First-in-human experience of the Bashir Endovascular Catheter in the treatment of iliocaval deep vein thrombosis.

Deep vein thrombosis (DVT) is a common disorder affecting approximately 900,000 new patients in the United States each year. Although the mainstay of treatment of DVT patients is therapeutic anticoagulation, some patients remain significantly symptomatic and therefore require more advanced interventions such as catheter-directed thrombolysis (CDT). We describe a case series of 13 patients with acute symptomatic inferior vena cava (IVC) and iliofemoral DVT that were treated with CDT using the Bashir Endovascular Catheter (BEC). We report the first-in-human use of BEC, which is a novel pharmacomechanical thrombolysis device. All the treated patients had complete and rapid resolution of their symptoms with excellent venous outflow. Despite initial promising results, larger studies using this catheter design will be needed to assess the role of BEC-directed therapy on rates of post-thrombotic syndrome and bleeding complications.

Outcomes of hospitalization in adults with Fontan palliation: The Mayo Clinic experience.

The outcomes of hospitalization in the Fontan population have not been specifically studied. The purpose of this study was to describe outcomes of hospitalization (frequency and indications for hospitalization, and in-hospital mortality) in this population and to determine how these outcomes differ from those of other adults with congenital heart disease (CHD). METHODS: This was a retrospective study of adult Fontan patients hospitalized at Mayo Clinic Rochester in 1990-2015. We selected age- and gender-matched control group of patients with repaired CHD and biventricular circulation hospitalized within the study period. RESULTS: A total of 367 Fontan patients (age 31±7 years and 259 [71%] with atriopulmonary Fontan) had 853 hospital admissions in 4 years (58 hospitalizations per 100 patient-years). The most common indications were arrhythmia (n=188, 22%), heart failure (n=169, 20%), and cardiac surgery (n=133, 16%). Overall in-hospital mortality was 4% (n=38), and the highest in-hospital mortality occurred in patients hospitalized for cardiac surgery (n=15, 11%) and heart failure (n=13, 8%). In comparison to the repaired CHD and biventricular circulation group, the Fontan group had more frequent hospitalizations (22 vs 58 per 100 patient-years, P<.001) and higher overall in-hospital mortality (1% vs 5%, P<.001), mortality after cardiac surgery (2% vs 11%, P=.01), and mortality for heart failure-related hospitalizations (2% vs 8%, P=.04). CONCLUSIONS: Adults with Fontan palliation had more frequent hospitalization and in-hospital mortality compared to the rest of the CHD population. Arrhythmia and heart failure were the most common indications for hospitalization. Perhaps optimal management of heart failure and arrhythmia may improve outcomes in this population.

Progressive right ventricular enlargement due to pulmonary regurgitation: Clinical characteristics of a "low-risk" group.

BACKGROUND: The optimal interval between serial cardiac magnetic resonance imaging (CMRI) scans for monitoring right ventricular (RV) enlargement in the setting of severe pulmonic valve regurgitation (PR) is unknown. The purposes of this study were to (1) determine the annual change in RV volume on serial CMRI scans and (2) identify the risk factors for rapid progression of RV enlargement. METHODS: A retrospective study of adults with postintervention native valve PR and ≥2 CMRI scans at Mayo Clinic Rochester from 2000 to 2015 was conducted. Rapid progression of RV enlargement was defined as first upper quartile of annual increase in RV end-diastolic volume index (RVEDVi) for the cohort. RESULTS: Of the 63 patients (age, 36 ±â€¯9 years) in the study, 43 (68%) had tetralogy of Fallot, whereas 20 (32%) had valvular pulmonic stenosis. Right ventricular outflow tract interventions that resulted in PR were balloon pulmonary valvuloplasty (n = 4; 7%), transannular patch repair (n = 30; 58%), and nontransannular patch repair (n = 18; 35%). Interval between baseline and second CMRI was 2 (1-4) years. In comparison to baseline CMRI, RVEDVi increased from 130 (109-141) to 135 (126-155) mL/m2 and median annual change in RVEDVi was 3.1 (1.7-5.9) mL/m2. Univariate risk factors for rapid progression of RV enlargement (annual increase in RVEDVi >6 mL/m2) were ≥moderate tricuspid regurgitation and RVEDVi >130 mL/m2. Among the 24 patients without these risk factors (low-risk subgroup), RVEDVi increased by only 3 (0-7) mL/m2 over 7 (5-9) years. CONCLUSIONS: Patients with PR without RVEDVi >130 mL/m2 and/or ≥moderate tricuspid regurgitation represent a low-risk subgroup that may be appropriate for clinical and echo follow-up but may potentially require infrequent CMRI follow-up.

Role of Doppler echocardiography for cardiac output assessment in Fontan patients.

BACKGROUND: To determine (1) correlation between Doppler stroke volume index (SVI) and cardiac magnetic resonance imaging (CMRI) SVI and (2) association between Doppler SVI and Fontan-associated diseases (FAD) and Fontan failure. METHODS: Review of Fontan patients who underwent same-day CMRI and transthoracic echocardiography (TTE), 2005 to 2015. We defined FAD as cardiac thrombus, protein-losing enteropathy, arrhythmia, and hospitalization for heart failure. Fontan failure was defined as Fontan conversion or revision, heart transplantation or listing, or death. RESULTS: Fifty-three patients with systemic left ventricle (LV) underwent 86 sets of TTE/CMRI. Mean (SD) age 31 (6) years. SVI (45 [16] vs 42 [13] mL/m2), CI (3.0 [1.1] vs 2.8 [0.8] L min-1 m-2), and ejection fraction (53 [4]% vs 51 [5]%) were similar for both modalities (P>.05 for all). Doppler SVI correlated with CMRI (r=0.68; P<.001). Sixteen patients had cirrhosis, and these patients had a higher CI (3.9 [0.9] vs 2.8 [1.0] L min-1 m-2; P<.01). Among the 37 patients without cirrhosis, Doppler SVI <39 mL/m2 was associated with FAD (odds ratio [OR], 2.11; 95% confidence limit, 1.26-3.14; P=.02); Fontan failure was more common in patients with CI was <2.5 L min-1 m-2 (3/9 [33%] vs 0/28 [0%], P=.01). Another 11 patients with systemic right ventricle (RV) underwent 17 sets of TTE/CMRI, mean (SD) age 17 (3) years, and CMRI SVI also correlated with Doppler SVI (r=0.75; P<.001). CONCLUSION: Doppler SVI correlated with CMRI SVI in patients with systemic LV and systemic RV. The association between output measures (SVI and CI) and FAD were seen only in single LV patients (single RV patients not assessed for this outcome due to small numbers). An association between low Doppler CI and Fontan failure was suggested in a small number of single LV patients.

Pelvic Vein Obstruction in Chronic Thromboembolic Pulmonary Hypertension: A Novel Association.

Background: Pelvic venous obstruction (PVO), defined as greater than 50% stenosis or occlusion of pelvic veins, is a known risk factor for deep vein thrombosis (DVT). DVT is a known risk factor for chronic thromboembolic pulmonary hypertension (CTEPH), but the prevalence of PVO in CTEPH is unknown. Methods: This cross-sectional study at Temple University's tertiary referral center for Pulmonary Hypertension, Right Heart Failure, and CTEPH sought to identify the presence of PVO in patients with CTEPH who underwent cardiac catheterization, pulmonary angiography, and venography. Results: A total of 193 CTEPH patients were referred for pulmonary angiography, and among these, 148 underwent venography. PVO was identified in 65 (44%) patients. Lower extremity (LE) DVT was associated with PVO (p = 0.004). The severity of pulmonary hypertension was similar with and without PVO (mean pulmonary artery pressure 43.0 ± 10.3 mm Hg vs. 43.8 ± 12.4 mm Hg, p = 0.70), as was the need for pulmonary thromboendarterectomy (69.2% vs. 61.4%, p = 0.32). Conclusions: Pelvic vein obstruction is common and a novel clinical association in patients with CTEPH, particularly in patients with a history of LE DVT. PVO and its role in CTEPH warrants further study, including the potential role of revascularization to mitigate further risk.

May-Thurner Anatomy in Patients With Chronic Thromboembolic Pulmonary Hypertension: An Important Clinical Association.

OBJECTIVES: The aim of this study was to investigate the incidence of May-Thurner anatomy (MTA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and identify its predictors. BACKGROUND: MTA is an anatomical variant characterized by compression of left common iliac vein by the overlying right iliac artery. Over time, this leads to venous intimal scarring, blood flow stasis, and the development of deep vein thrombosis (DVT). DVT is a known risk factor for the development of CTEPH. The prevalence of this anatomical variation in patients with CTEPH is unknown. METHODS: A retrospective chart review was conducted in patients referred to Temple University Hospital's cardiac catheterization laboratory for the evaluation of CTEPH between January 2016 and June 2020. Among these patients, those who underwent invasive venography were evaluated for the presence of angiographic MTA. Multivariate regression was used to identify factors associated with presence of MTA. RESULTS: A total of 193 patients with CTEPH were referred for pulmonary angiography, of whom 148 patients underwent invasive venography. MTA was identified in 44 patients (29.7%). Factors associated with the presence of MTA were lower extremity DVT (odds ratio: 3.5; 95% confidence interval: 1.58-7.8; P = 0.002), and left lower extremity post-thrombotic syndrome (odds ratio: 2.0; 95% confidence interval: 0.98-4.1; P = 0.05). Patients with MTA were more likely to undergo pulmonary thromboendarterectomy than patients without MTA (79.5% vs 58.7%; P = 0.015). CONCLUSIONS: MTA is very common in patients with CTEPH. History of lower extremity DVT and or left lower extremity post-thrombotic syndrome was associated with the presence of MTA.

Catheter-Directed Thrombolysis of Iliocaval Thrombosis in Patients With COVID-19 Infection.

We present the characteristics and outcomes of the first 2 cases of catheter-directed thrombolysis performed in patients presenting with coronavirus disease-2019 (COVID-19)-related iliocaval thrombosis. (Level of Difficulty: Beginner.).

Invasive and noninvasive hemodynamic assessment in adults with Fontan palliation.

BACKGROUND/OBJECTIVES: Although echocardiographic-Doppler cardiac index (CI) assessment is widely used to guide heart failure management in patients with biventricular circulation, this application has not been studied in the Fontan population. The objective of this study was to: (1) determine the correlation between Doppler and cardiac catheterization CI calculation; (2) determine the association between Doppler CI and the occurrence of Fontan failure. METHODS: Retrospective review of adult Fontan patients followed at Mayo Clinic Adult Congenital Heart Disease program, 1994-2015. Inclusion criteria were: systemic left ventricle and echocardiogram and cardiac catheterization performed within the same week. Fontan failure was defined as a composite of all-cause mortality, heart transplantation listing, and palliative care. RESULTS: 59 patients (age 29±6years; men 32[54%]) underwent 97 studies. Of the 59, 41[69%] had atriopulmonary Fontan and 12 (20%) had cirrhosis. Compared to patients without cirrhosis, patients with cirrhosis had higher Doppler CI (3.6±0.6 vs 2.8±0.4L/min∗m2, p=0.039); Fick CI (3.3 [2.5-3.7] vs 2.4 [1.6-3.1] L/min/m2, p=0.028); lower systemic vascular resistance (20±3 vs 25±4 WU∗m2, p=0.04). There was a positive correlation between Doppler and Fick CI (r=0.52; p<0.0001). Fontan failure occurred in 13 patients (22%) within 7.5±2.1years. In patients without cirrhosis, Fick CI and Doppler CI <2.5L/min/m2 were associated with Fontan failure (odds ratio [OR] 1.58, p=0.046) and (OR 1.43, p=0.051) respectively. CONCLUSIONS: Doppler CI assessment in feasible in a selected group of Fontan patients and it is predictive of clinical outcomes. The application of this concept in systemic right ventricles deserves further research.

Venous congestion and pulmonary vascular function in Fontan circulation: Implications for prognosis and treatment.

BACKGROUND: Elevation in central venous pressure (CVP) plays a fundamental pathophysiologic role in Fontan circulation. Because there is no sub-pulmonary ventricle in this system, CVP also provides the driving force for pulmonary blood flow. We hypothesized that this would make Fontan patients more susceptible to even low-level elevation in pulmonary vascular resistance index (PVRI), resulting in greater systemic venous congestion and adverse outcomes. METHODS: Adult Fontan patients and controls without congenital heart disease undergoing clinical evaluation that included cardiac catheterization and echocardiography were examined retrospectively. Outcomes including all-cause mortality and the development of Fontan associated diseases (FAD, defined as protein losing enteropathy, cirrhosis, heart failure hospitalization, arrhythmia, or thromboembolism) were assessed from longitudinal assessment. RESULTS: As compared to controls (n = 82), Fontan patients (n = 164) were younger (36 vs 45 years, p < 0.001), more likely to be on anticoagulation or antiplatelet therapy, and more likely to have atrial arrhythmia or cirrhosis. There was a strong correlation between CVP and PVRI in the Fontan group (r = 0.79, p < 0.001), but there was no such relationship in controls. Elevated PVRI identified patients at increased risk for FAD (HR 1.92, 95% CI 1.39-2.41, p = 0.01), and composite endpoint of FAD and/or death (HR 1.89, 95% CI 1.32-2.53, p = 0.01) per 1 WU∗m2 increment. CONCLUSIONS: Systemic venous congestion, which is the primary factor in the pathogenesis of FAD and death, is related to even low-level abnormalities in pulmonary vascular function. Multicenter studies are needed to determine whether interventions targeting pulmonary vascular structure and function can improve outcomes in the Fontan population.

Temporal changes in liver stiffness after Fontan operation: Results of serial magnetic resonance elastography.

BACKGROUND: The relationship between temporal progression of magnetic resonance elastography derived liver stiffness (MRE-LS) and progression of Fontan associated liver disease (FALD) is unknown. To assess this relationship, we hypothesized that progression of MRE-LS correlated with progression FALD severity and clinical outcomes. METHODS: Retrospective review of Fontan patients who had >1 liver MRE, 2010-2016. Annual change in MRE-LS was the quotient of the difference between baseline and subsequent MRE-LS, and the interval between scans. RESULTS: 22 patients were enrolled; median age 29(19-38) years, 14 (64%) males and 10 (46%) with atriopulmonary Fontan. Baseline and subsequent MRE-LS values were 5.4 ±â€¯1.1 kPa and 5.8 ±â€¯0.9 kPa" for clarity, interval between scans was 25 ±â€¯5 months, and annual change in MRE-LS was 0.3 ±â€¯0.2 kPa. Temporal change in MRE-LS correlated with temporal changes in model for end-stage liver disease (MELD) score (r = 0.84, p < 0.001) and model for end-stage liver disease excluding international normalized ratio (MELD-XI) score (r = 0.75, p = 0.001). The study cohort was divided into 2 groups using the mean annual change in MRE-LS as the cut point. Groups A and B comprised of patients with annual increase in MRE-LS ≥0.3 kPa (n = 6) and <0.3 kPa (n = 16) respectively. Composite adverse event endpoint (death, heart-liver transplant listing, palliative care, hospitalization, paracentesis) was more common in Group A (4 of 6, 67%) compared to Group B (3 of 16, 19%), p = 0.13 although this did not reach statistical significance due to small sample size. CONCLUSIONS: Progression of MRE-LS correlated with clinical deterioration as measured by worsening liver disease severity scores and the occurrence of adverse events.

Prognostic Role of Hypertensive Response to Exercise in Patients With Repaired Coarctation of Aorta.

BACKGROUND: This study aimed to determine the prevalence of hypertensive response to exercise (HRE) and its association with cardiovascular adverse events (CAEs) in patients with repaired coarctation of aorta (rCOA). METHODS: We retrospectively reviewed records of adult patients with rCOA who had cardiopulmonary exercise tests (CPETs) and follow-up from 1994 to 2014 at Mayo Clinic. Patients with residual COA, defined as aortic isthmus peak velocity >2.5 m/s, were excluded. HRE was defined as peak systolic blood pressure >200 mm Hg; CAEs were defined as cardiovascular death, stroke, acute coronary syndrome, heart failure hospitalization, and left ventricular ejection fraction (LVEF) < 35%. RESULTS: One hundred thirty-eight patients (82 men [59%]) underwent 213 CPETs, with follow-up of 85 ± 13 months. Age at initial COA repair was 9 ± 3 years; age at initial CPET was 40 ± 13 years. HRE occurred in 26 (19%) patients, and 24 (92%) of the patients with HRE had normal resting blood pressure. There were no differences in age, blood pressure at rest, and CPET findings between patients with HRE and those with normotensive response to exercise. There were 28 CAEs in 24 patients (17%), and HRE was an independent risk factor for CAE (hazard ratio [HR], 1.46 [1.13-2.52]; P = 0.04). CONCLUSIONS: HRE can occur even in the setting of normal blood pressure at rest, and it is a risk factor for CAE. We speculate that patients with HRE represent a high-risk group of patients who, presumably, have occult, advanced vascular dysfunction. CPET can identify these patients. The benefit of intensive antihypertension therapy needs to be confirmed.