tissues rather than formalin-fixed tissues. © 2011 Elsevier Masson SAS.

A critical infant with unrestrictive ventricular septal defect and absence of the left pulmonary artery underwent transcatheter re-canalization of the occluded arterial duct-left pulmonary artery complex and surgical banding of the right pulmonary artery. After this hybrid approach, the baby was discharged under mild anticongestive therapy in view of a later surgical repair.

SummaryBetween February 1986 and December 1991, 41 patients with pulmonary atresia and intact ventricular septum were treated in our institution following a multistage protocol of management. In all cases, the first step was to construct a right modified Blalock-Taussig shunt during the neonatal period regardless of the right ventricular anatomy. Then, in patients with well-developed right ventricles possessing all three components, we proceeded to early surgical repair. In contrast, in patients with right ventricles having obliteration of some components, yet deemed to be recoverable, the next step was to provide palliative relief of obstruction in the right ventricular outflow tract followed, if possible, by subsequent repair. Fontan's operation was performed in patients with right ventricles considered unsuitable from the outset to support the pulmonary circulation. Only one patient died following the initial shunt procedure (mortality of 2.43%). The subsequent program of treatment has now been concluded in 22 patients. In all those deemed to have favorable native anatomy (10 cases), the subsequent complete repair was successful. Among the 24 patients who required palliation of the outflow tract, five died while total repair was subsequently performed in eight. Fontan's operation was performed without mortality in five patients with small right ventricles, although one patient died while waiting for surgery. In all, 89 procedures were performed with an overall mortality of 14%.

The aim of this study was to compare the pulmonary artery (PA) growth after arterial duct (AD) stenting versus modified Blalock-Taussig shunt (MBTS) in neonates with congenital heart disease with duct-dependent pulmonary circulation (CHD-DPC).Arterial duct stenting is increasingly deemed a reliable alternative to surgical shunt in CHD-DPC. A stented duct might better adapt to the PA anatomy than a surgical conduit, thereby promoting a more uniform PA development.This study enrolled 27 patients with CHD-DPC submitted to AD stenting (n = 13, Group I) or MBTS (n = 14, Group II) at our institution. The PA growth was angiographically assessed with the Nakata and McGoon indexes as well as the individual PA z-scores. The right-to-left PA diameter ratio was considered as index of uniform growth.After 10 +/- 5 months, both options had promoted a significant increase of the Nakata index (from 136 +/- 72 mm/m(2) to 294 +/- 99 mm/m(2), p0.0001, Group I; from 151 +/- 74 mm/m(2) to 295 +/- 177 mm/m(2), p0.003, Group II) and McGoon ratio (from 1.5 +/- 0.3 to 2.1 +/- 0.3, p0.0001, Group I; from 1.6 +/- 0.3 to 2.0 +/- 0.5, p0.01, Group II). However, the surgical shunt had caused a worsening of the left-to-right PA diameter ratio compared with AD stenting (0.9 +/- 0.1 Group I vs. 1.6 +/- 0.9 Group II, p0.01), due to preferential growth of the PA contralateral to the shunt.Percutaneous AD stenting is as effective as MBTS in promoting a global PA growth in CHD-DPC. In addition, it ensures an even distribution of the pulmonary blood flow, thereby promoting a more balanced pulmonary vascular development than MBTS.

A 2 month-old infant with severe congestive heart failure due to unrestrictive ventricular septal defect and absence of the left pulmonary artery was submitted to a hybrid transcatheter-surgical palliation consisting in percutaneous re-canalization of the occluded arterial duct-left pulmonary artery complex and surgical banding of the right pulmonary artery. The post-operative course was uneventful and the baby was discharged in a few days under mild anti-congestive therapy. This hybrid approach was highly effective in stabilizing this critical infant in view of a later and safer surgical repair.

Arterial tortuosity syndrome is a rare connective tissue disorder characterized by elongation and tortuosity of large elastic arteries resulting in multiple vascular stenoses. This article reports on a novel hybrid transcatheter-surgical strategy to treat bilateral peripheral pulmonary artery stenoses due to this syndrome. Operation consisted of stent implantation by midline sternotomy and reductive reconstruction of the proximal pulmonary arteries. This approach resulted in right ventricular overload relief and sudden improvement of the patient's clinical condition.

Congenital heart diseases are the most common prenatal and postnatal malformations. Nowadays, fetal echocardiography is a widely practiced technique; however, the impact of prenatal diagnosis on prognosis of the newborns affected by congenital heart disease remains uncertain.To assess the outcome and the changes in the spectrum of prenatally detected congenital heart disease in our tertiary care centre in 12 years of activity (1995-2006).We detected 705 congenital heart diseases: 32% (223) were associated with extracardiac or chromosomal anomalies or both, and 68% (482) were isolated. Termination of pregnancy was chosen in 81% for associated anomalies and 37% for isolated anomalies (P0.001). Of these, more than one-third occurred in hypoplasic left heart cases. The general survival rate was 72%; it was significantly lower in the group with associated heart diseases (46 vs. 80%, P0.001). Over 12 years we noticed a reduction in the number of multimalformed fetuses and of the hypoplasic left heart cases, and a higher number of aortic arch anomalies detected. During the past 6 years of activity the survival rate obtained has significantly increased (55 to 84%, P0.05), the termination rate has significantly decreased (35 to 14%, P0.001) and the number of neonatal deaths has significantly decreased (39 to 10%, P0.001).The survival and the voluntary termination of fetuses with prenatally detected congenital heart diseases are strongly influenced by disease severity and by associated extracardiac or chromosomal anomalies, or both. Over 12 years, the spectrum of fetal congenital heart disease has changed and their outcome has significantly improved.

Objective: To evaluate the feasibility and results of stenting of the arterial duct in newborns with duct-dependent pulmonary circulation using low-profile, high-flexibility premounted coronary stents. Design: Prospective interventional and clinical follow-up study. Setting: Tertiary referral centre. Patient population: Between April 2003 and December 2006, 26 neonates (mean (SD) age 15.2 (19.9) days, mean (SD) weight 3.3 (0.8) kg) underwent attempts at stenting of the arterial duct. Main outcome measures: Procedural success and complication rates. Early and mid-term follow-up results. Results: The procedure was successfully completed in 24/26 (92.3%) cases. Minor complications occurred in 2/26 (7.7%) cases. No mortality occurred. After stenting, the ductal diameter increased from 1.2 (1.0) mm to 3.1 (0.4) mm (p

Background The aim of the study was to examine our experience with the implant of bileaflet mechanical prostheses and with a centralized management of anticoagulation and the related risks in patients aged older than 70 years, focusing on the resulting expectancy and quality of life. Methods Between January 1988 and January 2005, 681 consecutive patients older than 70 years (mean age, 73 ± 3.3 years) underwent bileaflet prostheses implantation in an isolated procedure (77%) or concomitant with other procedures (23%). Data were retrospectively collected, and follow-up was conducted by mean of outpatient chart review and outpatient clinic controls. Follow-up included assessment of perceived quality of life through the Medical Outcomes Trust Short Form 36-Item Health Survey tool (SF-36). The scores obtained by the patients were compared with those of the Italian general population matched for age and sex. Results Hospital mortality was 11.8%, and 74 late deaths (12.3%) occurred. Mean follow-up was 4.38 ± 2.85 years. Actuarial survival was 85.2% ± 0.014% at 1 year, 77.9% ± 0.017% at 5 years, 74.2% ± 0.02% at 10 years, and 71.8% ± 0.031% at 15 years. The mean international normalized ratio variability was 4.5% ± 1.2%. Freedom from bleeding was 98.7% ± 0.005% at 5 years and 98.3% ± 0.007% at 10 and 15 years. Freedom from thromboembolism was 99.1% ± 0.004% at 5 years, and 98.3% ± 0.007% at 10 and 15 years. The mean SF-36 scores in the study patients were significantly higher than those of the general population matched for age and sex ( p Conclusions Septuagenarian patients receiving mechanical valve prostheses did not experience increased rates of anticoagulation-related complications and perceived a satisfactory quality of life.

The association between large, left-sided patent ductus arteriosus and severe, peripheral, right pulmonary artery stenosis with no other cardiac malformation is an unreported condition that might be misdiagnosed as pulmonary hypertension due to long-standing ductal shunt. A 57-year-old man with supposed hypertensive patent ductus arteriosus underwent confirmatory cardiac catheterization. At angiography, a severe pre-hilar right pulmonary artery stenosis (peak pressure gradient, 65 mmHg) was found to complicate the hemodynamic picture of a moderate-to-large patent ductus arteriosus (QP/QS, 1.7:1), by causing pulmonary hypertension (mean pressure, 65 mmHg) and left-to-right pulmonary flow imbalance. Both lesions were treated in a single procedure of right pulmonary artery stenting and patent ductus arteriosus closure, after which the pulmonary artery pressure significantly decreased (mean, 35 mmHg). In our opinion, a thorough hemodynamic evaluation followed by pulmonary angiography should be mandatory before proceeding to patent ductus arteriosus closure in the adult patient who has “hypertensive” ductus, in whom possible associated malformations can be missed due to a poor echocardiographic window.

OBJECTIVE Transcatheter closure of atrial septal defects (ASDs) is currently a reliable alternative to surgery, even though challenging in the case of complex septal anatomy. The aim of this study was to evaluate the feasibility and mid-term results of percutaneous closure of complex ASDs in a tertiary referral centre compared with simple ASD closure. METHODS Between April 2000 and November 2004, 209 patients were submitted to transcatheter ASD closure; 83 patients (39.7%) presented with a complex defect (large ASDs with a deficient rim or a multifenestrated/aneurysmal septum) and were treated using different devices tailored to the atrial septal anatomy. RESULTS The transcatheter procedure was successful in 72 patients (86.8%), using a single device in 69 patients and two devices in the remaining three patients. Overall, 71 Amplatzer septal occluders, two multifenestrated Amplatzer septal occluders and two Cardioseal/Starflex devices were used. Procedural and fluoroscopy times were 141 +/- 45 min and 28 +/- 22 min, respectively (P < 0.0001 vs. simple ASD closure for both comparisons). Procedure-related complications were recorded in nine patients (12.5%) (P < 0.01 vs. simple ASD closure). One patient required surgical repair of a femoral arteriovenous fistula and another developed mitral valve dysfunction. Immediate ASD occlusion was recorded in 59.7% of patients, reaching 95.9% at the last follow-up control (P = NS vs. simple ASD closure for both comparisons). CONCLUSIONS Percutaneous closure of complex ASDs may be considered technically feasible, relatively safe and highly effective, although the procedure is still significantly more demanding than transcatheter closure of simple ASDs.

Aortic wall stress has been shown to increase locally at the convex aspect of the ascending tract when axial root motion is increased, as occurs in aortic valve regurgitation. The study aim was to assess the expression of extracellular matrix (ECM) proteins involved in stress-induced vascular remodeling in the convexity and the concavity of dilated ascending aortas with aortic valve regurgitation.Aortic wall specimens, harvested at the convexity and concavity of eight dilated ascending aortas with severe aortic valve regurgitation underwent morphometry, Western blot, RT-PCR and confocal immunohistochemistry. Five patients (group A) had congenital bicuspid aortic valve (BAV), and three (group B) had Marfan's syndrome. Specimens from the aorta of three multi-organ donors served as controls.At morphometry, medial degeneration was more severe in the convexity than in the concavity, especially in group A. Western blot, RT-PCR and immunohistochemistry disclosed an asymmetric pattern in the expression of some ECM proteins (laminin, tenascin, fibronectin). Fibronectin was increased in the convexity of both groups compared to controls at Western blot. Immunohistochemistry confirmed this pattern only in BAV. Higher levels of tenascin were found in the convexity in group A. The laminin content was greater in the concavity than in the convexity of both groups, but in group B the type of laminin was different, with the beta2 chain particularly expressed, and almost absent in non-Marfan patients. Type I and type III collagens were more markedly reduced in the convexity than in the concavity in BAV. In group B, type I collagen was decreased and type III increased, but without any significant difference between the two aspects of the aorta.A tissue remodeling response to valve disease-related wall stress may underlie aortic dilatation with BAV regurgitation. Although morphometry showed similar changes in Marfan aortas, molecular investigations differentiated this condition, qualitatively, from BAV.

Few data exist on infective endocarditis (IE) in intravenous drug abuse (IVDA) patients. In particular, clinical features, site of involvement and bacteriologic findings are controversial. Little is also known on the results of surgical treatment and on the long-term prognosis.The clinical and microbiological characteristics of IE in a series of 39 IVDA patients were retrospectively assessed and compared to those in 85 non-IVDA patients with a likely similar life expectancy. The total follow up of patients was 717.6 patient-years (119.9 pt-yr for IVDA, 597.7 pt-yr for non-IVDA).Although tricuspid involvement was significantly more frequent in IVDA cases than in non-IVDA cases (p = 0.001), left-sided endocarditis prevailed in both groups. In addition to Staphylococcus aureus (51.3%), Staph. epidermidis (15.4%) and streptococcal spp. (23.1%) were emerging pathogens in IVDA cases. A worse cardiac function (p0.002) and a higher rate of embolism (p = 0.04) characterized the preoperative status of IVDA patients. No difference was observed as to indications, emergency procedures and pathologic findings. Hospital and long-term survival did not significantly differ between the two groups. The rate of recurrence was higher in IVDA cases; this difference was mostly accounted for by early postoperative events.A new pattern of IE in IVDA is emerging, characterized by more frequent left heart involvement (61.5%), a severe clinical course, and a need for surgery in the active phase. Staph. epidermidis and streptococci are emerging pathogens. Drug abuse does not affect postoperative prognosis when an aggressive surgical attitude is combined with prolonged medical therapy. Higher rates of early recurrence are expected during the follow up period.

Left ventricular outflow tract (LVOT) obstruction due to anomalous tissue tag arising from the mitral valve is a rare congenital cardiac anomaly. It generally becomes symptomatic during the first decade of life as exercise intolerance, chest pain, or syncope at effort. To date, only a few cases of critical systemic obstruction due to isolated mitral valve anomaly in neonates have been reported. We report the case of a neonate who was a few hours old and was referred in severe clinical condition due to critical left ventricular outflow obstruction resulting from an anomalous tissue tag of mitral valve origin.

A neonate with severe aortic coarctation showed a double lumen transverse aorta (persistent fifth aortic arch) with both channels joining at the isthmus where the obstruction was confirmed by echocardiography and cardiac catheterization. Surgical repair was performed with a pantaloon-shaped patch. Persistent fifth aortic arch does not result in a vascular ring and, per se, is not hemodynamically significant unless associated with other cardiac malformations.

Bilateral arterial ducts feeding discontinuous pulmonary arteries are a very rare anatomic arrangement in complex heart malformations with pulmonary atresia. In this setting, neonatal ductal closure may result in abrupt pulmonary hypoperfusion and life-threatening systemic hypoxia. In high-risk patients, percutaneous arterial duct stenting might be an engaging and cost-effective alternative to surgery. This paper reports on a critical low-weight neonate with complex heart disease and discontinuous pulmonary arteries dependent on bilateral arterial ducts who underwent successful transcatheter ductal stenting as an alternative to a high-risk surgical palliation.

In 1997 Argenta and Morykwas 1 reported the use of negative pressure to enhance wound granulation and closure. Since then, more studies have reported good results with the application of vacuum in the treatment of deep sternal wound infection after cardiac surgery. 2,3 No study has described the use of negative pressure and the therapeutic intensity of vacuum in the neonatal age group. We report our experience in a 28-day-old female neonate with a thoracotomy wound infection treated with vacuum application. Clinical Summary

An 11-year-old boy was successfully treated by stent implantation for native aortic coarctation. At the 1-year control a severe re-coarctation was found at Doppler analysis and subsequent angiography revealed a transverse stent fracture. A stent-in-stent implantation was performed. Several hypotheses could explain this complication. Interventional cardiologists may pay more attention in following up these patients!

A 9-year-old female was referred to our institution with a history of palpitations at rest. She had no comorbidities or history of cardiovascular disease. Previous electrocardiograms (ECGs) and 24 h Holter monitoring failed to record any arrhythmias. The physical examination revealed a blood pressure of 100/ 60 mmHg, pulse of 85 b.p.m., and normal findings on auscultation. The 12-lead ECG was normal for age and sex. A transthoracic echocardiogram revealed an abnormal chamber connected to the lateral wall of the right atrium consistent with a large congenital right atrial aneurysm/diverticulum. A transoesophageal echocardiogram was then performed (Panel A) along with 3D off-line reconstruction. Multiplane 3D views showed a large diverticulum connected to the free wall of the right atrium with a 1 * 1.5 cm orifice (Panel B–E) with several trabeculae consistent with septation within the diverticulum (Panel D). The patient was referred for surgery owing to the increased risk of supraventricular arrhythmias and thrombus formation. Surgical exploration confirmed all the echocardiographic findings (Panel F). Excision of the diverticulum and direct suture of the connecting orifice was then performed. The patient was discharged 7 days after surgery and remains asymptomatic at 6-month follow-up.

The ideal palliation for infants with pulmonary atresia, ventricular septal defect and confluent pulmonary arteries should promote normal development of the pulmonary artery branches. In 26 patients who survived a modified Blalock-Taussig shunt (MBTS) in the first year of life, the right pulmonary artery was measured before and after operation by two dimensional (2D) echocardiography. In each patient its size was compared to the normal value for the same body surface area. The patients were divided according to this ratio and according to the real size (mm). Although increased in all cases but two, the size of the right pulmonary artery remained remarkably less than normal in patients with an initially small right pulmonary artery. In view of these results, early palliative enlargement of the right ventricular outflow tract is probably advisable in infants with diminutive pulmonary arteries who do not show adequate pulmonary branch development after MBTS.

Sudden death is a possible occurrence for newborns younger than 1 year with severe aortic coarctation (CoA) before surgical correction. In our previous study, we showed a significant increase of QTc-D and JTc-D in newborns with isolated severe aortic coarctation, electrocardiographic parameters that clinical and experimental studies have suggested could reflect the physiological variability of regional and ventricular repolarization and could provide a substrate for life-threatening ventricular arrhythmias. The aim of the current study was to evaluate the effect of surgical repair of CoA on QTc-d, JTc-d in severe aortic coarctation newborns with no associated congenital cardiac malformations. The study included 30 newborns (18M; 70±12 h old) affected by severe congenital aortic coarctation, without associated cardiac malformations. All newborns underwent to classic extended end-to-end repair. Echocardiographic and electrocardiographic measurements were performed in each patient 24 h before and 24 h after the interventional procedure and at the end of the follow-up period, 1 month after the surgical correction. All patients at baseline, 24 h and one month after CoA surgical repair did not significantly differ in terms of heart rate, weight, height, and echocardiographic parameters. There were no statistically significant differences in QTc-D (111.7±47.4 vs 111.9±63.8 ms vs 108.5±55.4 ms; P=0.4) and JTc-D (98.1±41.3 vs 111.4±47.5 vs 105.1±33.4 ms; P=0.3) before, 24 h and 1 month after CoA surgical correction. In conclusions, our study did not show a statistically significant decrease in QTc-D and JTc-D, suggesting the hypothesis that the acute left ventricular afterload reduction, related to successful CoA surgical correction, may not reduce the ventricular electrical instability in the short-term follow-up.