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.

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.

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.

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.

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.

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.

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.

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.

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.

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 transcatheter-surgical strategy in arterial tortuosity syndrome.

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.

Prosthesis-patient mismatch in the elderly: survival, ventricular mass regression, and quality of life.

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 < 0.001 in all domains). 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.

Changing spectrum and outcome of 705 fetal congenital heart disease cases: 12 years, experience in a third-level center.

INTRODUCTION: 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. OBJECTIVE: 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). METHODS AND RESULTS: 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 (P<0.001). 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%, P<0.001). 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%, P<0.05), the termination rate has significantly decreased (35 to 14%, P<0.001) and the number of neonatal deaths has significantly decreased (39 to 10%, P<0.001). CONCLUSION: 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.

Bilateral arterial duct 'stenting' in a low-weight neonate with complex congenital heart defect.

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.

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.