Systemic Central Venous Rehabilitation in Congenital Heart Disease.

Chronic venous obstructions resulting from indwelling lines, surgery and instrumentation, and congenital anomalies are increasingly common in patients with congenital heart disease (CHD) and other chronic illnesses. Venous obstruction results in threatened long-term vascular access and congestive symptoms. Endovascular therapies are safe and can be effective at rehabilitating obstructed and even occluded veins. The risk of recurrent obstruction is high, however. Post-rehabilitation monitoring and anticoagulation therapy are important, and reinterventions are common. Here, the authors describe techniques to address a variety of venous obstruction lesions that may be encountered in CHD patients and provide illustrative cases.

Use of Flow Restrictors in Congenital Heart Disease.

The surgical pulmonary artery band was first introduced in 1952 and, to this day, can produce challenges in regard to the ideal amount of restriction and the need for reoperations. A transcatheter option may be the ideal solution as it allows for a less-invasive approach for a better hemodynamic assessment and easier re-intervention. To date, multiple approaches have been developed with device modifications to create restrictions to flow, each with advantages and limitations. Continued experience is still necessary to determine the ideal device to use to create an adequate and modifiable level of restriction.

PDA Stent Placement *6: High-Impact Limitations of Angiography to Delineate a Complex PDA.

This is a case of an infant with duct-dependent pulmonary circulation, who required 6 stents delivered over three procedures to fully stent the arterial duct, which originated in a very unusual fashion. The attainable angiographic projections were unable to profile its origin, and only a CT scan was ultimately able to delineate the (stenotic) ductal origin from the aorta.

Hot topics in interventional cardiology: Proceedings from the society for cardiovascular angiography and interventions (SCAI) 2021 think tank.

The Society for Cardiovascular Angiography and Interventions (SCAI) Think Tank is a collaborative venture that brings together interventional cardiologists, administrative partners, and select members of the cardiovascular industry community annually for high-level field-wide discussions. The 2021 Think Tank was organized into four parallel sessions reflective of the field of interventional cardiology: (a) coronary intervention, (b) endovascular medicine, (c) structural heart disease, and (d) congenital heart disease. Each session was moderated by a senior content expert and co-moderated by a member of SCAI's Emerging Leader Mentorship program. This document presents the proceedings to the wider cardiovascular community in order to enhance participation in this discussion, create additional dialog from a broader base, and thereby aid SCAI, the industry community and external stakeholders in developing specific action items to move these areas forward.

Total anomalous origin of the coronary arteries from the pulmonary artery: a systematic review.

BACKGROUND: Although rare, coronary artery anomalies can have significant clinical implications. Total anomalous origin of the coronary arteries from the pulmonary artery (TCAPA) represents a rare subtype of coronary artery anomaly for which little is known. The aim of this review was to characterise the presentation, utilised diagnostic modalities, associated cardiac lesions, and treatment strategies in patients with TCAPA. METHODS: A systematic review was performed for cases of TCAPA using PubMed, Embase, and Web of Science. Keywords searched included "total anomalous origin of the coronary arteries from the pulmonary artery," "single ostium anomalous coronary artery from the pulmonary artery," and "anomalous origin of both coronary arteries from the pulmonary artery." RESULTS: Fifty-seven cases of TCAPA were identified in 50 manuscripts. Fifty-eight per cent of patients were male and the median age at presentation was 10 days (mean 1.71 ± 6.6 years, range 0 days-39 years). Most patients were symptomatic at the time of presentation; cyanosis (n = 22) and respiratory distress (n = 14) were the most common symptoms. Cases were most commonly diagnosed at autopsy (n = 26, 45.6%), but operative intervention was pursued in 22 cases (45.6%); aortic re-implantation (n = 14) and a Takeuchi-type repair (n = 7) were the most common routes of repair. CONCLUSIONS: The clinical presentation of patients with TCAPA was found to be variable, likely related to the presence of associated cardiac lesions. TCAPA should be considered in patients with suspected anomalous origin of the left coronary artery from the pulmonary artery for the serious consequences that can occur if not promptly corrected.

Critical Congenital Heart Disease Detection in the Screening Era: Do Not Neglect the Examination!

Pulse oximetry oxygen saturation (SpO 2 )-based critical congenital heart disease (CCHD) screening is effective in detection of cyanotic heart lesions. We report a full-term male infant with normal perfusion who had passed the CCHD screening at approximately 24 hours after birth with preductal SpO 2 of 99% and postductal SpO 2 of 97%. Detection of a loud systolic cardiac murmur before discharge led to the diagnosis of pulmonary atresia (PA) with ventricular septal defect (PA-VSD) by echocardiogram. The infant was transferred to a tertiary care center after initiation of prostaglandin E1 (PGE1) therapy. Throughout the initial course, he was breathing comfortably without respiratory distress or desaturations on pulse oximetry. We believe that this is the first documented report of PA missed by CCHD screening. Thorough and serial clinical examinations of the newborn infant proved vital in the timely diagnosis of this critical disease. We review the hemodynamics and the recent literature evaluating utility of CCHD screening in the diagnosis of PA-VSD. Pulse oximetry-based CCHD screening should be considered a tool to enhance CCHD detection with an emphasis on detailed serial physical examinations in newborn infants.

Resource Allocation and Decision Making for Pediatric and Congenital Cardiac Catheterization During the Novel Coronavirus SARS-CoV-2 (COVID-19) Pandemic: A U.S. Multi-Institutional Perspective.

BACKGROUND: The novel coronavirus (COVID-19) pandemic has placed severe stress on healthcare systems around the world. There is limited information on current practices in pediatric cardiac catheterization laboratories in the United States (US). OBJECTIVES: To describe current practice patterns and make recommendations regarding potential resource allocation for congenital cardiac catheterization during the COVID-19 pandemic. METHODS: A web-based survey was distributed regarding case candidacy and catheterization laboratory preparedness. Centers were categorized based on the current degree of disease burden in that community (as of April 1, 2020). Data and consensus opinion were utilized to develop recommendations. RESULTS: Respondents belonged to 56 unique US centers, with 27 (48.2%) located in counties with a high number of COVID-19 cases. All centers have canceled elective procedures. There was relative uniformity (>88% agreement) among centers as to which procedures were considered elective. To date, only three centers have performed a catheterization on a confirmed COVID-19 positive patient. Centers located in areas with a higher number of COVID-9 cases have been more involved in a simulation of donning and doffing personal protective equipment (PPE) than low-prevalence centers (46.7% vs 10.3%, respectively; P<.001). Currently, only a small fraction of operators has been reassigned to provide clinical services outside their scope of practice. CONCLUSIONS: At this stage in the COVID-19 pandemic, pediatric/congenital catheterization laboratories have dramatically reduced case volumes. This document serves to define current patterns and provides guidance and recommendations on the preservation and repurposing of resources to help pediatric cardiac programs develop strategies for patient care during this unprecedented crisis.

Procedural, pregnancy, and short-term outcomes after fetal aortic valvuloplasty.

OBJECTIVES: We aimed to evaluate the effect of technical aspects of fetal aortic valvuloplasty (FAV) on procedural risks and pregnancy outcomes. BACKGROUND: FAV is performed in cases of severe mid-gestation aortic stenosis with the goal of preventing hypoplastic left heart syndrome (HLHS). METHODS: The International Fetal Cardiac Intervention Registry was queried for fetuses who underwent FAV from 2002 to 2018, excluding one high-volume center. RESULTS: The 108 fetuses had an attempted cardiac puncture (mean gestational age [GA] 26.1 ± 3.3 weeks). 83.3% of attempted interventions were technically successful (increased forward flow/new aortic insufficiency). The interventional cannula was larger than 19 g in 70.4%. More than one cardiac puncture was performed in 25.0%. Intraprocedural complications occurred in 48.1%, including bradycardia (34.1%), pericardial (22.2%) or pleural effusion (2.7%) requiring drainage, and balloon rupture (5.6%). Death within 48 hr occurred in 16.7% of fetuses. Of the 81 patients born alive, 59 were discharged home, 34 of whom had biventricular circulation. More than one cardiac puncture was associated with higher complication rates (p < .001). Larger cannula size was associated with higher pericardial effusion rates (p = .044). On multivariate analysis, technical success (odds ratio [OR] = 10.9, 95% confidence interval [CI] = 2.2-53.5, p = .003) and later GA at intervention (OR = 1.5, 95% CI = 1.2-1.9, p = .002) were associated with increased odds of live birth. CONCLUSIONS: FAV is an often successful but high-risk procedure. Multiple cardiac punctures are associated with increased complication and fetal mortality rates. Later GA at intervention and technical success were independently associated with increased odds of live birth. However, performing the procedure later in gestation may miss the window to prevent progression to HLHS.

Stent treatment of ostial branch pulmonary artery stenosis: initial and medium-term outcomes and technical considerations to avoid and minimise stent malposition.

OBJECTIVE: Stenting of ostial pulmonary artery stenosis presents several unique challenges. These include difficulty in defining anatomy and need for precise stent placement in order to avoid missing the ostial stenosis or jailing either the contralateral branch pulmonary artery or the ipsilateral upper lobe branch. DESIGN: A retrospective review of outcomes was conducted in 1.5 or 2-ventricle patients who underwent stent placement for ostial branch pulmonary artery stenosis. Specific catheterisation lab techniques were reviewed. RESULTS: Forty-seven branch pulmonary arteries underwent stent placement for ostial stenosis in 43 patients. The median age and weight were 3.7 (0.3-18.1) years and 14.2 (5.6-70.0) kg, respectively. Three (2-8) angiographic projections were needed to profile the ostial stenosis. Open-cell stents were used in 23 and stents were modified in 5 cases. Following stent implantation, the minimum diameter improved from 3.6 (0.8-10.5) to 8.1 (4.2-16.5) mm (p < 0.001). The gradient improved from 21 (0-66) to 4 (0-27) mmHg (p < 0.001). Stent malposition occurred in eight (17%) of the stents placed. Five migrated distally causing suboptimal ostial coverage necessitating placement of a second stent in four. Three migrated proximally and partially jailed the contralateral pulmonary artery. Intentional jailing of the upper lobe branch occurred in four additional cases. At a follow-up of 2.4 (0.3-4.9) years, 15 stents underwent further dilation and 1 had a second stent placed within the exiting stent. CONCLUSION: Ostial branch pulmonary artery stenosis may require additional angiography to accurately define the ostial stenosis. Treatment with stents is effective but carries high rates of stent malposition.

Hybrid approach to ventricular septal defect enlargement.

The need for creation or enlargement of a ventricular septal defect (VSD) is a rare occurrence. It is most frequently required in patients with double-outlet right ventricle who develop restriction of a remote muscular VSD or obstruction of a perimembranous VSD secondary to atrioventricular (AV) valve attachments to the interventricular septum. Surgical and transcatheter options for VSD creation or enlargement are associated with several risks including heart block, AV valve injury, and perforation. We report the first description of a hybrid approach to VSD creation and enlargement in two patients.

Further percutaneous dilation of a Melody® valve in the mitral position to accommodate somatic growth in a small child: lessons learned.

A 15-month-old child underwent percutaneous expansion of a Melody transcatheter pulmonary valve in the mitral position to accommodate growth after initial surgical implantation during infancy, but transiently decompensated after valvuloplasty owing to stent malformation. The Melody valve in the mitral position of small patients can be further expanded by percutaneous dilation, but there are a number of potential complications and technical improvements to consider.

Recanalization of Occluded Central Veins in a Parenteral Nutrition-Dependent Child With No Access.

Central venous thromboses are common and pose challenges in the care of chronically ill pediatric patients. Among patients with intestinal failure (most commonly because of short bowel syndrome) who depend on parenteral nutrition, progressive loss of central venous access sites is a potentially fatal complication. We present the case of a 5-year-old girl with parenteral nutrition-dependent short bowel syndrome and no remaining standard central venous access sites despite medical anticoagulation, in whom angioplasty and stent implantations were used to reconstruct chronically occluded central veins. The patient presented with a bloodstream infection necessitating tunneled central venous line removal from the left internal jugular vein. All other standard access sites had known occlusions. The right iliofemoral vein (RIFV) and infrarenal inferior vena cava were recanalized and dilated with high-pressure balloons. The left internal jugular line was removed and a line was placed in the now-patent RIFV for antimicrobial therapy. After treatment, the RIFV line was removed and the vessels were stented open for future access. The occluded left innominate vein was recanalized and dilated to allow a new tunneled line to be placed. At 10 months, the line was functional and uninfected and the RIFV and inferior vena cava stents were patent without in-stent restenosis. We propose a new paradigm that uses these techniques to prevent access site exhaustion in patients who do not respond to anticoagulation therapy. This approach may reduce morbidity and mortality in patients with chronic access needs and the need for intestinal transplantation in patients with intestinal failure.

Unmasking the borderline coarctation: the utility of isoproterenol in the paediatric cardiac catheterisation laboratory.

BACKGROUND: One indication for intervention in coarctation of the aorta is a peak-to-peak gradient >20 mmHg. Gradients may be masked in patients under general anaesthesia and may be higher during exercise. Isoproterenol was given during cardiac catheterisation to simulate a more active physiologic state. OBJECTIVES: We aimed to describe the haemodynamic effects of isoproterenol in patients with coarctation and the impact of intervention on the elicited gradients. METHODS: A retrospective study was performed on two-ventricle patients who underwent cardiac catheterisation for coarctation with isoproterenol testing. RESULTS: 25 patients received isoproterenol before and after intervention. With isoproterenol, the mean diastolic (p=0.0015) and mean arterial (p=0.0065) blood pressures proximal to the coarctation decreased significantly. The mean systolic, diastolic, and mean arterial blood pressures distal to the coarctation decreased significantly (p20 mmHg. Post intervention, the median gradient decreased to 2 (0-29) mmHg, versus baseline, p=0.005, and with isoproterenol it decreased to 8 (0-27) mmHg, versus pre-intervention isoproterenol, p<0.0001. There were significant improvements in the gradients by Doppler (<0.0001) and by blood pressure cuff (p=0.0313). The gradients on isoproterenol best correlated with gradients by blood pressure cuff in the awake state (R2=0.76, p<0.0001). CONCLUSIONS: Isoproterenol can be a useful tool to assess the significance of a coarctation and the effectiveness of an intervention. Percutaneous interventions can effectively reduce the gradients elicited by isoproterenol.

Outcomes of Transcatheter Tricuspid Valve-in-Valve Implantation in Patients With Ebstein Anomaly.

We sought to describe the acute results and short- to medium-term durability of transcatheter tricuspid valve-in-valve (TVIV) implantation within surgical bioprostheses among patients with Ebstein anomaly (EA). Cases were identified from a voluntary, multicenter, international registry of 29 institutions that perform TVIV. Demographic, clinical, procedural, and follow-up data were analyzed. Eighty-one patients with EA underwent TVIV from 2008 to 2016. Thirty-four patients (42%) were New York Heart Association (NYHA) class 3/4 at time of TVIV. The most common indication for TVIV was the presence of moderate or severe tricuspid regurgitation (40%). Most patients received a Melody valve (64%). TVIV was ultimately successful in all patients, and there was no procedural mortality. Four patients (5%) developed acute valve thrombosis, 4 patients (5%) developed endocarditis, and 9 patients (11%) developed valve dysfunction not related to thrombosis or endocarditis. Eight patients (10%) underwent reintervention (2 transcatheter, 6 surgical) due to thrombosis (3), endocarditis (2), other valve dysfunction (2), and patient-prosthesis mismatch without valve dysfunction (1). Among 69 patients who were alive without reintervention at latest follow-up, 96% of those with NYHA status reported were class 1/2, a significant improvement from baseline (62% NYHA class 1/2, p <0.001). In conclusion, transcatheter TVIV offers a low-risk, minimally invasive alternative to surgical tricuspid valve re-replacement in patients with EA and a failing tricuspid valve bioprosthesis.

Predictive Factors for Patients Undergoing ASD Device Occlusion Who "Crossover" to Surgery.

The aim of this study was to define characteristics of those patients who are referred for device closure of an Atrial septal defect (ASD), but identified to "crossover" surgery. All patients who underwent surgical and device (Amplatzer or Helex occluder) closures of secundum ASDs from 2001 to 2010 were reviewed and organized into three groups: surgical closure, device closure, and "crossover" group. 369 patients underwent ASD closure (265 device, 104 surgical). 42 of the 265 patients referred for device closure "crossed over" to the surgical group at various stages of the catheterization procedure. The device group had defect size measuring 14.2 mm (mean) and an ASD index (Defect Size (mm)/BSA) of 14.0 compared to the corresponding values in the surgical group (20.1 mm, ASD index 25.9) (P < 0.001) and in the "crossover" group (20.7 mm, 22.6 ASD index) (P < 0.001). 79 patients in the device group had a deficient rim, and 86% were located in the retroaortic region. 33 patients in the "crossover" group had deficient rims with 70% deficiency in the posterior/inferior rim. The device group with deficient rims had an ASD index of 14.7 compared with the crossover group ASD index of 23.8 (P < 0.001). Comparing the device and "crossover" groups, an ASD index greater than 23.7 had a 90% specificity in "crossing over" to surgery. The crossover and surgical groups had statistically larger ASD defect size indexes compared with the device group. Deficient rim in the posterior/inferior rim is associated with a large ASD size index which is a predictive factor for crossing over to surgery. Catheterization did not negatively impact surgical results in the "crossover" group.