Outcomes of Transannular Repair of Tetralogy of Fallot With a Contegra® Monocuspid Patch.

BACKGROUND: Surgical repair of tetralogy of Fallot (ToF) depends on the anatomical variations of the heart defect. A group of patients with a hypoplastic pulmonary valve annulus required a transannular patch. This study aimed to evaluate the early and late outcomes of ToF repair with a transannular Contegra® monocuspid patch in a single center. METHODS: A retrospective review of medical records was conducted. This study included 224 children with a median age of 13 months who underwent ToF repair with a Contegra® transannular patch in over 20 years of observation. The primary outcomes were hospital mortality and need for early reoperations. The secondary outcomes were late death and event-free survival. RESULTS: The hospital mortality in our group was 3.1%, whereas two patients required early reoperation. Three patients were excluded from the study because follow-up data were not available. In the remaining group of patients (212 patients), the median follow-up was 116 (range, 1-206) months. One patient died because of sudden cardiac arrest at home six months after surgery. Event-free survival was observed in 181 patients (85.4%), whereas the remaining 30 patients (14.1%) required graft replacement. The median time to reoperation was 99 (range, 4-183) months. CONCLUSIONS: Although surgical treatment of ToF has been performed for more than 60 years worldwide, the optimal approach in children with a hypoplastic pulmonary valve annulus remains debatable. Among options, the Contegra® monocuspid patch can be effectively used in transannular repair of ToF with good long-term results.

Surgical Outcomes of Total Anomalous Pulmonary Venous Connection Repair.

Background and Objectives: Over the years, surgical repair of total anomalous pulmonary venous connection (TAPVC) outcomes have improved, however, morbidity and mortality still remain significant. This study aims to assess the early and long-term outcomes of surgical treatment of TAPVC children, operated on between 2006 and 2016, in one pediatric center in Poland. Materials and Methods: Diagnostics, surgical treatment, and follow-up data from 83 patients were collected. In addition, survival and risk factor analyses, control echocardiographic, and electrocardiographic examinations were performed. Results: In the analyzed group (n = 83), there were seven hospital deaths (within 30 days after the operation) (8.4%) and nine late deaths (10.8%). The mean follow-up time was 5.5 years, and, for patients who survived, it was 7.1 years. The mean survival time in patients with completed follow-up (n = 70) was 10.3 years; the overall five-year survival rate was 78.4%. Independent mortality risk factors were type I TAPVC, single ventricle physiology, time from admission to operation, intensive care unit stay, postoperative hospital stay, and temporary kidney insufficiency requiring dialysis. Conclusions: The presence of single ventricle physiology and the supracardiac subtype of TAPVC might be negative prognostic factors, while normal heart physiology presents with good post-repair results. This study indicates that cardiac arrhythmias may occur. Morbidity and mortality, related to surgical TAPVC correction, still remain significant.

Current applications and outcomes of venoarterial extracorporeal membrane oxygenation based on 6 years of experience: risk factors for in­hospital mortality.

INTRODUCTION: Data regarding venoarterial extracorporeal membrane oxygenation (VA ECMO) as a temporary circulatory support in cardiogenic shock (CS) for Central Europe are scarce. OBJECTIVES: The aim of the study was to disclose indications, in-hospital, and long-term (1-year) mortality along with risk factors. PATIENTS AND METHODS: The study is a retrospective investigation of patients who underwent VA ECMO for CS at a cardiosurgical tertiary center, from January 2013 to June 2018. A broad spectrum of pre- and postimplantation factors was tested using univariable analysis. RESULTS: A total of 198 patients met the inclusion criteria. The median (interquartile range) duration of support was 207 (91­339) hours, with no significant disparity among hospital survivors and nonsurvivors (P = 0.09). A total of 40.4% of patients died during ECMO support, while the joined in-hospital and 6-month mortality progressed to 65.2%, and 1-year mortality to 67.2%; 9% underwent a subsequent heart transplantation. Main adverse events were bleeding (76%), infection (56%), neurologic injury (15%), and limb ischemia (15%). Multiorgan failure was the most decisive risk factor of in-hospital mortality (odds ratio, 4.45; P <⁠0.001). Patients with postcardiotomy CS had a significantly lower out-of-hospital survival rate than the nonsurgical group (32.3% vs 45%; log-rank P = 0.037). CONCLUSION: The study showed survival benefit, despite frequent complications. The protocol focusing on proper candidate selection and timing can positively impact patient survival. Additional risk reduction can be achieved with a further increase of the team experience with ECMO.

Transcatheter pulmonary denervation in patients with left heart failure with reduced ejection fraction and combined precapillary and postcapillary pulmonary hypertension: A prospective single center experience.

OBJECTIVES: The present study was a prospective, single-center, single-arm study to investigate the efficacy of transcatheter pulmonary artery denervation (TPADN) in patients with combined postcapillary and precapillary PH (Cpc-PH) associated with left heart failure with reduced ejection fraction (HF-rEF). BACKGROUND: Pulmonary hypertension (PH) in patients with left ventricular systolic dysfunction has a negative impact on outcome. METHODS: The combination of pulmonary artery systolic pressure (PAPs) ≥60 mmHg, transpulmonary pressure gradient (TPG) ≥12 mmHg, nonreversible mean PAP, and pulmonary vascular resistance (PVR) ≥3.5 Wood Units was considered as too high risk for heart transplantation (HTx). The clinical efficacy endpoint was an improvement in 6-min walking test and the hemodynamic endpoints were changes in PAPs, PVR, and TPG between baseline and 6 months. Circumferential radiofrequency applications were delivered around distal main, left and right pulmonary arteries. At each ablation point temperature was 45°C and energy 10 W. RESULTS: TPADN was performed in 10 patients. At 6-month in 5 patients we observed reduction in PAP, PVR, TPG, and DPG and then 1 had successful HTx, 2 are on HTx waiting list, 2 received LVADs, 2 patients did not improve, and 3 patients died. CONCLUSIONS: TPADN may be beneficial in selected patients with HF-rEF and Cpc-PH.

Long term outcome of heart failure patients disqualified from heart transplantation.

BACKGROUND: The prognosis of patients with advanced heart failure is unfavourable. However, little is known about the survival of patients referred for heart transplantation but finally disqualified from transplantation due to contraindications. This study aimed to evaluate the prognosis of patients' disqualified from heart transplantation. METHODS: It was a retrospective study based on medical records of patients disqualified from heart transplantation. RESULTS: One hundred and fifty-one patients were included and 94 deaths were recorded during long-term follow-up (range 0.02-10.1 years). The survival rate at 5 years was 25%. The mean age of the studied population was 57.7 years and the majority of patients were males, 87.4%. The ischaemic aetiology (66.2%) was the most dominant aetiology of heart failure. In the Cox regression model, supervision by the specialist cardiology centre (HR 0.61;p = 0.04) and pharmacotherapy with beta-blockers (HR = 0.47;p = 0.02) positively influenced the prognosis. On the contrary, well-known heart failure risk factors like a renal failure (HR 1.59;p = 0.049), pulmonary hypertension (HR 1.55;p = 0.046), liver failure (HR 2.65;p = 0.02) were negative predictors of outcome. By Kaplan-Meier analysis, patients with other than pulmonary hypertension causes of disqualification from heart transplantation had a better survival rate, p = 0.047. CONCLUSIONS: The prognosis of patients disqualified from heart transplantation is unfavourable. However, some of the patients experience relatively long survival. Therefore, careful clinical assessment and identification of factors influencing prognosis may improve adequate patients' qualifications for heart transplantation.

Involvement in Health Behavior After Heart Transplantation: The Role of Personal Resources and Health Status. Single-Center Observational Study.

Introduction: Heart transplantation affects all spheres of the patients' functioning - their physical well-being and coping with everyday situations, as well as their identity and social functioning. Its long-term effects depend on the effective cooperation with the transplant team. Post-transplant patients are expected to be committed to adherence to recommendations. Patients' subjective characteristics could increase the risk of difficulties during treatment or might have a protective effect. The major aim of the study was to evaluate the level of engagement in health behavior in heart transplant recipients in relation to their personal resources, such as personality traits, and their health status. Material and Method: The observational ex post facto model was proposed. Participants completed a set of psychological questionnaires. In the study, there were used questionnaires regarding health behavior (HBI), personality traits (NEO-FFI), health locus of control (MHLC), self-efficacy (GSES) and health status (GHQ-28). The group included in the analyses consisted of 107 heart transplant patients. They ranged in age from 19 to 75 years; 10.3% of them were women. Results: According to norms, 71% patients reported high level of engagement in health behavior. There were significant differences in the level of dietary habits and other types of health behaviors. The best predictors of overall health behavior were conscientiousness (ß = 0.20, p < 0.05) and health locus of control (Powerful Others) (ß = 0.25, p < 0.05). The prophylaxis behavior was related significantly to the level of conscientiousness (p < 0.05) and health locus of control (Internal and Powerful Others) (p < 0.05; p < 0.01). The level of positive mental attitude was related significantly to agreeableness (p < 0.05), health locus of control (Powerful Others) (p < 0.01), and self-efficacy (p < 0.01). Everyday healthy practices were related significantly to openness to experience (p < 0.01) and health locus of control (all categories: Internal, Powerful Others and Chance) (p < 0.05; p < 0.01; p < 0.05, respectively). Conclusion: Majority of heart transplant patients is engaged in high level of health behavior. Among the various forms of health-relevant habits, heart transplant patients adhere significantly less frequently to a healthy diet. Among examined resources, the best predictors of caring about health are generalized self-efficacy and age at the time of HTx.

Titin-Related Dilated Cardiomyopathy: The Clinical Trajectory and the Role of Circulating Biomarkers in the Clinical Assessment.

Titin truncating variants (TTNtv) are known as the leading cause of inherited dilated cardiomyopathy (DCM). Nevertheless, it is unclear whether circulating cardiac biomarkers are helpful in detection and risk assessment. We sought to assess 1) early indicators of cardiotitinopathy including the serum biomarkers high-sensitivity cardiac troponin T (hs-cTnT) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) in clinically stable patients, and 2) predictors of outcome among TTNtv carriers. Our single-center cohort consisted of 108 TTNtv carriers (including 70 DCM patients) from 43 families. Clinical, laboratory and follow-up data were analyzed. The earliest abnormality was left ventricular dysfunction, present in 8, 26 and 47% of patients in the second, third and fourth decade of life, respectively. It was followed by symptoms of heart failure, linked to NT-proBNP elevation and severe left ventricular systolic dysfunction, and later by arrhythmias. Hs-cTnT serum levels were increased in the late stage of the disease only. During the median follow-up of 5.2 years, both malignant ventricular arrhythmia (MVA) and end-stage heart failure (esHF) occurred in 12% of TTNtv carriers. In multivariable analysis, NT-proBNP level ≥650 pg/mL was the best predictor of both composite endpoints (MVA and esHF) and of MVA alone. In conclusion, echocardiographic abnormalities are the first detectable anomalies in the course of cardiotitinopathies. The assessment of circulating cardiac biomarkers is not useful in the detection of the disease onset but may be helpful in risk assessment.

Accurate Noninvasive Assessment of Myocardial Iron Load in Advanced Heart Failure Patients.

BACKGROUND: Heart failure patients presenting with iron deficiency can benefit from systemic iron supplementation; however, there is the potential for iron overload to occur, which can seriously damage the heart. Therefore, myocardial iron (M-Iron) content should be precisely balanced, especially in already failing hearts. Unfortunately, the assessment of M-Iron via repeated heart biopsies or magnetic resonance imaging is unrealistic, and alternative serum markers must be found. This study is aimed at assessing M-Iron in patients with advanced heart failure (HF) and its association with a range of serum markers of iron metabolism. METHODS: Left ventricle (LV) myocardial biopsies and serum samples were collected from 33 consecutive HF patients (25 males) with LV dysfunction (LV ejection fraction 22 (11) %; NT-proBNP 5464 (3308) pg/ml) during heart transplantation. Myocardial ferritin (M-FR) and soluble transferrin receptor (M-sTfR1) were assessed by ELISA, and M-Iron was determined by Instrumental Neutron Activation Analysis in LV biopsies. Nonfailing hearts (n = 11) were used as control/reference tissue. Concentrations of serum iron-related proteins (FR and sTfR1) were assessed. RESULTS: LV M-Iron load was reduced in all HF patients and negatively associated with M-FR (r = -0.37, p = 0.05). Of the serum markers, sTfR1/logFR correlated with (r = -0.42; p = 0.04) and predicted (in a step-wise analysis, R 2 = 0.18; p = 0.04) LV M-Iron. LV M-Iron load (µg/g) can be calculated using the following formula: 210.24-22.869 × sTfR1/logFR. CONCLUSIONS: The sTfR1/logFR ratio can be used to predict LV M-Iron levels. Therefore, serum FR and sTfR1 levels could be used to indirectly assess LV M-Iron, thereby increasing the safety of iron repletion therapy in HF patients.

Can Circulating Cardiac Biomarkers Be Helpful in the Assessment of LMNA Mutation Carriers?

Mutations in the lamin A/C gene are variably phenotypically expressed; however, it is unclear whether circulating cardiac biomarkers are helpful in the detection and risk assessment of cardiolaminopathies. We sought to assess (1) clinical characteristics including serum biomarkers: high sensitivity troponin T (hsTnT) and N-terminal prohormone brain natriuretic peptide (NT-proBNP) in clinically stable cardiolaminopathy patients, and (2) outcome among pathogenic/likely pathogenic lamin A/C gene (LMNA) mutation carriers. Our single-centre cohort included 53 patients from 21 families. Clinical, laboratory, follow-up data were analysed. Median follow-up was 1522 days. The earliest abnormality, emerging in the second and third decades of life, was elevated hsTnT (in 12% and in 27% of patients, respectively), followed by the presence of atrioventricular block, heart failure, and malignant ventricular arrhythmia (MVA). In patients with missense vs. other mutations, we found no difference in MVA occurrence and, surprisingly, worse transplant-free survival. Increased levels of both hsTnT and NT-proBNP were strongly associated with MVA occurrence (HR > 13, p ≤ 0.02 in both) in univariable analysis. In multivariable analysis, NT-proBNP level > 150 pg/mL was the only independent indicator of MVA. We conclude that assessment of circulating cardiac biomarkers may help in the detection and risk assessment of cardiolaminopathies.

Fluctuations in circulating endothelial progenitor cell levels and acute cardiac graft rejection.

INTRODUCTION: Endothelial progenitor cells (EPCs) in nontransplant settings have reparative properties. However, their role in heart transplantation (HT) is not well defined. OBJECTIVES: The aim of this study was to prospectively evaluate changes in EPC levels in relation to post­HT rejection. PATIENTS AND METHODS: EPC levels were measured in 27 HT recipients for 6 months after HT. Acute cellular rejection (ACR) or antibody­mediated rejection (AMR) were assessed by right ventricular endomyocardial biopsy. RESULTS: ACR and AMR were observed in 7 (25.9%) and 6 (22.2%) patients, respectively. The ACR status at 1 month post­HT did not differ with respect to EPC immediately post­HT. At 1 month post­HT in patients without ACR or AMR, EPC levels were significantly reduced compared with the measurements immediately post­HT (P <0.001). On further follow­up, EPC levels were similar regardless of the rejection events. Nonetheless, greater changes (coefficient of variation) in EPClog (logarithmic transformation) were associated with the risk of AMR or ACR compared with those without any rejection event (median [lower-upper quartile], 15 [13-18] vs 8 [5-13]; P = 0.02 and 22 [14-26] vs 8 [5-13]; P = 0.01, respectively). The receiver operating characteristic curve showed that the coefficient of variation of EPClog of 12 was the optimal cutoff value for the prediction of rejection (area under the curve = 0.85). Higher levels were associated with greater risk of ACR or AMR (P <0.005). CONCLUSIONS: Early reduction of EPC levels was related to a lower risk of ACR or AMR. Greater changes of EPC­levels during follow­up were associated with a significantly higher risk of rejection.

Heart Transplantation and Risk of Cardiac Vasculopathy Development: What Factors Are Important?

BACKGROUND The aim of this study was to find the main risk factors for development of cardiac allograft vasculopathy (CAV), especially factors identified before the surgical procedure and factors related to the recipient profile and the medical history of the donor. MATERIAL AND METHODS There were 147 patients who had heart transplantation (HT) included in this study: mean age was 45.8±15.3 years. All study patients had coronary angiography after HT. Analyzed risk factors were: non-immunologic recipient risk factors (age of transplantation, smoking, hypertension, lipids, diabetes, obesity and weight gain after HT), immunologic recipient risk factors (acute cellular rejection (ACR), acute humoral rejection (AMR), cytomegalovirus (CMV) episodes), and donor-related risk factors (age, sex, catecholamine usage, ischemic time, compatibility of sex and blood groups, cause of death, cardiac arrest). RESULTS CAV was recognized in 48 patients (CAV group); mean age 53.6±13.6 years. There were 99 patients without CAV (nonCAV group); mean age 48.3±15.5 years. A univariate Cox analysis of the development of coronary disease showed statistical significance (p<0.05) for baseline high-density lipid (HDL), ACR, AMR, CMV, and donor age. Multivariate Cox regression model confirmed that only baseline HDL, episodes of ACR, donor age, and CMV infection are significant for the frequency of CAV after HT. CONCLUSIONS Older donor age is highly associated with CAV development. Older donor age and low level of HDL in heart recipients with the strongest influence of immunologic risk factors (ACR, CMV infection) were linked with development of CAV.

Titin Truncating Variants in Dilated Cardiomyopathy - Prevalence and Genotype-Phenotype Correlations.

TTN gene truncating variants are common in dilated cardiomyopathy (DCM), although data on their clinical significance is still limited. We sought to examine the frequency of truncating variants in TTN in patients with DCM, including familial DCM (FDCM), and to look for genotype-phenotype correlations. Clinical cardiovascular data, family histories and blood samples were collected from 72 DCM probands, mean age of 34 years, 45.8% FDCM. DNA samples were examined by next generation sequencing (NGS) with a focus on the TTN gene. Truncating mutations were followed up by segregation study among family members. We identified 16 TTN truncating variants (TTN trunc) in 17 probands (23.6% of all cases, 30.3% of FDCM, 17.9% of sporadic DCM). During mean 63 months from diagnosis, there was no difference in adverse cardiac events between probands with and without TTN truncating mutations. Among relatives 29 mutation carriers were identified, nine were definitely affected (31%), eight probably affected (27.6%) one possibly affected (3.4%) and eleven were not affected (37.9%). When relatives with all affected statuses were combined, disease penetrance was still incomplete (62.1%) even after exclusion of unaffected relatives under 40 (82%) and was higher in males versus females. In all mutation carriers, during follow-up, 17.4% had major adverse cardiac events, and prognosis was significantly worse in men than in women. In conclusion, TTN truncating variants were observed in nearly one fourth of young DCM patient population, in vast majority without conduction system disease. Incomplete penetrance suggests possible influence of other genetic and/or environmental factors on the course of cardiotitinopathy. Counseling should take into account sex and incomplete penetrance.