Diagnosis Of Structural Heart Disease Research Articles

STereotactic Arrhythmia Radioablation and its place in modern cardiac ElectroPhysiology: Results of an EHRA Survey

Abstract Background Stereotactic arrhythmia radioablation (STAR) is an emerging treatment option for recurrent ventricular tachycardia/fibrillation (VT/VF) in particular in structural heart disease (SHD). However, the current role and envisioned future role of STAR is in clinical practice as viewed by Cardiologists is unknown. Objective To assess the current role of STAR, barriers to its use, and expected future role, within the cardiology community. Methods An online survey was created with the support of the EHRA Scientific Initiatives committee and distributed via newsletters and social media. The survey consisted of 20 multiple-choice questions with a focus on baseline demographics, awareness / access, current use, and the future role of STAR. Results A total of 129 international participants completed the survey, mean age was 43±11 years, 25 (16.4%) were female. Ninety-one (59.9%) participants were cardiac electrophysiologists while 13 (8.6%) were general cardiologists. Nine participants (7%) were unaware of STAR as a therapeutic option. Sixty-four (49.6%) had access to STAR while 62 (48.1%) had treated / referred a patient for treatment. Median number of patients treated was 4 (range 1-100). Common primary indications for STAR were recurrent VT/VF in SDH (45%), recurrent VT/VF without SHD (7.8%) or PVC (3.9%). Based on their knowledge, 49% and 59% of survey participants responded they would consider performing / referring a patient for STAR, respectively. Reported main advantages of STAR were efficacy in the treatment of arrhythmias not amenable to conventional treatment (49%), non-invasive treatment approach with overall low expected acute and short-term procedural risk (23%) and possible higher efficacy than other available treatment options (23%). Survey respondents named one or more previous catheter ablations (CA) or contraindication for CA (79%), diagnosis of SHD (78%), recurrent monomorphic VT with >3 episodes within the preceding 3 months (68%), optimal antiarrhythmic medication (61%) or electrical storm (59%) as prerequisites for the use of STAR. Most respondents foresee a future clinical role for STAR in the treatment of VT/VF with or without underlying SHD (72% and 75%, respectively). Although, only a minority expected a first-line indication for it in with or without SHD (7% and 5%, respectively) and STAR was viewed rather as a bail-out option (48% and 35%, respectively). Conclusions STAR is a new option for the treatment of recurrent VT that appears to gain acceptance within the EP community. Randomized-controlled trials and an EHRA consensus document are critical to further define efficacy, patient populations, as well as the appropriate clinical use for the treatment of VT.Central figureFuture role of STAR

Evaluation of the heart sounds in children using a Doppler Phonolyser

BackgroundHeart auscultation is an easy and inexpensive tool for early diagnosis of congenital heart defects. In this regard, a simple device which can be used easily by physicians for heart murmur detection will be very useful. The current study was conducted to evaluate the validity of a Doppler-based device named “Doppler Phonolyser” for the diagnosis of structural heart diseases in pediatric patients. In this cross-sectional study, 1272 patients under 16 years who were referred between April 2021 and February 2022, to a pediatric cardiology clinic in Mofid Children Hospital, Tehran, Iran, were enrolled. All the patients were examined by a single experienced pediatric cardiologist using a conventional stethoscope at the first step and a Doppler Phonolyser device at the second step. Afterward, the patient underwent trans-thoracic echocardiography, and the echocardiogram results were compared with the conventional stethoscope as well as the Doppler Phonolyser findings.ResultsSensitivity of the Doppler Phonolyser for detecting congenital heart defects was 90.5%. The specificity of the Doppler Phonolyser in detecting heart disease was 68.9% in compared with the specificity of the conventional stethoscope, which was 94.8%. Among the most common congenital heart defects in our study population, the sensitivity of the Doppler Phonolyser was 100% for detection of tetralogy of Fallot (TOF); In contrast, sensitivity of both the conventional stethoscope and the Doppler Phonolyser was relatively low for detecting atrial septal defect.ConclusionsDoppler Phonolyser could be useful as a diagnostic tool for the detection of congenital heart defects. The main advantages of the Doppler Phonolyser over the conventional stethoscope are no need for operator experience, the ability to distinguish innocent murmurs from the pathologic ones and no effect of environmental sounds on the performance of the device.

Expected small left heart size in the presence of congenital diaphragmatic hernia: Fetal values and Z-scores for infants confirmed to have no heart disease postnatally.

In fetuses with left-sided congenital diaphragmatic hernia (CDH), left heart structures may appear small, but usually normalize after birth in the absence of structural cardiac anomalies. To decrease the possibility of an erroneous diagnosis of structural heart disease, we identify normal values for left heart structures in the presence of left CDH and secondarily investigate the relationship of left heart size and survival to neonatal hospital discharge. Left heart structures [mitral valve (MV) and aortic valve (AoV) annulus diameter, left ventricle (LV) length and width] were measured by fetal echocardiogram in fetuses with left CDH and no congenital heart disease. We generated linear regression models to establish the relationship of gestational age for each left heart structure using data from fetuses who survived after birth. We calculated z-scores (normalized to gestational age), and assessed the relationship of survival to the size of each structure. One hundred forty-two fetuses underwent fetal echocardiogram (median 25 weeks' gestation, IQR 23, 27 weeks). Left heart structures were deemed small when using published normative data from unaffected fetuses (z-scores: MV -1.09 ± 1.35, AoV -2.12 ± 1.16, LV length -1.36 ± 1.24, LV width -4.79 ± 0.79). CDH-specific models derived from log-transformed values yielded left-shifted distributions, reflecting the small structures (mean z-score lower by: MV 0.99 ± 0.30, AoV 2.04 ± 0.38, LV length 1.30 ± 0.36, and LV width 4.69 ± 0.28; p < 0.0001 for all comparisons). Non-survivors had worse z-scores than survivors for all measurements, but this did not reach statistical significance. Log-transformed linear models generated new normative data for fetal left heart structures in left CDH, which may be used to allay antenatal concerns regarding structural left heart anomalies. There were no significant differences in z-scores between survivors and non-survivors, suggesting that in the absence of true structural disease, cardiac evaluation is not predictive in isolation and that causes of mortality are likely multifactorial in this population.

Obstetric and fetal/neonatal outcomes in pregnant women with frequent premature ventricular complexes and structurally normal heart

BackgroundHigh premature ventricular complex (PVC) burden may increase the risk of left ventricular dysfunction and all-cause mortality. We aimed to evaluate maternal and neonatal outcomes of pregnant women with structurally normal heart having PVC burden ≥1%. MethodsThis retrospective cohort study used data from Chang Gung Research Database. Pregnancies from January 1, 2005, through June 30, 2020, with documented maternal PVC burden ≥1% by 24-h Holter monitor were identified. Pregnant women with a diagnosis of structural heart disease or arrhythmias other than PVC were excluded. We used propensity score matching (PSM) to balance the covariates between the PVC group and normal control group. The PVC group was classified into low-PVC (<10%) and high-PVC burden subgroups. The maternal and neonatal outcomes were assessed through 6 months after delivery or termination. ResultsAfter PSM, there were 214, 61, and 46 pregnant women enrolled in the normal control group, low-PVC burden, and high-PVC burden subgroups, respectively. The high-PVC and low-PVC burden subgroups had composite adverse maternal and neonatal events similar to the control group without use of antiarrhythmic drugs (AADs), but a higher proportion of placental abruption was observed in the high-PVC burden subgroup. Maternal age, diabetes, and overweight were significant predictors of composite adverse maternal events, whereas only maternal age was a significant predictor of composite adverse neonatal events. ConclusionsHigh PVC burden was not associated with poor composite adverse maternal and neonatal outcomes with no need of AADs therapy in pregnant women with structurally normal heart.

“Innocent” ventricular arrhythmia – can cardiovascular magnetic resonance augment diagnosis of structural heart disease?

Abstract Background Ventricular arrhythmias (VA) include a spectrum that ranges from premature ventricular beats (VPBs) to ventricular fibrillation (VF) and account for approximately 50% of all cardiovascular deaths. Echocardiography is commonly used to identify structural heart disease (SHD), the most frequent substrate of VA. Cardiovascular magnetic resonance (CMR) is recommended to complement echocardiography when image quality is suboptimal. Purpose This study sought to determine whether CMR may identify SHD in patients (pts) with VA, who had normal baseline ECGs and whose echocardiogram with fair technical conditions, ruled out pathological findings. Methods We included consecutive pts followed in arrythmia outpatient clinic of one clinical center from June 2014 to June 2021 for significant VA; this was categorized as &amp;gt;1,000 but &amp;lt;10,000 VPBs/24 h; ≥10,000 VPBs/24 h; nonsustained ventricular tachycardia (NSVT), sustained VT, or a history of resuscitated cardiac arrest, and no pathological findings at echocardiography, requiring a clinically indicated CMR. Primary endpoint was CMR detection of SHD. Data were collected at the end of the study. Results A total of 75 pts were included (no SHD: N=59; 45±15 years old; SHD: N=16, 53±15 years). All pts performed CMR, and Table 1 shows pts' baseline characteristics, VA diagnosis and CMR measurements. Definite SHD was diagnosed in 8 patients (11%): ischemic cardiopathy (three pts), myocarditis (1 pt), hypertrophic cardiomyopathy (1 pt), right ventricle arrhythmogenic disease (1 pt), non-compaction cardiomyopathy (1 pt); 1 patient presented higher myocardial T2 signal and was later diagnosed with sarcoidosis. Furthermore, abnormal findings not specific for a definite SHD diagnosis were found in other 8 pts (10%) who showed unspecific intra-myocardium enhancement. Discussion and conclusion CMR imaging identified SHD in around 20% of patients with normal ECG and echocardiogram. Among patients with identified SHD, ischemic cardiopathy was the most common finding, differently from the largest study that showed myocarditis as the main diagnosis. This may be associated to higher age in the SHD group. In conclusion, CMR allowed diagnosis of clinically relevant SHD even when echocardiography excluded it. Funding Acknowledgement Type of funding sources: None.

Is cardiac magnetic resonance of additional help in premature ventricular contraction work-up?

Abstract Background Premature ventricular contractions (PVCs) are common in routine medical practice. PVCs are commonly asymptomatic but can also result in palpitations, dyspnea, presyncope or PVC-induced cardiomyopathy that may lead to sudden death. Cardiovascular magnetic resonance (CMR) provides morphological, functional, and tissue characterization in a single setting. CMR imaging proved to reliably identify fibrotic lesions and, hence, has developed into a valuable tool for etiological orientation in patients with PVCs. Objectives The aims of this study were: 1) to evaluate which ventricular arrythmia (VA) characteristics predicted CMR abnormalities and 2) the diagnostic yield and the impact in the management of CMR-based imaging versus non-CMR-based imaging in patients referred for PVCs. Methods This monocentric retrospective study was conducted in patients with PVCs referred for CMR imaging. Their baseline interview and characteristics, physical examination, surface electrocardiogram (ECG) analysis, ambulatory monitoring, echocardiography and treadmill exercise test data were collected when they were available. The CMR (1,5 T and 3.0 T) protocol included cine imaging and late gadolinium enhancement (LGE) using standard cardiac geometries. Results We analyzed 168 patients (65% males, mean age 54.2±16.8 years) referred to our center for PVCs over a two-year period. CMR reported abnormal findings in 73 patients (43.5%) with the diagnosis of structural heart disease (SHD) in 46 patients (27.4%). Left-ventricular late gadolinium-enhancement was documented in 43 patients with abnormal CMR. Using a multivariate analysis, age (p=0.026), sustained ventricular tachycardia (p=0.002) and presence of cardiomyopathy on echocardiography (p&amp;lt;0.001) significantly predicted the presence of abnormal findings on CMR. Compared to non-CMR-based imaging, CMR had a diagnostic yield, by modifying the presumed diagnosis or by finding abnormalities requiring follow-up in 73 patients (43.5%). Conclusions Age, sustained ventricular tachycardia and presence of cardiomyopathy on echocardiography were the strongest predictors of positive CMR imaging results. Although echocardiography is generally used in first intention for the diagnosis of cardiomyopathy in patients with PVCs, this study showed that CMR-based imaging has a robust diagnostic yield in this indication. Funding Acknowledgement Type of funding sources: None.

RECHOmmend: An ECG-Based Machine Learning Approach for Identifying Patients at Increased Risk of Undiagnosed Structural Heart Disease Detectable by Echocardiography.

Background:Timely diagnosis of structural heart disease improves patient outcomes, yet many remain underdiagnosed. While population screening with echocardiography is impractical, ECG-based prediction models can help target high-risk patients. We developed a novel ECG-based machine learning approach to predict multiple structural heart conditions, hypothesizing that a composite model would yield higher prevalence and positive predictive values to facilitate meaningful recommendations for echocardiography.Methods:Using 2 232 130 ECGs linked to electronic health records and echocardiography reports from 484 765 adults between 1984 to 2021, we trained machine learning models to predict the presence or absence of any of 7 echocardiography-confirmed diseases within 1 year. This composite label included the following: moderate or severe valvular disease (aortic/mitral stenosis or regurgitation, tricuspid regurgitation), reduced ejection fraction <50%, or interventricular septal thickness >15 mm. We tested various combinations of input features (demographics, laboratory values, structured ECG data, ECG traces) and evaluated model performance using 5-fold cross-validation, multisite validation trained on 1 site and tested on 10 independent sites, and simulated retrospective deployment trained on pre-2010 data and deployed in 2010.Results:Our composite rECHOmmend model used age, sex, and ECG traces and had a 0.91 area under the receiver operating characteristic curve and a 42% positive predictive value at 90% sensitivity, with a composite label prevalence of 17.9%. Individual disease models had area under the receiver operating characteristic curves from 0.86 to 0.93 and lower positive predictive values from 1% to 31%. Area under the receiver operating characteristic curves for models using different input features ranged from 0.80 to 0.93, increasing with additional features. Multisite validation showed similar results to cross-validation, with an aggregate area under the receiver operating characteristic curve of 0.91 across our independent test set of 10 clinical sites after training on a separate site. Our simulated retrospective deployment showed that for ECGs acquired in patients without preexisting structural heart disease in the year 2010, 11% were classified as high risk and 41% (4.5% of total patients) developed true echocardiography-confirmed disease within 1 year.Conclusions:An ECG-based machine learning model using a composite end point can identify a high-risk population for having undiagnosed, clinically significant structural heart disease while outperforming single-disease models and improving practical utility with higher positive predictive values. This approach can facilitate targeted screening with echocardiography to improve underdiagnosis of structural heart disease.

29 Comparison between pure tachycardiomyopathy and acute heart failure and structural heart disease

Abstract Aims Tachycardiomyopathy (TCM) is a reversible cause of left ventricular dysfunction, secondary to rapid and/or asynchronous, irregular myocardial contraction. The disease can be divided in two main subgroups: arrhythmia-induced TCM also known as pure TCM, where the arrhythmia is the sole reason for the dysfunction, and impure TCM also known as arrhythmia mediated TCM, where the arrhythmia can exacerbate or worsen heart failure (HF) or an underlying heart disease. Pure TCM has already been described as affecting almost 1 out of 10 patients admitted for de novo acute HF. The aim of our study was to compare pure TCM and de novo acute HF and structural heart disease patients in terms of mortality and cardiovascular (CV)-related hospitalizations. Methods and results Prospective, observational study enrolling all consecutive patients with a confirmed diagnosis of TCM and all patients admitted for de novo acute HF. The TCM diagnosis was suspected in all patients admitted for HF-related symptoms, an ejection fraction &amp;lt;50% with concomitant persistent atrial or ventricular arrhythmia, and confirmed after clinical and echocardiographic recovery. Acute HF diagnosis was made in all patients with an ejection fraction &amp;lt;50%, new HF-like symptoms, diagnosis of structural heart disease and no evidence of clinical or echocardiographic recovery. For each patient, all-cause death and CV-related hospitalizations were recorded. One-hundred-and-ten patients with TCM (61.8% males, 68 ± 13 years old) were propensity matched with a control population of patients with HF and structural heart disease (76.6% males, 71 ± 15 years old, 75% ischaemic heart disease). After a median follow-up of 5.1 years (1st–3rd quartile 2.6–7.0 years) TCM patients showed an overall higher estimate of survival when compared to HF patients (78% vs. 58%; P = 0.031; Figure 1A) but a lower estimate of time free from CV-related hospitalization (31% vs. 57%, P = 0.014; Figure 1B). TCM patients got most often readmitted for AF-related elective procedures (60.8% of all hospitalizations) such ablation procedures or elective cardioversion, TCM recurrence (13.7%), and elective coronary angiography (5.9%). On the other hand, HF patients got readmitted for HF worsening (40.9%), cardiac or vascular surgery (22.7%), and elective coronary angiography (9.1%). Propensity-score matched analysis confirmed the results for all-cause death (81% vs. 49%; P = 0.006) and CV-related hospitalizations (29% vs. 54%; P = 0.007). Conclusions TCM is associated with higher rate of survival when compared to de novo acute HF, even after propensity score adjustment. On the other hand, patients with TCM got readmitted more frequently, requiring more often elective procedures in order to control the triggering arrhythmia.

764 Prevalence and clinical implications of cardiac involvement in individuals with paucisymptomatic SARS-CoV-2 infection

AimsMyocardial involvement has been reported in SARS-CoV-2 infection, especially in hospitalized patients during the acute phase of the disease. However, the exact prevalence and the clinical implications of cardiac involvement in young individuals with paucisymptomatic SARS-CoV-2 infection are debated.Methods and resultsWe gathered data on 100 young patients with previous paucisymptomatic SARS-CoV-2 infection, not undergoing hospitalization and without previous diagnosis of structural heart disease, who underwent cardiological evaluation in our clinic at IRCCS ICS Maugeri (Pavia, Italy). Results were validated in an external cohort of 28 patients who underwent cardiac magnetic resonance (MRI) at Humanitas Research Hospital (Rozzano, Italy). The study population included 100 patients with previous paucisymptomatic SARS-CoV-2 infection: 60 (60%) males; median age 36 years (IQR: 22–50 years); median time after SARS-CoV-2 infection 181 days (IQR: 76–218 days). At the cardiological evaluation, 31/100 (31%) of patients referred cardiological symptoms, including dyspnoea, palpitations, chest pain or syncope. Overall, 26/100 (26%) patients showed on or more of the following instrumental alterations at first level assessment: 4/100 (4%) increase of TnI; 7/100 (7%) electrocardiographic abnormalities, 12/100 (12%) ventricular arrhythmias, and 11/100 (11%) echocardiographic abnormalities. Of 32 patients who underwent cardiac MRI, myocardial involvement was detected in 6/32 (19%) patients (Figure 1), similarly to what was observed in the validation cohort [54% males; median age 47 years (IQR: 26–55 years); myocardial involvement at MRI 4/28, 14%]. Furthermore, the proportion of patients with myocardial involvement was significantly higher in patients with first-level cardiac alterations (6/18, 28%) as compared with patients without cardiac alterations at first-level examination (0/14, 0%, P = 0.024). When analysing possible predictors for the occurrence of cardiac involvement at the MRI, documentation of ventricular arrhythmias at Holter ECG or exercise test was associated with an 87-fold higher probability of cardiac involvement at the MRI (OR: 87.3; 95% CI: 4.0–1914.3; P < 0.001).ConclusionsAround 15–20% of patients with paucisymptomatic SARS-CoV-2 infection exhibit cardiac involvement documented at the cardiac MRI after a mean of 6 months from the onset of the disease. The presence of instrumental alterations detected with first level diagnostic tests, and in particular the documentation of ventricular arrhythmias at the 24 h-Holter ECG or at the exercise stress test, is a powerful predictor of myocardial involvement. 764 Figure 1Late gadolinium enhancement (A), extracellular volume (B), T1 mapping (C), and T2 mapping (D) show alterations compatible with sub-acute myocarditis secondary to SARS-CoV-2 infection in a 54-year-old patient.

Abstract 9599: Rechommend: An Ecg-Based Machine-Learning Approach for Identifying Patients at High-Risk of Undiagnosed Structural Heart Disease Detectable by Echocardiography

Introduction: Timely diagnosis of structural heart disease improves patient outcomes, yet millions remain undiagnosed. ECG-based prediction models can help identify high-risk patients for targeted screening, but existing individual disease models often have low positive predictive values (PPV) and limited clinical utility. Hypothesis: An ECG-based composite model can predict one of multiple, actionable structural heart conditions and yield higher prevalence and PPVs than individual models. Methods: Using 2,141,366 ECGs linked to echocardiography and EHR records from 461,466 adults from 1984-2021, we trained machine learning models to predict any of 7 echocardiography-confirmed diseases within 1 year. This composite label included: moderate or severe valvular disease (aortic stenosis or regurgitation, mitral stenosis or regurgitation, tricuspid regurgitation), reduced ejection fraction of &amp;lt;50%, or interventricular septal thickness &amp;gt;15mm. We tested various combinations of inputs and evaluated model performance with 1) cross-validation and 2) a simulated retrospective deployment. We measured area under the receiver operating characteristic curve (AUROC) and precision-recall curve (AUPRC), and PPV at 90% sensitivity. Results: Our composite "rECHOmmend" model using age, sex and ECG traces had an AUROC of 0.91, AUPRC of 0.78, and PPV of 52% at 90% sensitivity and 23% disease prevalence. Individual disease models had similar AUROCs (0.88-0.93), but lower AUPRCs (0.07-0.71) and PPVs (2%-41%; Figure). Across inputs, model AUROCs ranged from 0.85 to 0.93. Our simulated deployment model classified 22% of at-risk patients in 2010 as high-risk, of whom 40% developed true, echo-confirmed disease within 1 year. Conclusions: An ECG-based machine learning model using a composite endpoint can predict undiagnosed structural heart disease, outperforming single disease models with higher PPVs to facilitate targeted screening with echocardiography.

Long-term prognosis of tachycardiomyopathy without underlying heart disease

Abstract Background Tachycardiomyopathy (TCM) is a reversible cause of left ventricular dysfunction, secondary to rapid and/or asynchronous, irregular myocardial contraction. Two categories of the disease have been described: arrhythmia-induced TCM (pure TCM), where the arrhythmia is the sole reason for the dysfunction, and arrhythmia-mediated TCM (impure TCM), where the arrhythmia can exacerbate or worsen heart failure (HF) or an underlying heart disease. Pure TCM has already been described as affecting almost 1 out of 10 patients admitted for acute HF-like symptoms. On the other side, acute HF leads to a chronic irreversible condition which tends to worsen over time and is burdened by a high rate of complications. Purpose The aim of our study was to compare pure TCM and de novo acute HF patients (including all forms of structural heart disease) in terms of mortality and cardiovascular (CV)-related hospitalizations. Methods Prospective, observational study enrolling all consecutive patients with a confirmed diagnosis of TCM and all patients admitted for de novo acute HF. The TCM diagnosis was suspected in all patients admitted for HF-related symptoms, an ejection fraction &amp;lt;50% with concomitant persistent atrial or ventricular arrhythmia, and confirmed after clinical and echocardiographic recovery. Acute HF diagnosis was made in all patients with an ejection fraction &amp;lt;50%, new HF-like symptoms, diagnosis of structural heart disease and no evidence of clinical or echocardiographic recovery. For each patient, all-cause death and CV-related hospitalizations were recorded. Propensity score matching was used in order to compare the two populations through sensitivity analysis. Results One-hundred-and-ten patients with TCM (61.8% males, 68±13 years old) were propensity matched with a control population of patients with HF and structural heart disease (76.6% males, 71±15 years old, 75% ischemic heart disease). After a median follow-up of 5.1 years (1st-3rd quartile 2.6–7.0 years) TCM patients showed an overall higher estimate of survival when compared to HF patients (78% vs. 58%; p=0.031; figure 1A) but a lower estimate of time free from CV-related hospitalization (31% vs. 57%, p=0.014; figure 1B). TCM patients got most often readmitted for AF-related elective procedures (60.8% of all hospitalizations), TCM recurrence (13.7%) and elective coronary angiography (5.9%). On the other hand, HF patients got readmitted for HF worsening (40.9%), cardiac or vascular surgery (22.7%) and elective coronary angiography (9.1%). Propensity-score matched analysis confirmed the results for all-cause death (81% vs. 49%; p=0.006) and CV-related hospitalizations (29% vs. 54%; p=0.007). Conclusions TCM is associated with a better survival when compared to de novo acute HF, even after propensity score adjustment. On the other hand, patients with TCM got readmitted more frequently, requiring more often elective procedures in order to control the triggering arrhythmia. Funding Acknowledgement Type of funding sources: None.

Delivery Planning and Emergent Neonatal Intervention in Transposition of the Great Arteries.

* Abbreviations: CICU: : cardiac intensive care unit d-TGA: : dextro-transposition of the great arteries FIO2: : fraction of inspired oxygen A 20-year-old gravida 2, para 1 pregnant woman was referred to our fetal care center because of a diagnosis of structural heart disease in the fetus. The woman’s medical history included asthma, seasonal allergies, and depression, with no surgical history. She had 1 prior term vaginal delivery. This pregnancy had been complicated by late presentation to prenatal care at 20 weeks’ gestation. Her medications included asthma maintenance medications and prenatal vitamins. Pertinent family history included her partner who had a ventricular septal defect and a paternal male first cousin with transposition of the great arteries. Prenatal laboratory test results were unremarkable and genetic screening was declined. Imaging at the referring obstetrician’s office was significant for transposition of the great arteries with an intact ventricular septum and normal fetal biometry. She was referred to our center at 21 1/7 weeks’ gestation, with imaging and consultation with fetal cardiology at 21 6/7 weeks’ gestation. The following tests were performed: Figure 1. Four-chamber view of the heart at 21 weeks’ gestation, swept to the outflow tracts showing parallel arrangement of the great arteries with the pulmonary artery (PA) located posteriorly and arising from the left ventricle (LV) and the aorta (Ao) arising from the right ventricle (RV). Fetal position: right (R), left (L), anterior (A), posterior (P). At …

Diagnostic Workflow in Competitive Athletes with Ventricular Arrhythmias and Suspected Concealed Cardiomyopathies.

The diagnosis of structural heart disease in athletes with ventricular arrhythmias (VAs) and an apparently normal heart can be very challenging. Several pieces of evidence demonstrate the importance of an extensive diagnostic work-up in apparently healthy young patients for the characterization of concealed cardiomyopathies. This study shows the various diagnostic levels and tools to help identify which athletes need deeper investigation in order to unmask possible underlying heart disease.

Automatic recognition of murmurs of ventricular septal defect using convolutional recurrent neural networks with temporal attentive pooling

Recognizing specific heart sound patterns is important for the diagnosis of structural heart diseases. However, the correct recognition of heart murmur depends largely on clinical experience. Accurately identifying abnormal heart sound patterns is challenging for young and inexperienced clinicians. This study is aimed at the development of a novel algorithm that can automatically recognize systolic murmurs in patients with ventricular septal defects (VSDs). Heart sounds from 51 subjects with VSDs and 25 subjects without a significant heart malformation were obtained in this study. Subsequently, the soundtracks were divided into different training and testing sets to establish the recognition system and evaluate the performance. The automatic murmur recognition system was based on a novel temporal attentive pooling-convolutional recurrent neural network (TAP-CRNN) model. On analyzing the performance using the test data that comprised 178 VSD heart sounds and 60 normal heart sounds, a sensitivity rate of 96.0% was obtained along with a specificity of 96.7%. When analyzing the heart sounds recorded in the second aortic and tricuspid areas, both the sensitivity and specificity were 100%. We demonstrated that the proposed TAP-CRNN system can accurately recognize the systolic murmurs of VSD patients, showing promising potential for the development of software for classifying the heart murmurs of several other structural heart diseases.

Orthostatic vital signs do not predict 30 day serious outcomes in older emergency department patients with syncope: A multicenter observational study

BackgroundSyncope is a common chief complaint among older adults in the Emergency Department (ED), and orthostatic vital signs are often a part of their evaluation. We assessed whether abnormal orthostatic vital signs in the ED are associated with composite 30-day serious outcomes in older adults presenting with syncope. MethodsWe performed a secondary analysis of a prospective, observational study at 11 EDs in adults ≥ 60 years who presented with syncope or near syncope. We excluded patients lost to follow up. We used the standard definition of abnormal orthostatic vital signs or subjective symptoms of lightheadedness upon standing to define orthostasis. We determined the rate of composite 30-day serious outcomes, including those during the index ED visit, such as cardiac arrhythmias, myocardial infarction, cardiac intervention, new diagnosis of structural heart disease, stroke, pulmonary embolism, aortic dissection, subarachnoid hemorrhage, cardiopulmonary resuscitation, hemorrhage/anemia requiring transfusion, with major traumatic injury from fall, recurrent syncope, and death) between the groups with normal and abnormal orthostatic vital signs. ResultsThe study cohort included 1974 patients, of whom 51.2% were male and 725 patients (37.7%) had abnormal orthostatic vital signs. Comparing those with abnormal to those with normal orthostatic vital signs, we did not find a difference in composite 30-serious outcomes (111/725 (15.3%) vs 184/1249 (14.7%); unadjusted odds ratio, 1.05 [95%CI, 0.81–1.35], p = 0.73). After adjustment for gender, coronary artery disease, congestive heart failure (CHF), history of arrhythmia, dyspnea, hypotension, any abnormal ECG, physician risk assessment, medication classes and disposition, there was no association with composite 30-serious outcomes (adjusted odds ratio, 0.82 [95%CI, 0.62–1.09], p = 0.18). ConclusionsIn a cohort of older adult patients presenting with syncope who were able to have orthostatic vital signs evaluated, abnormal orthostatic vital signs did not independently predict composite 30-day serious outcomes.

Structural heart disease as the cause of syncope.

We described the clinical evolution of patients with structural heart disease presenting at the emergency room with syncope. Patients were stratified according to their syncope etiology and available scores for syncope prognostication. Cox proportional hazard models were used to investigate the relationship between etiology of the syncope and event-free survival. Of the 82,678 emergency visits during the study period, 160 (0.16%) patients were there due to syncope, having a previous diagnosis of structural heart disease. During the median follow-up of 33.8±13.8 months, mean age at the qualifying syncope event was 68.3 years and 40.6% of patients were male. Syncope was vasovagal in 32%, cardiogenic in 57%, orthostatic hypotension in 6%, and of unknown causes in 5% of patients. The primary composite endpoint death, readmission, and emergency visit in 30 days was 39.4% in vasovagal syncope and 60.6% cardiogenic syncope (P<0.001). Primary endpoint-free survival was lower for patients with cardiogenic syncope (HR=2.97, 95%CI=1.94-4.55; P<0.001). The scores were analyzed for diagnostic performance with area under the curve (AUC) and did not help differentiate patients with an increased risk of adverse events. The differential diagnosis of syncope causes in patients with structural heart disease is important, because vasovagal and postural hypotension have better survival and less probability of emergency room or hospital readmission. The available scores are not reliable tools for prognosis in this specific patient population.

New concepts for mitral valve imaging.

The high complexity of the mitral valve (MV) anatomy and function is not yet fully understood. Studying especially the dynamic movement and interaction of MV components to describe MV physiology during the cardiac cycle remains a challenge. Imaging is the key to assessing details of MV disease and to studying the lesion and dysfunction of MV according to Carpentier. With the advances of computational geometrical and biomechanical MV models, improved quantification and characterization of the MV has been realized. Geometrical models can be divided into rigid and dynamic models. Both models are based on reconstruction techniques of echocardiographic or computed tomographic data sets. They allow detailed analysis of MV morphology and dynamics throughout the cardiac cycle. Biomechanical models aim to simulate the biomechanics of MV to allow for examination and analysis of the MV structure with blood flow. Two categories of biomechanical MV models can be distinguished: structural models and fluid-structure interaction (FSI) models. The complex structure and dynamics of MV apparatus throughout the cardiac cycle can be analyzed with different types of computational models. These represent substantial progress in the diagnosis of structural heart disease since MV morphology and dynamics can be studied in unprecedented detail. It is conceivable that MV modeling will contribute significantly to the understanding of the MV.

Women Have a Lower Prevalence of Structural Heart Disease as a Precursor to Sudden Cardiac Arrest: The Ore-SUDS (Oregon Sudden Unexpected Death Study)

Our aim was to utilize a community-based approach to identify sex-related differences in risk factors for sudden cardiac arrest (SCA). There are significant sex-based differences in prevalence and manifestation of SCA. Any differences related to predictors of SCA in women versus men are likely to have implications for risk stratification and prevention. The Ore-SUDS (Oregon Sudden Unexpected Death Study) is an ongoing prospective investigation of SCA in the Portland, Oregon, metropolitan area (population approximately 1 million). All cases meeting criteria for SCA were ascertained using multiple sources. Medical records were reviewed to identify clinical conditions that may contribute to SCA risk, and comparisons were made between male and female SCA cases using Pearson's chi-square tests for categorical variables, t tests for continuous variables, and multivariate logistic regression analysis. During 2002 to 2007, 1,568 adult SCA cases were identified (women 36% vs. men 64%; p < 0.0001) and women were older (mean age 71 +/- 14 years vs. 65 +/- 14 years, p < 0.0001). There were no significant sex differences in prevalence of obesity, dyslipidemia, history of chronic obstructive pulmonary disease/asthma, left ventricular (LV) hypertrophy, or history of myocardial infarction. In multivariate analysis, women were significantly less likely to have severe LV dysfunction (odds ratio: 0.51; 95% confidence interval: 0.31 to 0.84) or previously recognized coronary artery disease (odds ratio: 0.34; 95% confidence interval: 0.20 to 0.60) compared with men. Women were significantly less likely than men to have a diagnosis of structural heart disease (LV dysfunction or coronary artery disease) before SCA. These findings suggest that fewer women may be eligible for prophylactic implantable cardioverter-defibrillator placement based on current guidelines and therefore may not have equal opportunity for prevention. Enhancement of SCA risk stratification may have even higher importance for women.

Use of pharmaceuticals in noninvasive cardiovascular diagnosis.

A number of pharmaceuticals are employed as diagnostic agents for cardiovascular diseases. Four groups of agents are reviewed here: 1) vasoactive substances employed as adjuncts to physical maneuvers in diagnosis of structural heart disease; 2) vasodilators used to produce heterogeneity of coronary flow; 3) sympathomimetic agents simulating the effects of exercise on the heart for the purpose of detection of coronary artery stenosis; and 4) ultrasonic contrast agents used to enhance myocardial imaging for the assessment of segmental wall motion. In the first group are amyl nitrate, a vasodilator, and methoxamine and phenylephrine, both vasopressors. The vasodilators of the second group are dipyridamole and adenosine. When combined with scintigraphic perfusion imaging or with echocardiographic assessment of segmental wall motion, these agents can detect single- or multiple-vessel coronary artery disease with sensitivity and specificity comparable to submaximal exercise. They are especially useful for preoperative risk assessment before noncardiac surgery. The sympathomimetic agents of the third group, dobutamine and arbutamine, increase myocardial contractility and heart rate, and dilate the peripheral vasculature. As with the vasodilators, when combined with nuclear or echocardiographic techniques they are equivalent to exercise in detection of coronary disease. They are especially useful in patients with bronchospastic disease and for assessment of myocardial viability. Agents from groups 2 and 3 have acceptable side-effect and safety profiles. The last group reviewed includes echocardiographic contrast agents that, in this investigative setting, are employed to enhance detection of segmental wall motion when used with agents from groups 2 and 3.

New developments in fetal echocardiography.

Fetal echocardiography has assumed an increasingly important role in the diagnosis of structural heart disease. Recently, the usefulness of this technique as a tool with which to observe the prenatal natural history of congenital heart disease has been appreciated. The application of spectral and color Doppler ultrasonographic interrogation has added to our ability to assess the integrity of the fetal and placental circuit. Only when we can consistently and accurately diagnose congenital heart disease in utero will we be able to successfully apply prenatal treatment for this condition.