Pulsus alternans in a child with dilated cardiomyopathy

2017 ◽  
Vol 28 (3) ◽  
pp. 479-481
Author(s):  
Hasti Sanandajifar ◽  
Sarosh P. Batlivala
Keyword(s):  
Dilated Cardiomyopathy ◽  
Pulse Wave ◽  
Severe Heart Failure ◽  
Clinical Information ◽  
Left Ventricular ◽  
Warning Sign ◽  
Pulse Wave Amplitude ◽  
Pulsus Alternans ◽  
Myocardial Oxygen Supply ◽  
New Onset

AbstractA previously healthy 21-month-old presented with new-onset dilated cardiomyopathy. Evaluation noted pulsus alternans, with beat-to-beat alternations in aortic pulse wave amplitude, both clinically and on diagnostic studies. Pulsus alternans is an infrequent, complex pathophysiologic sign often associated with severe heart failure. The mechanisms are incompletely understood, but theorised aetiologies include beat-to-beat changes in left ventricular loading conditions, variations in myocardial oxygen supply/demand, and alternations in myocardial contractility. Recognition of pulsus alternans is important as it provides significant clinical information, may suggest suboptimal medical management, and may be the first warning sign of severe cardiac dysfunction.

Abstract P294: Greater Peripheral Vasoconstriction During Mental Stress Predicts Adverse Cardiovascular Outcomes

Circulation ◽  
2018 ◽  
Vol 137 (suppl_1) ◽  
Author(s):  
Jeong Hwan Kim ◽  
Muhammad Hammadah ◽  
Ibhar Al Mheid ◽  
Kobina Wilmot ◽  
Ronnie Ramadan ◽  
...  
Keyword(s):  
Mental Stress ◽  
Wave Amplitude ◽  
Pulse Wave ◽  
Smoking Status ◽  
Left Ventricular ◽  
Cardiovascular Outcomes ◽  
Peripheral Vasoconstriction ◽  
Pulse Wave Amplitude ◽  
Digital Pulse ◽  
Peripheral Arterial

Introduction: Peripheral arterial vasoconstriction during mental stress (MS) has been associated with mental stress-induced myocardial ischemia, which in turn has been linked with worse cardiovascular outcomes. It is unknown whether the magnitude of peripheral vasoconstriction with MS is predictive of long term outcomes. Hypothesis: We hypothesized that greater peripheral arterial vasoconstriction during MS would be associated with adverse events among patients with coronary artery disease (CAD). Methods: Four hundred sixty-three patients with stable CAD and normal left ventricular function, (age 63±9, 75% male, 27% Black, EF 60±8 %) underwent MS testing with a standardized public speaking stressor. Digital pulse wave amplitude was continuously measured at baseline and during MS using peripheral arterial tonometry (PAT), and the PAT ratio of pulse wave amplitude (during mental stress/ baseline) was calculated. Cox proportional hazard models were calculated to examine the association between the PAT ratio and outcomes. Results: Median PAT ratio during MS was 0.68, indicating 32% average constriction with MS compared to rest. Subjects with greater peripheral constriction [low (<median) PAT ratio] were more likely to be male (80% vs. 70%, P=0.008) compared to those with PAT ratio ≥median, but their risk factor profiles were similar. During 2.8±0.5 year follow-up, 64 patients had adverse cardiac events including 7 cardiovascular deaths, 19 MI, and 54 revascularization events. After adjusting for age, sex, race, hypertension, diabetes, current smoking status, and prior MI, those with low PAT ratio had a greater risk of CV death/MI (HR[hazard ratio] 2.49, 95% CI [1.04-5.99]) and CV death/MI/revascularization (HR 1.77, 95% CI [1.03-3.04]) compared to those with high PAT ratio. Conclusion: Greater peripheral arterial vasoconstriction with MS is associated with a higher risk of adverse cardiovascular outcomes in patients with CAD.

Abstract 16801: Improved Transplant-Free Survival of Children With Dilated Cardiomyopathy: Analysis of Two Decades From the Pediatric Cardiomyopathy Registry

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Rakesh K Singh ◽  
Charles Canter ◽  
Ling Shi ◽  
Steven D Colan ◽  
Debra A Dodd ◽  
...  
Keyword(s):  
Dilated Cardiomyopathy ◽  
Competing Risks ◽  
Pediatric Patients ◽  
Cox Regression ◽  
Beta Blockers ◽  
Clinical Information ◽  
Left Ventricular ◽  
Pediatric Cardiomyopathy ◽  
Z Scores ◽  
Improvement In Survival

Background: Previous studies of pediatric patients with dilated cardiomyopathy (DCM) have suggested improvement in survival has been due to utilization of heart transplantation. We sought to characterize the changing clinical characteristics and outcomes of pediatric patients with DCM phenotype over the past two decades. Methods: Longitudinal data from 1953 children diagnosed with DCM in the NHLBI Pediatric Cardiomyopathy Registry (PCMR) from 1990-2009 were divided into two cohorts based upon year of diagnosis: “Era 1” (1990-1999, n=1199) and “Era 2” (2000-2009, n=754). Clinical information at diagnosis and incidence of transplant and death without transplant were analyzed by DCM etiology and era. Competing risks methodology was used to estimate the cumulative incidence of death or transplant by era. Results: The majority of patients in both groups had idiopathic DCM (71 vs. 71%, p=0.96). Median age (1.6 vs. 1.7 yrs, p=0.45), left ventricular end diastolic Z scores (+4.2 vs. +4.2, p=0.84) and fractional shortening (16 vs. 17%, p=0.28) at diagnosis were similar. Era 2 patients were more likely to be treated with ACE inhibitors (71 vs. 62%, p=0.004), beta-blockers (24 vs. 6%, p<0.001), and diuretics (89 vs. 84%, p=0.002). A total of 291 patients (15%) died without transplantation, with a median time from diagnosis to death of 0.4 years. Cox regression modeling demonstrated Era 1 was associated with a higher rate of death (HR=1.5, 95% CI=1.2-2.0, p=0.002) but not transplant (0.9, 95% CI=0.7-1.1, p=0.25) when controlling for etiology group. Competing risks estimate (Figure) showed that Era 1 was associated with a higher death rate (p<0.001), while heart transplant rate was not significantly different by era (p=0.068). Conclusions: Children with DCM phenotype have improved survival in the more recent era. This appears to be associated with factors other than the availability of transplantation, which was equally prevalent in both eras.

scholarly journals Three-Dimensional Right Ventriculo-Arterial Coupling as an Independent Correlate of Severe Heart Failure Symptoms in Patients with Dilated Cardiomyopathy

2021 ◽  
Author(s):  
Aura Vijiiac ◽  
Sebastian Onciul ◽  
Silvia Deaconu ◽  
Radu Vatasescu ◽  
Claudia Guzu ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Artery ◽  
Right Ventricular ◽  
Dilated Cardiomyopathy ◽  
Three Dimensional ◽  
Severe Heart Failure ◽  
Systolic Pressure ◽  
Left Ventricular ◽  
Pulmonary Artery Systolic Pressure ◽  
Characteristic Analysis

Background: Right ventricular-pulmonary artery coupling (RVPAC) is a predictor of outcome in pulmonary hypertension. However, the role of this parameter in dilated cardiomyopathy (DCM) remains to be established. The aim of this study was to assess the contribution of RVPAC to the occurrence of severe heart failure (HF) symptoms in patients with DCM using three-dimensional (3D) echocardiography. Methods: We prospectively screened 139 outpatients with DCM, 105 of whom were enrolled and underwent 3D echocardiographic assessment. RVPAC was estimated non-invasively as the 3D right ventricular stroke volume (SV) to end-systolic volume (ESV) ratio. Severe HF symptoms were defined by New York Heart Association (NYHA) class III or IV. We evaluated differences in RVPAC across NYHA classes and the ability of RVPAC to predict severe symptoms. Results: Mean left ventricular (LV) ejection fraction was 28±7%. Mean RVPAC was 0.77±0.30 and it was significantly more impaired with increasing symptom severity (p=0.001). RVPAC was the only independent correlate of severe HF symptoms, after adjusting for age, diuretic use, LV systolic function, LV diastolic function and pulmonary artery systolic pressure (OR 0.035 [95% CI, 0.004 – 0.312], p=0.003). By receiver-operating characteristic analysis, the RVPAC cut-off value for predicting severely symptomatic status was 0.54 (area under the curve=0.712, p<0.001). Conclusion: 3D echocardiographic SV/ESV ratio is an independent correlate of severe HF symptoms in patients with DCM. 3D RVPAC might prove to be a useful risk stratification tool for these patients, should it be further validated in larger studies.

Abstract 17964: Right Ventricular Reverse Remodeling during Long-Term Continuous-Flow Left Ventricular Assist Device Support in Patients with Dilated Cardiomyopathy

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Tetsuya Saito ◽  
Koichi Toda ◽  
Shigeru Miyagawa ◽  
Teruya Nakamura ◽  
Yasushi Yoshikawa ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Dilated Cardiomyopathy ◽  
Continuous Flow ◽  
Positive Impact ◽  
Interstitial Fibrosis ◽  
Severe Heart Failure ◽  
Left Ventricular ◽  
Reverse Remodeling ◽  
Lvad Support

Introduction: Although right ventricle (RV) failure as well as left ventricle (LV) failure can be of major concern in the treatment for severe heart failure, in clinical situation, only LV assist devices (LVAD) have been implanted despite the presence of RV failure. However, the circulatory and histological changes in the failed RV after long term continuous-flow (CF)-LVAD support have not been fully elucidated. In this study, we hypothesized that CF-LVAD long term support may ameliorate RV failure and have a positive impact on the distressed pathology of RV. Methods: This study included 16 dilated cardiomyopathy (DCM) patients who required long-term LVAD support, for 852 ± 84(129-1607) days, before heart transplantation (HTx). Echocardiograms were performed to analyze LV and RV functions. Transmural LV tissues and RV tissues sampled before LVAD implantation and after LVAD explantation were histopathologically analyzed for LV and RV remodeling. Results: Echocardiography showed significantly reduced LV end-diastolic and end-systolic dimensions during CF-LVAD support (73 ± 11 mm to 63 ± 13.6 mm, 67 ± 12 mm to 57 ± 17 mm, P<0.05, respectively). Regarding RV function, right ventricular end-diastolic dimension (RVDd) tended to decrease (48 ± 11 mm to 43 ± 10 mm, P=0.10) and the trans tricuspid pressure gradient (TRPG) was significantly decreased during long-term LVAD support (31 ± 14.8 mmHg to 12 ± 3.2 mmHg, P=0.01). Histopathologically, the sizes of cardiomyocytes in both the LV and the RV were significantly decreased during CF-LVAD support (left: 33.1±5.1 μm to 29.1±4.0 μm, P=0.05, right: 25.5 ± 2.4 μm to 21.6 ± 3.2 μm, P=0.01) and interstitial fibrosis tended to decrease in the RV (29.5 ± 3.1 % to 19.7 ± 7.2 %, P=0.10) despite there being no change in the LV (24.9 ± 10.8 % to 27.1 ± 12.1 %, P=0.14). Conclusion: Long-term CF-LVAD support ameliorates RV failure, possibly by reducing afterload on the RV followed by unloading of LV pressure overload, and thereby exerts a positive impact on RV reverse remodeling.

Intravenous immunoglobulins in children with new onset dilated cardiomyopathy

2017 ◽  
Vol 28 (1) ◽  
pp. 46-54 ◽  
Author(s):  
Josephine F. Heidendael ◽  
Suzanne L. Den Boer ◽  
Joanne G. Wildenbeest ◽  
Michiel Dalinghaus ◽  
Bart Straver ◽  
...  
Keyword(s):  
Dilated Cardiomyopathy ◽  
Viral Myocarditis ◽  
Intravenous Immunoglobulins ◽  
Left Ventricular ◽  
Hazard Ratio ◽  
Structural Defects ◽  
Free Survival ◽  
Systolic Left Ventricular Function ◽  
Median Period ◽  
New Onset

AbstractBackgroundDilated cardiomyopathy is a rare but serious disorder in children. No effective diagnostic or treatment tools are readily available. This study aimed to evaluate the efficacy of intravenous immunoglobulins in children with new onset dilated cardiomyopathy.Methods and resultsIn this retrospective cohort study, 94 children with new onset dilated cardiomyopathy were followed during a median period of 33 months. All patients with secondary dilated cardiomyopathy – for example, genetic, auto-immune or structural defects – had been excluded. Viral tests were performed in all patients and 18 (19%) children met the criteria for the diagnosis “probable or definite viral myocarditis”. Intravenous immunoglobulins were administered to 21 (22%) patients. Overall transplant-free survival was 75% in 5 years and did not differ between treatment groups. The treatment was associated with a higher recovery rate within 5 years, compared with non-treated children (70 versus 43%, log rank=0.045). After correction for possible confounders the hazard ratio for recovery with intravenous immunoglobulins was not significant (hazard ratio: 2.1; 95% CI: 1.0–4.6; p=0.056). Administration of intravenous immunoglobulins resulted in a greater improvement in the shortening fraction of the left ventricle.ConclusionIn our population of children with new onset dilated cardiomyopathy, of either viral or idiopathic origin, intravenous immunoglobulins were administered to a minority of the patients and did not influence transplant-free survival, but were associated with better improvement of systolic left ventricular function and with better recovery. Our results support the concept that children with new onset dilated cardiomyopathy might benefit from intravenous immunoglobulins.

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