Premature closure of the arterial duct presenting with right heart failure of the fetus and ductal aneurysm postnatally.

In utero idiopathic constriction of the arterial duct is a rare condition with only a handful reported cases. Ductal aneurysms with thrombus formations on the other hand are significantly more common. We report a case of a term infant who presented with right heart failure due to premature ductal closure and postnatal severe respiratory distress. Subsequent diagnostics revealed paresis of left laryngeal nerve and obstruction of the left pulmonary artery secondary to a ductal aneurysm. Consequently, surgical intervention was considered necessary. Post-operatively, right ventricular function and hoarseness resolved slowly.

Comparison of Patent Ductus Arteriosus Stent and Blalock-Taussig Shunt as Palliation for Neonates with Sole Source Ductal-Dependent Pulmonary Blood Flow: Results from the Congenital Catheterization Research Collaborative.

Patent ductus arteriosus (PDA) stenting is an accepted method for securing pulmonary blood flow in cyanotic neonates. In neonates with pulmonary atresia and single source ductal-dependent pulmonary blood flow (SSPBF), PDA stenting remains controversial. We sought to evaluate outcomes in neonates with SSPBF, comparing PDA stenting and surgical Blalock-Taussig shunt (BTS). Neonates with SSPBF who underwent PDA stenting or BTS at the four centers of the Congenital Catheterization Research Collaborative from January 2008 to December 2015 were retrospectively reviewed. Reintervention on the BTS or PDA stent prior to planned surgical repair served as the primary endpoint. Additional analyses of peri-procedural complications, interventions, and pulmonary artery growth were performed. A propensity score was utilized to adjust for differences in factors. Thirty-five patients with PDA stents and 156 patients with BTS were included. The cohorts had similar baseline characteristics, procedural complications, and mortality. Interstage reintervention rates were higher in the PDA stent cohort (48.6% vs. 15.4%, p < 0.001).

Total Transcatheter Stage 1: A Word of Caution.

For patients with single ventricle physiology, being able to initially establish systemic blood flow and control pulmonary blood flow is critical to their long-term health. Recently, there have been descriptions in achieving this by a purely transcatheter approach with stenting of the ductus arteriosus and implanting pulmonary flow restrictors, a very appealing prospect. We review a case series of 6 patients who underwent a percutaneous modified stage 1 approach using modified Microvascular plugs (MVP) at our center between September 2019 and December 2019. The initial procedure was technically successful in all patients with single-stage ductal stenting and placement of bilateral modified MVP via femoral access. Four patients underwent repeat cardiac catheterization prior to subsequent surgery that demonstrated elevated Qp:Qs (> 2:1) in 3 of the 4 patients with an elevated mean distal PA pressure > 20 mmHg in all patients. In some patients, the device migrated into the distal right pulmonary artery. One patient after Glenn shunt was found to have significant LPA stenosis requiring stenting. While the percutaneous modified stage 1 approach is a promising approach, we offer a word of caution against widespread adoption of this technique with the currently available devices.

Emergency surgical stent retrieval following stent dislodgement during ductal stenting for hybrid palliation.

Stenting of the ductus arteriosus is part of the hybrid treatment of high-risk patients with hypoplastic left heart syndrome. Dislodgement of a ductal stent is a rare complication. We present challenges faced in successful surgical retrieval of a dislodged ductal stent in a high-risk infant undergoing hybrid palliation for hypoplastic left heart syndrome.

Interventions on patent ductus arteriosus and its impact on congenital heart disease.

The ductus arteriosus (DA) connects the pulmonary artery to the aorta to bypass the pulmonary circulation in utero. It normally closes within 24-72 hours after birth due to increased pulmonary resistance from an increase in oxygen partial pressure with the baby's first breath. Medical treatment can help close the DA in certain situations where closure is delayed. However, in duct-dependent cardiac defects, the presence of the DA is crucial for survival and as such medical and surgical techniques have evolved to prevent closure. This review aims to outline the two main management options for keeping a ductus arteriosus patent. This includes stenting the PDA and shunting via a modified Blalock-Taussig shunt. Whilst both techniques exist, multicentre trials have found equal mortality end points but significantly reduced morbidity with stenting than shunting. This is also reflected by shorter recovery times, reduced requirement for extracorporeal membrane oxygenation (ECMO), and improved quality of life, although stent longevity remains a limiting factor.

Stent ductal en cardiopatía congénita con flujo pulmonar dependiente del ductus / Ductal stenting in congenital heart disease with duct dependent pulmonary blood flow

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Antenatal occlusion of a ductal arteriosus aneurysm: a potential postnatal surgical emergency. Case report and literature review.

Symptomatic presentation of ductal arteriosus aneurysm is usually a consequence of associated complications, including thromboembolism, infection, and compression of adjacent structures. In this case report, we present a thrombosed ductal aneurysm that developed antenatally with further postnatal progression of the thrombus and complete occlusion of the left pulmonary artery. Urgent surgical thrombectomy was successful and the post-operative course was uneventful.

AMP-Kinase Dysfunction Alters Notch Ligands to Impair Angiogenesis in Neonatal Pulmonary Hypertension.

Decreased angiogenesis contributes to persistent pulmonary hypertension of the newborn (PPHN); mechanisms remain unclear. AMPK (5'AMP activated protein kinase) is a key regulator of cell metabolism. We investigated the hypothesis that a decrease in AMPK function leads to mitochondrial dysfunction and altered balance of notch ligands delta-like 4 (DLL4) and Jagged 1 (Jag1) to impair angiogenesis in PPHN. Studies were done in fetal lambs with PPHN induced by prenatal ductus arteriosus constriction and gestation-matched control lambs. PPHN lambs were treated with saline or AMPK agonist metformin. Angiogenesis was assessed in lungs with micro-computed tomography angiography and histology. AMPK function; expression of mitochondrial electron transport chain (ETC) complex proteins I-V, Dll4, and Jag1; mitochondrial number; and in vitro angiogenesis function were assessed in pulmonary artery endothelial cells (PAEC) from control and PPHN lambs. AMPK function was decreased in PPHN PAEC and lung sections. Expression of mitochondrial transcription factor, PGC-1α, ETC complex proteins I-V, and mitochondrial number were decreased in PPHN. In vitro angiogenesis of PAEC and capillary number and vessel volume fraction in the lung were decreased in PPHN. Expression of DLL4 was increased and Jag1 was decreased in PAEC from PPHN lambs. AMPK agonists A769662 and metformin increased the mitochondrial complex proteins and number, in vitro angiogenesis, and Jag1 levels and decreased DLL4 levels in PPHN PAEC. Infusion of metformin in vivo increased the vessel density in PPHN lungs. Decreased AMPK function contributes to impaired angiogenesis in PPHN by altered balance of notch ligands in PPHN.

Sinus Superflex DS© early collapse in systemic arterial duct.

We implanted two sinus Superflex DS© stents in a systemic non-stenotic arterial duct of a newborn with hypoplastic left heart syndrome. Forty-eight hours later an important collapse of the stent was detected and treated with a balloon expandable stent. Sinus Superflex DS© has been specifically designed for systemic ducts. This complication generates doubts about its radial force in this scenario.

Repair of Rare Vascular Ring: Circumflex Aortic Arch with Associated Hypoplasia and Coarctation.

We describe a neonate with an unusual vascular ring formed by a right-sided aortic arch with associated coarctation and distal hypoplasia in the presence of an aberrant left subclavian artery. The descending aorta traveled behind the esophagus to descend on the left side of the spine. A left ductus arteriosus connected to the descending aorta completing the vascular ring, with notable esophageal compression. Surgical correction was accomplished through median sternotomy, resection of the hypoplastic circumflex arch, aortic arch advancement, and end-to-side anastomosis.

Stage 1 hybrid palliation of hypoplastic left heart syndrome: an initial experience in pulmonary trunk approach, procedural modifications, and complication management

Background/aim: Hypoplastic left heart syndrome (HLHS) is a rare pathology with a very high mortality rate. The present study aimed to share our initial experience with the ductus arteriosus stenting procedure using the pulmonary trunk approach in the treatment of HLHS, as well as provide some technical suggestions and discuss complications and their management. Materials and methods: The medical records of 9 neonates (age range: 1­8 days) with HLHS, who were operated on within a 12-month period, were reviewed retrospectively. Preprocedural planning was performed by computed tomography angiography and echocardiography. The operations were performed in a hybrid surgery room by interventional radiologists and pediatric vascular surgeons. Balloon-expandable stents were used in all of the operations. Results: All operations were successfully completed without any intraoperative mortality. All intraoperative complications were managed successfully during the stenting procedure. Conclusion: Stage 1 hybrid palliation for HLHS is a safe and effective procedure when several key points are kept in mind.

Drug-Eluting Stents Compared With Bare Metal Stents for Stenting the Ductus Arteriosus in Infants With Ductal-Dependent Pulmonary Blood Flow.

There have been no clinical studies evaluating the use of drug-eluting stents (DES) versus bare metal stents (BMS) for infants who underwent ductus arteriosus (DA) stent placement for ductal-dependent pulmonary blood flow (PBF). We aimed to compare the use of second-generation (fluoropolymer-coated everolimus) DES to BMS in infants who underwent DA stenting for ductal-dependent PBF. A retrospective study of infants who underwent DA stenting for ductal-dependent PBF from January 2004 to March 2018 at a single tertiary care pediatric hospital was performed. Of 94 infants identified, 71 (46 BMS and 25 DES) met inclusion criteria. Baseline characteristics of the DES and BMS cohorts were comparable. The patent lumen to stent diameter on subsequent angiographic evaluation was 81% in DES as compared with 50% in BMS group; p = 0.01. There were 2 deaths early in our experience, both in the BMS group. Unplanned reinterventions were less in the DES group (3, 12% patients) compared with the BMS group (13, 28%), p = 0.03. Pulmonary artery size as assessed using Nakata and pulmonary artery symmetry index was comparable in both the groups. There was no difference in infection rates between the groups. On multivariate analysis, prematurity, BMS, and lower oxygen saturations at discharge were associated with subsequent unplanned reintervention (p = 0.01, 0.03 and 0.03, respectively). In conclusion, our clinical experience suggests that in infants who underwent DA stenting for ductal-dependent PBF, (fluoropolymer-coated everolimus eluting) DES results in less luminal loss and lower unplanned reintervention for cyanosis as compared with BMS implantation.

Ductal Stenting to Retrain the Involuted Left Ventricle in d-Transposition of the Great Arteries.

BACKGROUND: Ductal stenting is performed to retrain involuted left ventricles (LVs) in patients with d-transposition of the great arteries and intact ventricular septum (TGA-IVS). However, its efficacy is largely unknown. This study aimed to determine the safety and efficacy of ductal stenting in retraining of the involuted LV in patients with TGA-IVS. METHODS: This was a single-center, retrospective study. Echocardiographic assessment of the LV geometry, mass, and free wall thickness was performed before stenting and before the arterial switch operation. Patients then underwent the arterial switch operation, and the postoperative outcomes were reviewed. RESULTS: There were 11 consecutive patients (male, 81.8%; mean age at stenting, 43.11 ± 18.19 days) with TGA-IVS with involuted LV who underwent LV retraining by ductal stenting from July 2013 to December 2017. Retraining by ductus stenting failed in 4 patients (36.3%). Two patients required pulmonary artery banding, and another 2 had an LV mass index of less than 35 g/m2. Patients in the successful group had improved LV mass index from 45.14 ± 17.91 to 81.86 ± 33.11g/m2 (p = 0.023) compared with 34.50 ± 10.47 to 20.50 ± 9.88 g/m2 (p = 0.169) and improved LV geometry after ductal stenting. The failed group was associated with an increased need for extracorporeal support (14.5% vs 50%, p = 0.012). An atrial septal defect-to-interatrial septum length ratio of more than 0.38 was associated with failed LV retraining. CONCLUSIONS: Ductal stenting is an effective method to retrain the involuted LV in TGA-IVS. A large atrial septal defect (atrial septal defect-to-interatrial septum length ratio >0.38) was associated with poor response to LV retraining.

Stenting of the ductus arteriosus for ductal-dependent pulmonary blood flow-current techniques and procedural considerations.

The use of prostaglandin-E1 immediately after birth and subsequent surgical creation of the modified Blalock-Taussig shunt (BTS) shunt have remarkably improved the prognosis and survival of children with congenital heart disease and ductal-dependent pulmonary blood flow (PBF). Despite the advancement in surgical techniques, bypass strategies, and postoperative management, significant morbidity and mortality after BTS still remain. Patent ductus arteriosus stenting has been shown to be as an acceptable alternative to BTS placement in select infants with ductal-dependent PBF. Newer procedural techniques and equipment, along with operator experience have all contributed to procedural refinement associated with improved outcomes over the recent years. In this article, we review the procedural and periprocedural details, with an emphasis on recent advances of this procedure.

Blalock-Taussig shunt versus patent ductus arteriosus stent as first palliation for ductal-dependent pulmonary circulation lesions: A review of the literature.

BACKGROUND: Infants with ductal-dependent pulmonary blood flow (PBF) often undergo a palliative procedure to provide a stable source of PBF prior to definitive palliation or repair. In the current era, a surgical shunt or ductal stent is used to provide PBF. We aimed to review the current literature comparing ductal stents to surgical shunts. METHODS AND RESULTS: Four small, single-center studies and two larger multicenter studies were identified comparing ductal stent to surgical shunt. Combined, these studies showed ductal stent resulted in similar or improved pulmonary artery growth, fewer complications, shorter length of stay, less diuretic use, and improved survival compared to surgical shunt. Despite inherent minor variability among the studies, ductal stent appears to be associated with more frequent reinterventions. CONCLUSIONS: Surgical shunts remain essential to the care of these patients, but ductal stent is a reasonable alternative, and may provide some advantages in select patients with ductal-dependent PBF.