Impact of Social Determinants of Health on Outcomes for Patients with Adult Congenital Heart Disease.

PURPOSE OF REVIEW: The study of adults with congenital heart disease (ACHD) is a rapidly growing field; however, more research is needed on the disparities affecting outcomes. With advances in medicine, a high percentage of patients with congenital heart disease (CHD) are advancing to adulthood, leading to an increase in the number of ACHD. This creates a pressing need to evaluate the factors, specifically the social determinants of health (SDOH) contributing to the outcomes for ACHD. RECENT FINDINGS: A myriad of factors, including, but not limited to, race, education, and socioeconomic status, have been shown to affect ACHD outcomes. Existing data from hospitalizations, mortality and morbidity, advanced care planning, patient and physician awareness, financial factors, and education alongside race and socioeconomic status present differences in ACHD outcomes. With SDOH having a significant impact on ACHD subspecialty care outcomes, ACHD centers need to be constantly adapting and innovating, incorporating SDOH into patient management, and providing additional healthcare resources to manage the care of ACHD.

Functionalization of endovascular devices with superparamagnetic iron oxide nanoparticles for interventional cardiovascular magnetic resonance imaging.

Presently, cardiovascular interventions such as stent deployment and balloon angioplasty are performed under x-ray guidance. However, x-ray fluoroscopy has poor soft tissue contrast and is limited by imaging in a single plane, resulting in imprecise navigation of endovascular instruments. Moreover, x-ray fluoroscopy exposes patients to ionizing radiation and iodinated contrast agents. Magnetic resonance imaging (MRI) is a safe and enabling modality for cardiovascular interventions. Interventional cardiovascular MR (iCMR) is a promising approach that is in stark contrast with x-ray fluoroscopy, offering high-resolution anatomic and physiologic information and imaging in multiple planes for enhanced navigational accuracy of catheter-based devices, all in an environment free of radiation and its deleterious effects. While iCMR has immense potential, its translation into the clinical arena is hindered by the limited availability of MRI-visible catheters, wire guides, angioplasty balloons, and stents. Herein, we aimed to create application-specific, devices suitable for iCMR, and demonstrate the potential of iCMR by performing cardiovascular catheterization procedures using these devices. Tools, including catheters, wire guides, stents, and angioplasty balloons, for endovascular interventions were functionalized with a polymer coating consisting of poly(lactide-co-glycolide) (PLGA) and superparamagnetic iron oxide (SPIO) nanoparticles, followed by endovascular deployment in the pig. Findings from this study highlight the ability to image and properly navigate SPIO-functionalized devices, enabling interventions such as successful stent deployment under MRI guidance. This study demonstrates proof-of-concept for rapid prototyping of iCMR-specific endovascular interventional devices that can take advantage of the capabilities of iCMR.

Intraoperative cone beam computed tomography-guided endovascular recanalization of an interrupted aorta.

A 42-year-old man sustained a stroke secondary to malignant hypertension and was found to have complete aortic interruption. We report a case of real-time image-guided endovascular repair to highlight the value of preprocedural planning and intraprocedural cone beam computed tomography. Two-dimensional fluoroscopy enhanced with three-dimensional landmarks from cone beam computed tomography was used to direct a Nykanen radiofrequency wire (Baylis Medical, Montreal, Quebec, Canada) through the interruption, avoiding critical adjacent structures. A covered Cheatham-Platinum stent (NuMED, Inc, Hopkinton, NY) was deployed successfully, recanalizing the thoracic aorta. The pressure gradient normalized, and the patient was discharged on postoperative day 1. At 10-month follow-up, the patient was on an antihypertensive regimen of two minimum-dose drugs.

Transcatheter closure of a sinus venosus atrial septal defect using 3D printing and image fusion guidance.

A 63-year-old man with cirrhosis, hepatocellular carcinoma, and coagulopathy was diagnosed with a sinus venosus atrial septal defect (ASD) and partial anomalous pulmonary venous return (PAPVR) of the right upper pulmonary vein (RUPV). Transcatheter repair by positioning a stent graft in the superior vena cava was planned. Based on three-dimensional (3D) reconstruction of gated cardiac CTA, a 28 mm × 7 cm Endurant II® aortic extension stent graft (Medtronic, MN) was chosen. A 3D model printed from the CTA was used to simulate device deployment, demonstrating successful exclusion of the sinus venosus ASD with return of the RUPV to the left atrium (LA). Post simulation, the 3D model was used for informed consent. The patient was then taken to the hybrid operating room. On-table cone beam CT was performed and registered with the CTA images. This enabled overlay of 3D regions of interest to live 2D fluoroscopy. The stent graft was then deployed using 3D regions of interest for guidance. Hemodynamics and angiography demonstrated successful exclusion of the sinus venosus ASD and unobstructed return of RUPV to the LA. This is the first report of comprehensive use of contemporary imaging for planning, simulation, patient consent, and procedural guidance for patient-centered complex structural intervention in repair of sinus venosus ASD with PAPVR. We propose this as a process model for continued innovation in structural interventions.

Building an Adult Congenital Heart Program: Critical Components and Important Allies.

PURPOSE OF THE REVIEW: The purpose of this review is to illustrate specific challenges and opportunities in the building of an adult congenital heart disease (ACHD) program and to highlight critical components and important allies. RECENT FINDINGS: With more than 1.4 million adults with congenital heart disease in the USA alone, access to specialized, compassionate, high-quality comprehensive care requires a shift toward more aggressive expansion of ACHD care, especially in the context of sparse ACHD provider representation in the vast majority of adult medical centers. The effective build of an ACHD program requires measured escalation in management of ACHD complexity matched with cultivation of key resources and clinical services ranging from congenital cardiac surgery and interventional cardiology to acquired heart disease as well as partnerships with non-cardiac specialists. By reframing ACHD care as a shared goal between patients, providers, hospitals, pharmaceutical and device industry, and payers, a potent business model can be built around the developing ACHD program to facilitate acquisition of these key resources.

Adult congenital heart disease: magnitude of the problem.

PURPOSE OF REVIEW: To define the magnitude of problems faced by patients with adult congenital heart disease (ACHD) and to identify unmet needs for this population. RECENT FINDINGS: The ACHD population is estimated to include more than 1 million people in the United States and continues to grow at a steady rate. Owing to the decline in early mortality in this group, modern medicine is now faced by the long-term complications associated with congenital heart disease such as chronic heart failure, increased endocarditis risk, elevated burden of arrhythmias, pulmonary hypertension, valvular dysfunction, and pregnancy. SUMMARY: Increasing access to ACHD care, evolution of imaging techniques and transcatheter technology and continued efforts at quality improvement will be key to successfully facing the challenges that are a product of the astounding success of pediatric cardiac surgery.

Imaging adult patients with coarctation of the aorta.

PURPOSE OF REVIEW: To provide an overview of current recommendations and techniques used to image coarctations of the aorta. RECENT FINDINGS: Imaging of coarctation of the aorta in adults is moving away from conventional techniques such as echocardiography and invasive angiography. Echocardiography may assist in making the diagnosis but is limited in its ability to quantify the severity of coarctations. Current guidelines recommend that every patient with a coarctation must undergo a computed tomography (CT) or MRI scan for accurate delineation of morphology. MRIs provide important hemodynamic data and this is rapidly evolving to include techniques such as 4D flow cardiac MRI which can provide time-resolved data; research on using MRIs in the interventional suite is underway. CT is important for surveillance and provides detailed information about repair-site anatomy. Additionally, the use of CT-based technology to guide transcatheter interventions may improve the safety and efficiency of angioplasty and stent deployment. SUMMARY: Although echocardiography is an important first line means to establish the diagnosis of aortic coarctations, CT and MRI-based techniques enable the provision of patient-specific management options, can guide transcatheter interventions and detect long-term post-intervention complications.

Sex affects myocardial blood flow and fatty acid substrate metabolism in humans with nonischemic heart failure.

BACKGROUND: In animal models of heart failure (HF), myocardial metabolism shifts from high-energy fatty acid (FA) metabolism toward glucose. However, FA (vs glucose) metabolism generates more ATP/mole; thus, FA metabolism may be especially advantageous in HF. Sex modulates myocardial blood flow (MBF) and substrate metabolism in normal humans. Whether sex affects MBF and metabolism in patients with HF is unknown. METHODS AND RESULTS: We studied 19 well-matched men and women with nonischemic HF (EF ≤ 35%). MBF and myocardial substrate metabolism were quantified using positron emission tomography. Women had higher MBF (mL/g/minute), FA uptake (mL/g/minute), and FA utilization (nmol/g/minute) (P < 0.005, P < 0.005, P < 0.05, respectively) and trended toward having higher FA oxidation than men (P = 0.09). These findings were independent of age, obesity, and insulin resistance. There were no sex-related differences in fasting myocardial glucose uptake or metabolism. Higher MBF was related to improved event-free survival (HR 0.31, P = 0.02). CONCLUSIONS: In nonischemic HF, women have higher MBF and FA uptake and metabolism than men, irrespective of age, obesity, or insulin resistance. Moreover, higher MBF portends a better prognosis. These sex-related differences should be taken into account in the development and targeting of novel agents aimed at modulating MBF and metabolism in HF.

Direct Aortic Access Transcatheter Aortic Valve Replacement: Three-Dimensional Computed Tomography Planning and Real-Time Fluoroscopic Image Guidance.

BACKGROUND AND AIM OF THE STUDY: Direct aortic access for transcatheter aortic valve replacement (DA-TAVR) is an important alternative approach in patients with hostile ileo-femoral vessels. Planning the transaortic puncture site and an 'ideal' trajectory towards the annulus plane is important for safe and successful valve implantation. The feasibility of three-dimensional (3D) planning and real-time fluoroscopic image guidance for DA-TAVR was evaluated using pre-procedural multi-detector computed tomography (MDCT) and intra-procedural Dyna CT co-registration approaches. METHODS: Between May 2012 and August 2014, a total of 44 patients (40 mini-sternotomies, four mini-thoracotomies) was selected for DA-TAVR using the authors' MDCT-Dyna CT co-registration approach (32 CoreValve, 12 SAPIEN). Pre-procedural contrast-enhanced multi-slice CT (MSCT) and intra- procedural non-contrast Dyna CT images were co-registered based on cardiac outline and aortic root calcifications. Using a prototype software, the aortic root was segmented and relevant landmarks identified automatically. The intersection of a virtual perpendicular trajectory from the annulus with the greater curvature of the aorta was marked as the planned DA puncture site. The planned DA puncture site, trajectory and relevant landmarks were overlaid onto real-time fluoroscopic images for image guidance during DA-TAVR. RESULTS: Real-time fluoroscopic overlay of planned trajectory was feasible in all 44 cases of DA-TAVR. The mean 2D projection distance error between the actual and planned aortic puncture sites was 1.60 +/- 1.1 cm. The mean angular difference error (measure of co-axiality) between actual and planned DA trajectory was 11.86 +/- 9.3. Errors in distance and co-axiality were lower with the mini-thoracotomy than with the mini-sternotomy approach. The Multi-Slice CT (MSCT)-Dyna CT co-registration technique resulted in significantly less contrast usage, and trended towards shorter fluoroscopy and operative times. There was also a trend towards a reduction in acute kidney injury, but no difference was identified in the degree of paravalvular regurgitation or mortality. CONCLUSION: 3D access planning and real-time image guidance for DA-TAVR is feasible using an MDCT/non-contrast Dyna CT image co-registration-based approach. Such image co-registration strategies improve the accuracy of case planning and safety of valve deployment with a direct aortic approach. Further studies are necessary to determine if these enhancements translate into an improvement in clinical outcomes.

Sedation and Anesthesia in Pediatric and Congenital Cardiac Catheterization: A Prospective Multicenter Experience.

Sedation/anesthesia is critical to cardiac catheterization in the pediatric/congenital heart patient. We sought to identify current sedation/anesthesia practices, the serious adverse event rate related to airway, sedation, or anesthesia, and the rate of intra-procedural conversion from procedural sedation to the use of assisted ventilation or an artificial airway. Data from 13,611 patients who underwent catheterization at eight institutions were prospectively collected from 2007 to 2010. Ninety-four (0.69 %) serious sedation/airway-related adverse events occurred; events were more likely to occur in smaller patients (<4 kg, OR 4.4, 95 % CI 2.3-8.2, p < 0.001), patients with non-cardiac comorbidities (OR 1.7, 95 % CI 1.1-26, p < 0.01), and patients with low mixed venous oxygen saturation (OR 2.3, 95 % CI 1.4-3.6, p < 0.001). Nine thousand three hundred and seventy-nine (69 %) patients were initially managed with general endotracheal anesthesia, LMA, or tracheostomy, whereas 4232 (31 %) were managed with procedural sedation without an artificial airway, of which 75 (1.77 %) patients were converted to assisted ventilation/general anesthesia. Young age (<12 months, OR 5.2, 95 % CI 2.3-11.4, p < 0.001), higher-risk procedure (category 4, OR 10.1, 95 % CI 6.5-15.6, p < 0.001), and continuous pressor/inotrope requirement (OR 11.0, 95 % CI 8.6-14.0, p < 0.001) were independently associated with conversion. Cardiac catheterization in pediatric/congenital patients was associated with a low rate of serious sedation/airway-related adverse events. Smaller patients with non-cardiac comorbidities or low mixed venous oxygen saturation may be at higher risk. Patients under 1 year of age, undergoing high-risk procedures, or requiring continuous pressor/inotrope support may be at higher risk of requiring conversion from procedural sedation to assisted ventilation/general anesthesia.

Incidence and management of life-threatening adverse events during cardiac catheterization for congenital heart disease.

Continued advancements in congenital cardiac catheterization and interventions have resulted in increased patient and procedural complexity. Anticipation of life-threatening events and required rescue measures is a critical component to preprocedural preparation. We sought to determine the incidence and nature of life-threatening adverse events in congenital and pediatric cardiac catheterization, risk factors, and resources necessary to anticipate and manage events. Data from 8905 cases performed at the 8 participating institutions of the Congenital Cardiac Catheterization Project on Outcomes were captured between 2007 and 2010 [median 1,095/site (range 133-3,802)]. The incidence of all life-threatening events was 2.1 % [95 % confidence interval (CI) 1.8-2.4 %], whereas mortality was 0.28 % (95 % CI 0.18-0.41 %). Fifty-seven life-threatening events required cardiopulmonary resuscitation, whereas 9 % required extracorporeal membrane oxygenation. Use of a risk adjustment model showed that age <1 year [odd ratio (OR) 1.9, 95 % CI 1.4-2.7, p < 0.001], hemodynamic vulnerability (OR 1.6, 95 % CI 1.1-2.3, p < 0.01), and procedure risk (category 3: OR 2.3, 95 % CI 1.3-4.1; category 4: OR 4.2, 95 % CI 2.4-7.4) were predictors of life-threatening events. Using this model, standardized life-threatening event ratios were calculated, thus showing that one institution had a life-threatening event rate greater than expected. Congenital cardiac catheterization and intervention can be performed safely with a low rate of life-threatening events and mortality; preprocedural evaluation of risk may optimize preparation of emergency rescue and bailout procedures. Risk predictors (age < 1, hemodynamic vulnerability, and procedure risk category) can enhance preprocedural patient risk stratification and planning.

Balloon Pulmonary Angioplasty for the Treatment of Chronic Thromboembolic Pulmonary Hypertension.

Chronic thromboembolic pulmonary hypertension is a rare form of pulmonary hypertension in patients who have evidence of chronic thromboembolic occlusion of the pulmonary vasculature. Historically, surgical pulmonary thromboendarterectomy has been the treatment of choice. However, with up to 40% of patients deemed inoperable, balloon pulmonary angioplasty has emerged as an additional treatment strategy. Balloon pulmonary angioplasty is a complementary strategy alongside surgical pulmonary thromboendarterectomy and offers the opportunity for pulmonary revascularization in patients who have more distal disease, higher comorbidities, or residual obstruction following operative intervention. This review examines the history of balloon pulmonary angioplasty, highlights its effectiveness, discusses important complications and risk reduction strategies, and emphasizes the importance of centers forming a multidisciplinary team of providers to manage the complexity of patients with chronic thromboembolic pulmonary hypertension.

Transcatheter ventricular septal defect (VSD) creation for restrictive VSD in double-outlet right ventricle.

BACKGROUND: Double-outlet right ventricle (DORV) with a restrictive ventricular septum is a rare but highly morbid phenomenon that can be complicated by progressive left ventricular hypertrophy, arrhythmias, aneurysm formation, severe pulmonary hypertension, and death in the newborn. Surgical creation or enlargement of a ventricular septal defect (VSD) is palliative but may damage the conduction system or the atrioventricular valves in the newborn. This report presents a transcatheter approach to palliation for a newborn that had DORV with a restrictive ventricular septum. METHODS/RESULTS: A full-term infant girl (2.9 kg) referred for hypoxia (80% with room air) and murmur was found to have DORV, interrupted inferior vena cava, and restrictive VSD (95-mmHg gradient). Transhepatic access was performed, and an internal mammary (IM) catheter was advanced through the atrial septal defect and into the left ventricle. By transesophageal echocardiographic guidance, a Baylis radiofrequency perforation wire was used to cross the ventricular septum, and the defect was enlarged using a 4-mm cutting balloon. A bare metal stent then was deployed to maintain the newly created VSD. The patient did well after the procedure but required pulmonary artery banding 4 days later. She returned 5 months later with cyanosis and the development of obstructing right ventricle muscle bundles, requiring further surgical palliation. CONCLUSIONS: This report describes the first transcatheter creation of VSD in DORV with a restrictive ventricular septum in a newborn infant. Use of the radiofrequency catheter in combination with cutting balloon dilation and stent implantation is an efficient method for creating a VSD in such a patient.

Tough Crossing: A Challenging Case of Valve-in-Valve Transcatheter Tricuspid Valve Replacement.

Transcatheter tricuspid valve replacement is a feasible treatment alternative in high-risk patients with degenerated tricuspid prosthesis. Either transjugular or transfemoral approaches are feasible, with the latter being used more commonly. We describe a challenging case of valve-in-valve transcatheter tricuspid valve replacement where we used a long sheath positioned in the right ventricular outflow tract to deliver the transcatheter heart valve.

Single-Center Experience With Aortic Coarctation Stenting in Adult Patients.

OBJECTIVE: Endovascular repair of aortic coarctation (CoA) has become an important tool in the treatment of an expanding patient population. In this study, we present our 10-year experience with endovascular repair of CoA. METHODS: Between January 2012 and January 2022, a total of 15 patients were treated at our Institution for CoA with catheter-based techniques. Demographics, intraprocedural data, and follow-up data were retrospectively collected from institutional databases and analyzed. The primary endpoint was technical success and secondary endpoints were intraoperative complications and short-, mid-, and long-term follow-up. RESULTS: Mean age was 44.87 ± 15.52 years (range, 15-64) and 12 patients (80%) were male. Fourteen patients (93.3%) were hypertensive, and 4 patients (26.7%) had a bicuspid aortic valve. Three patients (20%) had undergone open repair in the pediatric age. Fourteen patients (93.3%) received stenting of CoA and 1 patient (6.7%) received thoracic endovascular aortic repair and left subclavian artery stenting for proximal pseudoaneurysmatic dilation and symptomatic restenosis. Mean pretreatment trans-stenotic gradient was 23.25 ± 11.16 mm Hg and posttreatment trans-stenotic gradient was 1.3 ± 1.33 mm Hg. Primary technical success was achieved in 15 cases (100%). One right inguinal hematoma (6.7%) was observed. One patient (6.7%) had an aortic rupture at the left subclavian artery origin after poststent dilation. Mean follow-up time was 34.75 ± 34.38 months. A total of 2 patients had an increased trans-stenotic gradient at long-term follow-up, and 1 reintervention (6.7%) for somatic growth was performed. CONCLUSIONS: Endovascular repair of CoA is effective and safe, with excellent mid-term and long-term success rates.

Automated catheter tip repositioning for intra-cardiac echocardiography.

PURPOSE: Intra-Cardiac Echocardiography (ICE) is a powerful imaging modality for guiding cardiac electrophysiology and structural heart interventions. ICE provides real-time observation of anatomy and devices, while enabling direct monitoring of potential complications. In single operator settings, the physician needs to switch back-and-forth between the ICE catheter and therapy device, making continuous ICE support impossible. Two operator setups are sometimes implemented, but increase procedural costs and room occupation. METHODS: ICE catheter robotic control system is developed with automated catheter tip repositioning (i.e., view recovery) method, which can reproduce important views previously navigated to and saved by the user. The performance of the proposed method is demonstrated and evaluated in a combination of heart phantom and animal experiments. RESULTS: Automated ICE view recovery achieved catheter tip position accuracy of [Formula: see text] mm and catheter image orientation accuracy of [Formula: see text] in animal studies, and [Formula: see text]mm and [Formula: see text] in heart phantom studies, respectively. Our proposed method is also successfully used during transseptal puncture in animals without complications, showing the possibility for fluoro-less transseptal puncture with ICE catheter robot. CONCLUSION: Robotic ICE imaging has the potential to provide precise and reproducible anatomical views, which can reduce overall execution time, labor burden of procedures, and X-ray usage for a range of cardiac procedures.

Multimodality Image-Guided Embolization of Bronchial Artery Pseudoaneurysm in a Patient With Aortopathy.

A 59-year-old man received an incidental diagnosis of a 5-cm right para-aortic mass that was initially thought to be of venous origin. Multimodality imaging revealed a right bronchial artery pseudoaneurysm that was treated with endovascular embolization. Bronchial artery pseudoaneurysms are extremely rare and can be fatal if ruptured. (Level of Difficulty: Advanced.).

PFO Closure after Pulmonary Valve Intervention in Patients with Hypoxemia Due to Right-to-Left Intracardiac Shunt.

Four patients with pulmonary valve (PV) disease and patent foramen ovale (PFO) presented with dyspnea on exertion. Work-up revealed hypoxemia secondary to right-to-left intracardiac shunt. We demonstrate that correction of the primary culprit right heart overload lesion via PV replacement enabled safe PFO repair and resolution of hypoxemia.

Defect closure in the lipomatous hypertrophied atrial septum with the Amplatzer muscular ventricular septal defect closure device: a case series.

Variations in the anatomy of the atrial septum can confound the transcatheter closure of atrial septal defects and patent foramen ovale. While lipomatous hypertrophy of the atrial septum is not frequently encountered, attempts to use standard atrial septal occlusion devices in such patient can lead to malapposition and continued shunt. Some operators have suggested that atrial septal hypertrophy is a relative contraindication to transcatheter-based closure techniques. We present a novel technique exploiting the features of the Amplatzer muscular ventricular septal defect occluder to treat atrial septal defects in the setting of lipomatous hypertrophy of the atrial septum in two patients.