Nationwide trends of balloon pulmonary angioplasty and pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension (2012-2019).

Chronic thromboembolic pulmonary hypertension (CTEPH) is a sequela of a pulmonary embolus that occurs in approximately 1%-3% of patients. Pulmonary thromboendoarterectomy (PTE) can be a curative procedure, but balloon pulmonary angioplasty (BPA) has emerged as an option for poor surgical candidates. We used the National Inpatient Sample to query patients who underwent PTE or BPA between 2012 and 2019 with CTEPH. The primary outcome was a composite of in-hospital mortality, myocardial infarction, stroke, tracheostomy, and prolonged mechanical ventilation. Outcomes were compared between low- and high-volume centers, defined as 5 and 10 procedures per year for BPA and PTE, respectively. During our study period, 870 BPA and 2395 PTE were performed. There was a 328% relative increase in the number of PTE performed during the study period. Adverse events for BPA were rare. There was an increase in the primary composite outcome for low-volume centers compared to high-volume centers for PTE (24.4% vs. 12.1%, p = 0.003). Patients with hospitalizations for PTE in low-volume centers were more likely to have prolonged mechanical ventilation (20.0%% vs. 7.2%, p < 0.001) and tracheostomy (7.8% vs. 2.6%, p = 0.017). In summary, PTE rates have been rising over the past 10 years, while BPA rates have remained stable. While adverse outcomes are rare for BPA, patients with hospitalizations at low-volume centers for PTE were more likely to have adverse outcomes. For patients undergoing treatment of CTEPH with BPA or PTE, referral to high-volume centers with multidisciplinary teams should be encouraged for optimal outcomes.

Diagnosis and Management of Congenital Coronary Artery Fistulas in Adults.

PURPOSE OF REVIEW: This review describes the presentation, diagnosis, and management of congenital coronary artery fistulas (CAFs) in adults. RECENT FINDINGS: CAFs are classified as coronary-cameral or coronary arteriovenous fistulas. Fistulous connections at the distal coronary bed are more likely to be aneurysmal with higher risk of thrombosis and myocardial infarction (MI). Medium-to-large or symptomatic CAFs can manifest as ischemia, heart failure, and arrhythmias. CAF closure is recommended when there are attributable symptoms or evidence of adverse coronary remodeling. Closure is usually achievable using transcatheter techniques, though large fistulas may require surgical ligation with bypass. Given their anatomic complexity, cardiac CT with multiplanar 3-D reconstruction can enhance procedural planning of CAF closure. Antiplatelet and anticoagulation are essential therapies in CAF management. CAFs are rare cardiac anomalies with variable presentations and complex anatomy. CAF management strategies include indefinite medical therapy, percutaneous or surgical CAF closure, and lifelong patient surveillance.

Trends in the Incidence of Cardiogenic Shock, and Utilization of Mechanical Circulatory Support in Myocarditis: Insights from the National Inpatient Sample 2016 to 2019.

A subset of patients with myocarditis present with cardiogenic shock. There is a lack of contemporary data assessing the use of mechanical circulatory support (MCS) in these patients. Myocarditis hospitalizations were analyzed using the National Inpatient Sample between 2016 and 2019. Characteristics of patients with and without cardiogenic shock were assessed. Trends in mortality, MCS, right-sided cardiac catheterization (RHC) and endomyocardial biopsy were evaluated. The impact of RHC on consequent MCS and mortality was studied. A total of 38,300 hospitalizations for myocarditis were included in the study, of which 3,490 hospitalizations (9.1%) had cardiogenic shock. Patients with cardiogenic shock were older (p <0.001) and had more chronic kidney disease and atrial fibrillation. Between 2016 and 2019, there was an increase in myocarditis admissions but no difference in rates of cardiogenic shock and mortality and the use of extracorporeal membrane oxygenation, percutaneous ventricular assist devices, intra-aortic balloon pumps, left ventricular assist devices, and cardiac transplant. The most common form of MCS used in myocarditis was extracorporeal membrane oxygenation. The rates of RHC (p = 0.02) and endomyocardial biopsy (p = 0.03) increased over time. Patients who underwent RHC were more likely to receive mechanical support, and in patients with shock, RHC was associated with lower mortality (adjusted odds ratio 0.34, p <0.01). Myocarditis admissions increased over time but with no increase in the rates of cardiogenic shock and MCS. In patients with cardiogenic shock, RHC resulted in lower mortality.

A mimicker of constriction: a case report of a rare case of cardiac angiosarcoma.

Background: Cardiac angiosarcoma is an exceptionally rare primary malignant tumour with an aggressive course and typically poor prognosis. Diagnosis is difficult, and patients often present with metastatic disease. We report the rare case of a patient with cardiac angiosarcoma who presents with constrictive physiology due to tumour encasement. Case summary: A 65-year-old female with a past medical history of Hodgkin's lymphoma and limited scleroderma presented with progressive dyspnoea on exertion. Multimodality imaging and haemodynamics with echocardiography, cardiac magnetic resonance imaging (MRI), and cardiac catheterization showed findings of constrictive physiology. Cardiac MRI showed areas of pericardial enhancement, so she was initially started on colchicine, prednisone, and mycophenolate mofetil to treat pericardial inflammation. However, her symptoms progressed, and she underwent pericardiectomy with cardiac surgery. Pericardium was noted to be thickened and a mass-like substance was densely adherent and potentially invading the heart itself and could not be dissected free. Surgical pathology showed features consistent with epithelioid angiosarcoma. Patient had rapid progression of her disease and was started on chemotherapy. Her course, however, was complicated by acute gastrointestinal bleeding, atrial fibrillation with rapid rates, and persistent volume overload. She elected for comfort measures and passed away shortly after her diagnosis. Discussion: Our case shows an extremely rare diagnosis, cardiac angiosarcoma, presenting with typical findings of constrictive physiology. The case shows the typical features of constrictive physiology using multimodality imaging and haemodynamics and emphasizes the need to always think broadly in creating a differential diagnosis for constriction to ensure that rare diseases are considered.

Percutaneous closure of giant aneurysmal coronary artery-to-coronary sinus fistulae with guidance from three-dimensional printed models: a case series.

Background: Coronary artery fistulae are abnormal communications of coronary arteries with systemic vasculature, pulmonary vasculature, or cardiac chambers. Use of multimodality imaging can be paramount to understanding anatomical and functional features of these complex vascular lesions, therefore optimizing success of potential curative interventions. Case summary: We present two patients with incidentally discovered giant aneurysmal coronary arteries with distal fistulous connections to the coronary sinus, which were successfully closed percutaneously with Amplatzer Septal Occluders using the assistance of three-dimensional (3D) printed heart models. Conclusion: Computed tomography-guided reconstruction with 3D multiplanar, multicolour printed models can help augment visuospatial understanding of the size, origin, course, and drainage of giant aneurysmal coronary artery-to-coronary sinus fistulae, and with manual bench testing can assist with choosing accurately sized and shaped devices for closure.

Thirty-Day Readmission Rates after Takotsubo Syndrome with or without Malignancy: A Nationwide Readmissions Database Analysis.

The association between malignancy and readmission after Takotsubo syndrome (TTS) hospitalization has not been fully described. We sought to examine the rates, cause, and cost of 30-day readmissions of TTS, with or without malignancy, by utilizing Nationwide Readmissions Databases from 2010 to 2014. We identified 61,588 index hospitalizations for TTS. TTS patients with malignancy tended to be older (70.6 ± 0.2 vs. 66.1 ± 0.1, p < 0.001), and the overall burden of comorbidities was higher than in those without malignancy. TTS patients with malignancy had significantly higher 30-day readmission rates than those without malignancy (15.9% vs. 11.0%; odds ratio (OR), 1.35; 95% confidence interval (CI), 1.18-1.56). Non-cardiac causes were the most common causes of readmission for TTS patients with malignancy versus without malignancy (75.5% vs. 68.1%, p < 0.001). The 30-day readmission rate due to recurrent TTS was very low in both groups (0.4% and 0.5%; p = 0.47). The total costs were higher by 25% (p < 0.001) in TTS patients with vs. without malignancy. In summary, among patients hospitalized with TTS, the presence of malignancy was associated with increased risk of 30-day readmission and increased costs. These findings highlight the importance of optimized management for TTS patients with malignancy.

COVID-19 in Adults With Congenital Heart Disease.

BACKGROUND: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications. OBJECTIVES: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes. METHODS: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined. RESULTS: From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not. CONCLUSIONS: COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity.

Associations Between Hospital Length of Stay, 30-Day Readmission, and Costs in ST-Segment-Elevation Myocardial Infarction After Primary Percutaneous Coronary Intervention: A Nationwide Readmissions Database Analysis.

Background Readmission after ST-segment-elevation myocardial infarction (STEMI) poses an enormous economic burden to the US healthcare system. There are limited data on the association between length of hospital stay (LOS), readmission rate, and overall costs in patients who underwent primary percutaneous coronary intervention for STEMI. Methods and Results All STEMI hospitalizations were selected in the Nationwide Readmissions Database from 2010 to 2014. From the patients who underwent primary percutaneous coronary intervention, we examined the 30-day outcomes including readmission, mortality, reinfarction, repeat revascularization, and hospital charges/costs according to LOS (1-2, 3, 4, 5, and >5 days) stratified by infarct locations. The 30-day readmission rate after percutaneous coronary intervention for STEMI was 12.0% in the anterior wall (AW) STEMI group and 9.9% in the non-AW STEMI group. Patients with a very short LOS (1-2 days) were readmitted less frequently than those with a longer LOS regardless of infarct locations. However, patients with a very short LOS had significantly increased 30-day readmission mortality versus an LOS of 3 days (hazard ratio, 1.91; CI, 1.16-3.16 [P=0.01]) only in the AW STEMI group. Total costs (index admission+readmission) were the lowest in the very short LOS cohort in both the AW STEMI group (P<0.001) and the non-AW STEMI group (P<0.001). Conclusions For patients who underwent primary percutaneous coronary intervention for STEMI, a very short LOS was associated with significantly lower 30-day readmission and lower cumulative cost. However, a very short LOS was associated with higher 30-day mortality compared with at least a 3-day stay in the AW STEMI cohort.

Major aortopulmonary collateral arteries in a case of unrepaired tricuspid and pulmonary atresia with single ventricle physiology.

Tricuspid and pulmonary atresia with single ventricle physiology and major aortopulmonary collateral arteries (MAPCAs) is a complex cyanotic congenital heart disease with heterogeneous pulmonary artery morphology and arborization. The complex anatomy and physiology, coupled with a dearth of existing literature, pose imitable challenges to treatment. Although the exact surgical algorithm is still unclear, the goal is a well-developed, low-resistance pulmonary vascular bed. A precise understanding of the blood supply to each lung is a requisite for successful surgery, and a multimodality and multidisciplinary approach is compulsory. Herein, we describe a case of tricuspid and pulmonary atresia with single ventricle, MAPCAs and aortopulmonary collateral arteries.

Comprehensive Diagnostic Evaluation of Cardiovascular Physiology in Patients With Pulmonary Vascular Disease: Insights From the PVDOMICS Program.

BACKGROUND: Invasive hemodynamic evaluation through right heart catheterization plays an essential role in the diagnosis, categorization, and risk stratification of patients with pulmonary hypertension. METHODS: Subjects enrolled in the PVDOMICS (Redefining Pulmonary Hypertension through Pulmonary Vascular Disease Phenomics) program undergo an extensive invasive hemodynamic evaluation that includes repeated measurements at rest and during several provocative physiological challenges. It is a National Institutes of Health/National Heart, Lung, and Blood Institute initiative to reclassify pulmonary hypertension groups based on clustered phenotypic and phenomic characteristics. At a subset of centers, participants also undergo an invasive cardiopulmonary exercise test to assess changes in hemodynamics and gas exchange during exercise. CONCLUSIONS: When coupled with other physiological testing and blood -omic analyses involved in the PVDOMICS study, the comprehensive right heart catheterization protocol described here holds promise to clarify the diagnosis and clustering of pulmonary hypertension patients into cohorts beyond the traditional 5 World Symposium on Pulmonary Hypertension groups. This article will describe the methods applied for invasive hemodynamic characterization in the PVDOMICS program. Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT02980887.

Heart failure in adult congenital heart disease: tetralogy of Fallot.

The number of rTOF patients who survive into adulthood is steadily rising, with currently more than 90% reaching the third decade of life. However, rTOF patients are not cured, but rather have a lifelong increased risk for cardiac and non-cardiac complications. Heart failure is recognized as a significant complication. Its occurrence is strongly associated with adverse outcome. Unfortunately, conventional concepts of heart failure may not be directly applicable in this patient group. This article presents a review of the current knowledge on HF in rTOF patients, including incidence and prevalence, the most common mechanisms of heart failure, i.e., valvular pathologies, shunt lesions, left atrial hypertension, primary left heart and right heart failure, arrhythmias, and coronary artery disease. In addition, we will review information regarding extracardiac complications, risk factors for the development of heart failure, clinical impact and prognosis, and assessment possibilities, particularly of the right ventricle, as well as management strategies. We explore potential future concepts that may stimulate further research into this field.

Tricuspid Stenosis in Pregnancy: A Valve-in-Valve Conundrum.

Redo tricuspid valve replacement has high surgical operative mortality. Transcatheter valve-in-valve provides a viable option for valve replacement. We discuss the decision-making process involved in performing transcatheter tricuspid valve-in-valve replacement in a 23-week pregnant woman with multiple comorbidities and symptomatic severe bioprosthetic stenosis. (Level of Difficulty: Intermediate.).

Predictors of Late Mortality in D-Transposition of the Great Arteries After Atrial Switch Repair: Systematic Review and Meta-Analysis.

Background Existing data on predictors of late mortality and prevention of sudden cardiac death after atrial switch repair surgery for D-transposition of the great arteries (D-TGA) are heterogeneous and limited by statistical power. Methods and Results We conducted a systematic review and meta-analysis of 29 observational studies, comprising 5035 patients, that reported mortality after atrial switch repair with a minimum follow-up of 10 years. We also examined 4 additional studies comprising 105 patients who reported rates of implantable cardioverter-defibrillator therapy in this population. Average survival dropped to 65% at 40 years after atrial switch repair, with sudden cardiac death accounting for 45% of all reported deaths. Mortality was significantly lower in cohorts that were more recent and operated on younger patients. Patient-level risk factors for late mortality were history of supraventricular tachycardia (odds ratio [OR] 3.8, 95% CI 1.4-10.7), Mustard procedure compared with Senning (OR 2.9, 95% CI 1.9-4.5) and complex D-TGA compared with simple D-TGA (OR 4.4, 95% CI 2.2-8.8). Significant risk factors for sudden cardiac death were history of supraventricular tachycardia (OR 4.7, 95% CI 2.2-9.8), Mustard procedure (OR 2.2, 95% CI 1.1-4.1), and complex D-TGA (OR 5.7, 95% CI 1.8-18.0). Out of a total 124 implantable cardioverter-defibrillator discharges over 330 patient-years in patients with implantable cardioverter-defibrillators for primary prevention, only 8% were appropriate. Conclusions Patient-level risk of both mortality and sudden cardiac death after atrial switch repair are significantly increased by history of supraventricular tachycardia, Mustard procedure, and complex D-TGA. This knowledge may help refine current selection practices for primary prevention implantable cardioverter-defibrillator implantation, given disproportionately high rates of inappropriate discharges.

"Treat-to-close": Non-repairable ASD-PAH in the adult: Results from the North American ASD-PAH (NAAP) Multicenter Registry.

BACKGROUND: Adults presenting with an unrepaired atrial septal defect and pulmonary arterial hypertension (ASD-PAH) are typically classified as "correctable" or "non-correctable". The use of directed PAH medical therapy in non-correctable ASD-PAH leading to favorable closure candidacy, repair status and long-term follow-up is not well studied. We therefore sought to characterize response to PAH targeted therapy in 'non-correctable' ASD-PAH. METHODS AND RESULTS: Nine North American tertiary care centers submitted retrospective data from adults with unrepaired ASD-PAH that did not meet recommendations for repair at initial presentation (1996-2017). Sixty-nine patients (women 51(74%), 40 ±â€¯15 years, mean pulmonary artery pressure (mPA) 51 ±â€¯13 mm Hg, pulmonary vascular resistance (PVR) 8.7 ±â€¯4.9 Wood units, Qp:Qs 1.6 ±â€¯0.4) were enrolled. All patients were prescribed PAH targeted therapy and late shunt repair occurred in 19(28%) (Women 15(29%) vs. Men 4(22%), p = 0.6). At late follow-up (4.4 ±â€¯2.9 years) 6-minute walk test distance (6MWTD) was significantly better in the group that underwent repair (486 ±â€¯89 m vs. 375 ±â€¯139 m, p < 0.05). Transthoracic echo showed significant improvement in right ventricular (RV) function (severe dysfunction in repaired 8(40%) vs. unrepaired groups 35(69%), p < 0.05). Divergent survival curves suggest that with larger studies and more follow-up, differences in survival between repaired and unrepaired groups may be important. (repaired: 17(94%) vs. unrepaired: 32(81%), p = 0.18). CONCLUSIONS: This is the first and largest multicenter study evaluating the "treat-to-close" approach in non-correctable ASD-PAH. Our new data supports further study of this strategy in patients who have reversibility of PAH in response to targeted therapy. We demonstrate that in the carefully selected patient with non-correctable ASD-PAH, successful shunt repair is possible if post-therapy PVR is ≤6.5 Wood units. Patients who underwent repair had improved RV function following PAH targeted therapy. Divergent survival curves suggest that with further study, defect repair may affect medium-term to late survival.