Infrared thermography as an adjunctive tool for detection of femoral arterial thrombosis after cardiac catheterization: A prospective, pilot study.

OBJECTIVES: To assess the utility of infrared thermography (IRT), to map skin temperature, in the detection of femoral arterial (FA) thrombosis after cardiac catheterization. BACKGROUND: Ultrasound is a validated method for thrombus detection but is generally reserved as a confirmatory test for clinical suspicion due to various constraints. METHODS: Prospective study of infants and children undergoing cardiac catheterization via FA access, comparing IRT and pulse examination. The thermograms, displayed in a color map with each pixel representing a temperature, were examined by qualitative assessment of symmetry in thermal patterns and quantitative image analysis with abnormal thermographic asymmetry defined as a difference of >10% between limbs. RESULTS: In the 20 children enrolled, excellent agreement was found between the two methods with a Kappa value of 0.89. The median thermographic asymmetry in the nine children with pulse loss was 36 (13-76)%. Using receiver operating characteristic analysis, the asymmetrical pattern of ≥18% between limbs predicted the need for anticoagulation with a sensitivity of 100% and specificity of 89%. The area under the curve was 0.97 (95% confidence interval: 0.95-1). Children with absent pulse requiring anticoagulation showed a slower recovery in thermal asymmetry compared to those with a reduced pulse. By qualitative IRT assessment, all children with absent pulse requiring anticoagulation were correctly identified by 10 independent assessors. CONCLUSIONS: This pilot study showed that IRT is feasible and reliable as an adjunctive tool for thrombus detection postcatheterization and treatment monitoring. Specific advantages of IRT include portability, affordability, and contactless image acquisition.

Is there a role for endovascular stent implantation in the management of postoperative right ventricular outflow tract obstruction in the era of transcatheter valve implantation?

BACKGROUND: The optimal management pathway for the dysfunctional right ventricular outflow tract (RVOT) is uncertain. We evaluated the long-term outcomes and clinical impact of stent implantation for obstructed RVOTs in an era of rapidly progressing transcatheter pulmonary valve technology. METHODS: Retrospective review of 151 children with a biventricular repair who underwent stenting of obstructed RVOT between 1991 and 2017. RESULTS: RVOT stenting resulted in significant changes in peak right ventricle (RV)-to-pulmonary artery (PA) gradient (39.4 ± 17.1-14.9 ± 8.3; p < 0.001) and RV-to-aortic pressure ratio (0.78 ± 0.22-0.49 ± 0.13; p < 0.001). Subsequent percutaneous reinterventions in 51 children to palliate recurrent stenosis were similarly effective. Ninety-nine (66%) children reached the primary outcome of subsequent pulmonary valve replacement (PVR). Freedom from PVR from the time of stent implantation was 91%, 51%, and 23% at 1, 5, and 10 years, respectively. Small balloon diameters for stent deployment were associated with shorter freedom from PVR. When additional children without stent palliation (with RV-to-PA conduits) were added to the stent cohort (total 506 children), the multistate analysis showed the longest freedom from PVR in those with stent palliation and subsequent catheter reintervention. Pulmonary regurgitation was well-tolerated clinically. Indexed RV dimensions and function estimated by echocardiography remained stable at last follow up or before primary outcome. CONCLUSION: Prolongation of conduit longevity with stent implant remains an important strategy to allow for somatic growth to optimize the risk-benefit profile for subsequent surgical or transcatheter pulmonary valve replacement performed at an older age.

Mixed-reality view of cardiac specimens: a new approach to understanding complex intracardiac congenital lesions.

Digital reality is an emerging platform for three-dimensional representation of medical imaging data. In this technical innovation paper, the authors evaluated the accuracy and utility of mixed-reality technology in the morphological evaluation of complex congenital heart disease. The authors converted CT datasets of 12 heart specimens with different subtypes of double-outlet right ventricle to stereoscopic images and interrogated them using a mixed-reality system. The morphological features identified on the stereoscopic models were compared with findings at macroscopic examination of the actual heart specimens. The results showed that the mixed-reality system provided highly accurate stereoscopic display of spatially complex congenital cardiac lesions, with interactive features that might enhance 3-D understanding of morphology. Additionally, the authors found that high-resolution digital reproduction of cardiac specimens using clinical CT scanners is feasible for preservation and educational purposes.

An Overview of Contemporary Outcomes in Fetal Cardiac Intervention: A Case for High-Volume Superspecialization?

Fetal cardiac interventions (FCI) offer the opportunity to rescue a fetus at risk of intrauterine death, or more ambitiously to alter disease progression. Most of these fetuses require multiple additional postnatal procedures, and it is difficult to disentangle the effect of the fetal procedure from that of the postnatal management sequence. The true clinical impact of FCI may only be discernible in large-volume institutions that can commit to a standardized postnatal approach and have sufficient case volume to overcome their FCI learning curve.

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

BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Outcome after transcatheter occlusion of patent ductus arteriosus in infants less than 6 kg: A national study from United Kingdom and Ireland.

OBJECTIVES: This study aimed to report our national experience with transcatheter patent ductus arteriosus (PDA) occlusion in infants weighing <6 kg. BACKGROUND: The technique of transcatheter PDA closure has evolved in the past two decades and is increasingly used in smaller patients but data on safety and efficacy are limited. METHODS: Patients weighing < 6 kg in whom transcatheter PDA occlusion was attempted in 13 tertiary paediatric cardiology units in the United Kingdom and Ireland were retrospectively analyzed to review the outcome and complications. RESULTS: A total of 408 patients underwent attempted transcatheter PDA closure between January 2004 and December 2014. The mean weight at catheterization was 4.9 ± 1.0 kg and mean age was 5.7 ± 3.0 months. Successful device implantation was achieved in 374 (92%) patients without major complication and of these, complete occlusion was achieved in 356 (95%) patients at last available follow-up. Device embolization occurred in 20 cases (5%). The incidence of device related obstruction to the left pulmonary artery or aorta and access related peripheral vascular injury were low. There were no deaths related to the procedure. CONCLUSIONS: Transcatheter closure of PDA can be accomplished in selected infants weighing <6 kg despite the manufacturer's recommended weight limit of 6 kg for most ductal occluders. The embolization rate is higher than previously reported in larger patients. Retrievability of the occluder and duct morphology needs careful consideration before deciding whether surgical ligation or transcatheter therapy is the better treatment option.

Stent Therapy for Aortic Coarctation in Children <30 kg: Use of the Low Profile Valeo Stent.

To report our experience of coarctation stent therapy in small children weighing less than 30 kg, with the low profile dilatable Valeo stent and review the literature on coarctation stent therapy in this patient population. Coarctation stent implantation was undertaken in 14 consecutive children using the Bard Valeo Stent. Demographic, angiographic, echocardiographic and clinical data were reviewed retrospectively. The median age at the time of procedure was 5.1 (2.6-7.5) years and median weight was 20.8 (14.7-27) kg. There was improvement in median coarctation diameter from 4 (1.3-5.2) to 9.5 (5.8-12.7) mm, p < 0.001; and a reduction in the median peak pressure gradient across the coarctation from 35 (20-49) to 9 (0-15) mmHg, p < 0.001. Median stent recoil was 7.9 (0-20)%. There was one case of access related complication that resolved without sequelae. Follow-up was a median of 15 (3.0-57.2) months. CT angiogram performed at a median time of 3.3 (2.6-10.2) months post procedure showed no aortic wall injury and preserved stent integrity in all cases. Two children underwent re-intervention for stent dilation and further stent implantation due to in-stent stenosis and somatic growth after 3 years. Six of fourteen children remained on a single antihypertensive agent post-intervention at last follow-up. Implantation of the dilatable Valeo stent is a feasible treatment strategy in native or recurrent coarctation in small children, accepting that additional stent implantation may be necessary with somatic growth. Further study is required to determine longer-term stent efficacy and clinical outcome.

Clinical Characteristics and Outcomes of Cardiomyopathy in Barth Syndrome: The UK Experience.

Barth syndrome (BTHS) is an X-linked disorder characterised by cardiomyopathy, neutropenia, skeletal myopathy and growth delay. This study describes the UK national clinical experience and outcome of cardiomyopathy in BTHS. The clinical course and echocardiographic changes of all patients with BTHS in the UK were reviewed from 2004 to 2014. In addition, strain analysis using 2D speckle tracking echocardiography was performed to further assess left ventricular function in the most recent follow-up. At last follow-up, 22 of 27 patients were alive with a median age of 12.6 (2.0-23.8) years; seven underwent cardiac transplantation at a median age of 2 (0.33-3.6) years, and five died (18.5%) at a median age of 1.8 (0.02-4.22) years. All deaths were related to cardiomyopathy or its management. Left ventricular diastolic dimension and systolic function measured by fractional shortening tended to normalise and stabilise after the first 3 years of life in the majority of patients. However, patients with BTHS (n = 16) had statistically significant reduction in global longitudinal and circumferential strain compared to controls (n = 18), (p < 0.001), despite apparent normal conventional measures of function. There was also reduced or reversed apical rotation and reduced left ventricular twist. Sustained ventricular arrhythmia was not seen at follow-up. Cardiac phenotype in BTHS is variable; however, longer-term outcome in our cohort suggests good prognosis after the first 5 years of life. Most patients appeared to have recovered near normal cardiac function by conventional echocardiographic measures, but strain analysis showed abnormal myocardial deformation and rotational mechanics.

Longer-term outcome of perventricular device closure of muscular ventricular septal defects in children.

OBJECTIVE: To describe the longer-term clinical experience and follow-up with perventricular device closure of ventricular septal defects (VSD) in children. METHODS: Between January 2005 and December 2013, muscular ventricular septal defect closure with the Amplatzer Muscular VSD Occluder was undertaken using a hybrid perventricular approach. Data including demographic, echocardiographic parameter, and clinical outcome were reviewed retrospectively. RESULTS: Median age at the time of procedure was 8.9 months (range 1.9-31.0 months) and median weight was 6.6 kg (range 4.5-12.9 kg). All had a moderate to large muscular VSD, three had more than one VSD, four had previous coarctation repair, and five had previous pulmonary artery (PA) banding. A single Amplatzer muscular occluder (range 8-18 mm) was deployed in each patient without cardiopulmonary bypass under echocardiographic guidance. Two of ten patients subsequently required a short period of cardiopulmonary bypass for reconstruction of PA after de-banding and closure of atrial septal defect in one. Occluder removal was necessary in one patient due to entrapment of the tricuspid valve and progressive tricuspid regurgitation. This patient underwent surgical repair with a good result and no device-related valve damage. In the remaining nine patients, no severe complications such as device embolization, arrhythmia, or significant valve regurgitation were noted in the post-operative period or follow-up. At a median time of 6.5 years (range 0.9-8.4 years) post device implantation, complete closure was achieved in five patients and four had small residual leaks, which were not hemodynamically significant. CONCLUSION: Perventricular muscular VSD closure is effective in small children with suitable muscular defects and may avoid the morbidity associated with cardiopulmonary bypass and conventional surgical repair. There have been no late complications with this approach.

Foetal supraventricular tachycardia with hydrops fetalis: a role for direct intraperitoneal amiodarone.

INTRODUCTION: Persistent foetal tachyarrythmias complicated by hydrops fetalis carry a poor prognosis, with foetal death reported in excess of a quarter despite treatment. We present our experience with direct intraperitoneal amiodarone administration in eight hydropic foetuses with resistant supraventricular tachycardia. METHODS: Amiodarone was injected slowly into foetal peritoneal cavity under ultrasound guidance. All mothers were loaded with oral amiodarone before the procedure and maintained on it. The procedure was repeated guided by foetal rhythm. RESULT: All eight cases had severe hydrops with a median foetal heart rate of 255 bpm (range 240-300 bpm), and the median gestational age was 27+1 weeks (range 21-33+3 weeks) at presentation. In six cases, the average time for supraventricular tachycardia to revert to sinus rhythm from the first procedure was 11.5 days. In one case, intravascular injection of amiodarone into the umbilical vein was performed before intraperitoneal injection, which resulted in conversion to sinus rhythm sustained until delivery. In the last case, supraventricular tachycardia and severe hydrops persisted and the baby was delivered 5 days later at 34 weeks' gestation. Hydrops resolved in five foetuses with a mean resolution time of 28.4 days. The mean gestational age at delivery was 34+5 days and seven of eight cases survived beyond the neonatal period with good postnatal outcomes. CONCLUSION: Intraperitoneal administration of amiodarone is a relatively simple and effective strategy in refractory supraventricular tachycardia complicated by severe hydrops. The intraperitoneal route assures delivery of the drug to the severely hydropic foetus and enables a bolus dose to be delivered for sustained absorption.

Long-term clinical experience with Amplatzer Ductal Occluder II for closure of the persistent arterial duct in children.

OBJECTIVES: To describe the long-term clinical experience and follow-up with the Amplatzer Ductal Occluder II (ADO II) in children. METHODS: All patients undergoing attempted transcatheter closure of patent arterial duct (PDA) with the ADO II were included. Data collected included demographic, clinical, and echocardiographic parameters. RESULTS: From March 2008 until March 2013, 62 patients with a median age of 1.2 years (range 0.43-11.1 years) and median weight of 9 kg (range 4.7-31.4 kg) underwent the procedure. The median measurement for minimal ductal diameter was 2.7 mm (range 1.3-5 mm). An ADO II was implanted in 60 patients (96.8%). Two patients had significant residual shunting following deployment of the ADO II and underwent closure with the Amplatzer ductal occluder I (ADO I) during the same procedure. In six patients, the initial ADO II was unsatisfactory, and after recapture a different size ADO II was deployed. Device embolization of the ADO II to the pulmonary artery occurred in 6.7% of patients. Of these, one underwent surgical closure and three were closed with an ADO I. Complete occlusion on echocardiography was noted prior to discharge in 87.5% of the deployed occluders and 100% at first follow-up. Five year follow-up (n = 25) revealed a 100% occlusion rate. There were three cases of persistent mild left pulmonary artery stenosis at long-term follow-up. CONCLUSIONS: The ADO II is effective for occlusion of PDA with variable anatomy from either arterial or venous approaches with a low profile delivery system. Stable occluder position is highly dependent on accurate device sizing, good quality imaging to visualize device configuration after deployment and operator experience.

Interrupted aortic arch with retrograde subclavian perfusion.

Type B interrupted aortic arch results in the left subclavian artery being perfused by a patent ductus arteriosus and antegrade flow is usually seen in the left subclavian artery. We describe a case of retrograde perfusion of the left subclavian artery through the circle of Willis in the presence of interrupted aortic arch type B and a restrictive patent ductus arteriosus. This is similar to subclavian steal syndrome when the perfusion pressure from the cerebral circuit is greater than that from the left subclavian artery. Early recognition of this phenomenon may alter management by indicating restriction at ductal level. This case highlights the need for a comprehensive review of both ventricular systolic function and ductal haemodynamics when retrograde flow is seen in the subclavian artery.

Outcome of patent ductus arteriosus ligation in premature infants in the East of England: a prospective cohort study.

BACKGROUND: Surgical ligation of patent ductus arteriosus is considered when medical treatment fails or is contraindicated. This study aims to determine the mortality and morbidity of preterm neonates referred for patent ductus arteriosus ligation. METHODS: A prospective study was conducted in the East of England to follow the outcome of premature infants under 37 weeks' gestation undergoing patent ductus arteriosus ligation. A standardised proforma was used to collect information before and after the procedure. RESULTS: A total of 102 premature infants were recruited, and patent ductus arteriosus ligation was performed in 92. Surgical complications occurred in 8.7% (8/92), which included pneumothorax (5/8), recurrent laryngeal nerve palsy (2/8), and chylothorax (1/8). Morbidity outcome data were not available for all infants. The incidence of chronic lung disease was 88% (88/99); intraventricular haemorrhage was 49% (49/100); necrotising enterocolitis 39% (39/99), and retinopathy of prematurity 42% (41/97). The overall mortality rate in our study was 7.8% (8/102). Mortality rate in infants who had patent ductus arteriosus ligation was 4.3% (4/92). The 30-day survival rate after ligation was 99% (91/92). Beyond 30 days post-ligation, three infants died from other causes that were not directly related to surgery. CONCLUSION: Patent ductus arteriosus ligation in premature infants is associated with low mortality and complication rates; however, there is a high incidence of neonatal morbidity. Surgical capacity for patent ductus arteriosus ligation needs to be carefully planned nationally as the duration of ''waiting time'' and transport to another surgical centre could adversely affect outcomes in this high-risk population.

Mortality among infants born with orofacial clefts in a single cleft network.

OBJECTIVE: To determine mortality rate and cause of death in infants born with orofacial clefts. METHODS: Retrospective case note review of all children with orofacial clefts within the East of England Cleft Network who have died. RESULTS: Between 2002 and 2010, of 638 children born with orofacial clefts, 23 died at a median age of 5 months (range, 1 day to 4 years). The overall mortality rate was 36 per 1000 cleft births. A total of 21 deaths were in the isolated cleft palate group, with a calculated death rate of 68.1 per 1000. One child each from the cleft lip and the cleft lip and palate groups died. Of the children who died, 21 (91%) had other structural abnormalities. Of these children, 19 (83%) died before their first birthday. The causes of death were mainly due to associated congenital anomalies (61%) and infection (17%). CONCLUSIONS: Children born with cleft palate have a 15-fold increase in mortality compared with the regional East of England infant mortality rate and at least a 10-fold increase when compared with other clefts. Pediatricians should be involved in the care of these complicated patients.

Case report of a left superior vena cava to left atrial connection treated with percutaneous covered stent placement.

Background: Persistent left superior vena cava (LSVC) with connection to the left atrium (LA) is a rare anomaly with serious clinical implications. Depending on the direction of flow through the intracardiac shunt, clinical presentation varies from cyanosis or paradoxical embolism to overt right heart failure. Case summary: A 26-year-old man with repaired ventricular septal defect (VSD) during infancy presented with symptoms of progressive exercise intolerance. Cardiac imaging revealed a large defect at the posterior wall of the LSVC above its entry to the coronary sinus (CS), connecting the LSVC with the LA and resulting in diversion of pulmonary venous return to the CS. All pulmonary veins connect normally to the LA. The large left-to-right intracardiac shunt led to significant right ventricular dilation and tricuspid regurgitation. He underwent successful anatomical repair with transcatheter implantation of covered stent from LSVC to the CS, redirecting pulmonary venous return to the LA. At 1 year follow up, his exercise capacity had improved, and cardiac imaging showed complete seal of the LSVC defect without obstruction to pulmonary venous return. Discussion: Our case is the first to our knowledge to report this unusual anatomic variant of LSVC to LA connection, and complete repair by transcatheter treatment. Previous case reports of other forms of LSVC to LA connection were treated with surgery or device occlusion without reconnection of LSVC. This case highlights the efficacy and safety of innovative percutaneous techniques in the management of complex congenital heart lesions. Meticulous procedural planning through 3D modelling and simulation is vital to mitigate the risks of these innovative procedures.

Renaissance of Cardiac Imaging to Assist Percutaneous Interventions in Congenital Heart Diseases:The Role of Three-Dimensional Echocardiography and Multimodality Imaging.

Percutaneous interventions have completely refashioned the management of children with congenital heart diseases (CHD) and the use of non-invasive imaging has become the gold standard to plan and guide these procedures in the modern era. We are now facing a dual challenge to improve the standard of care in low-risk patients, and to shift our strategies from the classic open chest surgery to imaging-guided percutaneous interventions in high-risk patients. Such rapid evolution of ultrasound technologies over the last 20 years have permitted the integration of transthoracic, transesophageal and intracardiac echocardiography into the interventional workflow to improve image guidance and reduce radiation burden from fluoroscopy and angiography. Specifically, miniaturization of transesophageal probe and advances in three-dimensional (3D) imaging techniques have enabled real-time 3D image guidance during complex interventional procedure, In addition, multimodality and fusion imaging techniques harness the strengths of different modalities to enhance understanding of anatomical and spatial relationship between different structures, improving communication and coordination between interventionalists and imaging specialists. In this review, we aim to provide an overview of 3D imaging modalities and multimodal fusion in procedural planning and live guidance of percutaneous interventions. At the present times, 3D imaging can no longer be considered a luxury but a routine clinical tool to improve procedural success and patient outcomes.

Long term outcomes in CDH: Cardiopulmonary outcomes and health related quality of life.

BACKGROUND: With improvements in clinical management and an increase in CDH survivorship there is a crucial need for better understanding of long-term health outcomes in CDH. AIM: To investigate the prevalence of cardiopulmonary health morbidity and health related quality of life (HRQoL) in CDH survivors. METHODS: We included all studies (n = 65) investigating long-term cardiopulmonary outcomes in CDH patients more than 2 years published in the last 30 years. The Newcastle-Ottawa Scale and the CASP checklist for cohort studies were utilized to assess study quality. Results were reported descriptively and collated by age group where possible. RESULTS: The incidence of pulmonary hypertension was highly variable (4.5-38%), though rates (%) appeared to diminish after 5 years of age. Lung function indices and radiological outcomes were frequently abnormal, and Health Related Quality of Life (HRQoL) reduced also. Long term diseases notably emphysema and COPD are not yet fully described in the contemporary literature. CONCLUSION: This study underscores cardiopulmonary health morbidity and a reduced HRQoL among CDH survivors. Where not already available dedicated multidisciplinary follow-up clinics should be established to support these vulnerable patients transition safely into adulthood. Future research is therefore needed to investigate the risk factors for cardiopulmonary ill health and morbidity in CDH survivors. TYPE OF STUDY: Systematic review of case control and cohort studies.

Biventricular Repair in Borderline Left Hearts: Insights From Cardiac Magnetic Resonance Imaging.

Background: Cardiac magnetic resonance imaging (CMR) may augment 2-dimensional (2D) echocardiography in decision-making for biventricular repair in borderline hypoplastic left hearts. Objectives: This study evaluates: 1) the relationship between 2D echocardiography and CMR; 2) imaging variables affecting assignment to biventricular vs non-biventricular management; and 3) variables affecting transplant-free biventricular survival. Methods: We reviewed clinical, echocardiographic, and CMR data in 67 infants, including CMR-determined ascending aortic (AAo) flow and comparable left ventricular end-diastolic volume indexed (LVEDVi) by 2D-echocardiography and CMR. Results: Treatment assignment to biventricular repair was either direct (BV, n = 45) or with a bridging hybrid procedure (H1-BV, n = 12). Echocardiographic LVEDVi was <20 mL/m2 in 83% of biventricular repair infants and underestimated CMR-LVEDVi by 16.8 mL/m2. AAo flows had no/weak correlation with aortic and mitral valve z-scores or LVEDVi. AAo flows differed between BV, H1-BV, and single-ventricle groups (median): 2.1, 1.7, and 0.7 L/min/m2, respectively. Important variables for treatment assignment were presence of endocardial fibroelastosis, AAo flow, and mitral valve z-score. Biventricular repair was achieved in 54. The median follow-up was 8.0 (0.1-16.4) years. Transplant-free biventricular survival was 96%, 82%, and 77% at 1, 5, and 10 years, respectively. Patients without aortic coarctation repair were at higher risk of death, transplantation, or single-ventricle conversion (HR: 54.3; 95% CI: 6.3-47.1; P < 0.001) during follow-up. AAo flow had a smaller nonlinear effect with hazard ratio increasing at lower flows. Conclusions: Historical 2D echocardiographic criteria would have precluded many patients from successful biventricular repair. AAo flow, an integrative index of left heart performance, was important in assigning patients to a biventricular circulation and affected survival. Biventricular survival was strongly associated with the need for aortic coarctation repair.

Transcatheter creation of a Potts shunt with the Occlutech Atrial Flow Regulator: Feasibility in a pig model.

BACKGROUND: Creation of a Potts shunt, a connection between the left pulmonary artery (LPA) and descending aorta (DAo), improves functional status and survival in drug-refractory suprasystemic pulmonary arterial hypertension. We investigated a new approach to transcatheter Potts shunt creation in pigs. METHODS AND RESULTS: In six pigs, a steerable SureFlex sheath was used to optimize the trajectory of perforation from the DAo into LPA using a 0.035″ radiofrequency wire. The combination of a larger perforation, stiffer radiofrequency wire and smooth dilator-to-sheath transition allowed sheath entry into the LPA without requiring an arterio-venous wire circuit. The Occlutech Atrial Flow Regulator (AFR), a double-disc device with a central fenestration, was deployed through this sheath with apposition of the distal disc to the posterior LPA wall and the proximal disc to the anterior DAo wall. The AFR is compliant and crumpling of the central fenestration was resolved by balloon dilation. It was feasible to implant a stent within the fenestration (n = 3). Aortography confirmed a left-to-right shunt through the AFR without contrast extravasation. Autopsy demonstrated anchoring of both discs against the vessel walls, patency of the fenestration and secure placement of the stent with no intra-thoracic bleeding. CONCLUSIONS: In an acute pig model, we have demonstrated the feasibility of creating a transcatheter Potts shunt with a simplified technique using a steerable sheath, a double-disc device with a central fenestration that acts as the shunt channel and optional stenting of the fenestration.

Balloon Angioplasty for Native Aortic Coarctation in 3- to 12-Month-Old Infants.

BACKGROUND: Balloon angioplasty for native coarctation of the aorta (CoA) is successful in children and adults but in neonates results in frequent restenosis. The efficacy of balloon angioplasty for native CoA during infancy beyond the neonatal period was examined in infants aged 3 to 12 months of age. METHODS: A retrospective review of 68 infants who underwent balloon angioplasty for native CoA. 95% CI are in parentheses. RESULTS: Procedural age was (mean±SD) 6±3.4 months and weight was 7±1.8 kg. Balloon angioplasty produced a large decrease in both the noninvasive arm-to-leg blood pressure gradient (41.2±18.7 to 5.6±9.6 mm Hg) and the invasive peak systolic pressure gradient (34±12 to 11±9 mm Hg). Balloon angioplasty increased the CoA diameter from 2.7±1 mm to 4.6±1.2 mm. One patient was lost to follow-up. A catheter reintervention was required in 11.8% and surgery in 10.3%. The hazard of reintervention was highest early. Median freedom from reintervention was 89% (95% CI, 80%-96%) at 1 year, 83% (95% CI, 73%-92%) at 5 years, and 81% (95% CI, 69%-90%) at 10 years. Femoral artery thrombosis was documented in 6 (9%) infants without any long-term consequence. One patient developed a small aortic aneurysm late and has not required treatment. A robust estimate of the frequency of aortic aneurysms remains to be determined as the majority of subjects have not had cross-sectional imaging. CONCLUSIONS: Balloon angioplasty of native CoA is effective and safe in infants aged 3 to 12 months with outcomes comparable to those in older children and adults. Catheter reinterventions can avoid the need for surgery in most patients.