Fisiopatología de la hernia diafragmática congénita en el recién nacido / Pathophysiology of congenital diaphramatic hernia in the newborn

La hernia diafragmática congénita es una discontinuidad del diafragma con herniación de los órganos abdominales a la cavidad torácica, actualmente se postula una hipótesis dual para su origen. Su fisiopatología está determinada por la hipoplasia pulmonar, la hipertensión pulmonar y la disfunción ventricular, entender estos elementos es necesario para un adecuado manejo y la mejoría del pronóstico.

Congenital diaphragmatic hernia is a discontinuity of the diaphragm with herniation of the abdominal organs into the thoracic cavity, currently a dual hypothesis for its origin is postulated. Its pathophysiology is determined by pulmonary hypoplasia, pulmonary hypertension and ventricular dysfunction, understanding these elements is necessary for adequate management and improve prognosis.

Exercise-induced cardiac troponin elevations and cardiac ventricular dysfunction assessed by tissue Doppler echocardiography and speckle tracking among non-elite runners in Beijing marathon.

OBJECTIVES: We aimed to identify the major determinants of cardiac troponin changes response to exercise among non-elite runners participating in the Beijing 2022 marathon, with a particular focus on the associations with the cardiac function assessed by tissue Doppler echocardiography and speckle tracking. DESIGN: A prospective study. METHODS: A total of 33 non-elite participants in the 2022 Beijing Marathon were included in the study. Echocardiographic assessment and blood sample collection were conducted before, immediately after, and two weeks after the marathon. Blood samples were analyzed using the same Abbot high-sensitivity cTnI STAT assay. Echocardiography included tissue Doppler and speckle tracking echocardiography. RESULTS: Following the marathon, significant increases were observed in cardiac biomarkers, with hs-cTnI elevating from 3.1 [2.3-6.7] to 49.6 [32.5-76.9] ng/L (P < 0.0001). Over 72 % of participants had post-race hs-TnI levels surpassing the 99th percentile upper reference limit. There was a notable correlation between pre-marathon hs-cTnI levels (ß coefficient, 0.56 [0.05, 1.07]; P = 0.042), weekly average training (ß coefficient, -1.15 [-1.95, -0.35]; P = 0.009), and hs-cTnI rise post-marathon. Echocardiography revealed significant post-race cardiac function changes, including decreased E/A ratio (P < 0.0001), GWI (P < 0.0001), and GCW (P < 0.0001), with LVEF (ß coefficients, 0.112 [0.01, 0.21]; P = 0.042) and RV GLS (ß coefficients, 0.124 [0.01, 0.23]; P = 0.035) changes significantly associated with hs-TnI alterations. All echocardiographic and laboratory indicators reverted to baseline levels within two weeks. CONCLUSIONS: Baseline hs-cTnI levels and weekly average training influence exercise-induced hs-cTnI elevation in non-elite runners. Echocardiography revealed post-race changes in cardiac function, with LVEF and RV GLS significantly associated with hs-TnI alterations. These findings contribute to understanding the cardiac response to exercise and could guide training and recovery strategies.

Cardiac Function Before Sepsis and Clinical Outcomes.

This cohort study characterizes heterogeneity in cardiac function prior to sepsis and describes associations with hospitalization outcomes and mortality.

Improving Valvular Pathologies and Ventricular Dysfunction Diagnostic Efficiency Using Combined Auscultation and Electrocardiography Data: A Multimodal AI Approach.

Simple sensor-based procedures, including auscultation and electrocardiography (ECG), can facilitate early diagnosis of valvular diseases, resulting in timely treatment. This study assessed the impact of combining these sensor-based procedures with machine learning on diagnosing valvular abnormalities and ventricular dysfunction. Data from auscultation at three distinct locations and 12-lead ECGs were collected from 1052 patients undergoing echocardiography. An independent cohort of 103 patients was used for clinical validation. These patients were screened for severe aortic stenosis (AS), severe mitral regurgitation (MR), and left ventricular dysfunction (LVD) with ejection fractions ≤ 40%. Optimal neural networks were identified by a fourfold cross-validation training process using heart sounds and various ECG leads, and their outputs were combined using a stacking technique. This composite sensor model had high diagnostic efficiency (area under the receiver operating characteristic curve (AUC) values: AS, 0.93; MR, 0.80; LVD, 0.75). Notably, the contribution of individual sensors to disease detection was found to be disease-specific, underscoring the synergistic potential of the sensor fusion approach. Thus, machine learning models that integrate auscultation and ECG can efficiently detect conditions typically diagnosed via imaging. Moreover, this study highlights the potential of multimodal artificial intelligence applications.

Genetic Atrial Cardiomyopathies: Common Features, Specific Differences, and Broader Relevance to Understanding Atrial Cardiomyopathy.

Atrial cardiomyopathy is a condition that causes electrical and contractile dysfunction of the atria, often along with structural and functional changes. Atrial cardiomyopathy most commonly occurs in conjunction with ventricular dysfunction, in which case it is difficult to discern the atrial features that are secondary to ventricular dysfunction from those that arise as a result of primary atrial abnormalities. Isolated atrial cardiomyopathy (atrial-selective cardiomyopathy [ASCM], with minimal or no ventricular function disturbance) is relatively uncommon and has most frequently been reported in association with deleterious rare genetic variants. The genes involved can affect proteins responsible for various biological functions, not necessarily limited to the heart but also involving extracardiac tissues. Atrial enlargement and atrial fibrillation are common complications of ASCM and are often the predominant clinical features. Despite progress in identifying disease-causing rare variants, an overarching understanding and approach to the molecular pathogenesis, phenotypic spectrum, and treatment of genetic ASCM is still lacking. In this review, we aim to analyze the literature relevant to genetic ASCM to understand the key features of this rather rare condition, as well as to identify distinct characteristics of ASCM and its arrhythmic complications that are related to specific genotypes. We outline the insights that have been gained using basic research models of genetic ASCM in vitro and in vivo and correlate these with patient outcomes. Finally, we provide suggestions for the future investigation of patients with genetic ASCM and improvements to basic scientific models and systems. Overall, a better understanding of the genetic underpinnings of ASCM will not only provide a better understanding of this condition but also promises to clarify our appreciation of the more commonly occurring forms of atrial cardiomyopathy associated with ventricular dysfunction.

Long-term follow-up of atrioventricular valve function in Fontan patients: effect of atrioventricular valve surgery.

OBJECTIVES: The aim of this study was to evaluate the relationship between atrioventricular valve and ventricular function in Fontan survivors, including the effect of atrioventricular valve surgery. METHODS: Analysis focused on transplant-free survival and the need for atrioventricular valve surgery in single ventricle patients after Fontan completion. Longitudinal echocardiographic examination of long-term valve and ventricular function was performed. RESULTS: Fontan completion was performed in 113 patients, having a right univentricular morphology in 33.6%, a left ventricle morphology in 62.8% and ambiguous in 3.6%. Perioperative mortality was 2.7% (n = 3). Within a median follow-up of 16.3 years (interquartile range 10.6-23.6), transplant-free survival was 96.1 ± 1.9% and 90.4 ± 5.8% at 10-25 years. Twenty AV valve procedures were performed in 14 (12.4%) children, respectively, pre-Fontan (n = 10), per-Fontan (n = 8) and post-Fontan (n = 2), resulting in a cumulative incidence of AV valve surgery is 5.7 ± 2.2% and 12.3 ± 3.2% at 1-5 years. Atrio-ventricular valve function deteriorated over time [hazard ratio (HR) 1.112, 95% confidence interval (CI) 1.089-1.138, P < 0.001], without difference for valve morphology (P = 0.736) or ventricular dominance (P = 0.484). AV valve dysfunction was greater in patients requiring AV valve surgery (HR 20.383, 95% CI 6.223-36.762, P < 0.001) but showed a comparable evolution since repair to those without valve surgery (HR 1.070, 95% CI 0.987-1.160, P = 0.099). Progressive time-related ventricular dysfunction was observed (HR 1.141, 95% CI 1.097-1.182, P < 0.001), significantly less in left ventricle-dominance (HR 0.927, 95% CI 0.860-0.999, P = 0.047) but more after AV valve surgery (HR 1.103, 95% CI 1.014-1.167, P = 0.022). CONCLUSIONS: In a homogeneously treated Fontan population, 25-year transplant-free survival is encouraging. Atrio-ventricular valve surgery was necessary in 12.4%, resulting mostly in a durable valve function. However, a slow time-related decline of atrioventricular valve function as of ventricular function is worrisome, evoking a role for additional heart failure therapy.

Association of Interstage Monitoring Era and Likelihood of Hemodynamic Compromise at Intervention for Recoarctation Following the Norwood Operation.

Background Intensive monitoring has been associated with a lower death rate between the Norwood operation and superior cavopulmonary connection, possibly due to early identification and effective treatment of residual anatomic lesions like recoarctation before lasting harm occurs. Methods and Results Neonates undergoing a Norwood operation and receiving interstage care at a single center between January 1, 2005, and September 18, 2020, were studied. In those with recoarctation, we evaluated association of era ([1] preinterstage monitoring, [2] a transitional phase, [3] current era) and likelihood of hemodynamic compromise (progression to moderate or greater ventricular dysfunction/atrioventricular valve regurgitation, initiation/escalation of vasoactive/respiratory support, cardiac arrest preceding catheterization, or interstage death with recoarctation on autopsy). We also analyzed whether era was associated with technical success of transcatheter recoarctation interventions, major adverse events, and transplant-free survival. A total of 483 subjects were studied, with 22% (n=106) treated for recoarctation during the interstage period. Number of catheterizations per Norwood increased (P=0.005) over the interstage eras, with no significant change in the proportion of subjects with recoarctation (P=0.36). In parallel, there was a lower likelihood of hemodynamic compromise in subjects with recoarctation that was not statistically significant (P=0.06), with a significant difference in the proportion with ventricular dysfunction at intervention (P=0.002). Rates of technical success, procedural major adverse events, and transplant-free survival did not differ (P>0.05). Conclusions Periods with interstage monitoring were associated with increased referral for catheterization but also reduced likelihood of ventricular dysfunction (and a suggestion of lower likelihood of hemodynamic compromise) in subjects with recoarctation. Further study is needed to guide optimal interstage care of this vulnerable population.

Impact of Ventricular Dysfunction and Atrioventricular Valve Regurgitation on Pre-Fontan Attrition.

BACKGROUND: There is a significant incidence of pre-Fontan attrition-defined as failure to undergo Fontan completion-after superior cavopulmonary connection. This study investigated the impact of at least moderate ventricular dysfunction (VD) and atrioventricular valve regurgitation (AVVR) on pre-Fontan attrition. METHODS: This single-center retrospective cohort study included all infants who underwent Norwood palliation from 2008 to 2020 and subsequently underwent superior cavopulmonary connection. Pre-Fontan attrition was defined as death, listing for heart transplantation before Fontan completion, or unsuitability for Fontan completion. The study's secondary outcome was transplant-free survival. RESULTS: Pre-Fontan attrition occurred in 34 of 267 patients (12.7%). Isolated VD was not associated with attrition. However, patients with isolated AVVR had 5 times the odds of attrition (odds ratio, 5.4; 95% CI 1.8-16.2), and patients with both VD and AVVR had 20 times the odds of attrition (odds ratio, 20.1; 95% CI 7.7-52.8) compared with patients without VD or AVVR. Only patients with both VD and AVVR had significantly worse transplant-free survival compared with patients without VD or AVVR (hazard ratio, 7.7; 95% CI 2.8-21.6). CONCLUSIONS: The additive effect of VD and AVVR is a powerful contributor to pre-Fontan attrition. Future research investigating therapies that can mitigate the degree of AVVR may help improve Fontan completion rates and long-term outcomes.

Can myocardial dysfunction be detected in patients with rheumatoid arthritis with no cardiac symptoms?

OBJECTIVE: The aim of our study was to investigate the role of tissue Doppler and Myocardial Performance Index (MPI) in evaluating cardiac involvement in patients with rheumatoid arthritis (RA) with no cardiac symptoms, to determine whether these measurements differ between healthy controls and RA patients, and whether they can be used to determine the risk of cardiovascular disease and predict prognosis. PATIENTS AND METHODS: 50 RA patients fulfilling the 2010 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) RA criteria and 50 healthy volunteering controls were included in the study. All patients and controls were assessed using electrocardiography (ECG), echocardiography, conventional Doppler echocardiography and tissue Doppler echocardiography. MPI values were calculated. In addition, RA patients were compared after being divided into two subgroups: seropositive and seronegative RA. Disease activity levels of the patients were determined based on Disease Activity Score in 28 Joints (DAS28). RESULTS: The control group and RA group were compared in terms of PR interval, left atrial diameter, E/A, E/e', and MPI values. Comparisons between the groups yielded statistically significant differences in left atrial diameter, E/A, E/e', and MPI values and no significant difference in PR intervals. These parameters were also compared between seropositive and seronegative patients. Left atrial diameter was significantly higher in seronegative patients than in seropositive patients. There was no significant difference in the other values. DAS28 scores had no correlation with cardiac parameters. CONCLUSIONS: Early detection of ventricular dysfunction in RA may be useful in clinical practice when predicting prognosis and optimizing treatment. The present study found that RA patients had impaired tissue Doppler measurements and MPI results compared to controls. MPI and tissue Doppler may be useful in early detection of ventricular dysfunction.

Severe Pulmonary Arterial Hypertension in Healthy Young Infants: Single Center Experience.

OBJECTIVE: We studied the clinical presentation and management of acute pulmonary arterial hypertension (PAH) in healthy young infants, and the effect of thiamine therapy. METHODS: Review of hospital records was conducted for 56 healthy infants (aged below 6 month) who developed sudden onset of pulmonary arterial hypertension as diagnosed on 2D echocardiography, and were admitted at our institution. RESULTS: All patients received supportive care and pulmonary vasodilator therapy, whereas those admitted after Sep-tember, 2019 (n=28) received thiamine in addition, as per the institute's protocol. Overall, complete recovery was seen in 80% (n=45). Infants who died had significantly lower mean pH (7.05 vs 7.27; P=0.001) and serum bicarbonate (9.1 vs 14.9; P=0.007), higher arterial lactate (72.7 vs 61.5; P=0.92), ventricular dysfunction (16 vs 10; P=0.01) and shock (7 vs 9; P=0.008) when compared to those who survived. Baseline characteristics, severity of acidosis and pulmonary hypertension, time taken to recover from PAH, presence of ventricular dysfunction were comparable among those who received thiamine and those who did not receive it. Similarly, recovery (89% vs 71%; P=0.17) and mortality (11% vs 29%) were also comparable between the two groups. CONCLUSIONS: A significant proportion of infants with PAH improve with supportive treatment and pulmonary vasodilator therapy. Thiamine supplementation may not give any additional benefit in these patients.

Cardiac Outcome of Children With SARS-CoV-2 Related Multisystem Inflammatory Syndrome.

OBJECTIVE: To study the cardiac outcomes of patients with multisystem inflammatory syndrome in children (MIS-C) after 6-month of diagnosis. METHODS: This review of hospital records was conducted on MIS-C patients (aged <21 year) who completed a six-month follow up. The baseline demographic, clinical, laboratory, and treatment characteristics during the acute phase, and echocardiographic findings during follow-up were collected. RESULTS: 116 patients (61.2% male, median age 7 years) with MIS-C were included in the study. At the time of admission, cardiac abnormalities were present in 70.7% of MIS-C patients, and the most common cardiac abnormalities were valve failure (50.9%), followed by ventricular dysfunction (39.7%), and pericardial effusion (23.3%). Six month after diagnosis, cardiac abnormalities were found in 10.3% of patients, and patients had lower rates of ventricular dysfunction (P<0.001), valve failure (P<0.001), pericardial effusion (P<0.001), and coronary involvement (P<0.001) as composed to the baseline. Intravenous immunoglobulin (IVIG) and steroid treatment significantly reduced the odds of occurrence of ventricular dysfunction (P=0.002), valve failure (P=0.004), and low ejection fraction (P=0.002) in comparison to IVIG treatment. CONCLUSION: While most MIS-C patients had abnormal echocardiographic findings at admission, only 10.3% of patients had cardiac abnormalities during follow up.

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.

Left atrial strain parameters derived by echocardiography are impaired in patients with acute myocarditis and preserved systolic left ventricular function.

PURPOSE: Data derived by cardiac magnetic resonance (CMR) feature tracking suggest that not only left ventricular but also left atrial function is impaired in patients with acute myocarditis. Therefore, we investigated the diagnostic value of speckle tracking echocardiography of the left ventricle and left atrium in patients with acute myocarditis and normal left ventricular ejection fraction (LVEF). METHODS AND RESULTS: 30 patients with acute myocarditis confirmed by CMR according to the Lake Louise criteria and 20 healthy controls were analyzed including global longitudinal strain (GLS) and left atrial (LA) strain parameters. Although preserved LVEF was present in both groups, GLS was significantly lower in patients with acute myocarditis (GLS - 19.1 ± 1.8% vs. GLS - 22.1 ± 1.7%, p < 0.001). Further diastolic dysfunction measured by E/e' mean was significantly deteriorated in the myocarditis group compared to the control group (E/e' mean 6.4 ± 1.6 vs. 5.5 ± 1.0, p = 0.038). LA reservoir function (47.6 ± 10.4% vs. 55.5 ± 10.8%, p = 0.013) and LA conduit function (-33.0 ± 9.6% vs. -39.4 ± 9.5%, p = 0.024) were significantly reduced in patients with acute myocarditis compared to healthy controls. Also left atrial stiffness index (0.15 ± 0.05 vs. 0.10 ± 0.03, p = 0.003) as well as left atrial filling index (1.67 ± 0.47 vs. 1.29 ± 0.34, p = 0.004) were deteriorated in patients with myocarditis compared to the control group. CONCLUSION: In patients with acute myocarditis and preserved LVEF not only GLS but also LA reservoir function, LA conduit function and left atrial stiffness index as well as left atrial filling index were impaired compared to healthy controls indicating ventricular diastolic dysfunction and elevated LV filling pressures.

Improvement in electrocardiographic parameters of repolarization related to sudden death in patients with ventricular dysfunction and left bundle branch block after cardiac resynchronization through His bundle pacing.

BACKGROUND: Cardiac resynchronization therapy (CRT) through permanent His bundle pacing (p-HBP) normalizes interventricular conduction disorders and QRS. Similarly, there are immediate and long-term changes in repolarization, which could be prognostic of a lower risk of sudden death (SD) at follow-up. We aimed to compare the changes in different electrocardiographic (ECG) repolarization parameters related to the risk of SD before and after CRT through p-HBP. METHODS: In this prospective, descriptive single-center study (May 2019 to December 2021), we compared the ECG parameters of repolarization related to SD in patients with non-ischemic dilated cardiomyopathy, left bundle branch block (LBBB), and CRT indications, at baseline and after CRT through p-HBP. RESULTS: Forty-three patients were included. Compared to baseline, after CRT through p-HBP, there were immediate significant changes in the QT interval (ms): 445 [407.5-480] vs 410 [385-440] (p = 0.006), QT dispersion (ms): 80 [60-100] vs 40 [40-65] (p < 0.001), Tp-Te (ms): 90 [80-110] vs 80 [60-95] (p < 0.001), Tp-Te/QT ratio: 0.22 [0.19-0.23] vs 0.19 [0.16-0.21] (p < 0.001), T wave amplitude (mm): 6.25 [4.88-10] vs - 2.5 [- 7-2.25] (p < 0.001), and T wave duration (ms): 190 [157.5-200] vs 140 [120-160] (p = 0.001). In the cases of the corrected QT (Bazzett and Friederichia) and the Tp-Te dispersion, changes only became significant at 1 month post-implant (468.5 [428.8-501.5] vs 440 [410-475.25] (p = 0.015); 462.5 [420.8-488.8] vs 440 [400-452.5] (p = 0.004), and 40 [30-52.5] vs 30 [20-40] (p < 0.001), respectively) (Table 1). Finally, two parameters did not improve until 6 months post-implant: the rdT/JT index, 0.25 [0.21-0.28] baseline vs 0.20 [0.19-0.23] 6 months post-implant (p = 0.011), and the JT interval, 300 [240-340] baseline vs 280 [257-302] 6 months post-implant (p = 0.027). Additionally, most of the parameters continued improving as compared with immediate post-implantation. CONCLUSIONS: After CRT through His bundle pacing and LBBB correction, there was an improvement in all parameters of repolarization related to increased SD reported in the literature.

Impact of Rheumatic Process in Left and Right Ventricular Function in Patients with Mitral Regurgitation.

Background: Mitral regurgitation (MR) burdens the left and right ventricles with a volume or pressure overload that leads to a series of compensatory adaptations that eventually lead to ventricular dysfunction, and it is well known that in rheumatic heart disease (RHD) that the inflammatory process not only occurs in the valve but also involves the myocardial and pericardial layers. However, whether the inflammatory process in rheumatic MR is associated with ventricular function besides hemodynamic changes is not yet established. Purpose: Evaluate whether rheumatic etiology is associated with ventricular dysfunction in patients with chronic MR. Methods: The study population comprised patients aged 18 years or older included in the registry who had echocardiography performed at the National Cardiovascular Center Harapan Kita in Indonesia during the study period with isolated primary MR due to rheumatic etiology and degenerative process with at least moderate regurgitation. Results: The current study included 1,130 patients with significant isolated degenerative MR and 276 patients with rheumatic MR. Patients with rheumatic MR were younger and had a higher prevalence of atrial fibrillation and pulmonary hypertension, worse left ventricle (LV) ejection fraction and tricuspid annular plane systolic excursion (TAPSE) value, and larger left atrium (LA) dimension compared to patients with degenerative mitral regurgitation (MR). Gender, age, LV end-systolic diameter, rheumatic etiology, and TAPSE were independently associated with more impaired LV ejection fraction. Whereas low LV ejection fraction, LV end-systolic diameter, and tricuspid peak velocity (TR) peak velocity >3.4 m/s were independently associated with more reduced right ventricle (RV) systolic function (Table 3). Conclusions: Rheumatic etiology was independently associated with more impaired left ventricular function; however, rheumatic etiology was not associated with reduced right ventricular systolic function in a patient with significant chronic MR.