Percutaneous Balloon-Expandable Stent Implantation to Treat Transverse Aortic Arch Obstruction: Medium- to Long-Term Outcomes of a Retrospective Multicenter Study.

BACKGROUND: Transverse aortic arch obstruction is a challenging lesion for which stent implantation provides a potentially important alternate therapy. The objectives were to evaluate the technical, procedural, and medium-to-long-term clinical outcomes of percutaneous stent implantation of transverse aortic arch obstruction. METHODS: This is a retrospective, multicenter study of transverse aortic arch stent implantation. Univariable and multivariable analyses were performed. RESULTS: Index catheterization included 187 stent implants in 146 patients. The median age is 14.3 years (interquartile range, 9.3-19), weight is 53 kg (30-69), and follow-up is 53 months (12-120). The most common stent design was open cell (n=90, 48%). Stents overlapped 142 arch vessels (37 carotid arteries) in 118 (81%) cases. Technical and procedural success rates were 100% and 88%, respectively. Lower weight (P=0.018), body surface area (P=0.013), and minimum-to-descending aortic diameter ratio (P<0.001) were associated with higher baseline aortic gradient. The residual gradient was inversely associated with implant and final dilation diameters (P<0.001). The combined incidence of aortic injury and stent-related complications was 14%. There were no reports of abnormal brain scans or stroke. Blood pressure cuff gradient, echocardiographic arch velocity, and hypertension rates improved within 1-year follow-up with increased antihypertensive medication use. Reintervention was reported in 60 (41%) patients at a median of 84 (22-148) months to first reintervention. On multivariable logistic regression, residual aortic gradient >10 mm Hg was associated with increased odds of reintervention at all time points when controlling for each final dilation diameter, weight, and minimum-to-descending aortic diameter ratio. CONCLUSIONS: Transverse aortic arch stent implantation has high rates of technical, procedural, and medium-to-long-term clinical success. Aortic gradient >10 mm Hg is associated with increased odds of reintervention at 1-year and most recent follow-ups. Open cell stent design was frequently used for its advantages in conformability, perfusion of arch vessels, low fracture rate, and the ability to perform effective angioplasty of side cells.

Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension due to septic emboli.

We present the case of a 28-year-old woman with a history of tricuspid valve endocarditis leading to chronic thromboembolic pulmonary hypertension (CTEPH) with multiple pulmonary artery chronic total occlusions (CTOs) due to septic emboli. Following a multidisciplinary care discussion, the patient was brought forward for balloon pulmonary angioplasty (BPA) with successful revascularization of all chronically occluded territories. This case highlights advances in pulmonary artery CTO interventions and demonstrates the feasibility of BPA for CTEPH patients with a history of septic emboli.

Right ventricular outflow tract obstruction associated with neointimal tissue accumulation and distortion of the Harmony TPV25 stent frame: Potential mechanisms and treatment.

BACKGROUND: The Harmony TPV25 transcatheter pulmonary valve (Medtronic Inc.) is constructed with a self-expanding stent frame comprising six zigged nitinol wires sewn together and covered with knitted polyester fabric, with flared inflow and outflow ends and a porcine pericardial valve sutured to the central portion of the device. It was approved for treatment of pulmonary regurgitation after prior right ventricular outflow tract repair in 2021. Early outcomes of this procedure have been excellent, but little is known about valve durability or ultimate mechanisms of dysfunction. METHODS: We collected data on patients who underwent reintervention for TPV25 dysfunction and described findings related to distortion of the stent frame and tissue accumulation. RESULTS: We describe six patients who underwent valve-in-valve implant for TPV25 obstruction (peak catheterization gradient peak 28-73 mmHg) 10-28 months after implant. In all cases, there was tissue accumulation within the inflow and valve-housing segments of the device and deformation of the self-expanding valve frame characterized by variable circumferential narrowing at the junction between the valve housing and the inflow and outflow portions of the device, with additional geometric changes in all segments. All six patients underwent valve-in-valve implant that results in a final peak gradient ≤10 mmHg and no regurgitation. DISCUSSION: The occurrence of short-term Harmony TPV25 dysfunction in multiple patients with a similar appearance of frame distortion and tissue accumulation within the inflow and valve housing portions of the device suggests that this may be an important failure mechanism for this valve. Potential causes of the observed findings are discussed. It is possible to treat this mechanism of TPV25 dysfunction with valve-in-valve implant using balloon expandable transcatheter valves.

Clinically significant myocardial bridging.

Myocardial bridging is a common anatomical variant in which a major epicardial coronary artery takes an intramyocardial course, leading to dynamic systolic compression. Because coronary perfusion occurs primarily during diastole, most patients with this anatomical variant have no associated perfusion abnormalities or symptoms. Despite this, there is a subset of patients with myocardial bridging who experience ischaemic symptoms. Determining which anatomical variants are benign and which are clinically relevant remains a challenge. Further complicating the picture, functional factors such as diastolic dysfunction and coronary vasospasm may exacerbate myocardial bridging-related ischaemia. In patients with ischaemic symptoms in the absence of alternative explanations, a detailed assessment of myocardial bridging with invasive physiology should be performed to define the significance of the lesion and guide tailored medical therapy. Patients with refractory symptoms despite maximally tolerated medical therapy should be considered for surgical coronary unroofing.

Establishing a balloon pulmonary angioplasty program for chronic thromboembolic pulmonary hypertension: A United States single-center experience.

INTRODUCTION: Balloon pulmonary angioplasty (BPA) is a less invasive treatment alternative for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are unable to move forward with pulmonary thromboendarterectomy. This report describes a single-center experience with a nascent BPA program in the United States (US). METHODS: All patients who underwent BPA between August 2018-2021 were included in this retrospective, single-center observational cohort. Pre- and post-procedure clinical information was collected, along with procedural characteristics. RESULTS: Thirty patients began their BPA series during the study period. The majority of patients had segmental disease (n = 25, 83.3%). A total of 135 BPA procedures were performed on 417 segments. On average, patients completed 4.5 sessions and the majority of patients (n = 23, 76.7%) underwent more than 2. There were 24 episodes of hemoptysis and 20 procedural events that required treatment, typically with either heparin reversal or balloon tamponade. Of 26 participants with completed series, mean PA pressure (-6 mmHg, 95% CI -9 to -4 mmHg, p = 0.0001), PVR (-1.9 Wood units, 95% CI -2.9 to -1.0, p = 0.0002), and pulmonary compliance (-1.0 mL/mmHg, 95% CI -1.5 to -0.5, p = 0.0002) improved. Improvement was also seen in NYHA functional classification and walk distance (p = 0.01). Two deaths occurred, with one death peri-procedurally. CONCLUSION: This paper describes an early experience with BPA at a single US center. Improvement in non-invasive and invasive metrics were seen without adding a significant morbidity to an already high-risk patient population.

The Simplified Extraction of Atrial Tumor With Targeted Loop Electricity (SEATTLE) Procedure.

The removal of intramyocardial masses has long been thought of as a surgical procedure and is generally reserved for patients with obstructive symptoms when the mass is thought to be benign. Thus, many patients who are incidentally diagnosed with intracardiac masses are either subjected to protracted follow-up with serial imaging awaiting tumor growth before surgical excision is ultimately offered. We report a novel procedure in which a 54-year-old man with an atrial myxoma underwent successful percutaneous resection using electrosurgery followed by removal with a novel endovascular retrieval system. This approach provides an alternative to either surgical excision or watchful waiting in patients with small- to medium-sized intracardiac tumors. (Level of Difficulty: Advanced.).

Use of the PK Papyrus covered coronary stent in the treatment of Kawasaki disease-associated giant coronary artery aneurysms.

Kawasaki disease (KD) is an acute vasculitis that can cause coronary artery inflammation and aneurysm formation leading to early obstructive disease. We describe the use of PK Papyrus covered stents (Biotronic, Inc.) in three pediatric patients to exclude coronary artery aneurysms (CAA) from the circulation and relieve aneurysm associated stenoses. Follow-up angiography at 11-17 months postprocedure demonstrated persistent exclusion of CAA and varying degrees of in-stent restenosis (ISR). Two patients required percutaneous coronary intervention with drug eluting stent (DES) implantation to relieve in-stent stenosis. Our findings suggest that CAA exclusion with the PK Papyrus stent is possible and may be a valuable tool in simultaneously treating stenotic and thrombogenic CAA in pediatric KD patients. ISR of these non-DES remains an issue and may require additional interventions within the short-term to maintain vessel patency.

Thyroid-stimulating hormone and mortality in pulmonary arterial hypertension.

INTRODUCTION: Pulmonary arterial hypertension (PAH) remains a serious and life-threatening illness. Thyroid dysfunction is relatively understudied in individuals with PAH but is known to affect cardiac function and vascular tone in other diseases. The aim of this observational study was to evaluate the association between thyroid-stimulating hormone (TSH), mortal and non-mortal outcomes in individuals with PAH. METHODS: The Seattle Right Ventricle Translational Science (Servetus) Study is an observational cohort that enrolled participants with PAH between 2014 and 2016 and then followed them for 3 years. TSH was measured irrespective of a clinical suspicion of thyroid disease for all participants in the cohort. Linear regression was used to estimate the relationships between TSH and right ventricular basal diameter, tricuspid annular plane systolic excursion and 6-minute walk distance. Logistic regression was used to estimate the relationship with New York Heart Association Functional Class, and Cox proportional hazards were used to estimate the relationship with mortality. Staged models included unadjusted models and models accounting for age, sex at birth and aetiology of pulmonary hypertension with or without further adjustment for N-terminal-pro hormone brain natriuretic peptide. RESULTS: Among 112 participants with PAH, TSH was strongly associated with mortality irrespective of adjustment. There was no clear consistent association between TSH and other markers of severity in a cohort with PAH. DISCUSSION: This report reinforces the important observation that TSH is associated with survival in patients with PAH, and future study of thyroid dysfunction as a potential remediable contributor to mortality in PAH is warranted.

Balloon Pulmonary Angioplasty in Chronic Totally Occluded Pulmonary Arteries: Applying Lessons Learned From the Treatment of Coronary Artery Chronic Total Occlusions.

OBJECTIVES: This study aims to describe the safety and efficacy of revascularizing chronic total occlusions (CTOs) of the pulmonary arteries with balloon pulmonary angioplasty (BPA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH). BACKGROUND: BPA has emerged as an effective treatment for CTEPH patients when surgical treatment is not possible. Experience to date has suggested treating CTOs may be associated with excess risk and less procedural success relative to other lesion types. METHODS: This study is a retrospective case series of all BPAs on CTOs for individuals with CTEPH at a single institution. Procedural approach, complications, and success rate over a 6-month period are described. RESULTS: During the study period, 6 individuals with 15 CTOs were identified and intervened upon during 21 interventions. Success rate for revascularization was 62% per attempt and 87% per lesion. Techniques used for successful intervention include true to true lumen wiring (n = 7) and subintimal dissection re-entry with subintimal tracking and re-entry (n = 3), Stingray balloon (Boston Scientific) assisted re-entry (n = 2), and direct wire re-entry (n = 1). Wire perforations were relatively common and occurred in 62% of interventions, but rarely resulted in a change in clinical status. CONCLUSIONS: Although important barriers to routine intervention on CTOs in CTEPH remain, the current series suggests a higher success rate than previously reported experiences using CTO revascularization techniques including subintimal tracking and re-entry and Stingray balloon-assisted re-entry. Although the frequency of wire perforation was relatively high, the clinical ramifications of these complications were mild.

Intravascular coronary brachytherapy combined with a drug-coated balloon.

BACKGROUND: Coronary artery disease leads to stenosis of the major cardiac vessels, resulting in ischemia and infarction. Percutaneous intervention (PCI) with balloon angioplasty can re-open stenosed vessels. Drug eluting stents (DES) and intravascular brachytherapy (IVBT) and drug-coated balloons (DCBs) are proven to decrease the likelihood of another restenosis after PCI, but neither is completely effective. Due to the limited long-term effectiveness of IVBT or DCB used separately for salvage PCI, we combined the two in some poor prognosis patients. METHODS: Combined IVBT+DCB was intended for a total of 36 patients from 2015-2020. PCI with some combination of ballooning, laser and directional/rotational atherectomy was used to maximally open the stenotic region prior to IVBT+DCB. Beta-radiation brachytherapy for all patients was done with a Novoste Beta-Cath. Lutonix 4.0 x 40 mm paclitaxel-coated balloons (Bard, Murray Hill, NJ) were employed. RESULTS: Overall survival at two years was 88%. Nine patients had follow-up angiograms, all for cardiac symptoms. Time from IVBT+DCB to follow-up angiography ranged from 4 to 33 months. The average months PCI-free interval before brachy therapy was 11.1 mos (95% CI 1.03-23.25) versus 23.3 mos after VBT (23.3 95% CI 12.3-32.3). The mean difference was 11.2 mos (95% CI 1.06-21.4, p < 0.031). None of the follow-up angiographic procedures displayed evidence of what could be interpreted as radiation damage. CONCLUSIONS: In this uncontrolled series, IVBT plus DCB appeared to lengthen the ISR-free interval relative to what had been achieved prior to the combined intervention. We view these results as mildly encouraging, worthy of further study.

Associations of Angiopoietins With Heart Failure Incidence and Severity.

BACKGROUND: Angiopoietin-1 and 2 (Ang1, Ang2) are important mediators of angiogenesis. Angiopoietin levels are perturbed in cardiovascular disease, but it is unclear whether angiopoietin signaling is causative, an adaptive response, or merely epiphenomenon of disease activity. METHODS AND RESULTS: In a cohort free of cardiovascular disease at baseline (Multi-Ethnic Study of Atherosclerosis [MESA]), relationships between angiopoietins, cardiac morphology, and subsequent incidence of heart failure or cardiovascular death were evaluated. In cohorts with pulmonary arterial hypertension or left heart disease, associations between angiopoietins, invasive hemodynamics, and adverse clinical outcomes were evaluated. In MESA, Ang2 was associated with a higher incidence of heart failure or cardiovascular death (hazard ratio 1.21 per standard deviation, P < .001). Ang2 was associated with increased right atrial pressure (pulmonary arterial hypertension cohort) and increased wedge pressure and right atrial pressure (left heart disease cohort). Elevated Ang2 was associated with mortality in the pulmonary arterial hypertension cohort. CONCLUSIONS: Ang2 was associated with incident heart failure or death among adults without cardiovascular disease at baseline and with disease severity in individuals with existing heart failure. Our finding that Ang2 is increased before disease onset and that elevations reflect disease severity, suggests Ang2 may contribute to heart failure pathogenesis.

Ebstein's Anomaly of the Tricuspid Valve: an Overview of Pathology and Management.

PURPOSE OF REVIEW: Ebstein's anomaly (EA) is a rare, but complex form of congenital heart disease consisting of a right ventricular myopathy and morphologic tricuspid valve disease leading to a high incidence of right ventricular dysfunction and arrhythmias. This review offers an updated overview of the current understanding and management of patients with EA with a focus on the adult population. RECENT FINDINGS: Increased understanding of anatomic accessory atrioventricular pathways in EA has resulted in an improvement in ablation techniques and long-term freedom of atrial arrhythmia recurrence. Despite an improvement in understanding and recognition of EA, significant disease heterogeneity and complex treatment options continue to challenge providers, with the best outcomes achieved at expert congenital heart disease centers.

Utility of biomarkers in adult Fontan patients with decompensated heart failure.

BACKGROUND: The incidence of heart failure is increasing within the Fontan population. The use of serological markers, including B-type natriuretic peptide, has been limited in this patient population. METHODS: This was a single-centre retrospective study of Fontan patients in acute decompensated heart failure. Fontan patients underwent a 1:2 match with non-Fontan patients for each heart failure hospitalisation for comparative analysis. A univariate logistic regression model was used to assess associations between laboratory and echocardiographic markers and a prolonged length of stay of 7 days or greater. RESULTS: B-type natriuretic peptide levels were significantly lower in Fontan patients admitted for heart failure than that in non-Fontan patients [390.9 (±378.7) pg/ml versus 1245.6 (±1160.7) pg/ml, respectively, p < 0.0001] and were higher in Fontan patients with systemic ventricular systolic or diastolic dysfunction than that in Fontan patients with normal systemic ventricular function [833.6 (±1547.2) pg/ml versus 138.6 (±134.0) pg/ml, p = 0.017]. The change from the last known outpatient value was smaller in Fontan patients in comparison with non-Fontan patients [65.7 (±185.7) pg/ml versus 1638.0 (±1444.7) pg/ml, respectively, p < 0.0001]. Low haemoglobin and high blood urea nitrogen levels were associated with a prolonged length of stay. CONCLUSION: B-type natriuretic peptide levels do not accurately reflect decompensated heart failure in Fontan patients when compared to non-Fontan heart failure patients and should, therefore, be used with caution in this patient population.

Platypnea Orthodeoxia Syndrome Secondary to Intracardiac Shunt Following Orthotopic Liver Transplantation.

Platypnea orthodeoxia syndrome (POS) occurs when an upright position results in acute-onset hypoxemia and is relieved with recumbency. POS can be due to intracardiac shunting, intrapulmonary shunting, ventilation-perfusion mismatch, or a combination of these. We report a case of POS that developed 3 days post liver transplantation as a result of new-onset right to left shunting across a patent foramen ovale. Right heart catheterization revealed a posteriorly directed inferior vena cava likely due to altered inferior vena cava-right atrial junction anatomy as a result of liver transplantation. The patient underwent successful transcatheter patent foramen ovale closure with a 25-mm Gore Cardioform septal occluder device with immediate and sustained improvement in hypoxia.

What Every Cardiologist Should Know About the 2018 Updated Adult Congenital Cardiology Guidelines.

PURPOSE OF REVIEW: Review the recently updated guidelines for the management of patients with adult congenital heart disease (ACHD) with a focus on the changes between these guidelines and the prior guidelines. RECENT FINDINGS: The 2018 guidelines for the management of patients with ACHD focused on utilizing the available data and limiting the number recommendations based only on expert opinion. These guidelines implement a new anatomic and physiological classification scheme to guide management of patients, which takes into account both the underlying anatomy as well as residual cardiac disease and symptoms. Given a lack of robust outcomes data for many types of CHD, the new guidelines provide fewer total recommendations than the prior version, emphasizing the data that is available and drawing attention to the need for additional data. The 2018 guidelines provide the field with a comprehensive update in the management of ACHD patients with an emphasis on the available data.

Constrictive Pericarditis After Lung Transplantation.

As a rare complication after lung transplant, cardiac constriction should not be missed. Physical exam, echocardiography, and catheterization are essential for diagnosis A 65-year-old man with previous coronary artery disease and idiopathic pulmonary fibrosis underwent bilateral lung transplant and subsequently presented for progressive dyspnea and volume overload. Cardiac imaging and cardiac catheterization confirmed constriction, and complete pericardiectomy was performed. The patient had rapid resolution of heart failure symptoms. Pericardial constriction is a rare complication following lung transplant, and we provide a review of the literature and discussion of potential contributing factors. (Level of Difficulty: Intermediate.).

Endothelin-1, cardiac morphology, and heart failure: the MESA angiogenesis study.

BACKGROUND: Circulating levels of endothelin-1 (ET1) are elevated in heart failure and predict poor prognosis. However, it is not clear whether ET1 elevation is an adaptive response, maladaptive response, or an epiphenomenon of heart failure. In this study, we evaluated the relationships between ET1, cardiac morphology, and incident heart failure or cardiovascular death in participants with no evidence of clinical cardiovascular disease at the time ET1 was measured. METHODS AND RESULTS: ET1 was measured in 1,361 participants in the Multi-Ethnic Study of Atherosclerosis Angiogenesis Sub-Study. As suggested by linear regression, participants with lower circulating ET1 levels tended to be older, non-white, more likely to have smoked heavily, and less likely to report intentional exercise. Participants with higher ET1 levels had smaller left ventricular end-diastolic volumes (8.9 ml smaller per log increase in ET1, 95% confidence interval 17.1-0.7, p = 0.03) with an increased left ventricular ejection fraction (2.8% per log increase in ET1, 95% confidence interval 0.5%-5.2%, p = 0.02). As suggested by Cox Proportional Hazards estimates, participants with higher ET1 levels had a lower risk for the composite outcome of heart failure or cardiovascular death in models that were unadjusted or had limited adjustment (p = 0.03 and p = 0.05, respectively). Lower risk for heart failure with higher ET1 levels could not be clearly shown in a model including health behaviors. CONCLUSIONS: These results suggest, but do not confirm, that elevated levels of circulating ET1 are associated with a more favorable cardiac phenotype. The relationship between ET1 and outcomes was not fully independent of one or more covariates.

Feasibility of pulmonary valve imaging using transesophageal echocardiography upper esophageal view.

BACKGROUND: The present study designed to evaluate feasibility of transesophageal echocardiographic (TEE) imaging of the pulmonary valve (PV) at the transaortic upper esophageal (TAUE) window. We hypothesized that patients with larger aorta would be more likely to have visualization of the PV from this TAUE window. METHODS: 2D TEE images of the PV were prospectively acquired by one operator at the TAUE window looking through the aortic arch. Patients were divided into four groups based on image quality of PV (group 1, not visualized; group 2, barely visualized; group 3, sufficient visualization; group 4, excellent visualization). Clinical, echocardiographic, and radiologic parameters were collected. RESULTS: A total of 212 consecutive patients (54 ± 14 years, 63.7% male) were enrolled. Group distribution was as follows: group 1, n = 60 (28.3%); group 2 n = 39 (18.4%); group 3, n = 27 (12.7%); group 4, n = 86 (40.6%). There were no differences between groups' baseline clinical characteristics. There was a weak although statistically negative correlation between PV image quality and aortic arch dimension (r = -0.17 P = 0.01). There was a stronger positive correlation between PV thickness (r = 0.38 P < 0.001) and PV image quality. Retrospective subgroup analysis of 76 patients with recent chest CT showed similar, but not significant trends as by TEE. CONCLUSION: The use of 2D TEE TAUE results in diagnostic image quality of the PV in the majority of patients and outstanding image quality in a subset of patients. PV image quality is negatively affected by increasing aorta diameter and positively affected by PV thickness.