Ductal-dependent Pulmonary Blood Flow Research Articles

Correlation of ductus arteriosus length and morphology between computed tomographic angiography and catheter angiography and their relation to ductal stent length.

Patent ductus arteriosus (PDA) stent placement in infants with ductal-dependent pulmonary blood flow is being increasingly used in clinical practice. To correlate computed tomographic (CT) angiography morphology and length of the PDA with catheter angiography and its relation to eventual PDA stent length. We retrospectively identified all pediatric patients who underwent PDA stenting at our institute from 2004 to 2018. We included children who had CT angiography prior to stenting. PDA length was measured by a radiologist blinded to the catheter angiography data, using Syngo-via post-processing software (Siemens, Erlangen, Germany). Vessel centerline technique was used. We measured the actual length of the duct as well as straight length between aortic and pulmonary ends. PDA morphology tortuosity index was classified as straight (Type I), mildly tortuous with 1 turn (Type II) and tortuous with >1 turn (Type III), and the PDA origin was noted. The PDA was also measured and morphology classified on catheter angiography by an interventional cardiologist blinded to the CT angiography findings. We compared the CT angiography and catheter angiography lengths, straight lengths and stent length using scatter plots and intraclass correlation coefficient (ICC). A total of 83 children who had PDA stenting were identified, of whom 17 had prior CT angiography. Fifteen of these were neonates. There was agreement between CT angiography and catheter angiography regarding the PDA morphology tortuosity index in 94% of cases and PDA origin in 100% of cases. There was moderate agreement between CT angiography and catheter angiography actual and straight PDA lengths, with ICC coefficients of 0.65 and 0.68, respectively. There was moderate agreement between CT angiography actual length, CT angiography straight length, catheter angiography actual length and eventual stented PDA length, with ICCs of 0.57, 0.67 and 0.73, respectively. There was poor agreement between catheter angiography straight length and eventual stented PDA length, with an ICC of 0.39. PDA length and morphology description on CT angiography correlates well with catheter angiography and can be a reliable guide for the interventional cardiologist in decision-making regarding appropriate choice of PDA stent length.

Preprocedural three-dimensional planning aids in transcatheter ductal stent placement: A single-center experience.

Describe the use of three-dimensional (3D) patent ductus arteriosus (PDA) modeling to better define ductal anatomy to improve preprocedural planning for ductal stent placement. Ductal stenting is an alternative to surgical shunting in patients with ductal dependent pulmonary blood flow. Ductal anatomy is often complex with extreme tortuosity and risk of pulmonary artery isolation, thus increasing procedural risks. CT angiograms were segmented to produce 3D PDA models. Ductal morphology was characterized with attention to access approach, degree of pulmonary artery offset/risk of isolation and ductal tortuosity. 3D models were retrospectively compared with biplane angiography. 3D modeling was performed in 12 patients with adequate image quality for complete analysis in 11; median (interquartile range) age/weight 17 days (8-20 days) and 3.1 kg (2.4-3.9 kg). The PDA was reverse oriented in nine with average length of 17.2 ± 2.5 mm and high tortuosity (mean tortuosity index 52, range 3-108). From 3D modeling, two patients were excluded from ductal stenting-extreme ductal tortuosity and threatened pulmonary artery discontinuity, respectively. Ductal stenting was successful in the remaining nine with no major procedural complications. 3D modeling predicted a successful access approach based on the aortic orientation of the ductus in all patients (five carotid, two axillary, two femoral). When comparing 2D angiography with 3D models, angiography consistently underestimated ductal length (-3.2 mm ± 1.6 mm) and tortuosity (-14.8 ± 7.2). 3D modeling prior to ductal stent placement for ductal dependent pulmonary blood flow is useful in procedural planning, specifically for eligibility, access approach, and accurate ductal measurements. Further studies are needed to determine if 3D planning improves procedural outcomes.

Correction to: Comparison of Outcomes at Time of Superior Cavopulmonary Connection Between Single Ventricle Patients With Ductal-Dependent Pulmonary Blood Flow Initially Palliated With Either Blalock-Taussig Shunt or Ductus Arteriosus Stent: Results From the Congenital Catheterization Research Collaborative

HomeCirculation: Cardiovascular InterventionsVol. 12, No. 11Correction to: Comparison of Outcomes at Time of Superior Cavopulmonary Connection Between Single Ventricle Patients With Ductal-Dependent Pulmonary Blood Flow Initially Palliated With Either Blalock-Taussig Shunt or Ductus Arteriosus Stent: Results From the Congenital Catheterization Research Collaborative Free AccessCorrectionPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessCorrectionPDF/EPUBCorrection to: Comparison of Outcomes at Time of Superior Cavopulmonary Connection Between Single Ventricle Patients With Ductal-Dependent Pulmonary Blood Flow Initially Palliated With Either Blalock-Taussig Shunt or Ductus Arteriosus Stent: Results From the Congenital Catheterization Research Collaborative Originally published19 Nov 2019https://doi.org/10.1161/HCV.0000000000000045Circulation: Cardiovascular Interventions. 2019;12:e000045In the article by Meadows et al, “Comparison of Outcomes at Time of Superior Cavopulmonary Connection Between Single Ventricle Patients With Ductal-Dependent Pulmonary Blood Flow Initially Palliated With Either Blalock-Taussig Shunt or Ductus Arteriosus Stent: Results From the Congenital Catheterization Research Collaborative,” which appeared in the October 2019 issue of the journal (Circ Cardiovasc Interv. 2019;12:e008110. doi: 10.1161/CIRCINTERVENTIONS.119.008110), corrections were needed.In the Results section of the Abstract the sentence “Preoperative hemodynamics and overall pulmonary atresia growth were similar, although right pulmonary artery growth was better with DAS (change in z-score: 1.57 versus 0.65, P=0.026)” should be “Preoperative hemodynamics and overall pulmonary artery growth were similar, although right pulmonary artery growth was better with DAS (change in z-score: 1.57 versus 0.65, P=0.026).”In the Conclusions section of the Abstract, the sentence “Both groups demonstrated good pulmonary atresia growth” should be “Both groups demonstrated good pulmonary artery growth.”Additionally, Figures 1 and 2 were incorrect. Figure 1 should have been Figure 2, and Figure 2 should have been Figure 1, and both were missing units of “(Months)” on the x axis.This correction has been made to the article, which is available at: https://www.ahajournals.org/doi/10.1161/CIRCINTERVENTIONS.119.008110. Previous Back to top Next FiguresReferencesRelatedDetails November 2019Vol 12, Issue 11 Advertisement Article InformationMetrics © 2019 American Heart Association, Inc.https://doi.org/10.1161/HCV.0000000000000045 Originally publishedNovember 19, 2019 PDF download Advertisement

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.

Stenting the vertical neonatal ductus arteriosus via the percutaneous axillary approach.

Stenting the ductus arteriosus (DAS) has become an alternative to surgical systemic to pulmonary artery shunts in neonates with ductal-dependent pulmonary blood flow (PBF). Femoral approach for a vertical ductus can be difficult secondary to the acute angle and tortuous course, thus alternative access sites have been explored. Carotid access complications have been reported in 5%-10%. The extensive use of an axillary arterial approach in the United States has not been reported. The aim of this study is to describe our experience with DAS using the axillary approach. We reviewed all patients with DAS with an axillary approach in neonates with ductal-dependent PBF (May 2017-May 2018) in our institution. Procedural reports, angiograms, and clinical records of all consecutive patients were reviewed. Procedural technique, procedural outcomes, adverse events, and post-hospital courses are reported. Seven consecutive patients who received DAS utilizing axillary approach. All patients had ductal-dependent PBF through a vertical, tortuous ductus. Five had pulmonary atresia or near atresia, one had compromised PBF due to dynamic subvalvar obstruction, and one had Tetralogy of Fallot with isolated left pulmonary artery. Axillary access with 3.3 or 4 French sheath was obtained using ultrasound guidance. Bare metal coronary stents were deployed successfully in all. Intra-procedure, one developed in stent thrombus requiring re-stenting. There were no procedural mortalities or major adverse events from axillary access. There is a steep learning curve. Hemostasis was achieved with manual compression. Two patients had reintervention at 6-8weeks. All patients underwent successful planned surgeries. This series suggests DAS in neonates utilizing an axillary approach is a feasible and effective alternative for establishing PBF. Axillary arterial approach may be preferred as there is no risk to neurological sequelae and very low risk of limb complications. Larger series are needed to validate this approach.

Classification scheme for ductal morphology in cyanotic patients with ductal dependent pulmonary blood flow and association with outcomes of patent ductus arteriosus stenting.

To devise a classification scheme for ductal morphology in patients with ductal dependent pulmonary blood flow (PBF) that can be used to assess outcomes. The impact of ductal morphology on outcomes following patent ductus arteriosus (PDA) stenting is not well defined. Patients <1 year of age who underwent PDA stenting for ductal dependent PBF at the four centers comprising the Congenital Catheterization Research Collaborative (CCRC) were included. A classification scheme for PDA morphology was devised based on a tortuosity index (TI)-Type I (straight), Type II (one turn), and Type III (multiple turns). A subtype classification was used based upon the ductal origin. One hundred and five patients underwent PDA stenting. TI was Type I in 58, Type II in 24, and Type III in 23 PDAs, respectively. There was a significant association between ductal origin and vascular access site (p < 0.001). Procedure times and need for >1 stent did not differ based on TI. Greater TI was associated with pulmonary artery (PA) jailing (p = 0.003). Twelve (11.4%) patients underwent unplanned reintervention, more commonly with greater TI (p = 0.022) and PA jailing (p < 0.001). At the time of subsequent surgical repair/palliative staging, PA arterioplasty was performed in 32 patients, more commonly when a PA was jailed (p = 0.048). PA jailing did not affect PA size at follow up. The proposed qualitative and quantitative PDA morphology classification scheme may be helpful in anticipating outcomes in patients with ductal dependent PBF undergoing PDA stenting.

Total Correction of Tetralogy of Fallot in the First 60 Days of Life in Symptomatic Infants: Is It The Gold Standard?

The timing of surgical repair of tetralogy of Fallot (TOF) is a key to alleviate complications and for long-term survival. Total correction was usually performed at the age of 6 months or older under the notion of decreasing the surgical risk. However, avoiding palliation with an aortopulmonary shunt and early correction of systemic hypoxia appear to be of more benefit than the inborn surgical risk in low body weight patients. Our objective was to assess early/midterm survival and operative complications and to analyze patients, surgical techniques, and morphological risk factors to determine their effects on outcomes. We retrospectively reviewed 152 patients with TOF who were ≤60 days of age when they underwent total correction of TOF. All patients had either duct-dependent pulmonary blood flow or arterial blood oxygen saturation less than 65% on room air requiring urgent surgical correction. Exclusion criteria included TOF with pulmonary atresia, TOF with nonconfluent pulmonary arteries, TOF with multiple aortopulmonary collateral arteries, and associated complete atrioventricular septal defects. The mean age at repair was 34 ± 19 days, and the mean weight was 3.8 ± 0.9 kg. Before surgery, 96 patients received an infusion of prostaglandin, 45 were mechanically ventilated, and 32 required inotropic support. Right ventricular outflow tract obstruction was managed with a transannular patch in 112 patients, and all the others had a main pulmonary artery patch. Cardiopulmonary bypass (CPB) with moderate hypothermia was the standard, and the CPB time averaged 48 ± 21 minutes. The postoperative intensive care unit stay was 5.7 ± 6 days, with 2.8 ± 4 days of mechanical ventilation. Early mortality was 4.6% (7 of 152), and actuarial survival rates were 95% at 1 year and 92% at 5 years. Univariable and multivariable analyses of the patients' demographics, anatomical characteristics, and operative techniques revealed the presence of small pulmonary arteries and low body weight to be the only independent risk factors for death. Early total correction of TOF during the first 60 days of life can be performed with low mortality and good intermediate-term survival and, from our point of view, "should be the gold standard for TOFs."

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

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. 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. 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.

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.

Fetal hemodymanic effects on ductus arteriosus development and influences on postnatal management in infants with ductal-dependent pulmonary blood flow.

The ductus arteriosus (DA) has been studied since Galen. Initially after birth in neonates with obstruction to pulmonary blood flow, DA patency is integral to ensure output and oxygenation. While DA stenting dates back 25 years, there is emerging interest in better understanding how and when to utilize this strategy as an alternative to surgical shunt placement or ongoing prostaglandin administration. Understanding the normal fetal circulation and the perturbations that affect flow and oxygenation is integral to comprehending how normal DA anatomy and morphology may change and how this may influence technical and clinical considerations. In the normal human fetus the great majority of descending aorta circulation comes from the DA, whereas this is a small minority in pulmonary outflow lesions, resulting in size and angle abnormalities. Study of the DA morphology has previously sought to identify patients requiring early intervention and more novel classifications are contributing to knowledge of complications and increasing the likelihood of success. As well, optimal patient selection for aorto-pulmonary shunt vs DA stent remains unclear. This review seeks to convey how fetal circulation can affect the DA, how other clinical considerations such as neurocognitive development support these finding and influence management, and emphasize that the variability in the DA will affect suitability for stenting, which requires further study as guidelines and standards are developed.

Abstract 16867: Comparison of Ductal 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

Introduction: Ductus arteriosus stenting (DAS) is an accepted method for securing pulmonary blood flow in cyanotic neonates. However, in neonates with pulmonary atresia and single source ductal-dependent pulmonary blood flow (SSPBF), DAS remains controversial. Published outcomes in this population are limited. We sought to evaluate outcomes in neonates with SSPBF pts, comparing DAS and surgical Blalock-Taussig shunt (BTS). Methods: Neonates with SSPBF who underwent DAS or BTS from four centers of the Congenital Catheterization Research Collaborative from January 2008 to December 2015 were retrospectively reviewed. Peri-procedural complications, reintervention prior to ultimate surgical repair, pulmonary artery (PA) diameter and interval growth were compared using available imaging studies. A propensity score was utilized to compare cohorts adjusted for differences in patient factors. Results: Forty patients with DAS and 157 patients with BTS were included. At palliation, the DAS and BTS cohorts had similar age, weight, and expected ultimate physiology. Rates of procedural complications following BTS and DAS were also similar. Following initial palliation, DAS patients were less likely to be treated with inotropes (p &lt;0.01) or diuretics (p = 0.046). Both groups had similar median lengths of hospital stay DAS 19 days (9-38), BTS 13 (10-23), p=0.19 and rates of unplanned reinterventions (17.5% of DAS and 21% in BTS, p= 0.62). Pre-palliation PA diameter was smaller (p=0.02), and PA symmetry was lower (p=0.02) in the DAS cohort. However, by time of definitive repair there was no difference in PA symmetry (0.80 in DAS cohort vs 0.82 in BTS cohort, p=0.64), in PA diameter (Nakata 161mm 2 /m 2 (137-166) in DAS vs 152mm 2 /m2 (94-227) in BTS, p=0.62) or in PA growth (change in Nakata 51mm 2 /m 2 (-21-61) in DAS vs 24mm 2 /m 2 (-28-131) in BTS, p=0.79). Rates of PA plasty and post-repair PA interventions did not differ between the cohorts. Conclusions: In neonates with SSPBF, DAS and BTS appear to have equivalent outcomes post-palliation and at time of subsequent surgical repair. Despite concerns about DAS in SSPBF, it appears to be a durable approach with unchanged PA growth compared to BTS.

Duct Stenting Versus Modified Blalock-Taussig Shunt in Neonates With Duct-Dependent Pulmonary Blood Flow: Associations With Clinical Outcomes in a Multicenter National Study.

Infants born with cardiac abnormalities causing dependence on the arterial duct for pulmonary blood flow are often palliated with a shunt usually between the subclavian artery and either pulmonary artery. A so-called modified Blalock-Taussig shunt allows progress through early life to an age and weight at which repair or further more stable palliation can be safely achieved. Modified Blalock-Taussig shunts continue to present concern for postprocedural instability and early mortality such that other alternatives continue to be explored. Duct stenting (DS) is emerging as one such alternative with potential for greater early stability and improved survival. The purpose of this study was to compare postprocedural outcomes and survival to next-stage palliative or reparative surgery between patients undergoing a modified Blalock-Taussig shunt or a DS in infants with duct-dependent pulmonary blood flow. All patients undergoing cardiac surgery and congenital interventions in the United Kingdom are prospectively recruited to an externally validated national outcome audit. From this audit, participating UK centers identified infants <30 days of age undergoing either a Blalock-Taussig shunt or a DS for cardiac conditions with duct-dependent pulmonary blood flow between January 2012 and December 31, 2015. One hundred seventy-one patients underwent a modified Blalock-Taussig shunt, and in 83 patients, DS was attempted. Primary and secondary outcomes of survival and need for extracorporeal support were analyzed with multivariable logistic regression. Longer-term mortality before repair and reintervention were analyzed with Cox proportional hazards regression. All multivariable analyses accommodated a propensity score to balance patient characteristics between the groups. There was an early (to discharge) survival advantage for infants before next-stage surgery in the DS group (odds ratio, 4.24; 95% confidence interval, 1.37-13.14; P=0.012). There was also a difference in the need for postprocedural extracorporeal support in favor of the DS group (odds ratio, 0.22; 95% confidence interval, 0.05-1.05; P=0.058). Longer-term survival outcomes showed a reduced risk of death before repair in the DS group (hazard ratio, 0.25; 95% confidence interval, 0.07-0.85; P=0.026) but a slightly increased risk of reintervention (hazard ratio, 1.50; 95% confidence interval, 0.85-2.64; P=0.165). DS is emerging as a preferred alternative to a surgical shunt for neonatal palliation with evidence for greater postprocedural stability and improved patient survival to destination surgical treatment.

Stenting of the ductus arteriosus in infants with functionally univentricular heart disease and ductal-dependent pulmonary blood flow: A single-center experience.

To determine the short- and medium-term outcomes of ductal stenting (DS) in patients with functionally univentricular hearts (FUHs) and ductal-dependent pulmonary blood flow. Several studies have evaluated the outcomes of DS in a limited number of patients with FUHs. Nonetheless, there is still no consensus regarding the indications for this procedure, and no appropriate patient selection criteria have been devised. From 2005 to 2015, cardiac catheterization for DS was performed in 68 patients with FUHs. Of these patients, 49 had single source pulmonary blood flow from ductus arteriosus. Procedural and follow-up data were evaluated. The median weight of the patients was 3.6 kg (2.3-6.8 kg), and the median age was 26 days (3 days-8 months). The technical success rate of the procedure was 95% (65 of 68 patients). Mean oxygen saturation increased from 70% ± 7.6% to 87% ± 4.6% (P < 0.0001). Among patients in whom DS was successful, 55 (84.6%) were bridged to a Glenn procedure after a median of 9.1 months (6.4-14 months), and 41 (63%) were successfully bridged to a Glenn procedure without additional interventions. Of the 16 patients with preexisting mild pulmonary artery stenosis, five required a surgical shunt due to progressive branch pulmonary artery stenosis, while nine were bridged to a Glenn operation without shunt placement. Five (7.3%) patients died, including patients who were sent to surgery. DS is a reasonable and effective alternative to surgical shunt placement as a first-stage palliative procedure in patients with FUHs. © 2016 Wiley Periodicals, Inc.

Ductus arteriosus stenting: A promising percutaneous palliation in patients with duct-dependent pulmonary circulation

ObjectivesWe aimed to study the feasibility and outcomes of ductal stenting in patients with duct-dependent pulmonary blood flow (PBF). MethodsDuct-dependent hypoxic patients with confluent pulmonary artery (PA) branches were enrolled for ductal stenting and followed regularly. ResultsSixty patients, with a median age of 12 (1-1095) days and weight of 2.8 (2.2-8.9) kg, were enrolled. Median right PA (RPA) and left PA (LPA) Z-scores were -1.23 (-10.54 to 2.81) and -0.96 (-8.03 to 3.0), respectively. Mean narrowest ductal diameter was 1.73±0.57 mm and length was 12.78±3.32 mm. Sixty-four stents with mean diameter of 4.21±0.32 mm and length of 14.34±3.44 mm were deployed in 59 patients. The procedure was unsuccessful in one. Post-stenting mean oxygen saturation (SO2) increased significantly from baseline of 68.88±7.47% to 90.43±6.04% (p<0001). Complications included pulmonary edema in one patient and acute stent occlusion in another. At a median follow-up of eight (2-14) months, mean SO2 (80.04±7.54%) was significantly higher than baseline (p<0.0001). Median RPA and LPA Z-scores, 0.56 (-2.89 to 3.29) and -0.02 (-2.81 to 3.86), respectively, were significantly higher than baseline. Six patients required re-interventions (shunt in three and angioplasty in three). Six patients died, three due to sepsis and another three with worsened cyanosis due to impaired PBF, probably due to ductal occlusion. ConclusionDuctal stenting is an effective palliation in patients with duct-dependent PBF. It maintains adequate SO2 and promotes balanced PA growth at mid-term follow-up.

Ductus arteriosus stenting: A promising percutaneous palliation in patients with duct-dependent pulmonary circulation

ObjectivesWe aimed to study the feasibility and outcomes of ductal stenting in patients with duct-dependent pulmonary blood flow (PBF). MethodsDuct-dependent hypoxic patients with confluent pulmonary artery (PA) branches were enrolled for ductal stenting and followed regularly. ResultsSixty patients, with a median age of 12 (1-1095) days and weight of 2.8 (2.2-8.9) kg, were enrolled. Median right PA (RPA) and left PA (LPA) Z-scores were -1.23 (-10.54 to 2.81) and -0.96 (-8.03 to 3.0), respectively. Mean narrowest ductal diameter was 1.73±0.57 mm and length was 12.78±3.32 mm. Sixty-four stents with mean diameter of 4.21±0.32 mm and length of 14.34±3.44 mm were deployed in 59 patients. The procedure was unsuccessful in one. Post-stenting mean oxygen saturation (SO2) increased significantly from baseline of 68.88±7.47% to 90.43±6.04% (p<0001). Complications included pulmonary edema in one patient and acute stent occlusion in another. At a median follow-up of eight (2-14) months, mean SO2 (80.04±7.54%) was significantly higher than baseline (p<0.0001). Median RPA and LPA Z-scores, 0.56 (-2.89 to 3.29) and -0.02 (-2.81 to 3.86), respectively, were significantly higher than baseline. Six patients required re-interventions (shunt in three and angioplasty in three). Six patients died, three due to sepsis and another three with worsened cyanosis due to impaired PBF, probably due to ductal occlusion. ConclusionDuctal stenting is an effective palliation in patients with duct-dependent PBF. It maintains adequate SO2 and promotes balanced PA growth at mid-term follow-up.

Pulmonary artery growth after arterial duct stenting in completely duct-dependent pulmonary circulation

ObjectiveSignificant and balanced pulmonary artery (PA) growth following arterial duct (AD) stenting has already been reported in literature. However, no data are so far available about the role of this...

Non-Surgical Management of Ductal Dependent Lesion as a Safe Option: A Case Report and Review of Literature

Introduction: Critical congenital heart disease with ductal dependent pulmonary blood flow can present in early neonatal period as a cardiac emergency. Case Presentation: We herein reported a case of critical pulmonary stenosis in a newborn who presented with cyanosis and breathlessness. Conclusions: Initially managed with prostaglandin, an emergency balloon pulmonary valvuloplasty proved to be an effective and safe option.

Routine Use of Pulse Oximetry in the Newborn to Detect Critical Congenital Heart Disease

Critical Congenital Heart Disease’s (CCHD’s) are potentially life-threatening cardiac abnormalities where the systemic or pulmonary circulation is dependent on a patent ductus arteriosus and include those with duct-dependent pulmonary blood flow, inter-circulatory mixing, and systemic hypoperfusion. The seven main CCHD’s screening targets for neonatal pulse oximetry are hypoplastic left heart syndrome, pulmonary atresia (with intact ventricular septum), Tetralogy of Fallot, total anomalous pulmonary venous return, transposition of the great arteries, tricuspid atresia and truncus arteriosus. Early diagnosis improves outcomes. Systematic review and metaanalysis of pulse oximetry screening for critical congenital heart defects in asymptomatic newborn babies revealed that the overall sensitivity was 76.5% and specificity was 99.9%. The false-positive rate was low when it was done after 24 hours from birth [1]. The American Heart Association and the American Academy of Pediatrics suggest that routine pulse oximetry done on asymptomatic newborns after 24 hours of life but before discharge may detect critical CHD, incurs very low cost, and there is a low risk of harm [2]. European studies suggest that screening is cost-effective in identifying newborns with CCHD [3,4]. Over half of babies discharged with CHD detected in the first year of life were discharged from the hospital with a normal routine neonatal examination and the 6 week exam missed one-third of the patients [5]. In many countries, births are not attended and infants are discharged within hours of birth without medical evaluation by a qualified practioner. CCHD’s will go undetected in this setting.

Ebstein's anomaly and Down's syndrome : case report

We report on two cases presenting with a rare combination of Ebstein's anomaly and Down's syndrome. The first patient presented with respiratory distress, mild cyanosis and right heart failure immediately after delivery. The symptoms improved with heart failure medication. The patient remained asymptomatic on follow up. The second patient was diagnosed antenatally with marked apical displacement of the tricuspid valve and a very small functional right ventricle compared to the left ventricle. At birth, the patient presented with an extreme form of Ebstein's anomaly with severe cyanosis, marked right heart failure and ductal-dependent pulmonary blood flow. The patient died within days of birth.

Neither age at repair nor previous palliation affects outcome in tetralogy of Fallot repair

The study aimed to evaluate the results following complete repair of tetralogy of Fallot (TOF) in relation to age at surgery and to assess the role of palliation in the current era. A retrospective review of 251 consecutive patients with TOF repaired between 2003 and 2011 at the Great Ormond Street Hospital was performed. Children were divided into two groups: Group A, younger than 6 months (n = 78) and B, older than 6 months (n = 173). Early clinical outcomes and reoperation/reintervention rates were studied as well as indication for a palliation. There was 1 (0.4%) early and 1 (0.4%) late death after a median follow-up time of 4.5 years. Forty-three patients (17%) underwent repair after initial palliation with inter-stage mortality of 5%. Groups A and B were similar in terms of surgical approach, postoperative complications and length of stay. Significant differences were found in terms of more frequent use of a transannular patch (P = 0.05), longer surgeries (P = 0.02) and a greater proportion of palliated patients (P = 0.002) in older patients. There was no difference in rates of reoperation/reintervention between groups and following both primary and staged repair. Palliated patients were more symptomatic (duct-dependent pulmonary blood flow; P < 0.01, cyanotic spells; P < 0.01), had more extracardiac/genetic anomalies (P < 0.01), coronary anomalies (P = 0.015) and significantly smaller pulmonary annulus, right pulmonary artery (RPA) and left pulmonary artery (LPA) Z-scores (P < 0.01 for all). Age at complete repair was not linked to early clinical outcome or reoperation/reintervention rate. Palliative procedures postponed the timing of complete repair, but did not increase the reintervention rate.