PATHFINDER-CHD: prospective registry on adults with congenital heart disease, abnormal ventricular function, and/or heart failure as a foundation for establishing rehabilitative, prehabilitative, preventive, and health-promoting measures: rationale, aims, design and methods.

BACKGROUND: Adults with congenital heart defects (ACHD) globally constitute a notably medically underserved patient population. Despite therapeutic advancements, these individuals often confront substantial physical and psychosocial residua or sequelae, requiring specialized, integrative cardiological care throughout their lifespan. Heart failure (HF) is a critical challenge in this population, markedly impacting morbidity and mortality. AIMS: The primary aim of this study is to establish a comprehensive, prospective registry to enhance understanding and management of HF in ACHD. Named PATHFINDER-CHD, this registry aims to establish foundational data for treatment strategies as well as the development of rehabilitative, prehabilitative, preventive, and health-promoting interventions, ultimately aiming to mitigate the elevated morbidity and mortality rates associated with congenital heart defects (CHD). METHODS: This multicenter survey will be conducted across various German university facilities with expertise in ACHD. Data collection will encompass real-world treatment scenarios and clinical trajectories in ACHD with manifest HF or at risk for its development, including those undergoing medical or interventional cardiac therapies, cardiac surgery, inclusive of pacemaker or ICD implantation, resynchronization therapy, assist devices, and those on solid organ transplantation. DESIGN: The study adopts an observational, exploratory design, prospectively gathering data from participating centers, with a focus on patient management and outcomes. The study is non-confirmatory, aiming to accumulate a broad spectrum of data to inform future hypotheses and studies. PROCESSES: Regular follow-ups will be conducted, systematically collecting data during routine clinical visits or hospital admissions, encompassing alterations in therapy or CHD-related complications, with visit schedules tailored to individual clinical needs. ASSESSMENTS: Baseline assessments and regular follow-ups will entail comprehensive assessments of medical history, ongoing treatments, and outcomes, with a focus on HF symptoms, cardiac function, and overall health status. DISCUSSION OF THE DESIGN: The design of the PATHFINDER-CHD Registry is tailored to capture a wide range of data, prioritizing real-world HF management in ACHD. Its prospective nature facilitates longitudinal data acquisition, pivotal for comprehending for disease progression and treatment impacts. CONCLUSION: The PATHFINDER-CHD Registry is poised to offer valuable insights into HF management in ACHD, bridging current knowledge gaps, enhancing patient care, and shaping future research endeavors in this domain.

Longitudinal analysis of systemic ventricular function and atrioventricular valve function after the Norwood procedure.

OBJECTIVES: To evaluate longitudinal systemic ventricular function and atrioventricular valve regurgitation in patients after the neonatal Norwood procedure. METHODS: Serial postoperative echocardiographic images before Fontan completion were assessed in neonates who underwent the Norwood procedure between 2001 and 2020. Ventricular function and atrioventricular valve regurgitation were compared between patients with modified Blalock-Taussig shunt and right ventricle to pulmonary artery conduit. RESULTS: A total of 335 patients were identified including 273 hypoplastic left heart syndrome and 62 of its variants. Median age at Norwood was 8 (7-12) days. Modified Blalock-Taussig shunt was performed in 171 patients and the right ventricle to pulmonary artery conduit in 164 patients. Longitudinal ventricular function and atrioventricular valve regurgitation were evaluated using a total of 4352 echocardiograms. After the Norwood procedure, ventricular function was initially worse (1-30 days) but thereafter better (30 days to stage II) in the right ventricle to pulmonary artery conduit group (P < 0.001). After stage II, the ventricular function was inferior in the right ventricle to the pulmonary artery conduit group (P < 0.001). Atrioventricular valve regurgitation between the Norwood procedure and stage II was more frequent in the modified Blalock-Taussig shunt group (P < 0.001). After stage II, there was no significant difference in atrioventricular valve regurgitation between the groups (P = 0.171). CONCLUSIONS: The effect of shunt type on haemodynamics after the Norwood procedure seems to vary according to the stage of palliation. After the Norwood, the modified Blalock-Taussig shunt is associated with poorer ventricular function and worse atrioventricular valve regurgitation compared to right ventricle to pulmonary artery conduit. Whereas, after stage II, modified Blalock-Taussig shunt is associated with better ventricular function and comparable atrioventricular valve regurgitation, compared to the right ventricle to pulmonary artery conduit.

Outcomes of coronary artery bypass surgery using modified del Nido cardioplegia in patients with poor ventricular function.

BACKGROUND: del Nido cardioplegia (DN) has been shown to be safe in adult patients undergoing isolated coronary artery bypass grafting with normal left ventricular ejection fraction. We sought to determine whether it was also safe in adult patients with diminished left ventricular function. METHODS: All patients with preoperative left ventricular ejection fraction ≤ 40% undergoing isolated coronary artery bypass grafting between 1/1/2019 and 7/10/2022 were retrospectively analyzed. Off-pump and beating heart cases were excluded. Patients were divided by surgeon preference between conventional cardioplegia (CCP) and DN. Baseline and intraoperative characteristics and short-term postoperative outcomes were compared. RESULTS: Six surgeons performed 829 isolated coronary artery bypass operations during the study. Two-hundred seventy-two met study criteria. Three surgeons used exclusively CCP for the duration of the study, two used exclusively DN and one switched from CCP to DN mid-way through. Group totals were: CCP n = 181 and DN n = 91. There were no significant differences in baseline characteristics including mean left ventricular ejection fraction (CCP 32.5 ± 7.4% vs. DN 33.4 ± 7.29%, p = 0.939). Other than a significant decrease in bypass time for DN (113.20 ± 37.2 vs. 122.43 ± 34.3 min, p = 0.043) there were no intergroup differences in urgency, number of grafts, ischemic time or incidence of blood transfusion. Postoperative outcomes between CCP and DN were similar including incidence of atrial fibrillation (12.2% vs. 8.8%, p = 0.403), intensive care length of stay (3.7 ± 2.3 vs. 4.3 ± 3.7 days, p = 0.886), total length of stay (5.7 ± 3.7 vs. 6.3 ± 4.4 days, p = 0.922) and 30-day mortality (3.85% vs. 1.10%, p = 0.205). CONCLUSION: Compared to conventional cardioplegia, del Nido cardioplegia provides equivalent short-term outcomes in patients with low left ventricular ejection fraction undergoing isolated coronary artery bypass grafting.

Cardiac radiation improves ventricular function in mice and humans with cardiomyopathy.

BACKGROUND: Rapidly dividing cells are more sensitive to radiation therapy (RT) than quiescent cells. In the failing myocardium, macrophages and fibroblasts mediate collateral tissue injury, leading to progressive myocardial remodeling, fibrosis, and pump failure. Because these cells divide more rapidly than cardiomyocytes, we hypothesized that macrophages and fibroblasts would be more susceptible to lower doses of radiation and that cardiac radiation could therefore attenuate myocardial remodeling. METHODS: In three independent murine heart failure models, including models of metabolic stress, ischemia, and pressure overload, mice underwent 5 Gy cardiac radiation or sham treatment followed by echocardiography. Immunofluorescence, flow cytometry, and non-invasive PET imaging were employed to evaluate cardiac macrophages and fibroblasts. Serial cardiac magnetic resonance imaging (cMRI) from patients with cardiomyopathy treated with 25 Gy cardiac RT for ventricular tachycardia (VT) was evaluated to determine changes in cardiac function. FINDINGS: In murine heart failure models, cardiac radiation significantly increased LV ejection fraction and reduced end-diastolic volume vs. sham. Radiation resulted in reduced mRNA abundance of B-type natriuretic peptide and fibrotic genes, and histological assessment of the LV showed reduced fibrosis. PET and flow cytometry demonstrated reductions in pro-inflammatory macrophages, and immunofluorescence demonstrated reduced proliferation of macrophages and fibroblasts with RT. In patients who were treated with RT for VT, cMRI demonstrated decreases in LV end-diastolic volume and improvements in LV ejection fraction early after treatment. CONCLUSIONS: These results suggest that 5 Gy cardiac radiation attenuates cardiac remodeling in mice and humans with heart failure. FUNDING: NIH, ASTRO, AHA, Longer Life Foundation.

Influence of recombinant human B-type natriuretic peptide on improving ventricular function in patients with ST elevation myocardial infarction.

OBJECTIVE: The aim of this study was to investigate the effect of recombinant human B-type natriuretic peptide (rhBNP) on improving ventricular function in patients with ST-elevation myocardial infarction (STEMI). PATIENTS AND METHODS: In this retrospective study, 96 patients with STEMI admitted to Cangzhou Central Hospital from June 2017 to June 2019 were recruited and randomized to either a control group or an experimental group, with 48 patients in each group. Patients in both groups were given conventional pharmacological therapy, and an emergency coronary intervention was performed within 12 hours. Patients in the experimental group received rhBNP intravenously postoperatively, whereas patients in the control group received an equal amount of 0.9% NaCl solution through an intravenous drip. Postoperative recovery indicators were compared between the two groups. RESULTS: Patients treated with rhBNP showed better postoperative respiratory frequency, heart rate, blood oxygen saturation, pleural effusion, acute left heart remodeling after surgery and central venous pressure at 1-3 days after surgery than those without (p<0.05). Early diastolic blood flow velocity/early diastolic motion velocity (E/Em) and wall-motion score indices (WMSI) of patients in the experimental group were markedly lower compared to the control group one week after surgery (p<0.05). Patients receiving rhBNP had better left ventricular ejection fraction (LVEF) and WMSI six months after surgery and higher left ventricular end diastolic volume (LVEDV) and LVEF one week after surgery than the controls (p<0.05). Administration of rhBNP for patients with STMI provided a higher treatment safety by significantly reducing the incidence of left ventricular remodeling and complication than conventional medication (p<0.05). CONCLUSIONS: Intervention with rhBNP in STEMI patients could effectively inhibit ventricular remodeling, alleviate symptoms, reduce adverse complications and improve ventricular function.

Ventricular function and biomarkers in relation to repair and pulmonary valve replacement for tetralogy of Fallot.

OBJECTIVE: Cardiac surgery may cause temporarily impaired ventricular performance and myocardial injury. We aim to characterise the response to perioperative injury for patients undergoing repair or pulmonary valve replacement (PVR) for tetralogy of Fallot (ToF). METHODS: We enrolled children undergoing ToF repair or PVR from four tertiary centres in a prospective observational study. Assessment-including blood sampling and speckle tracking echocardiography-occurred before surgery (T1), at the first follow-up (T2) and 1 year after the procedures (T3). Ninety-two serum biomarkers were expressed as principal components to reduce multiple statistical testing. RNA Sequencing was performed on right ventricular (RV) outflow tract samples. RESULTS: We included 45 patients with ToF repair aged 4.3 (3.4 - 6.5) months and 16 patients with PVR aged 10.4 (7.8 - 12.7) years. Ventricular function following ToF repair showed a fall-and-rise pattern for left ventricular global longitudinal strain (GLS) (-18±4 to -13±4 to -20±2, p < 0.001 for each comparison) and RV GLS (-19±5 to -14±4 to 20±4, p < 0.002 for each comparison). This pattern was not seen for patients undergoing PVR. Serum biomarkers were expressed as three principal components. These phenotypes are related to: (1) surgery type, (2) uncorrected ToF and (3) early postoperative status. Principal component 3 scores were increased at T2. This increase was higher for ToF repair than PVR. The transcriptomes of RV outflow tract tissue are related to patients' sex, rather than ToF-related phenotypes in a subset of the study population. CONCLUSIONS: The response to perioperative injury following ToF repair and PVR is characterised by specific functional and immunological responses. However, we did not identify factors relating to (dis)advantageous recovery from perioperative injury. TRIAL REGISTRATION NUMBER: Netherlands Trial Register: NL5129.

Avaliação ecocardiográfica do paciente com choque circulatório: uma abordagem contemporânea / Echocardiographic evaluation of a patient in circulatory shock: a contemporary approach

O choque circulatório é caracterizado por um estado de ineficiência da oferta de oxigênio tecidual e disfunção múltipla de órgãos. Necessita de diagnóstico e terapias rápidas e assertivas para redução de sua alta letalidade. O ecocardiograma já se estabeleceu como método fundamental no manejo do paciente com choque circulatório. Auxilia de forma crucial no diagnóstico etiológico, prognóstico, monitorização hemodinâmica e estimativa volêmica desses pacientes, tendo como potenciais vantagens a portabilidade, ausência de contraste ou radiação, baixo custo e avaliação em tempo real e de forma seriada. Em ambiente de UTI, demonstra alta correlação com formas invasivas (cateter de artéria pulmonar) e minimamente invasivas (termodiluição transpulmonar) de monitorização hemodinâmica. Atualmente, outras técnicas, como ultrassom pulmonar e VExUS score, têm se agregado à avaliação ecocardiográfica, tornando o método mais abrangente e acurado. Essas técnicas acrescentam dados relevantes na estimativa da volemia do paciente crítico, influenciando na decisão probabilística de fluidoresponsividade e agregando informações no raciocínio diagnóstico das causas do choque, otimizando o prognóstico desses pacientes. O point of care ultrasound (POCUS) tem como objetivo tornar mais acessível, ao médico não especialista em radiologia, habilidades para se obter informações a beira leito, por meio do ultrassom, que o ajudem na tomada de decisões. Esse artigo aborda as diversas aplicabilidades do ecocardiograma em pacientes com choque circulatório, incluindo avaliação prognóstica e diagnóstico etiológico por meio dos parâmetros encontrados nas principais causas de choque, além da monitorização hemodinâmica, avaliação de fluido-responsividade e utilização prática do ultrassom pulmonar.(AU)

Circulatory shock is characterized by a state of inefficient tissue oxygen supply and multiple organ dysfunction. Patients with circulatory shock require fast and assertive diagnosis and therapies to reduce its high lethality. Echocardiography has already been established as a fundamental method in managing patients with circulatory shock. It provides crucial assistance in etiological diagnosis, prognosis, hemodynamic monitoring, and volume estimation in these patients; its potential advantages include portability, absence of contrast or radiation, low cost, and real-time serial assessment. In the intensive care unit setting, it demonstrates a high correlation with invasive (pulmonary artery catheter) and minimally invasive (transpulmonary thermodilution) forms of hemodynamic monitoring. Currently, other techniques, such as pulmonary ultrasound and VExUS score, have been added to echocardiographic assessment, making the method more comprehensive and accurate. These techniques add relevant data to blood volume estimation in critical patients, influencing the probabilistic decision of fluid responsiveness and providing additional information in the diagnostic reasoning of the causes of shock, thus optimizing these patients' prognosis. Point of care ultrasound (POCUS) aims to make abilities to obtain information at the bedside more accessible to physicians who are not specialists in radiology, by means of ultrasound, which assists them in decision-making. This article addresses the diverse applications of echocardiography in patients with circulatory shock, including prognostic evaluation and etiological diagnosis by means of the parameters found in the main causes of shock, in addition to hemodynamic monitoring, evaluation of fluid responsiveness, and practical use of pulmonary ultrasound.(AU)

Longitudinal analysis of systemic ventricular function and atrioventricular valve function after the Fontan procedure.

OBJECTIVES: This study aimed to determine the longitudinal change of systemic ventricular function and atrioventricular valve (AVV) regurgitation after total cavopulmonary connection (TCPC). METHODS: In 620 patients who underwent TCPC between 1994 and 2021, 4219 longitudinal echocardiographic examinations of systemic ventricular function and AVV regurgitation were evaluated retrospectively. RESULTS: The most frequent primary diagnosis was hypoplastic left heart syndrome in 172, followed by single ventricle in 131, tricuspid atresia in 95 and double inlet left ventricle (LV) in 91 patients. Dominant right ventricle (RV) was observed in 329 (53%) and dominant LV in 291 (47%). The median age at TCPC was 2.3 (1.8-3.4) years. Transplant-free survival at 5, 10 and 15 years after TCPC was 96.3%, 94.7% and 93.6%, respectively, in patients with dominant RV and 97.3%, 94.6% and 94.6%, respectively, in those with dominant LV (P = 0.987). Longitudinal analysis of systemic ventricular function was similar in both groups during the first 10 years postoperatively. Thereafter, systemic ventricular function worsened significantly in patients with dominant RV, compared with those with dominant LV (15 years: P = 0.007, 20 years: P = 0.03). AVV regurgitation was more frequent after TCPC in patients with dominant RV compared with those with dominant LV (P < 0.001 at 3 months, 3 years, 5 years, 10 years and 15 years, P = 0.023 at 20 years). There was a significant correlation between postoperative systemic ventricular dysfunction and AVV regurgitation (P < 0.001). CONCLUSIONS: There were no transplant-free survival difference and no difference in ventricular function between dominant RV and dominant LV for the first 10 years after TCPC. Thereafter, ventricular function in dominant RV was inferior to that in dominant LV. The degree of AVV regurgitation was significantly higher in dominant RV, compared with dominant LV, and it was positively associated with ventricular dysfunction, especially in dominant RV.

The Supported vs Unsupported Ross in Pediatric Patients: Neoaortic Root and Ventricular Function.

BACKGROUND: The supported Ross is used to mitigate the neoaortic root dilation that has been described with the unsupported Ross. There is limited literature assessing the efficacy of the supported Ross in young patients. In this study, the fate of the neoaortic root was compared in the supported and unsupported Ross procedure in adolescent patients. METHODS: A retrospective review was performed of patients who underwent the Ross procedure between 1996 and 2019. An analysis was conducted of patients aged 10 to 18 years who underwent the supported and unsupported Ross operation, without a Konno enlargement, to assess for longitudinal echocardiographic changes. Given differences in follow-up time, both regression analysis and Mann-Whitney nonparametric tests were used to correct for time from discharge to most recent follow-up. RESULTS: The median follow-up time for supported and unsupported Ross patients without a Konno enlargement was 2.90 years (0.21-13.03 years) and 12.13 years (2.63-19.47 years), respectively. Unsupported Ross patients experienced a higher rate of change per year in the aortic annulus (P = .003 and P = .014) and aortic sinus (P = .002 and P = .002) diameters, respectively. There was no significant difference in the rate of change of end-diastolic left ventricular internal diameter (P = .703 and P = .92) and aortic insufficiency (P = .687 and P = .215) between the supported and unsupported Ross patients. CONCLUSIONS: Progressive dilation of the neoaortic root in unsupported Ross patients is significantly mitigated with the supported Ross with excellent stability. The supported Ross is safe and effective and may play an increasing role in the management of children with aortic disease.

Comportamiento de strain ventrículo derecho en pacientes tratados con quimioterapia y/o radioterapia en una coherte de pacientes / Right ventricular strain behavior in patients treated with chemotherapy/radiation therapy in patient cohort

Introducción: es bien conocida la utilidad del strain global longitudinal (SGL) del ventrículo izquierdo (VI) en pacientes que reciben antineoplásicos. Estudios recientes sugieren una conducta similar del strain longitudinal de la pared libre (SLPL) del ventrículo derecho (VD), pero en la literatura aún existen muchas controversias que limitan su valoración, por lo que este estudio es un aporte a esta necesidad de practicidad. Objetivo: evaluar el comportamiento del SLPL del VD en los pacientes tratados con quimioterapia y/o radioterapia, y su correlación con el SGL del VI. Metodología: se aplicó un protocolo de recolección de datos que incluía variables demográficas y antecedentes de pacientes con diagnóstico de cáncer, tratados con quimioterapia y/o radioterapia, que acudieron a realizarse un ecocardiograma con strain, desde noviembre 2020 a marzo 2021. El ecocardiograma se hizo acorde con los estándares de las guías de la Sociedad Americana de Ecocardiografía, que incluyera SGL del VI y el SLPL del VD. Los datos fueron analizados en programa SPSS. Resultados: de 90 pacientes, 6 excluidos para un total de 84 recopilados. 89 % mujeres, edad promedio 59 ±11 años, 73 % cáncer de mama. 54 % hipertensos, 27 % obesos, 23 % con dislipidemia, 19 % diabéticos y 11 % eran o fueron fumadores. 46 % había recibido quimioterapia y radioterapia concomitantes y el 48 % había recibido tratamiento hace más de un año. En hallazgos ecocardiográficos: fracción de eyección del VI (FE) promedio 64±7; sin embargo, 82 % tenía SGL del VI reducido (44 % menor a -15 %). De manera similar, el 74 % tenía reducción del SLPL del VD, pero solo el 15 % de la onda S del Doppler tisular del VD estaba anormal, 7 % del TAPSE estaba reducido y 6 % el cambio del área fraccional (CAF). El coeficiente de correlación de Pearson entre el SGL del VI y el SLPL del VD fue de 0.463, con un valor de p <.001, indicando una significativa relación lineal positiva moderada. Conclusión: nuestro estudio sugiere que el SLPL del VD tiene un comportamiento similar al SGL del VI y es un mejor predictor de disfunción ventricular derecha, por encima de los parámetros convencionales. Por esta razón, debería incorporarse como una medida rutinaria en la evaluación ecocardiográfica de los pacientes que reciben terapias antineoplásicas.

Introduction: It is well known the usefulness of left ventricle (LV) longitudinal global strain (GLS) in patients receiving antineoplastic therapy. Recent studies suggested a similar behavior of the right ventricle (RV) free wall longitudinal strain (FWLS), but in the literature there are still many controversies that limit its evaluation, so this study is a contribution to this need for practicality. Objective: To assess the behavior and usefulness of RV FWLS in patients treated with chemotherapy and/or radiotherapy and its correlation with the LV GLS. Method: A data collection protocol that included demographic variables and personal history was applied to patients diagnosed with cancer treated with chemotherapy and/ or radiotherapy who underwent an echocardiogram with strain from November 2020 to March 2021. The echocardiogram was performed accordingly with American Society of Echocardiography guidelines standards, including a LV GLS and a RV FWLS. The data was analyzed in the SPSS program. Results: Of 90 patients, 6 excluded for a total of 84 collected. 89% were women, mean age 59 ±11 years, 73% breast cancer. 54% had hypertension, 27% obesity, 23% dyslipidemia, 19% were diabetic and 11% are or use to be smokers. 46% had received concomitantly chemotherapy and radiotherapy and 48% were treated more than a year ago. In echocardiographic findings, mean LV ejection fraction (EF) was 64±7 %, however, 82% had reduced LV GLS (44% being less than -15%). Similarly, 74% of RV FWLS was reduced, but only 15% of the S wave of the RV by tissue Doppler was abnormal, 7% of TAPSE was reduced, and 6% of fractional area change (FAC). The Pearson correlation coefficient between LV GLS and RV FWLS was of 0.463 with a p value of <.001 indicating a significant moderate positive linear relationship. Conclusion: Our study suggests that RV FWLS has a similar behavior as LV GLS and is a better predictor of right ventricular dysfunction over conventional parameters. Whereby should be incorporated as a routine measurement in the echocardiographic evaluation of patients receiving antineoplastic therapy.

Ventricular morphology of single-ventricle hearts has a significant impact on outcomes after Fontan palliation: a meta-analysis.

OBJECTIVES: A conclusive interpretation of the role of ventricular dominance in outcomes after Fontan palliation has not been formulated yet. We conducted a systematic review and meta-analysis of scientific literature to give an insight into the impact of ventricular morphology in single-ventricle palliation, focusing on its influence on survival, morbidities, ventricular performance and functional capacity. METHODS: A systematic review of PubMed, Web of Science and Scopus databases was performed. A random-effect meta-analysis was conducted, and survival data were reconstructed using the published Kaplan-Meier survival curves. RESULTS: Twenty-seven studies were selected, for a total of 4529 left-dominant versus 4844 right-dominant patients. Estimated survival at 1, 5, 10, 20 and 30 years of follow-up was 0.99 [95% confidence interval (CI) = 0.98-0.99], 0.95 [95% CI = 0.94-0.96], 0.92 [95% CI = 0.91-0.93], 0.86 [95% CI = 0.84-0.88] and 0.68 [95% CI = 0.65-0.83] for left-dominant patients and 0.94 [95% CI = 0.93-0.95], 0.89 [95% CI = 0.88-0.9], 0.85 [95% CI = 0.83-0.87], 0.69 [95% CI = 0.63-0.75] and 0.59 [95% CI = 0.5-0.69] for right-dominant patients, respectively. Survival was statistically lower for right-dominant patients (P < 0.001), with an hazard ratio for the mortality of 2.38 (2.03-2.80); also, they displayed significantly longer hospital stay, worse ventricular function, larger ventricular volumes and a higher incidence of moderate or severe atrioventricular valve regurgitation when compared to left-dominant patients. CONCLUSIONS: According to our meta-analysis, the morphology of the dominant ventricle has a significant impact on outcomes after Fontan palliation. Right-dominant patients experience an inferior long-term survival when the anatomical right ventricle is included in the systemic circulation.

Introduction of Lazy Luna an automatic software-driven multilevel comparison of ventricular function quantification in cardiovascular magnetic resonance imaging.

Cardiovascular magnetic resonance imaging is the gold standard for cardiac function assessment. Quantification of clinical results (CR) requires precise segmentation. Clinicians statistically compare CRs to ensure reproducibility. Convolutional Neural Network developers compare their results via metrics. Aim: Introducing software capable of automatic multilevel comparison. A multilevel analysis covering segmentations and CRs builds on a generic software backend. Metrics and CRs are calculated with geometric accuracy. Segmentations and CRs are connected to track errors and their effects. An interactive GUI makes the software accessible to different users. The software's multilevel comparison was tested on a use case based on cardiac function assessment. The software shows good reader agreement in CRs and segmentation metrics (Dice > 90%). Decomposing differences by cardiac position revealed excellent agreement in midventricular slices: > 90% but poorer segmentations in apical (> 71%) and basal slices (> 74%). Further decomposition by contour type locates the largest millilitre differences in the basal right cavity (> 3 ml). Visual inspection shows these differences being caused by different basal slice choices. The software illuminated reader differences on several levels. Producing spreadsheets and figures concerning metric values and CR differences was automated. A multilevel reader comparison is feasible and extendable to other cardiac structures in the future.

Cavopulmonary support with a modified cannulation technique in a failing Fontan patient.

Failing Fontan patients present a unique challenge for mechanical circulatory support. We report on a 17-year-old patient with Fontan failure and preserved ventricular function who underwent mechanical cavopulmonary support using a novel cannulation technique.

Extreme altitude induces divergent mass reduction of right and left ventricle in mountain climbers.

Mountain climbing at high altitude implies exposure to low levels of oxygen, low temperature, wind, physical and psychological stress, and nutritional insufficiencies. We examined whether right ventricular (RV) and left ventricular (LV) myocardial masses were reversibly altered by exposure to extreme altitude. Magnetic resonance imaging and echocardiography of the heart, dual x-ray absorptiometry scan of body composition, and blood samples were obtained from ten mountain climbers before departure to Mount Everest or Dhaulagiri (baseline), 13.5 ± 1.5 days after peaking the mountain (post-hypoxia), and six weeks and six months after expeditions exceeding 8000 meters above sea level. RV mass was unaltered after extreme altitude, in contrast to a reduction in LV mass by 11.8 ± 3.4 g post-hypoxia (p = 0.001). The reduction in LV mass correlated with a reduction in skeletal muscle mass. After six weeks, LV myocardial mass was restored to baseline values. Extreme altitude induced a reduction in LV end-diastolic volume (20.8 ± 7.7 ml, p = 0.011) and reduced E', indicating diastolic dysfunction, which were restored after six weeks follow-up. Elevated circulating interleukin-18 after extreme altitude compared to follow-up levels, might have contributed to reduced muscle mass and diastolic dysfunction. In conclusion, the mass of the RV, possibly exposed to elevated afterload, was not changed after extreme altitude, whereas LV mass was reduced. The reduction in LV mass correlated with reduced skeletal muscle mass, indicating a common denominator, and elevated circulating interleukin-18 might be a mechanism for reduced muscle mass after extreme altitude.

Late outcome, therapy and systemic ventricular function in patients with a systemic right ventricle: data of the German National Register for Congenital Heart Defects.

BACKGROUND: Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure. METHODS: A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included. RESULTS: Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation. CONCLUSION: A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.

Novel Transcatheter Mitral Prosthesis Designed to Preserve Physiological Ventricular Flow Dynamics.

BACKGROUND: Current mitral bioprostheses are akin to the aortic valve and therefore abolish the left ventricular (LV) physiological vortex. We evaluated the hemodynamic performance and the effects on intraventricular flow dynamics (IFD) of a novel mitral bioprosthesis that presents an innovative design mimicking the native valve. METHODS: A D-shaped self-expandable stent-bovine pericardium monoleaflet valve was designed to provide physiological asymmetric intraventricular flow. Transapical implantation was consecutively performed in 12 juvenile sheep. Postimplant studies using Doppler echocardiography and IFD using echo particle imaging velocimetry were obtained immediately after the implantation and at 3 months to assess the hemodynamic performance of the prostheses. RESULTS: There were 3 deaths during follow-up, 1 due to valve misplacement because of poor imaging visualization and 2 not valve related. The mean transvalvular gradient and effective orifice area were 2.2 ± 1.2 mm Hg and 4.0 ± 1.1 cm2 after implantation and 3.3 ± 1.5 mm Hg and 3.5 ± 0.5 cm2 at 3 months, respectively. LV vortex dimension, orientation, and physiological anticlockwise rotation were preserved compared with preoperative normal LV flow pattern. One animal showed a moderate paravalvular leak, others mild or none. LV outflow tract obstruction, valve thrombosis, and hemolysis were not observed. CONCLUSIONS: Our preclinical in vivo results confirm the good hemodynamic performance of this new transcatheter bioprosthesis with preservation of the physiological IFD. Clinical studies are needed to document whether these characteristics will foster LV recovery and improve the clinical outcome of patients with mitral regurgitation.

Effect of heavy metals on ventricular repolarization and depolarization in the Metropolitan Manila Development Authority (MMDA) traffic enforcers' health study.

We examined the relationships between blood heavy metals [cadmium (B-Cd), mercury (B-Hg), and lead (B-Pb)] and heart rate-corrected QT interval (QTc), JT interval (JTc), and QRS complex duration (QRSc), electrocardiogram markers of ventricular repolarization and depolarization among 60 traffic enforcers in the MMDA traffic enforcers' health study. We fitted regression models to estimate the mean change effect on QTc, JTc, and QRSc, of B-Cd, B-Hg, and B-Pb concentrations, adjusted for potential confounding factors. We looked at effect modification by sex and smoking status. An interquartile range increase in B-Cd (0.9 µg/L) was related to a 6.6% increase in mean QRSc [95% confidence interval (CI): 2.5, 10.8], and a 1.7% increase in mean QTc (95% CI: 0.2, 3.3). We also found that the associations between B-Cd and QRSc and QTc were higher among participants who were never smokers than ever smokers. Moreover, the association between B-Cd and QRSc was also higher among males than females.

Effects of graft preservation conditions on coronary endothelium and cardiac functional recovery in a rat model of donation after circulatory death.

BACKGROUND: Use of cardiac grafts obtained with donation after circulatory death (DCD) could significantly improve donor heart availability. As DCD hearts undergo potentially deleterious warm ischemia and reperfusion, clinical protocols require optimization to ensure graft quality. Thus, we investigated effects of alternative preservation conditions on endothelial and/or vascular and contractile function in comparison with the current clinical standard. METHODS: Using a rat DCD model, we compared currently used graft preservation conditions, St. Thomas n°2 (St. T) at 4°C, with potentially more suitable conditions for DCD hearts, adenosine-lidocaine preservation solution (A-L) at 4°C or 22°C. Following general anesthesia and diaphragm transection, hearts underwent either 0 or 18 min of in-situ warm ischemia, were explanted, flushed and stored for 15 min with either St. T at 4°C or A-L at 4°C or 22°C, and then reperfused under normothermic, aerobic conditions. Endothelial integrity and contractile function were determined. RESULTS: Compared to 4°C preservation, 22°C A-L significantly increased endothelial nitric oxide synthase (eNOS) dimerization and reduced oxidative tissue damage (p < 0.05 for all). Furthermore, A-L at 22°C better preserved the endothelial glycocalyx and coronary flow compared with St. T, tended to reduce tissue calcium overload, and stimulated pro-survival signaling. No significant differences were observed in cardiac function among ischemic groups. CONCLUSIONS: Twenty-two-degree Celsius A-L solution better preserves the coronary endothelium compared to 4°C St. T, which likely results from greater eNOS dimerization, reduced oxidative stress, and activation of the reperfusion injury salvage kinase (RISK) pathway. Improving heart preservation conditions immediately following warm ischemia constitutes a promising approach for the optimization of clinical protocols in DCD heart transplantation.