Biventricular performance in adults with a systemic right ventricle: new insights from myocardial work analysis.

To evaluate biventricular mechanics by means of echo-derived myocardial work (MW) analysis in patients with a systemic right ventricle (sRV). Comprehensive echo data were collected in all patients with a sRV who underwent transthoracic echocardiography at our tertiary centre between 2020 and 2021 including sRV function indices, global longitudinal strain (GLS) of right and left ventricle (RV/LV), biventricular MW, and atrial strain in those with congenitally corrected transposition of the great arteries (ccTGA). Fifty-six patients (37 [30.97-45.87]years, 59% male) and 49 healthy individuals matched per age and sex were included for comparison. Global work index (GWI:1106 [869.80-1293.10] Vs 314.2 [281.5-358.2]mmHg%, p < 0.0001) and global constructive work(GCW: 1542.50 [1338.9-1718.50] Vs 416.4 [365.70-464]mmHg%, p < 0.0001) were both increased for sRV compared to normal RV, reflecting exposition to a systemic afterload, with a contemporary raise in wasted work (GWW:197 [138.50-322.20] Vs 26.09 [17.80-43.48]mmHg%, p < 0.0001) and impaired efficiency (GWE:89 [83-93.54] Vs 93.67 [91.67-96] %, p < 0.0001). Conversely, sRV showed reduced MW indices in comparison to normal LV(p < 0.0001 for all). Non-systemic LV demonstrated normal GLS values (19.51 ± 3.9%), but reduced GWI (479 [368-665] Vs 2172 [1978-2386]mmHg%, p < 0.0001) and GCW (708 [490-815]mmHg% Vs 86.5 [59.25-118], p < 0.0001). Nevertherless, non-systemic LV showed also impaired efficiency (91 [88-94] Vs 95 [94-97]%, p < 0.0001). LVGLS values were related to RVGLS (R = 0.5, p = 0.00019), suggesting a consistent interventricular dependency. Atrial strain could be assessed in 16 out of 20 (80%) patients with ccTGA: both atria had reduced strain values compared to their normal counterparts. Moreover, pulmonary atrial strain during the reservoir phase was related to LVGWE (R = 0.58, p = 0.047) and inversely related to LVGLS (R = - 0.71, p = 0.0043). MW analysis is feasible in sRV and may provide additional clinical data. In our cohort MW revealed biventricular impairment, in particular for non-systemic LV, in spite of normal GLS values.

External Validation of a Risk Score Model for Predicting Major Clinical Events in Adults After Atrial Switch.

BACKGROUND: A risk model has been proposed to provide a patient individualized estimation of risk for major clinical events (heart failure events, ventricular arrhythmia, all-cause mortality) in patients with transposition of the great arteries and atrial switch surgery. We aimed to externally validate the model. METHODS AND RESULTS: A retrospective, multicentric, longitudinal cohort of 417 patients with transposition of the great arteries (median age, 24 years at baseline [interquartile range, 18-30]; 63% men) independent of the model development and internal validation cohort was studied. The performance of the prediction model in predicting risk at 5 years was assessed, and additional predictors of major clinical events were evaluated separately in our cohort. Twenty-five patients (5.9%) met the major clinical events end point within 5 years. Model validation showed good discrimination between high and low 5-year risk patients (Harrell C index of 0.73 [95% CI, 0.65-0.81]) but tended to overestimate this risk (calibration slope of 0.20 [95% CI, 0.03-0.36]). In our population, the strongest independent predictors of major clinical events were a history of heart failure and at least mild impairment of the subpulmonary left ventricle function. CONCLUSIONS: We reported the first external validation of a major clinical events risk model in a large cohort of adults with transposition of the great arteries. The model allows for distinguishing patients at low risk from those at intermediate to high risk. Previous episode of heart failure and subpulmonary left ventricle dysfunction appear to be key markers in the prognosis of patients. Further optimizing risk models are needed to individualize risk predictions in patients with transposition of the great arteries.

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.

Fulminant biventricular multivalvular infective endocarditis in complex congenital heart disease: a case report.

Background: Infective endocarditis (IE) is a major issue during follow-up of adults with congenital heart disease (ACHD), leading to significant mortality. Case summary: A 37-year-old woman with transposition of great arteries and previous Mustard operation developed a drug-resistant pneumonia shortly after a pacemaker implant procedure performed at a local hospital. After referral to the ACHD centre, the patient was diagnosed with multivalvular IE with biventricular involvement by methicillin-resistant Staphylococcus aureus. On admission, the patient was already in acute respiratory distress and presented both systemic and pulmonary embolization. Despite adequate treatment was promptly started, the patient developed multiorgan failure. Discussion: This case depicts a particularly aggressive of infective endocarditis forms caused with biventricular involvement and multiple embolization. Patients with congenital heart disease are at high risk of IE with adverse impact on the prognosis. Early recognition and treatment are the keys to improve prognosis. Therefore, suspicion should be high, especially following invasive procedure, which should be preferably performed at ACHD specialized centres.

Telemedicine in Adult Congenital Heart Disease: Usefulness of Digital Health Technology in the Assistance of Critical Patients.

The number of adults with congenital heart disease (ACHD) has progressively increased in recent years to surpass that of children. This population growth has produced a new demand for health care. Moreover, the 2019 coronavirus pandemic has caused significant changes and has underlined the need for an overhaul of healthcare delivery. As a result, telemedicine has emerged as a new strategy to support a patient-based model of specialist care. In this review, we would like to highlight the background knowledge and offer an integrated care strategy for the longitudinal assistance of ACHD patients. In particular, the emphasis is on recognizing these patients as a special population with special requirements in order to deliver effective digital healthcare.

Mid-term follow-up after COVID-19 vaccination in adults with CHD: a prospective study.

BACKGROUND: Long-term data on COVID-19 vaccine safety, immunogenicity, and acceptance in adults with CHD are lacking. METHODS: This is a prospective study including adults with CHD patients undergoing COVID-19 vaccination from January 2021 to June 2022. Data on adverse events, antispike IgG titre, previous or subsequent COVID-19 infection, booster doses, and patients' attitude towards vaccination were collected. RESULTS: Four hundred and ninety CHD patients (36 ± 13 years, 53% male, 94% with moderate/complex defects) were prospectively included: 433 (88%) received a Pfizer-BioNTech mRNA vaccine, 31 (6%) Moderna mRNA vaccine, 23 (5%) AstraZeneca-Oxford ChAdOx1 nCov-19 vaccine, and 3 (0.6%) Janssen Vaccine; 310 (63%) received a booster dose. Median follow-up after vaccination was 1.53 [1.41-1.58] years. No major adverse event was reported. Eighty-two fully vaccinated patients contracted COVID-19 during follow-up after a median of 5.4 [4.3-6.5] months from the last dose. One patient with Ebstein's disease died from severe COVID-19. Symptoms' duration in patients who tested positive after vaccination was significantly shorter than in the group tested positive before vaccination (5.5 [3-8] versus 9 [2.2-15] days, p = 0.04). Median antispike IgG titre measured in 280 individuals (57%) at a median of 1.4 [0.7-3.3] months from the last dose was 2381 [901-8307] BAU/ml. Sixty patients (12%) also showed positive antinucleocapsid antibodies, demonstrating previous SARS-COV2 exposure. Twenty-nine percent appeared to have concerns regarding vaccine safety and 42% reported fearing potential effects of the vaccine on their cardiac disease before discussing with their CHD cardiologist. CONCLUSION: COVID-19 vaccines appear safe in the mid-term follow-up in adults with CHD with satisfactory immunogenicity and reduction of symptoms' duration in case of infection.

Infective Endocarditis in Adults With Congenital Heart Disease: Never Stop Digging-The Role of Multimodality Imaging.

A challenging case of infective endocarditis in a young woman with repaired tetralogy of Fallot and a diagnosis of ankylosing spondylitis is described. Despite the presence of multiple confounding factors, a multidisciplinary approach with the use of multimodality cardiac imaging allowed a correct diagnosis and effective medical treatment. (Level of Difficulty: Intermediate.).

Advanced echocardiographic assessment in adults with repaired aortic coarctation: myocardial work analysis provides novel insights on left ventricular mechanics.

AIM: To analyze left ventricular (LV) mechanics through advanced echocardiography, including speckle tracking analysis and myocardial work (MW) in a cohort of adults with repaired aortic coarctation (CoA). METHODS: Data on standard echocardiography, LV speckle-tracking and MW analysis were collected in CoA patients > 18 years with no significant recoartation or valvular disease and normal LV ejection fraction at the time of the exam. MW indices were calculated using the blood pressure measured in the right arm. A group of healthy subjects with comparable sex, age and body surface area was included for comparison. RESULTS: Eighty-nine CoA patients and 70 healthy subjects were included. Patients had higher systolic blood pressure (p < 0.0001), LV mass index (p < 0.0001), left atrial volume index (p = 0.005) and E/E' ratio (p = 0.001). Despite similar LV ejection fraction, speckle tracking analysis revealed lower global longitudinal strain (GLS: - 18.3[17-19] vs - 20.7[19-22]%, p < 0.0001) and increased peak systolic dispersion (PSD: 45[40-54] vs 37.5[32-43] ms, p < 0.0001) in CoA patients. Global work index (GWI) and global constructive work were similar to healthy controls (p = 0.6 and 0.5, respectively), whereas CoA patients showed significant increased wasted work (GWW: 125[90-185] vs 89.5[64-127]mmHg%, p < 0.0001) and a mild but significant reduction in global work efficiency (GWE: 93%[92-95] vs 95%[94-97], p < 0.0001). Moreover, when stratifying for GLS values, MW analysis showed increased GWW and PSD with impaired GWE in 54(61%) patients with normal GLS compared to healthy individuals. Spearman's linear method illustrated an inverse relation between GWE and PSD (r: - 0.53, p < 0.0001), while GCW was associated with peak (r: 0.2, p = 0.01) and mean gradient across the descending aorta (r: 0.3, p = 0.004) and with systolic blood pressure (r: 0.48, p < 0.0001). PSD was the sole univariate predictor of GWE on linear regression analysis (ß: - 0.1 [- 0.16 to - 0.07], p < 0.0001), whereas female sex, SBP and gradients across the descending aorta were independently associated with higher GCW values. When CoA patients were divided based on the history of redo CoA repair and arterial hypertension, no significant differences in MW indices were found. CONCLUSIONS: MW is a novel echocardiographic tool, which provides additional information on LV performance in CoA patients over GLS allowing a more comprehensive understanding of LV dysfunction mechanisms in a setting of increased afterload.

Safety and Efficacy of Sacubitril/Valsartan in Patients With a Failing Systemic Right Ventricle: A Prospective Single-Center Study.

BACKGROUND: Sacubitril/valsartan was demonstrated to reduce hospitalization rate and mortality in patients with heart failure with reduced ejection fraction. Data on the effects of sacubitril/valsartan in patients with a systemic right ventricle are still lacking. METHODS: Patients with transposition of the great arteries following Senning/Mustard procedure or congenitally corrected transposition of the great arteries with impaired systemic right ventricle systolic function were prospectively included. Primary end points included sacubitril/valsartan safety and efficacy. Primary efficacy end points were NT-proBNP (N-terminal pro-B-type natriuretic peptide) and systolic function improvement. Secondary end points included New York Heart Association class, 6-minute walking distance, and quality of life change. RESULTS: Fifty patients (38±12 years, 60% male, 35% congenitally corrected transposition of the great arteries) were included and followed for 1 year. No major adverse events occurred. Two (4%) patients ceased treatment due to hypotension and 1 (2%) developed a nephrotic syndrome. The target dose was reached in 20 (42%) patients. NT-proBNP values decreased significantly immediately after treatment initiation, while returned to baseline at 1 year. Echocardiography showed progressive fractional area change increase (29.2±5.8 versus 34.9±5.1%; P<0.001), and right ventricle global longitudinal strain (-13.9 [-15.1, -11.8] versus -15.3 [-17.2, -13.4]%; P<0.001) and free-wall global longitudinal strain (-14.3 [-17.3, -12.3] versus -17.2 [-19.3, -15.8]%; P<0.001) raise, whereas tricuspid regurgitation severity improved only in transposition of the great arteries patients (P=0.006). Moreover, 3-dimensional echocardiography demonstrated right ventricle volumes reduction (end-diastolic volume: 181±63 versus 156±50 mL; P=0.002; end-systolic volume: 117±48 versus 89±33 mL; P<0.001), and significantly increased systemic right ventricle ejection fraction (35.6±8.1 versus 41.5±7.5%; P<0.001). Clinical improvement was suggested by New York Heart Association class change (P<0.001), increased 6-minute walking distance (425 [333, 480] versus 500 [443, 560] m; P<0.001) as well as improved quality of life at 1-year follow-up. Beneficial effects were observed irrespective of the underlying anatomy and were more pronounced in those on target dose. CONCLUSIONS: Our data showed that sacubitril/valsartan is well tolerated and is associated with systemic right ventricle remodeling and improved systolic function as well as improved clinical status, supporting its use in this complex population.

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.

Wearable cardioverter-defibrillator (life-vest): A feasible bridging treatment in adult congenital heart disease.

BACKGROUND: Wearable cardioverter-defibrillators (WCDs) are currently used in patients at temporarily heightened risk for sudden cardiac death (SCD) who are temporarily unable to receive an implantable cardioverter-defibrillator (ICD). WCD can safely record and terminate life-threatening arrhythmias through a non-invasive electrode-based system. The current clinical indications for WCD use are varied and keep evolving as experience with this technology increases. METHODS: We reviewed and explored the data behind indications for WCD use and discuss its usefulness in congenital heart disease (CHD) patients. RESULTS: We considered 8 consecutive patients (mean age 35.25 years, range 18-51 years, average duration of WCD use 4 months, range 3-6 months) with complex CHD, in which a WCD was used between June 2018 and January 2022. No sustained ventricular arrhythmias requiring shocks were recorded in the observation period. No inappropriate shocks were recorded. All the patients showed a good compliance and a very high mean wear time per day (21.2 ± 1 h a day). Four patients implanted a permanent device (3 CRT-D, 1 ICD), three underwent cardiac surgery at the end of the WCD period and one is still on the waiting list for the operation. CONCLUSIONS: Larger trial could confirm the possible conceivable benefit from an extended use of the WCD in certain populations with complex CHD as in our case series, especially in patients with life-treating ventricular arrhythmias waiting for surgery for residual cardiac defects or in the early phases following the surgical/hemodynamic interventions, patients with tachycardiomyopathy expected to improve after the arrhythmias are removed and patients awaiting implantation of an ICD at high risk due to active infection.

A new score for life-threatening ventricular arrhythmias and sudden cardiac death in adults with transposition of the great arteries and a systemic right ventricle.

AIMS: To investigate the incidence of major adverse ventricular arrhythmias and related events (MAREs) and to develop a stratification tool predicting MAREs in adults with a systemic right ventricle (sRV). METHODS AND RESULTS: In a multicentre approach, all adults (≥16 years old) with a sRV undergoing follow-up between 2000 and 2018 were identified. The incidence of MAREs, defined as sudden cardiac death, sustained ventricular tachycardia, and appropriate implantable cardioverter-defibrillator (ICD) therapy, was analysed. The association of MAREs with clinical, electrical, and echocardiographic parameters was evaluated. A total of 1184 patients (median age 27.1 years; interquartile range 19.9-34.9 years; 59% male; 70% with atrial switch repair for D-transposition of the great arteries) were included. The incidence of MAREs was 6.3 per 1000 patient-years. On multivariate analysis, age, history of heart failure, syncope, QRS duration, severe sRV dysfunction and at least moderate left ventricular outflow tract obstruction were retained in the final model with a C-index of 0.78 [95% confidence interval (CI) 0.72-0.83] and a calibration slope of 0.93 (95% CI 0.64-1.21). For every five ICDs implanted in patients with a 5-year MARE risk >10%, one patient may potentially be spared from a MARE. CONCLUSION: Sudden cardiac death remains a devastating cause of death in a contemporary adult cohort with a sRV. A prediction model based on clinical, electrocardiographic, and echocardiographic parameters was devised to estimate MARE risk and to identify high-risk patients who may benefit from primary prevention ICD implantation.

Prognostic Relevance of Thyroid Disorders in Adults With Congenital Heart Disease.

Adults with congenital heart disease (ACHD) are frequently affected by thyroid diseases (TDs). However, the clinical relevance of TD in ACHD remains unknown. We aimed to describe the prevalence of TD in the ACHD population and to ascertain whether TD are associated with worse outcome. Patients with ACHD >18 years attending our tertiary center for a day-case between 2014 and 2019 were included. Clinical data between patients' first visit and December 2020 were collected. Primary end point was a combination of death, hospitalization for heart failure (HF), and new-onset of arrhythmic events. Secondary end points were each part of the primary outcome as separate end points. A total of 495 patients with ACHD (32.2 [24.5 to 45.6] years; 54% women) were included. Median follow-up was 9.4 (4.5 to 13.1) years. The prevalence of TD was 30%. TD group showed worse clinical status, as demonstrated by N-terminal pro b-type natriuretic peptide values (243.5 [96.5 to 523] vs 94 [45 to 207] pg/ml, p <0.001) and New York Heart Association class (27% vs 13% in class III to IV, p <0.0001) with higher incident rate of adverse events at follow-up (4.45 [3.43 to 5.69] % vs 1.29[0.94 to 1.75] % per person-year, p <0.001). TD were independently associated with higher risk of death (hazard ratio [HR] 4.1, p = 0.009), arrhythmic events (HR 3.8, p <0.0001), and hospitalization for HF (HR 8.02, p <0.0001). There was a fourfold increased risk of primary end point in the TD group even after propensity score matching for clinical variables including age, gender, disease complexity, physiological stage, previous palliative surgery, ventricular function, pulmonary arterial hypertension, cyanosis, and presence of systemic right ventricle (HR 4.47, p <0.0001). In conclusion, TD are predictive of adverse outcome in the ACHD population. Routine screening of thyroid function during follow-up in this population may be helpful to identify those with higher risk of death, arrhythmias, and HF.

Chylothorax Due to Superior Vena Cava Obstruction in a Patient With Complex Congenital Heart Disease.

Obstruction of the superior vena cava represents an under-recognized cause of chylothorax in the adult population. Our case report describes the successful conservative management of chylothorax due to bilateral superior vena cava obstruction in an adult patient with complex congenital heart disease. (Level of Difficulty: Beginner.).

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.