Global Longitudinal Strain of the Systemic Ventricle Is Correlated with Plasma Galectin-3 and Predicts Major Cardiovascular Events in Adult Patients with Congenital Heart Disease.

Backround and Objective: We sought to assess in adult congenital heart disease (ACHD) patients the prognostic value of plasma galectin-3 (Gal-3) levels and systemic ventricular global longitudinal strain (SV GLS) as well as their association with NTproBNP and arrhythmogenesis. Materials and Methods: We studied 58 patients (26 men, mean age 37 ± 16.8 years) with various congenital heart diseases. Patients underwent echocardiogram, 24 h ambulatory ECG monitoring, while NTproBNP and Gal-3 were measured. They were followed up (median of 790.5 days -IQR 350.3 days) and major cardiovascular events (MACE) were recorded. Results. Mean Gal-3 levels were 17.07 ± 6.38 ng/m. Plasma Gal-3 was correlated with LogNTproBNP (r = 0.456, p = 0.001).Gal-3 levels associated with supraventricular tachycardia (SVT) (p < 0.001) and ventricular tachycardia (VT) (p < 0.001), but was not associated with MACE (HR 1.018, 95% CI 0.944-1.098, p = 0.641).Mean SVGLS in patients with systemic left ventricle was -15.91% ± 4.09%, which was significantly lower compared to patients with systemic right ventricle and patients with single ventricle (-11.42% ± 3.37% and -11.9% ± 5.06%, respectively, p = 0.021).SV GLS correlated with plasma Gal-3 (r = 0.313, p = 0.027) and logNTproBNP (r = 0.479, p < 0.001). SVGLS correlated with VT arrhythmias (p = 0.004). NTproBNP predicted MACE (AUC 0.750, p = 0.03). SVGLS also predicted MACE (AUC 0.745, p = 0.03. In multivariate analysis, SVGLS and logNTproBNP maintained their predictive value (p = 0.004 and p = 0.009, respectively) Conclusion: In ACHD patients, SV GLS was found to predict MACE independently from NTproBNP and correlated with VT. Gal-3 correlated with NTproBNP and SVGLS as well as SVT and VT, but has not been shown to bear significant prognostic potential.

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.

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.

Pregnancy in a Patient With Tetralogy of Fallot and Borderline Pulmonary Arterial Hypertension.

Management of pregnancy in patients with complex congenital heart disease and pulmonary arterial hypertension has always been a challenge. We are presenting a patient with complex congenital heart disease and borderline pulmonary arterial hypertension who complicated with pulmonary embolism during pregnancy. (Level of Difficulty: Beginner.).

Atrial Fibrillation or Flutter During Pregnancy in Patients With Structural Heart Disease: Data From the ROPAC (Registry on Pregnancy and Cardiac Disease).

OBJECTIVES: Atrial fibrillation (AF)/atrial flutter (AFL) during pregnancy in these women is associated with adverse outcome of pregnancy. BACKGROUND: The incidence, timing, and consequences of AF or AFL during pregnancy in patients with heart disease are not well known. METHODS: Between January 2008 to June 2011, 60 hospitals in 28 countries prospectively enrolled 1,321 pregnant women with congenital heart disease, valvular heart disease, ischemic heart disease, or cardiomyopathy in the ROPAC (Registry of Pregnancy and Cardiac Disease). We studied the incidence, onset, and predictors of AF/AFL during pregnancy and assessed the pregnancy outcome. An overview of the existing literature is provided. RESULTS: Seventeen women (1.3%) developed AF/AFL during pregnancy, mainly in the second trimester (61.5%). Univariable analysis identified the following pre-pregnancy risk factors for AF/AFL in pregnancy: AF/AFL before pregnancy (odds ratio [OR]: 7.1, 95% confidence interval [CI]: 1.5 to 32.8); mitral valvular heart disease (OR: 6.9, 95% CI: 2.6 to 18.3); beta-blocker use (OR: 3.3, 95% CI: 1.2 to 9.0); and left-sided lesions (OR: 2.9, 95% CI: 1.0 to 8.3). Maternal mortality was higher in women with than in women without AF/AFL (11.8% vs. 0.9%; p = 0.01), although heart failure was not seen more often. Low birth weight (<2,500 g) occurred more often in women with than in women without AF/AFL (35% vs. 14%; p = 0.02). CONCLUSIONS: AF/AFL occurs in 1.3% of pregnant patients with structural heart disease with a peak at the end of the second trimester. AF/AFL during pregnancy in cardiac patients is associated with unfavorable maternal outcome and also has an impact on fetal birth weight.

Assessing the failing systemic right ventricle.

The purpose of this article is to address main clinical issues and the way to assess right ventricular (RV) function of adult patients with transposition complexes and systemic RV failure. Echo, cardiopulmonary exercise test, radionuclide ventriculography, cardiac magnetic resonance imaging, multislice computed tomography, and cardiac catheterization may be performed in adult patients with transposition complexes to evaluate clinical status and RV function. Echo is an everyday clinical tool that can give valuable information on RV function, tricuspid regurgitation, concomitant lesions, or postoperative complications. Cardiac magnetic resonance is the gold standard in the estimation of RV volumes and function. Cardiopulmonary exercise test can provide objective evaluation of patients' clinical status. Other imaging modalities are useful when appropriate. In conclusion, for all transposition complexes adult patients should undergo a regular follow-up to recognize early signs of systemic RV failure.