Interdigitating Technique for Repair of Aortic Arch Obstruction to Reduce Reintervention Rates.

BACKGROUND: The incidence of reintervention for aortic arch obstruction is 5% to 14% after coarctation or hypoplastic aortic arch repair and 25% after the Norwood procedure. Institutional practice review indicated higher than reported reintervention rates. Our aim was to assess the impact of an interdigitating reconstruction technique on reintervention rates for recurrent aortic arch obstruction. METHODS: Children (<18 years) were included if they had undergone aortic arch reconstruction by sternotomy or the Norwood procedure. Three surgeons participated in the intervention with staggered rollout dates between June 2017 and January 2019, with the study ending December 2020 and review for reinterventions ending February 2022. Preintervention cohorts represented patients who underwent aortic arch reconstructions with patch augmentation, and postintervention cohorts represented patients who underwent an interdigitating reconstruction technique. Reinterventions by cardiac catheterization or operation were measured within 1 year of initial operation. Wilcoxon rank sum and χ2 tests were used to compare preintervention and postintervention cohorts. RESULTS: Overall, 237 patients were included for participation in this study, with 84 patients in the preintervention cohort and 153 in the postintervention cohort. Patients undergoing the Norwood procedure represented 30% (n = 25) of the retrospective cohort and 35% (n = 53) of the intervention cohort. Overall reinterventions were significantly decreased after the study intervention from 31% (n = 26/84) to 13% (n = 20/153; P < .001). Reintervention rates were decreased for each intervention cohort: aortic arch hypoplasia (24% [n = 14/59] vs 10% [n = 10/100]; P = .019) and Norwood procedure (48% [n = 12/25] vs 19% [n = 10/53]; P = .008). CONCLUSIONS: The interdigitating reconstruction technique for obstructive aortic arch lesions was successfully implemented and is associated with a decrease in reinterventions.

Extubation failure after cardiac surgery in children with Down syndrome.

Extubation failure (EF) after cardiac surgery is associated with poorer outcomes. Approximately 50% of children with Down syndrome (DS) have congenital heart disease. Our primary aim was to describe the frequency of EF and identify risk factors for its occurrence in a population of patients with DS after cardiac surgery. Secondary aims were to describe complications, length of hospital stay, and mortality rates. This report was a retrospective case-control study and was carried out in a national reference congenital heart disease repair center of Chile. This study includes all infants 0-12 months old with DS who were admitted to pediatric intensive care unit after cardiac surgery between January 2010 and November 2020. Patients with EF (cases) were matched 1:1 with children who did not fail their extubation (controls) using the following criteria: age at surgery, sex, and type of congenital heart disease. Overall, 27/226 (11.3%) failed their first extubation. In the first analysis, before matching of cases and controls was made, we found association between EF and younger age (3.8 months vs 5 months; p = 0.003) and presence of coarctation of the aorta (p = 0.005). In the case-control univariate analysis, we found association between an increased cardiothoracic ratio (CTR) (p = 0.03; OR 5 (95% CI 1.6-16.7) for a CTR > 0.59) and marked hypotonia (27% vs 0%; p = 0.01) with the risk of EF. No differences were found in ventilatory management. CONCLUSIONS: In pediatric patients with DS, EF after cardiac surgery is associated with younger age, presence of aortic coarctation, higher CTR reflecting the degree of cardiomegaly and hypotonia. Recognition of these factors may be helpful when planning extubation for these patients. WHAT IS KNOWN: • Extubation failure after cardiac surgery is associated with higher morbidity and mortality rates. Some studies report higher rates of extubation failure in patients with Down syndrome. WHAT IS NEW: • In children with Down syndrome, extubation failure after cardiac surgery is associated with younger age, presence of aortic coarctation, higher CTR reflecting cardiomegaly and severe hypotonia.

[Ascending aorta and aortic arch hypoplasia in a patient with connective tissue dysplasia and multiple deficit of coagulation factors]. / Gipoplaziya voskhodyashchego otdela i dugi aorty u bol'nogo s displaziei soedinitel'noi tkani i kombinirovannym defitsitom plazmennykh faktorov svertyvaniya.

Connective tissue dysplasia (CTD) is an urgent problem, especially in young and employable people. Damage to cardiovascular system and, in particular, aorta is predominant factor determining the incidence of various complications and mortality in patients with CTD. The authors report surgical treatment of aortic hypoplasia in an adolescent patient with connective tissue dysplasia syndrome and combined deficiency of coagulation factors.

Identified plasma proteins related to vascular structure are associated with coarctation of the aorta in children.

BACKGROUND: Coarctation of the aorta (CoA), presenting with local stenosis of the aorta is involved in many cardiovascular processes. However, there has been little research on the mechanism of coarctation of the aorta. METHODS: Altered proteins were identified by isobaric tag for relative and absolute quantitation (iTRAQ) technology in 8 participants, and further analysed by heatmap, Gene Ontology (GO), Kyoto Encyclopedia of Genes and Genomes pathway (KEGG) and Search Tool for the Retrieval of Interacting Gene (STRING). Of these, two vascular structure-related proteins were further validated by using enzyme-linked immunosorbent assay (ELISA) in a new cohort of CoA patients. RESULTS: 39 differentially expressed plasma proteins were first identified in patients with coarctation of the aorta by iTRAQ. Of these, fibulin-1 (FBLN1) and insulin-like growth factor-binding protein complex acid labile subunit (ALS) were considered candidates and further validation also showed that the level of FBLN1 in the CoA group (8.92 ± 2.36 µg/ml) was significantly higher compared with control group (6.13 ± 1.94 µg/ml), and the level of ALS in CoA children (348.08 ± 216.74 ng/ml) was significantly lower than the level in normal children (619.46 ± 274.08 ng/ml). CONCLUSIONS: The differentially expressed proteins identified in the plasma from CoA patients indicated that they may play critical roles in CoA and that they could potentially be utilized as biomarkers for diagnosis. Altered vascular related proteins were associated with COA. These results provide a foundation for further understanding and studying the aetiology and pathogenesis of coarctation of the aorta.

Does Ascending Aorta Size Affect Norwood Outcomes in Hypoplastic Left Heart With Aortic Atresia?

BACKGROUND: Conflicting data exist regarding the impact of ascending aorta size on outcomes after the Norwood procedure. Results from multi-institutional studies have largely relied on heterogeneous populations undergoing this surgery for different anatomic defects. Using data from the Single Ventricle Reconstruction Trial, we analyzed the impact of preoperative ascending aortic diameter on Norwood outcomes for patients with aortic atresia variants of hypoplastic left heart syndrome. METHODS: Neonates with aortic atresia and no ventricular septal defect were included and classified into four groups, based on their baseline ascending aorta echocardiographic measurements: less than or equal to 1.5 mm, 1.6 to 1.9 mm, 2.0 to 3.9 mm, and greater than or equal to 4.0 mm. Outcomes included 14-day mortality, transplant-free survival at 1 and 14 months, need for extracorporeal membrane oxygenation, length of ventilation, intensive care, and hospital stay, intensive care unit (ICU)-free days, right ventricular function, and incidence of recoarctation by 14 months. RESULTS: Overall, 292 patients were analyzed. Median length of ICU stay was significantly longer for infants with small aortas, and ICU-free days were significantly lower. There was no difference in length of mechanical ventilation or hospitalization between groups. Long-term right ventricular function and tricuspid regurgitation did not differ. Aortic arch recoarctation incidence was higher in patients with small aortic diameters. Patients with aortas less than or equal to 1.5 mm had decreased 30-day transplant-free survival. CONCLUSIONS: Infants with aortic atresia variants of hypoplastic left heart syndrome and baseline ascending aortic diameter less than or equal to 1.5 mm appear to suffer the greatest morbidity and mortality early after Norwood procedure. These infants also experienced longer stays in the ICU and higher rates of recoarctation. Ascending aortic diameter does not appear to affect long-term ventricular function.

An unexpected association in a patient with heart failure presenting a surgical challenge.

Bicuspid aortic valve (BAV) is the most common form of congenital heart disease and frequently leads to premature valvular dysfunction. BAV is associated with aortic wall abnormalities and a high prevalence of ascending aorta dilatation and coarctation of the aorta (CoA). Consequently, in patients with BAV a careful assessment of the valve, and also of the aortic root and the ascending aorta, should be performed. The most feared complication is aortic dissection, however, the actual incidence of this complication is low. We report the case of a 58-year-old man who presented with New York Heart Association class III heart failure. The work-up revealed BAV with severe stenosis and severe compromise of left ventricle systolic function. In addition, CoA in the isthmus region, and type B dissection of the aorta were diagnosed.

A case of PHACE syndrome with growth hormone deficiency and abnormal thyroid functions.

Background PHACE syndrome is a rare vascular neurocutaneous disorder characterized by posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies and eye anomalies. Growth hormone deficiency (GHD) has been infrequently described. Case presentation We report a girl with PHACE syndrome. Endocrine abnormalities including abnormal thyroid functions and GHD have recently been described in similar cases. Conclusions This case suggests the necessity to screen pituitary functions in all patients with PHACE syndrome with abnormal hypothalamus and pituitary (HP) anatomy. Likewise, growth parameters and thyroid function test (TFT) should be monitored in all patients with PHACE syndrome at regular intervals.

Aorta coarctation and systemic lupus erythematosus: A case report.

RATIONALE: Vasculitis is one of the common pathological hallmarks of systemic lupus erythematosus (SLE). Vascular lesions in SLE commonly involve medium- and small-sized vessels. Rarely, vasculitis in SLE may involve large vessels such as the aorta leading to life-threatening complications. Reported cases of large vessel lesions in SLE included aortic aneurysm and aortic dissection. PATIENT CONCERNS: Here, we report a 52-year-old Chinese woman with SLE, who was stable on oral glucocorticoid, but showed sudden intractable hypertension and heavy proteinuria before we found aorta coarctation in her computed tomography (CT) scan of the aorta. DIAGNOSES: This patient's large vascular lesions were likely secondary and not a primary manifestation of lupus activity. INTERVENTIONS AND OUTCOMES: After endovascular stent graft repair of the abdominal aorta, her hypertension and proteinuria were controlled. LESSONS: In the context of reported cases of large vessel lesions in SLE, our case further supports the significance of having a wide differential for vascular lesions in SLE, especially when an SLE patient presents sudden hypertension and heavy proteinuria. This case also demonstrates that vascular lesions in SLE may lead to serious, potentially fatal consequences.

Endocrine manifestations of PHACE syndrome.

PHACE syndrome is an uncommon disorder of posterior fossa anomalies, cervicofacial infantile hemangiomas, arterial anomalies, cardiac defects, eye anomalies, and midline/ventral defects. Endocrine abnormalities including hypopituitarism and ectopic thyroid were rarely described. In this article we review occurrence, onset, presenting symptoms, hormonal treatments and outcomes of all endocrine abnormalities in PHACE syndrome. Eleven of 20 (55%) had hypothalamic-pituitary dysfunction and 10 of 20 (50%) had thyroid dysgenesis. A thorough understanding of the endocrine manifestations is important for clinicians to early identify endocrine involvement in PHACE and develop plans for monitoring and treatment of its complications.

Experience of stent implantation for recurrent aortic arch obstruction following Norwood or Damus-Kaye-Stansel operation over the last decade.

BACKGROUND: Recurrent aortic arch obstruction following the Norwood procedure is recognised as an important complication. Balloon arch angioplasty is associated with a high recoarctation rate. METHODS: We sought to evaluate the prevalence and outcome of stent implantation for recoarctation in children following Norwood or Damus-Kaye-Stansel procedure over the past decade at a single national cardiology centre. RESULTS: Of 114 children who underwent Norwood procedure or Damus-Kaye-Stansel procedure between January 2003 and June 2013, 80 patients survived. Of these 15 children underwent stent implantation for recoarctation. Six of these patients had previous balloon angioplasty. The median age at stent implantation was 4.4 months (range 2-82 months). The median peak aortic arch gradient at catheterisation decreased from 26mmHg (range 10-70mmHg) to 2mmHg (range 0-20mmHg). The median luminal diameter increased from 4.7 mm (range 3.2-7.9 mm) to 8.6 mm (range 6.2-10.9 mm). The median coarctation index increased by 0.49 (range = 0.24-0.64). A Valeo stent was employed in 11 children, a Palmaz Genesis stent in 2 patients, a MultiLink stent in 1 child, and a Jomed covered stent in 1 child. Two factors were associated with the need for stent placement: previous arch angioplasty (p valve < 0.001, χ-square 11.5) and borderline left ventricle (p = 0.04, χ-square = 4.1). Stent migration occurred in one child. There were two deaths related to poor right ventricular systolic function and severe tricuspid regurgitation. Six patients underwent redilation of the stent with no complications. CONCLUSIONS: The prevalence of recurrent aortic arch obstruction following Norwood/Damus-Kaye-Stansel procedure was 18%. Stent implantation is safe and reliably eliminates the aortic obstruction. Redilation can be successfully achieved to accommodate somatic growth or development of stent recoarctation.

Natural history of PHACE syndrome: A survey of adults with PHACE.

BACKGROUND: Awareness of PHACE syndrome has increased; however, little information exists regarding its natural history, especially in patients over the age of 18. We aim to describe the natural history of PHACE to enhance clinical management and counseling of patients. METHODS: A cohort of patients ≥ 18 years was identified through the PHACE Syndrome Registry and a Vascular Anomalies Clinic Database. A cross-sectional survey was designed after a review of the literature by PHACE experts (IF, JP, DS). Questions were selected by consensus, and the survey was conducted using the Qualtrics platform and via in-person interviews. A 75% response rate was found. RESULTS: Eighteen adults-17 females and one transgender male-completed the survey. Respondents ranged in age from 18 to 59, with 24 being the mean age. Eighty-nine percent reported experiencing headaches, and 17% reported experiencing acute but transient symptoms mimicking acute ischemic stroke, later diagnosed as atypical migraines. Thirty-three percent reported hearing loss, and 67% endorsed dental issues. One patient experienced two arterial dissections. Three-fourths who attempted conception were successful, and none of their children had clinical features of PHACE. Because results were based on a retrospective survey, data captured were prone to recall bias and not objective. Results were limited by a small sample size. CONCLUSIONS: Health care providers should be aware of a possible increased risk of neurovascular complications, including atypical migraines mimicking transient ischemic attacks and arterial dissection, in adults with PHACE. Heritability has not been demonstrated, and future studies are needed to assess the risk of infertility.

Aortic angle is associated with neo-aortic root dilatation and regurgitation following arterial switch operation.

INTRODUCTION: Neo-aortic root dilatation and regurgitation are common progressive long-term complications of the arterial switch operation (ASO) for transposition of the great arteries (TGA) with increasing clinical burden. While several risk factors have been identified, most are constitutional. The acute aortic angle commonly seen after ASO might alter aortic dynamics and facilitate progression of the neo-aortic root dilatation and aortic regurgitation, but insufficient data is available. We intend to assess the effect of the aortic angle in the extent of neo-aortic root dilatation and presence of regurgitation. METHODS: Retrospective analysis of TGA patients undergoing CMR after ASO at a single tertiary centre from November 2010 to July 2017. RESULTS: 180 patients were analysed, 157 of which having adequate imaging of the aortic arch and root. Neo-aortic root Z score was normally distributed with 73% of patients having a Z score > 2. The aortic angle had a significant (p < 0,001) inverse relationship with the neo-aortic root Z score both in univariate and multivariate linear regression. Other significant associations were male gender and the concomitant presence of a VSD or a dysplastic neo-aortic valve. The presence of neo-aortic regurgitation was also inversely correlated with the aortic angle. The presence of a bicuspid neo-aortic valve was another significant association, further correlating with the more severe forms. CONCLUSIONS: Acute aortic angles associate more extensive neo-aortic root dilatation and higher incidence of regurgitation. We believe a surgical technique promoting less acute aortic angles has potential for ameliorating the long-term outcomes of TGA.

Cervical left aortic arch with distal tortuosity causing coarctation and aneurysmal formation in a child.

We present a case of a 10-year-old female with a cervical aortic arch and distal transverse arch tortuosity causing coarctation and aneurysmal formation and describe the surgical repair of this rare congenital anomaly.

Aortopulmonary collaterals in neonates with d-transposition of the great arteries - Clinical significance early after arterial switch operation.

OBJECTIVES: Purpose of this study is to evaluate the clinical significance of major aortopulmonary collateral arteries (MAPCAs) during the early postoperative course after arterial switch operation (ASO) in d-transposition of the great arteries (dTGA). METHODS: Clinical data of 98 patients with simple dTGA between January 2007 and December 2016 at the University Children's Hospital Zurich, Switzerland were analyzed retrospectively. RESULTS: 37 from 98 patients (38%) required cardiac catheterization (CC) due to an early complicated postoperative course or difficult coronary transfer due to special coronary anatomy. In 15 (15%) patients, hemodynamically relevant MAPCAs were found during CC and coil embolization was performed. Patients with relevant MAPCAs had a significantly longer PICU stay (7 versus 6 days, p = 0.021), longer hospital stay (41 versus 27 days, p = 0.005), longer mechanical ventilation time (5 versus 3 days, p = 0.005), longer need for inotropic support (5 versus 4 days, p = 0.001) and delayed chest closure time (3 versus 2 days, p = 0.030) in those in whom it was left open in comparison to all other patients. In patients having CC, pre-surgery oxygen saturation was significantly lower in patients with relevant MAPCAs (58% vs 70%, p 0.019). Echocardiography had a sensitivity of 53% and a specificity of 100% in detecting relevant MAPCAs, accurately. CONCLUSIONS: MAPCAs are frequently found in dTGA patients and can be associated with lower baseline oxygen saturation and a prolonged postoperative course after ASO. Transthoracic echocardiography cannot replace CC as diagnostic tool. If significant MAPCAs are suspected, early CC should be performed for diagnostic and therapeutic reasons.

An unusual case of interrupted cervical aortic arch associated with long segment coarctation of the descending thoracic aorta.

Interrupted aortic arch is a rare congenital anomaly in newborns and infants and is commonly associated with other cardiovascular anomalies. Here, we report an unusual case of type A interrupted cervical aortic arch associated with long segment coarctation of the descending thoracic aorta. Patent ductus arteriosus reconstituted the descending thoracic aorta. Proximal segments of the left common carotid and left subclavian arteries were atretic. Echocardiography-gated multidetector CT angiography not only identified the type of aortic arch interruption in the neonate but also delineated the exact anatomical details.

Early results of aortic arch reconstruction and bilateral pulmonary artery banding: modification of the Norwood operation for treatment of hypoplastic left heart syndrome.

BACKGROUND: In the period from 2003 to the end of 2015, 96 Norwood I procedures were performed in the Paediatric Heart Surgery Department in Katowice, Poland, in children with hypoplastic left heart syndrome (HLHS). AIM: This paper presents a retrospective analysis of early surgical results. METHODS: The patients consisted of two groups: group I included 59 children operated on in the years 2003-2012, in whom the stage I Norwood procedure with the Sano modification was performed with the aortic arch reconstructed by use of a ho-mogenous pulmonary artery patch or a bovine pericardial patch. Group II included 37 children after our modification of the Norwood I procedure, in which the aortic arch was reconstructed with an extracellular matrix patch and bilateral pulmonary artery banding was done. RESULTS: Aortic cross-clamping time was significantly shorter in group II (mean 52; range 38-62 min) than in group I (mean 57; range 39-72 min; p < 0.009). Eighteen (30.5%) children in group I and six (16.2%) in group II died. Although this dif-ference did not reach statistical significance (p = 0.12), it suggested that improved outcomes with the modified procedure are possible. The cause of death in group I was significantly more frequently due to massive postoperative bleeding (n = 6; 33.3%) than in group II (n = 1; 16.7%; p = 0.046). CONCLUSIONS: The introduction of this new surgical technique reduced postoperative bleeding rates, shortened the operation time, and might improve the mortality rate in the first-stage surgical treatment of children with HLHS.

Coarctation of the aorta-stenting via Glidesheath Slender in a newborn with recoarctation early after a Norwood operation.

In this article, we report on a newborn with hypoplastic left heart syndrome in whom recoarctation of the aorta was treated with a bare metal stent (Cook Formula 414 Stent) in the early postoperative period after a Norwood procedure. To reduce the risk for scarring and occluding the femoral artery the stent was implanted via 5F Glidesheath Slender sheath.