End-stage heart failure in congenitally corrected transposition of the great arteries: a multicentre study.

BACKGROUND AND AIMS: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear. METHODS: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death. RESULTS: From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P < .001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome. CONCLUSIONS: Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery.

Right Heart Thrombus in Transit: Unusual Echocardiographic Finding in a patient with Acute Pulmonary Embolism.

Eighty year old male patient with heart failure preserved ejection fraction (EF), Obstructive sleep apnea, peripherovascular disease admitted with increasing shortness of breath and found with pulmonary emboli. Baseline 2D-echo-cardiogram performed demonstrated preserved ejection fraction and a right thrombus in transit. Anticoagulation with weight based-low molecular weight heparin was given for six days. Follow-up echo performed demonstrated complete dissolution of right heart thrombi. Since there was complete dis- solution of thrombi seen on right atrium, anticoagulation with Rivaroxaban was given instead.

Right Heart Thrombus in Transit: Unusual Echocardiographic Finding in a patient with Acute Pulmonary Embolism.

Eighty year old male patient with heart failure preserved ejection fraction (EF), Obstructive sleep apnea, peripherovascular disease admitted with increasing shortness of breath and found with pulmonary emboli. Baseline 2D-echocardiogram performed demonstrated preserved ejection fraction and a right thrombus in transit. Anticoagulation with weight based-low molecular weight heparin was given for six days. Follow-up echo performed demonstrated complete dissolution of right heart thrombi. Since there was complete dissolution of thrombi seen on right atrium, anticoagulation with Rivaroxaban was given instead.

Case Report: Prothesis-patient mismatch after aortic valve replacement.

Valve replacement is the standard surgical treatment of diseased valves that cannot be repaired. The main goal of replacement is to exchange the diseased valve with one that has the engineering and hemodynamics as close as possible to the disease free native valve. However due to mechanical and fluid dynamic constraints all prosthetic heart valves (PHVs) are smaller than normal and thus are inherently stenotic. This represents a challenge when it comes time to replace a valve. The correct valve with the correct and matching profile has to be selected before the procedure to avoid possible complications. It is well recognized that patients are also prone to patient-prosthesis mismatch at long term which could have consequences in the clinical outcomes (1). The evaluation of patient-prosthesis mismatch (PPM) has not been sufficiently emphasized in common practice. Failure to recognize this fact may lead to significant hemodynamic impairment and worsening of the clinical status over the time. Making efforts to identifying patients at risk may decrease the prevalence of PPM, the economic impact to our health system, the morbidity and mortality involved in these cases as well as creates efforts to standardized pre-operative protocols to minimized risk of PPM. We present a case of a 78 years old male patient who underwent aortic valve replacement due severe aortic stenosis, afterwards his clinical course got complicated with several admissions for shortness of breath and decompensated congestive heart failure (CHF).

COVID-19-Related Thrombotic and Bleeding Events in Adults With Congenital Heart Disease.

Background: Altered coagulation is a striking feature of COVID-19. Adult patients with congenital heart disease (ACHD) are prone to thromboembolic (TE) and bleeding complications. Objectives: The purpose of this study was to investigate the prevalence and risk factors for COVID-19 TE/bleeding complications in ACHD patients. Methods: COVID-19-positive ACHD patients were included between May 2020 and November 2021. TE events included ischemic cerebrovascular accident, systemic and pulmonary embolism, deep venous thrombosis, myocardial infarction, and intracardiac thrombosis. Major bleeding included cases with hemoglobin drop >2 g/dl, involvement of critical sites, or fatal bleeding. Severe infection was defined as need for intensive care unit, endotracheal intubation, renal replacement therapy, extracorporeal membrane oxygenation, or death. Patients with TE/bleeding were compared to those without events. Factors associated with TE/bleeding were determined using logistic regression. Results: Of 1,988 patients (age 32 [IQR: 25-42] years, 47% male, 59 ACHD centers), 30 (1.5%) had significant TE/bleeding: 12 TE events, 12 major bleeds, and 6 with both TE and bleeding. Patients with TE/bleeding had higher in-hospital mortality compared to the remainder cohort (33% vs 1.7%; P < 0.0001) and were in more advanced physiological stage (P = 0.032) and NYHA functional class (P = 0.01), had lower baseline oxygen saturation (P = 0.0001), and more frequently had a history of atrial arrhythmia (P < 0.0001), previous hospitalization for heart failure (P < 0.0007), and were more likely hospitalized for COVID-19 (P < 0.0001). By multivariable logistic regression, prior anticoagulation (OR: 4.92; 95% CI: 2-11.76; P = 0.0003), cardiac injury (OR: 5.34; 95% CI: 1.98-14.76; P = 0.0009), and severe COVID-19 (OR: 17.39; 95% CI: 6.67-45.32; P < 0.0001) were independently associated with increased risk of TE/bleeding complications. Conclusions: ACHD patients with TE/bleeding during COVID-19 infection have a higher in-hospital mortality from the illness. Risk of coagulation disorders is related to severe COVID-19, cardiac injury during infection, and use of anticoagulants.

Rheumatoid pericardial mass producing right ventricle inflow tract restriction: a case report.

Rheumatoid arthritis (RA) is an autoimmune disease that causes chronic inflammation of the joints affecting multiple organs in the body. Pericardial disease is a relative common systemic manifestation of RA. A small number of cases are described in the literature related to pericardial involvement in RA. We described a case of a 69 year-old-male with chronic RA who presented with progressively worsening shortness of breath at exertion. Previous echocardiogram performed one year ago had normal ejection fraction and no structural abnormalities. Laboratories showed no significant abnormalities. The echocardiogram and MRI performed at the time of evaluation, revealed a mass in the right ventricle free wall pericardia. On the pathology report the pericardial mass resection showed mild chronic inflammation, extensive fibrosis, fat necrosis and branchial cyst surrounded with mild chronic inflammation. After surgical resection the patient improved clinically.

Long-Term Outcomes After Atrial Switch Operation for Transposition of the Great Arteries.

BACKGROUND: For patients with d-loop transposition of the great arteries (d-TGA) with a systemic right ventricle after an atrial switch operation, there is a need to identify risks for end-stage heart failure outcomes. OBJECTIVES: The authors aimed to determine factors associated with survival in a large cohort of such individuals. METHODS: This multicenter, retrospective cohort study included adults with d-TGA and prior atrial switch surgery seen at a congenital heart center. Clinical data from initial and most recent visits were obtained. The composite primary outcome was death, transplantation, or mechanical circulatory support (MCS). RESULTS: From 1,168 patients (38% female, age at first visit 29 ± 7.2 years) during a median 9.2 years of follow-up, 91 (8.8% per 10 person-years) met the outcome (66 deaths, 19 transplantations, 6 MCS). Patients experiencing sudden/arrhythmic death were younger than those dying of other causes (32.6 ± 6.4 years vs 42.4 ± 6.8 years; P < 0.001). There was a long duration between sentinel clinical events and end-stage heart failure. Age, atrial arrhythmia, pacemaker, biventricular enlargement, systolic dysfunction, and tricuspid regurgitation were all associated with the primary outcome. Independent 5-year predictors of primary outcome were prior ventricular arrhythmia, heart failure admission, complex anatomy, QRS duration >120 ms, and severe right ventricle dysfunction based on echocardiography. CONCLUSIONS: For most adults with d-TGA after atrial switch, progress to end-stage heart failure or death is slow. A simplified prediction score for 5-year adverse outcome is derived to help identify those at greatest risk.

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.