Ten-years, single-center experience with arterial duct stenting in duct-dependent pulmonary circulation: early results, learning-curve changes, and mid-term outcome.

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 < 0.001 for all comparisons), without significant changes of left-to-right PA diameter ratio. PA growth was significantly better in patients with severely hypoplastic PAs at the time of duct stabilization (Nakata Index increase 194 ± 115 vs. 75 ± 61%, P < 0.001). CONCLUSIONS: AD stenting is feasible and effective at low-risk in a high percentage of patients with CHD-DPC, promoting significant and balanced PA growth mainly in patients with hypoplastic main PAs at duct stabilization.

Heterogeneity of ventricular repolarization in newborns with severe aortic coarctation.

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.

Predictors of ascending aortic dilatation with bicuspid aortic valve: a wide spectrum of disease expression.

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: <30 years) and severe aortic stenosis (OR=23.8) independently predicted mid-ascending dilatation (p<0.001). Male gender (OR=4.1, p=0.001), age >60 (OR=2.6, p=0.022) and severe regurgitation (OR=3.9, p=0.011) were determinants of root involvement, while stenosis (> or =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.

Spatial patterns of matrix protein expression in dilated ascending aorta with aortic regurgitation: congenital bicuspid valve versus Marfan's syndrome.

BACKGROUND AND AIM OF THE STUDY: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Infective endocarditis in intravenous drug abusers: patterns of presentation and long-term outcomes of surgical treatment.

BACKGROUND AND AIM OF THE STUDY: 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. METHODS: 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). RESULTS: 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 (p < 0.002) 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. CONCLUSION: 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.

Transcatheter treatment of "pulmonary artery hypertension" due to patent ductus arteriosus and pulmonary artery stenosis.

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.

Transcatheter palliation of congenital heart disease with reduced pulmonary blood flow.

BACKGROUND: Although surgical shunt is still considered as the best palliation of congenital heart disease with reduced pulmonary blood flow, stent implantation may be technically simpler, safer and more cost-effective than surgery in high-risk patients. This study evaluated the feasibility and results of this option in patients with duct-dependent pulmonary blood flow or systemic-to-pulmonary shunt malfunction. METHODS: Between April 2003 and July 2004, 9 patients (age 11 days-52 years, weight 2.1-52 kg) with complex congenital heart disease underwent stent implantation inside the patent ductus arteriosus (4 patients) or a stenotic surgical shunt (5 patients). RESULTS: The stenting procedure was successfully completed in all cases. The procedural time was 162 +/- 36 min (range 90-225 min). The fluoroscopy time was 33.8 +/- 6.8 min. No patient died. The morbidity rate was 22.2% (1 patient had local infection at the site of puncture and 1 had transient femoral artery pulse loss). After the procedure, the ductus/shunt diameter increased from 1.2 +/- 0.6 to 3.6 +/- 0.6 mm (p < 0.0001) and oxygen saturation improved from 74.0 +/- 6.5 to 85.2 +/- 3.3% (p < 0.01). Three patients underwent corrective surgery without technical problems after 8.0 +/- 1.0 months while oxygen saturation remained constantly >80% in patients still waiting for surgical repair (follow-up 5.3 +/- 3.1 months). CONCLUSIONS: Stent implantation is a technically feasible, safe and effective palliative option in high-risk surgical patients with congenital heart disease and reduced pulmonary blood flow. Although larger series are required to define the cost-effective clinical impact of this therapeutic option, it is reasonable to hypothesize a further extension of its indication even to elective and low-risk surgical patients.

Fate of Hypoplastic Pulmonary Arteries After Arterial Duct Stenting in Congenital Heart Disease With Duct-Dependent Pulmonary Circulation.

OBJECTIVES: This 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). BACKGROUND: Significant 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. METHODS: Pre-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 mm(2)/m(2), Group I [n = 15] vs. Nakata Index >100 mm(2)/m(2), Group II [n = 30]). RESULTS: Control 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 mm(2)/m(2) to 270 ± 88 mm(2)/m(2) (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 mm(2)/m(2) vs. 282 ± 78 mm(2)/m(2), p = NS). CONCLUSIONS: Percutaneous 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.

Two decades of experience with the Ross operation in neonates, infants and children from the Italian Paediatric Ross Registry.

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.

Hybrid palliation in complex congenital heart malformation with duct-dependent isolated pulmonary artery.

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.

Aortic erosion during negative pressure therapy in a pediatric heart transplant recipient.

A happy-ending "series of unfortunate events" is reported of the successful emergency treatment of an erosion of the ascending aorta during negative pressure sternal wound therapy for a relapse of Berlin Heart driveline infection in a pediatric transplant recipient. Several key issues related to assist device-related infections and negative pressure complications are discussed in this peculiar setting.

Search of somatic GATA4 and NKX2.5 gene mutations in sporadic septal heart defects.

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.

Late percutaneous re-canalization of arterial duct-dependent isolated pulmonary artery.

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.

Hybrid transcatheter--surgical approach in complex pulmonary artery stenosis due to arterial tortuosity syndrome.

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.

Pulmonary artery growth after palliation of congenital heart disease with duct-dependent pulmonary circulation: arterial duct stenting versus surgical shunt.

OBJECTIVES: 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). BACKGROUND: 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. METHODS: 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. RESULTS: 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), p < 0.0001, Group I; from 151 +/- 74 mm/m(2) to 295 +/- 177 mm/m(2), p < 0.003, Group II) and McGoon ratio (from 1.5 +/- 0.3 to 2.1 +/- 0.3, p < 0.0001, Group I; from 1.6 +/- 0.3 to 2.0 +/- 0.5, p < 0.01, 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, p < 0.01), due to preferential growth of the PA contralateral to the shunt. CONCLUSIONS: 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.

Aortic coarctation with persistent fifth left aortic arch.

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.

Hybrid approach in a case of arterial tortuosity syndrome.

Arterial tortuosity syndrome is a rare connective tissue disorder characterised by elongation, tortuosity, stenosis and aneurysms of the large and middle-sized arteries. The symptomatology is correlated to the artery affected by the pathology with correlated stenosis. We describe our hybrid surgical procedure in the treatment of a case of kinking of the pulmonary branches with significant gradient and hypertension. Aortic arch and supraaortic vessels presented various deviousness without hemodynamic alterations.

Stenting of bilateral arterial ducts in complex congenital heart disease.

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.

Tissue versus mechanical prostheses: quality of life in octogenarians.

BACKGROUND: 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. METHODS: 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. RESULTS: 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, p < 0.001) 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. CONCLUSIONS: 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.