Normal Left Ventricular Function Research Articles

The role of rhythm control in patients with first diagnosed atrial fibrillation in a real world collective

Abstract Funding Acknowledgements Type of funding sources: Private company. Main funding source(s): Daiichi Sankyo Bayer. Background There has been conflicting evidence regarding the importance of rhythm control as opposed to rate control in patients with atrial fibrillation (AF). Thus, no clear recommendation on early rhythm control for risk reduction is given in the current guidelines. However, some evidence suggests that rhythm control is superior to rate control in patients with a recent diagnosis of AF. Purpose We aim to characterize patients with first diagnosed AF and evaluate the effect of a rhythm control strategy at the time of diagnosis on long term cardiovascular outcomes. Methods This is a single center, retrospective observational sub study based as a part of the HERA-FIB project. HERA-FIB included 10.222 patients presenting to the emergency department (ED) of the department of cardiology of a university hospital from June 2009 to March 2020. We consecutively included all patients with a history AF or current AF upon presentation, regardless of the reason for presentation. A structured follow-up regarding survival and major cardiovascular events was performed. Mean follow up was 23 months (IQR 12-35). Patient care was not influenced. The project was performed according to the declaration of Helsinki and was approved by the local ethics committee and registered at ClinicalTrials.gov (Identifier: NCT05995561). Results. After adjudication we included 10.222 patients in this study. Of those 2758 (27%) had no previous history of AF and were first diagnosed with AF upon presentation. There were major differences in baseline characteristics as patients with first diagnosed AF were younger (73 years vs 76 years, p<0.0001), showed a normal left ventricular function more often (47.9% vs 40.2%, p<0.0001) and had fewer histories of coronary artery disease (CAD) (30.2% vs 48.0%, p<0.0001) and fewer histories of stroke or transient ischemic attack (TIA) (9.0% vs 14.0%, p<0.0001). In contrast they had higher heart rates (113 bpm vs 84 bpm, p<0.0001) on admission. Among the patients with first diagnosed AF 24.6% received a cardioversion (either pharmacological of electrical). Patients with first diagnosed AF undergoing rhythm control treatment showed lower rates for all-cause mortality (10.4% vs 18.2%, p<0.0001) and myocardial infarction (2.4% vs. 5.2%, p=0.0045) as compared to patients first diagnosed with AF not recieving a cardioversion. However, no differences for strokes (2.7% vs 3.3%, p=0.6158) or severe bleeding according to ISTH major bleeding criteria (4.0% vs. 5.3%, p=0.2252) could be detected. Conclusions Patients presenting to an ED with first diagnosed AF are younger and show fewer comorbidities compared to patients with pre-existing AF. Patients with first diagnosed AF seem to profit from early rhythm control strategies showing a reduction for the rates of all-cause mortality and myocardial infarction. However, no difference for stroke or major bleeding events could be detected. Additional, prospective studies are required to verify this hypothesis.

Enasidenib‐induced hepatitis in an individual with Type II D2‐hydroxyglutaric aciduria

AbstractType II D‐2‐Hydroxyglutaric aciduria (T2D2HGA) is caused by a gain‐of‐function pathogenic variant in Isocitrate Dehydrogenase 2 (IDH2). Patients with T2D2HGA commonly present with developmental delay, seizures, cardiomyopathy, and arrhythmias. The recently approved IDH2‐inhibitor Enasidenib targets the p.Arg140Gln pathogenic IDH2 variant and decreases production of D2HGA. We present a 7‐year‐old female with T2D2HGA due to the p.Arg140Gln variant. She was diagnosed at 3‐years‐old after presenting with global developmental delay, leukoencephalopathy, communicating hydrocephalus, seizures, and dilated cardiomyopathy. At age 3 years 11 months, 50 mg Enasidenib daily was initiated. Primary outcomes included seizure frequency, hospital admissions, development, and cardiac structure. Laboratories were monitored biweekly for common Enasidenib side effects. Our patient tolerated Enasidenib well. Urine 2‐HGA decreased significantly from 244 mg/g creatinine to undetectable within 2 weeks of treatment. Inpatient admissions decreased from 8 during the 2 years preceding treatment to 1 during treatment. She has been seizure‐free since Enasidenib initiation. Echocardiography showed improvement in dilated cardiomyopathy with normal left ventricular systolic function. Developmental assessment demonstrated improvements in gross motor, fine motor, language, and socialization domains. Treatment was complicated by mild elevations in alanine transaminase (118 IU/L, range 0–28) and creatine kinase (334 U/L, range 45–198) that resolved by decreasing Enasidenib dosing frequency to three times weekly. Enasidenib is a viable treatment for Type II D2HGA with benefits including developmental gains, fewer acute medical interventions, and cardiomyopathy improvement. While drug‐induced hepatitis is a novel adverse effect of Enasidenib, it can be ameliorated by decreasing dose frequency.

Noninvasive measurement of tracer extraction efficiency in tissue, illustrated with Tc-99m-MAG3.

The aim of this study is to develop a noninvasive technique for measuring tissue tracer extraction efficiency (E) and illustrate it for Tc-99m-mercaptoacetyltriglycine (MAG3) and kidney. E was measured in 10 patients with normal MAG3 renography. E is the ratio of tissue clearance-to-blood flow (Ki/F). For single-photon tracers, attenuation constants are unknown, so Ki and F cannot be separately measured. However, by deriving attenuation-uncorrected Ki' and F' from the same regions of interests (ROIs), these constants cancel out, giving E. Using a lung ROI for blood activity, F was measured from first-pass and Ki' from Gjedde-Patlak-Rutland (GPR) analysis up to 130 s. Because of interference from right ventricle, a left ventricular ROI (LV) is unsuitable for F' but was used in GPR analysis, making an adjustment for the ratio of respective blood pool signals arising from lung and LV ROIs in early frames (60-90 s). A lung ROI underestimates F' by 4% at normal LV function. Chest wall interstitial activity (I), which does not affect F', amounted to 53 and 30% of the lung and LV signals at 20 min, and 12 and 6% at 130 s, resulting in underestimations of Ki of 4 and 2%, respectively. Ignoring these opposing errors, E based on lung ROI for left and right kidneys was 43.5 (SD 8)% and 47.3 (9)%, and based on LV ROI for GPR analysis was 44.5 (10.9)% and 48.3 (10.6)%. E can be measured by combining blood flow from first-pass with clearance from GPR analysis, and has potential value both clinically and in clinical research.

Congenital Diaphragmatic Hernia Patients with Left Heart Hypoplasia and Left Ventricular Dysfunction Have Highest Odds of Mortality

To describe the scope of left ventricular (LV) dysfunction and left heart hypoplasia (LHH) in infants with congenital diaphragmatic hernia (CDH), to determine associations with CDH severity, and to evaluate the odds of extracorporeal membrane oxygenation (ECMO) and death with categories of left heart disease. Demographic and clinical variables were collected from a single-center, retrospective cohort of CDH patients from January 2017 through May 2022. Quantitative measures of LV function and LHH were prospectively performed on initial echocardiograms. LHH was defined as ≥2 of the following: Z-score ≤ -2 of any left heart structure or LV end-diastolic volume <3 mL. LV dysfunction was defined as shortening fraction <28%, ejection fraction <60%, or global longitudinal strain <20%. The exposure was operationalized as a four-group categorical variable (LV dysfunction +/-, LHH +/-). Logistic regression models evaluated associations with ECMO and death, adjusting for CDH severity. One-hundred and eight-two patients (80.8% left CDH, 63.2% liver herniation, 23.6% ECMO, 12.1% mortality) were included. Twenty percent demonstrated normal LV function and no LHH (LV dysfunction-/LHH-), 37% normal LV function with LHH (LV dysfunction-/LHH+), 14% LV dysfunction without LHH (LV dysfunction+/LHH-), and 28% both LV dysfunction and LHH (LV dysfunction+/LHH+). There was a dose-response effect between increasing severity of left heart disease, ECMO use, and mortality. LV dysfunction+/LHH+ infants had the highest odds of ECMO use and death, after adjustment for CDH severity [OR (95% CI); 1.76 (1.20,2.62) for ECMO, 2.76 (1.63, 5.17) for death]. In our large single-center cohort, CDH patients with LV dysfunction+/LHH+ had the highest risk of ECMO use and death.

Mortality in patients with normal left ventricular function requiring emergency VA-ECMO for postcardiotomy cardiogenic shock due to coronary malperfusion.

To analyze outcomes in patients with normal preoperative left ventricular ejection fraction (LVEF) undergoing venoarterial extracorporeal membrane oxygenation (VA-ECMO) therapy due to postcardiotomy cardiogenic shock (PCCS) related to coronary malperfusion. Retrospective single-center analysis in patients with normal preoperative LVEF treated with VA-ECMO for coronary malperfusion-related PCCS between May 1998 and May 2018. The primary outcome was 30-day mortality, which was compared using the Kaplan-Meier method and the log-rank test. Multivariable logistic regression was performed to identify predictors of mortality. During the study period, a total of 62,125 patients underwent cardiac surgery at our institution. Amongst them, 59 patients (0.1%) with normal preoperative LVEF required VA-ECMO support due to coronary malperfusion-related PCCS. The mean duration of VA-ECMO support was 6 days (interquartile range 4-7 days). The 30-day mortality was 50.8%. Under VA-ECMO therapy, a complication composite outcome of bleeding, re-exploration for bleeding, acute renal failure, acute liver failure, and sepsis occurred in 51 (86.4%) patients. Independent predictors of 30-day mortality were lactate levels > 9.9 mmol/l before VA-ECMO implantation (odds ratio [OR]: 3.3; 95% confidence interval [CI] 1.5-7.0; p = 0.002), delay until revascularization > 278 minutes (OR: 2.9; 95% CI 1.3-6.4; p = 0.008) and peripheral arterial artery disease (OR: 3.3; 95% 1.6-7.5; p = 0.001). Mortality rates are high in patients with normal preoperative LVEF who develop PCCS due to coronary malperfusion. The early implantation of VA-ECMO before the development of profound tissue hypoxia and early coronary revascularization increases the likelihood of survival. Lactate levels are useful to define optimal timing for the VA-ECMO initiation.

Cardiac biopsies reveal differences in transcriptomics between left and right ventricle in patients with or without diagnostic signs of heart failure

New or mild heart failure (HF) is mainly caused by left ventricular dysfunction. We hypothesised that gene expression differ between the left (LV) and right ventricle (RV) and secondly by type of LV dysfunction. We compared gene expression through myocardial biopsies from LV and RV of patients undergoing elective coronary bypass surgery (CABG). Patients were categorised based on LV ejection fraction (EF), diastolic function and NT-proBNP into pEF (preserved; LVEF ≥ 45%), rEF (reduced; LVEF < 45%) or normal LV function. Principal component analysis of gene expression displayed two clusters corresponding to LV and RV. Up-regulated genes in LV included natriuretic peptides NPPA and NPPB, transcription factors/coactivators STAT4 and VGLL2, ion channel related HCN2 and LRRC38 associated with cardiac muscle contraction, cytoskeleton, and cellular component movement. Patients with pEF phenotype versus normal differed in gene expression predominantly in LV, supporting that diastolic dysfunction and structural changes reflect early LV disease in pEF. DKK2 was overexpressed in LV of HFpEF phenotype, potentially leading to lower expression levels of β-catenin, α-SMA (smooth muscle actin), and enhanced apoptosis, and could be a possible factor in the development of HFpEF. CXCL14 was down-regulated in both pEF and rEF, and may play a role to promote development of HF.

Atrial secondary tricuspid regurgitation: pathophysiology, definition, diagnosis, and treatment.

Atrial secondary tricuspid regurgitation (A-STR) is a distinct phenotype of secondary tricuspid regurgitation with predominant dilation of the right atrium and normal right and left ventricular function. Atrial secondary tricuspid regurgitation occurs most commonly in elderly women with atrial fibrillation and in heart failure with preserved ejection fraction in sinus rhythm. In A-STR, the main mechanism of leaflet malcoaptation is related to the presence of a significant dilation of the tricuspid annulus secondary to right atrial enlargement. In addition, there is an insufficient adaptive growth of tricuspid valve leaflets that become unable to cover the enlarged annular area. As opposed to the ventricular phenotype, in A-STR, the tricuspid valve leaflet tethering is typically trivial. The A-STR phenotype accounts for 10%-15% of clinically relevant tricuspid regurgitation and has better outcomes compared with the more prevalent ventricular phenotype. Recent data suggest that patients with A-STR may benefit from more aggressive rhythm control and timely valve interventions. However, little is mentioned in current guidelines on how to identify, evaluate, and manage these patients due to the lack of consistent evidence and variable definitions of this entity in recent investigations. This interdisciplinary expert opinion document focusing on A-STR is intended to help physicians understand this complex and rapidly evolving topic by reviewing its distinct pathophysiology, diagnosis, and multi-modality imaging characteristics. It first defines A-STR by proposing specific quantitative criteria for defining the atrial phenotype and for discriminating it from the ventricular phenotype, in order to facilitate standardization and consistency in research.

Prognostic value of visual and quantitative CMR regional myocardial function in patients with suspected myocarditis

According to updated Lake-Louise Criteria, impaired regional myocardial function serves as a supportive criterion in diagnosing myocarditis. This study aimed to assess visual regional wall motional abnormalities (RWMA) and novel quantitative regional longitudinal peak strain (RLS) for risk stratification in the clinical setting of myocarditis. In patients undergoing CMR and meeting clinical criteria for suspected myocarditis global longitudinal strain (GLS), late gadolinium enhancement (LGE), RWMA and RLS were assessed in the anterior, septal, inferior, and lateral regions and correlated to the occurrence of major adverse cardiac events (MACE), including heart failure hospitalization, sustained ventricular tachycardia, recurrent myocarditis, and all-cause death. In 690 consecutive patients (age: 48.0 ± 16.0 years; 37.7% female) with suspected myocarditis impaired RLS was correlated with RWMA and LV-GLS but not with the presence of LGE. At median follow up of 3.8 years, MACE occurred in 116 (16.8%) patients. Both, RWMA and RLS in anterior-, septal-, inferior-, and lateral- locations were univariately associated with outcomes (all p < 0.001), but not after adjusting for clinical characteristics and LV-GLS. In the subgroup of patients with normal LV function, RWMA were not predictive of outcomes, whereas septal RLS had incremental and independent prognostic value over clinical characteristics (HRadjusted = 1.132, 95% CI 1.020–1.256; p = 0.020). RWMA and RLS can be used to assess regional impairment of myocardial function in myocarditis but are of limited prognostic value in the overall population. However, in the subgroup of patients with normal LV function, septal RLS represents a distinctive marker of regional LV dysfunction, offering potential for risk-stratification.Graphical abstractCI: confidence interval, CMR: cardiac magnetic resonance imaging, HR: hazard ratio, MACE major adverse cardiovascular events

Results of the Double Switch Operation in Patients Who Previously Underwent Left Ventricular Retraining.

Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart disease that has numerous subtypes. While most patients with CC-TGA have a large ventricular septal defect (VSD) and pulmonary stenosis, there are some patients who have either no VSD or a highly restrictive VSD. These patients will require left ventricular (LV) retraining prior to double switch. The purpose of this study was to review our experience with the double switch procedure in patients who had previously undergone LV retraining. This was a retrospective review of a single institution experience with the double switch procedure in patients who had undergone LV retraining (2002-present). Forty-five patients underwent double switch following LV retraining. Of these, 39 had an arterial switch with hemi-Mustard/bidirectional Glenn and six had a Senning. The median cross-clamp time was 135 min (range 71-272) and median bypass time was 202 min (range 140-430 min). Median hospital length of stay was eight days (range 4-108). There were no in-hospital deaths. Median duration of follow-up was 30 months (range 0-175). One patient subsequently underwent heart transplantation and died 65 months following double switch. At follow-up, 41 of the 44 survivors (93%) have normal or low normal LV function and 40 of the 44 survivors (91%) have no or trace mitral regurgitation. The data demonstrate early and mid-term survival of 100% and 97%. Ninety-three percent had preserved LV function. These results suggest that patients with CC-TGA who undergo LV retraining and double switch can have excellent clinical outcomes.

Diverse Cardiac Phenotype of Becker Muscular Dystrophy: Under-Recognized Subclinical Cardiomyopathy Due to Partial Dystrophin Deficiency in a Contemporary Era.

Becker muscular dystrophy (BMD) is an X-linked recessive disorder responsible for mild skeletal muscle involvement and variable degree of cardiomyopathy. The characteristics of cardiac phenotype of BMD in childhood remain elusive. Clinical manifestations, genotype, serum biomarkers, and echocardiogram were retrospectively reviewed in BMD patients. Cardiac phenotype was classified into acute progressive (AP), chronic persistent (CP), and latent (L) groups based upon symptoms and echocardiographic findings. Twenty-five BMD patients were studied over 9.5 ± 2.5years. Sixteen patients presented initially with variable degree of muscle weakness whereas 9 were asymptomatic. Three patients developed medically refractory heart failure by age 18 with progressive dilated cardiomyopathy (DCM) (AP). Six patients developed mild to moderate left ventricular (LV) systolic dysfunction with LV dilatation but remained asymptomatic (CP). Although 16 patients continued to show normal LV function (L), they demonstrated variable degrees of skeletal muscle involvement. The AP groups presented with significantly larger LV size and LV mass index (LVMI) at the initial encounter than groups CP or L, suggesting early myocardial remodeling predicts rapid disease progression. None presented with atrophic myocardial phenotype commonly observed in Duchenne muscular dystrophy (DMD). Wide availability of genetic testing has changed the scope of clinical presentation of BMD. Cardiomyopathy in BMD presents with a diverse clinical spectrum with variable progression of DCM where larger LV dimension and mass at the time of diagnosis may predict the progressiveness of cardiomyopathy.

Systematic review and meta-analysis of early aortic valve replacement versus conservative therapy in patients with asymptomatic aortic valve stenosis with preserved left ventricle systolic function

BackgroundA quarter of patients with severe aortic stenosis (AS) were asymptomatic, and only a third of them survived at the end of 4 years. Only a select subset of these...

Association between Time to Thrombolysis and Left Ventricular Systolic Function in Patients with St-Elevation Myocardial Infarction: Unraveling the Relationship

OBJECTIVES To determine the association between time to thrombolysis and left ventricular (LV) systolic function in patients with ST-elevation Myocardial Infarction (STEMI). METHODOLOGY T This descriptive case series study compared the time to thrombolysis and LV systolic function of 149 STEMI patients. LV systolic function was evaluated following thrombolysis, and data were collected and analyzed using SPSS version 21.0 software. This study was conducted at the Ayub Teaching Hospital Cardiology Department in Abbottabad from August 2022 to January 2023. RESULTSThe study examined how LV systolic function and symptoms in STEMI patients related to when thrombolysis was administered. The 149 patients analyzed included 28 with normal LV function, 86 with mildly impaired function, 26 with moderately impaired function, and 9 with severely impaired function. In contrast to delayed thrombolysis, which was linked to higher levels of impairment, immediate thrombolysis was correlated with a higher percentage of normal LV function. The prevalence of patients with severely compromised LV function was also higher in those who received thrombolysis within 3 to 6 hours and 9 to 12 hours. LV function was correlated with the type of myocardial infarction, with varying degrees of impairment seen in AWMI and IWMI patients. Gender had no discernible effect on LV function. CONCLUSION Improved LV function is associated with early thrombolysis within hours of STEMI symptoms, highlighting the significance of prompt intervention and minimizing wait times for better patient outcomes.

Association of Atrial Fibrillation Progression With Left Atrial Functional Reserve and Its Reversibility.

Atrial fibrillation (AF) progression is closely related to heart failure occurrence, and catheter ablation carries a beneficial effect for heart failure prevention. Recently, particular attention has been given to left atrial (LA) function and functional reserve in the pathogenesis linking AF and heart failure, although its significance and reversibility is not well studied. We prospectively investigated 164 patients with AF with normal left ventricular systolic function and free from heart failure who underwent first catheter ablation and pre-/postprocedural echocardiography. Conventional and speckle-tracking echocardiography were performed at rest and during passive leg lifting to assess LA size, LA reservoir strain (LARS), and functional reserve calculated as passive leg lifting-LARS - rest-LARS. Patients were categorized into 3 AF subtypes: paroxysmal AF (N=95), persistent AF (PeAF; N=50), and long-standing persistent AF (LS-PeAF; N=19). The PeAF and LS-PeAF groups had larger LA size and reduced LARS compared with the paroxysmal AF group (all P<0.05). LA functional reserve was significantly impaired in the LS-PeAF group (P=0.003). In multivariable analysis, LS-PeAF and advanced age were significantly associated with impaired LA functional reserve. Among 149 patients with sinus rhythm 1 to 2 days after catheter ablation, LARS was significantly improved in both PeAF and LS-PeAF groups but was still lower than that in the paroxysmal AF group. Sinus rhythm restoration also led to amelioration of LA functional reserve in patients with LS-PeAF. AF progression was related to impaired LARS and LA functional reserve, and restoration of sinus rhythm might contribute to early LA reverse remodeling.

Correlation of ventricular pacing burden and left ventricular function in patients with heart failure with reduced ejection fraction.

Pacemaker-induced cardiomyopathy is a well described phenomenon in patients with preserved ejection fraction at the time of permanent pacemaker implant. One of the identified risk factors for pacemaker-induced cardiomyopathy is the degree of ventricular pacing burden. However, it is unclear how a high right ventricular pacing burden affects patients with depressed left ventricular function at the time of pacemaker implantation. We sought to assess the relationship between right ventricular pacing and change in left ventricular function over time. We conducted an analysis of all patients who had received either a single or dual lead cardiac implantable electronic devices, excluding biventricular devices, and had a prior transthoracic echocardiogram demonstrating an ejection fraction of less than 50%. The primary end-point was the correlation between the percentage of ventricular pacing and the change in LV ejection fraction. Fifty eight patients with preceding heart failure had pacemakers implanted and had follow up echocardiograms. There was no correlation between the degree of ventricular pacing and the absolute change in LV function (r = .04, p = .979). None of the previously identified risk factors for pacemaker induced cardiomyopathy were predictive of a significant fall in ejection fraction. The degree of RV pacing and other established risk factors for pacemaker-induced cardiomyopathy in patients with normal left ventricular function at the time of implantation do not appear to carry the same risk in patients with pre-existing heart failure who receive either single or dual lead pacemakers.

Role of Left Ventricular Dysfunction in Systemic Sclerosis-Related Pulmonary Hypertension

In systemic sclerosis (SSc), pulmonary hypertension remains a significant cause of morbidity and mortality. Although conventionally classified as group 1 pulmonary arterial hypertension, systemic sclerosis-related pulmonary hypertension (SSc-PH) is a heterogeneous disease. The contribution of left-sided cardiac disease in SSc-PH remains poorly understood. How often does left ventricular (LV) dysfunction occur in SSc among patients undergoing right heart catheterization and how does coexistent LV dysfunction with SSc-PH affect all-cause mortality in this patient population? We conducted a retrospective, observational study of 165 patients with SSc who underwent both echocardiography and right heart catheterization. LV dysfunction was identified using LV global longitudinal strain (GLS) on speckle-tracking echocardiography based on a defined threshold of >-18%. SSc-PH was defined by a mean pulmonary artery pressure > 20mmHg. Among patients with SSc who have undergone right heart catheterization, LV dysfunction occurred in 74.2%with SSc-PH and 51.2%without SSc-PH. The median survival of patients with SSc-PH and LV dysfunction was 67.9 (95%CI, 38.3-102.0) months, with a hazard ratio of 12.64 (95%CI, 1.73-92.60) for all-cause mortality when adjusted for age, sex, SSc disease duration, and FVC compared with patients with SSc without pulmonary hypertension with normal LV function. LV dysfunction is common in SSc-PH. Patients with SSc-PH and LV dysfunction by LV GLS have increased all-cause mortality. This suggests that LV GLS may be helpful in identifying underlying LV dysfunction and in risk assessment of patients with SSc-PH.

Echocardiography, apical 2-chamber views showing sustained normal LV function (ejection fraction 55%) at 4-year follow up.

Echocardiography, apical 2-chamber views showing sustained normal LV function (ejection fraction 55%) at 4-year follow up.

Echocardiography, apical 4-chamber view showing sustained normal LV function (ejection fraction 55%) at 4-year follow up.

Echocardiography, apical 4-chamber view showing sustained normal LV function (ejection fraction 55%) at 4-year follow up.

Cardiac MRI six months after the onset of multisystem inflammatory syndrome in children and adolescents temporally related to COVID-19: A retrospective follow-up study.

In 2020, reports revealed cases called multisystem inflammatory syndrome in children and adolescents temporally related to COVID-19 or multisystem inflammatory syndrome in children. A small proportion of patients suffer from persistent left ventricular dysfunction at discharge. The primary aim was to investigate if myocardial impairment persists during follow-up in these patients. Children fulfilling the criteria for multisystem inflammatory syndrome in children with cardiac involvement hospitalized between December 2020 and February 2022 were included in this retrospective single centre study. Cardiac MRI was performed six months after the onset of symptoms to evaluate possible persistent myocardial damage. Fifteen patients (80% male) with a median age of 8 years (interquartile range 4.5 - 13.5 years) were included. Upon admission, eight patients (53%) presented with reduced left ventricular function, with a median left ventricular ejection fraction of 54% (interquartile range 49.5%-61.5%) on transthoracic echocardiography. Elevated levels of cardiac-specific markers were found in 14 patients (93%). Cardiac MRI was performed in 12 patients at a median of 190.5 days after the onset of symptoms. Nine patients (75%) had normal left ventricular function, with a median left ventricular ejection fraction of 59.45%, while the remaining patients showed mildly to moderately reduced values. None of the patients showed signs of late gadolinium enhancement, indicating the absence of persistent myocardial scarring. During a follow-up of 6.2 months, mild to moderate cardiac impairment was revealed in 25% of patients evaluated by cardiac MRI. Although a majority of patients do not show signs of cardiac impairment, close follow-ups should be performed in a proportion of patients.

Reference Values for Inward Displacement in the Normal Left Ventricle: A Novel Method of Regional Left Ventricular Function Assessment.

Regional functional left ventricular (LV) assessment using current imaging techniques remains limited. Inward displacement (InD) has been developed as a novel technique to assess regional LV function via measurement of the regional displacement of the LV endocardial border across each of the 17 LV segments. Currently, normal ranges for InD are not available for clinical use. The aim of this study was to validate the normal reference limits of InD in healthy adults across all LV segments. InD was analyzed in 120 healthy subjects with a normal LV ejection fraction, using the three standard long-axis views obtained during cardiac MRI that quantified the degree of inward endocardial wall motion towards the true LV center of contraction. For all LV segments, InD was measured in mm and expressed as a percentage of the theoretical degree of maximal segment contraction towards the true LV centerline. The arithmetic average InD was obtained for each of the 17 segments. The LV was divided into three regions, obtaining average InD at the LV base (segments 1-6), mid-cavity (segments 7-12) and apex (segments 13-17). Average InD was 33.4 ± 4.3%. InD was higher in basal and mid-cavity LV segments (32.8 ± 4.1% and 38.1 ± 5.8%) compared to apical LV segments (28.6 ± 7.7%). Interobserver variability correlations for InD were strong (R = 0.80, p < 0.0001). We provide clinically meaningful reference ranges for InD in subjects with normal LV function, which will emerge as an important screening and assessment imaging tool for a range of HFrEF therapies.

Postpartum cardiogenic shock with rapid recovery in a Titin gene variant: The role of genetic predisposition in peripartum cardiomyopathy

Peripartum cardiomyopathy is defined as heart failure secondary to left ventricular (LV) dysfunction with a left ventricular ejection fraction &lt;45% occurring towards the end of pregnancy or in the months following delivery without other identifiable cause. Pathogenesis of peripartum cardiomyopathy and the role of genetic variants are unknown. We present a previously healthy 33-year-old woman in her first pregnancy with acute onset of heart failure postpartum. She developed cardiogenic shock four days after caesarean section and was treated with levosimendan, cabergoline, ramipril and bisoprolol. She used a wearable cardioverter/defibrillator for 3 months. After 8 months, she was free of symptoms with normal left ventricular function and brain-natriuretic-peptide-levels. Genetic analysis was carried out due to a positive family history and disclosed a heterozygous variant c7627dupA in the TTN gene. Genetic analysis in patients with a positive family history should be carried out since that may provide insights in the pathogenesis of heart failure due to systolic dysfunction including peripartum cardiomyopathy.