Concurrent congenital pseudoaneurysm of mitral-aortic intervalvular fibrosa and left sinus of Valsalva aneurysm

2019 ◽  
Vol 8 (2) ◽  
Author(s):  
Valdano Manuel

Either the pseudoaneurysm of mitral-aortic intervalvular fibrosa or the aneurysms of Valsalva are rare cardiac conditions. Concomitant diagnosis in the same patient is extremely rare with only one case report. Herein, we described an extremely rare case of concomitant pseudoaneurysm of mitral-aortic intervalvular fibrosa and aneurysm of left sinus of Valsalva in 21-year-old black man admitted with new-onset of heart failure (NYHA class III) diagnosed by transesophageal echocardiography. Despite medical therapy optimization for heart failure, the patient died waiting for cardiac surgery. Regarding the case, we discussed possible etiology, clinical presentation and the role of echocardiography in the diagnosis and surgical approach of this very rare association.

2019 ◽  
Vol 11 (4) ◽  
pp. NP232-NP234
Author(s):  
Federica Caldaroni ◽  
Mauro Lo Rito ◽  
Antonio Saracino ◽  
Alessandro Varrica ◽  
Luca Giugno ◽  
...  

Congenital sinus of Valsalva aneurysm is a rare disorder, mostly involving the right and noncoronary sinuses, in which intracardiac rupture is more likely foreseen into the right chambers. Because of the unfavorable prognosis, which includes heart failure and sudden death, detection itself is an indication for treatment, which may be performed either surgically or percutaneously. We present a case of a four-year-old patient with aorto-right atrial tunnel, in which a transcatheter attempt of closure was performed, complicated by new onset of aortic valve regurgitation, requiring surgical intervention.


2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Claire E. Raphael ◽  
Frances Mitchell ◽  
Gajen Sunthar Kanaganayagam ◽  
Alphonsus C. Liew ◽  
Elisa Di Pietro ◽  
...  

Abstract Introduction Heart failure (HF) in hypertrophic cardiomyopathy (HCM) is associated with high morbidity and mortality. Predictors of HF, in particular the role of myocardial fibrosis and microvascular ischemia remain unclear. We assessed the predictive value of cardiovascular magnetic resonance (CMR) for development of HF in HCM in an observational cohort study. Methods Serial patients with HCM underwent CMR, including adenosine first-pass perfusion, left atrial (LA) and left ventricular (LV) volumes indexed to body surface area (i) and late gadolinium enhancement (%LGE- as a % of total myocardial mass). We used a composite endpoint of HF death, cardiac transplantation, and progression to NYHA class III/IV. Results A total of 543 patients with HCM underwent CMR, of whom 94 met the composite endpoint at baseline. The remaining 449 patients were followed for a median of 5.6 years. Thirty nine patients (8.7%) reached the composite endpoint of HF death (n = 7), cardiac transplantation (n = 2) and progression to NYHA class III/IV (n = 20). The annual incidence of HF was 2.0 per 100 person-years, 95% CI (1.6–2.6). Age, previous non-sustained ventricular tachycardia, LV end-systolic volume indexed to body surface area (LVESVI), LA volume index ; LV ejection fraction, %LGE and presence of mitral regurgitation were significant univariable predictors of HF, with LVESVI (Hazard ratio (HR) 1.44, 95% confidence interval (95% CI) 1.16–1.78, p = 0.001), %LGE per 10% (HR 1.44, 95%CI 1.14–1.82, p = 0.002) age (HR 1.37, 95% CI 1.06–1.77, p = 0.02) and mitral regurgitation (HR 2.6, p = 0.02) remaining independently predictive on multivariable analysis. The presence or extent of inducible perfusion defect assessed using a visual score did not predict outcome (p = 0.16, p = 0.27 respectively). Discussion The annual incidence of HF in a contemporary ambulatory HCM population undergoing CMR is low. Myocardial fibrosis and LVESVI are strongly predictive of future HF, however CMR visual assessment of myocardial perfusion was not.


2019 ◽  
Vol 25 (4) ◽  
pp. 234-239
Author(s):  
Justas Simonavičius ◽  
Dalia Jarašūnienė

Sinus of Valsalva aneurysm is an uncommon finding. The present paper describes a case report of a 57-year-old female patient who developed angina pectoris due to the left sinus of Valsalva aneurysm. Clinical presentation, localization, unruptured status and concomitant coarctation of the aorta make the case presented in this document extremely rare and interesting. Current knowledge about the disease is briefly discussed.


Author(s):  
Ignacio Juaneda ◽  
Alejandro E. Contreras ◽  
Alejandro R. Peirone ◽  
Ignacio Juaneda

A 20-year-old boy with Down´s syndrome presented to the emergency department with new onset acute heart failure. Right side chest effusion and cardiomegaly was observed on chest x-ray. A mobile aneurysmal sac protruding into the right atrium was detected and a ruptured aneurysm of the right coronary sinus of Valsalva was observed in transthoracic echocardiogram. Cardiac angiography catheterization confirmed the diagnosis. Surgical repair consisting of aneurysm resection and defect closure was performed shortly after with pericardium reinforced suture through the right atrium. The patient was discharged uneventfully.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Aadhavi Sridharan ◽  
Martin S Maron ◽  
Dou Huang ◽  
Craig Cooper ◽  
Jennifer Drummond ◽  
...  

Background: The impact of comorbid disease states and lifestyle on the natural history of patients with hypertrophic cardiomyopathy (HCM) remains unknown. Objective: Evaluate the association of non-HCM comorbidities including obesity, hypertension, diabetes, obstructive sleep apnea, kidney disease, tobacco use, alcohol use, and lung disease with disease progression in a large cohort of HCM patients. Methods: 2269 patients evaluated at the Tufts HCM Institute between 2004 to 2019, ≥ 18 years of age (54 ± 15 years; 1392 male), and followed for an average of 4 ± 3.4 years for disease progression including progressive heart failure (HF) symptoms (from NYHA class I/II to NYHA class III/IV), new-onset atrial fibrillation (AF), or sudden death (SD) event (including appropriate defibrillation for ventricular arrhythmias, resuscitated cardiac arrest, or SD). Results: Of 1376 patients with NYHA class I/II symptoms at initial clinical evaluation, 252 (18%) developed progressive HF symptoms to NYHA class III/IV over follow-up (5%/year). Obesity (BMI ≥ 30) was significantly more prevalent in patients who had progressive HF during follow-up (43%) compared to those who remained without HF (34%, p = 0.014). In contrast, other comorbidities were not significantly associated with progressive HF symptoms (p > 0.10 for all other comorbidities). Of the 1823 patients without AF history at initial clinical visit, 198 (11%) developed new-onset AF over follow-up (3%/year). No comorbidities were significantly associated with new-onset AF in HCM (p > 0.10), although obesity was more common in patients who developed new-onset AF (48%) compared to those who had no AF (41%, p = 0.08). Notably, SD events were not associated with non-HCM comorbidities (p > 0.10 for all comorbidities), and patients with SD events were less likely to have comorbidities than patients without SD events. Conclusions: In adult HCM patients, obesity is associated with progressive symptoms and outcomes supporting weight loss as an important modifier in obese HCM patients to potentially help prevent HCM complications. In contrast, other non-HCM comorbidities do not appear to impact disease course, and SD events are not associated with comorbidities in HCM.


2015 ◽  
Vol 2015 ◽  
pp. 1-10 ◽  
Author(s):  
Aderville Cabassi ◽  
Simone Maurizio Binno ◽  
Stefano Tedeschi ◽  
Gallia Graiani ◽  
Cinzia Galizia ◽  
...  

Rationale. Heart failure (HF) is accompanied by the development of an imbalance between oxygen- and nitric oxide-derived free radical production leading to protein nitration. Both chlorinating and peroxidase cycle of Myeloperoxidase (MPO) contribute to oxidative and nitrosative stress and are involved in tyrosine nitration of protein. Ceruloplasmin (Cp) has antioxidant function through its ferroxidase I (FeOxI) activity and has recently been proposed as a physiological defense mechanism against MPO inappropriate actions.Objective. We investigated the relationship between plasma MPO-related chlorinating activity, Cp and FeOxI, and nitrosative stress, inflammatory, neurohormonal, and nutritional biomarkers in HF patients.Methods and Results. In chronic HF patients (n=81, 76±9 years, NYHA Class II (26); Class III (29); Class IV (26)) and age-matched controls (n=17, 75±11 years, CTR), plasma MPO chlorinating activity, Cp, FeOxI, nitrated protein, free Malondialdehyde, BNP, norepinephrine, hsCRP, albumin, and prealbumin were measured. Plasma MPO chlorinating activity, Cp, BNP, norepinephrine, and hsCRP were increased in HF versus CTR. FeOxI, albumin, and prealbumin were decreased in HF. MPO-related chlorinating activity was positively related to Cp (r= 0.363,P<0.001), nitrated protein, hsCRP, and BNP and inversely to albumin.Conclusions. Plasma MPO chlorinated activity is increased in elderly chronic HF patients and positively associated with Cp, inflammatory, neurohormonal, and nitrosative parameters suggesting a role in HF progression.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
E Calero ◽  
E Hidalgo ◽  
R Marin ◽  
L Rosenfeld ◽  
I Fernandez ◽  
...  

Abstract Background Self-care is a crucial factor in the education of patients with heart failure (HF) and directly impacts in the progression of the disease. However, little is published about its major clinical implications as admission or mortality in patients with HF. Aims and methods The aim of the study was to analyze time to admission due to acute heart failure and mortality associated with poor self-care in patients with chronic HF. We prospectively recruited consecutive patients with stable chronic HF referred to a nurse-led HF programme. Selfcare was evaluated at baseline with the 9 item European Heart Failure Self-Care Behavior Scale. Scores were standardized and reversed from 0 (worst selfcare) to 100 (better self care). For the purpose of this study we analyzed the associations of worse self-care (defined as scores below the lower tertile of the scale) with demographic, disease-related (clinical) and psychosocial factors in all patients at baseline. Results We included 1123 patients, mean age 72±11, 639 (60%) were male, mean LVEF 45±17 and 454 (40,4%) were in NYHA class III or IV. Mean score of the 9-item ESCBE was 69±28. Score below 55 (lower tertile) defined impaired selfcare behaviour. Those patients with worse self-care had more ischaemic heart disease, more COPD, and they achieved less distance in the 6 minute walking test. Regarding psychosocial items patients in lower tertile of self-care needed a caregiver more frequently, they present more cognitive impairment, depressive symptoms and worse score in terms of health self-perception. Multivariate Cox Models showed that a score below 55 points in 9-item ESCBE was independently associated with higher readmission due to acute heart failure [HR 1.26 (1.02–1.57), p value=0.034] and with mortality [HR 1.24 CI95% (1.02–1.50), p value=0.028] Conclusion Poor self-care measured with the modified 9-item ESCBE was associated with higher risk of admission due to acute decompensation and higher risk of mortality in patients with chronic heart failure. These results highlight the importance of assessing self-care and provide measures to improve them. Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Hospital Univesitario de Bellvitge


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
U Zeymer ◽  
L.H Lund ◽  
V Barrios ◽  
C Fonseca ◽  
A.L Clark ◽  
...  

Abstract Background Heart failure (HF) is a major medical and economic burden that is often managed in office based practices. Recently, the angiotensin receptor neprilysin inhibitor (ARNI) sacubitril/valsartan (S/V) was introduced as novel therapeutic option into European guidelines for the management of HF. The ARIADNE registry aims to provide information on how outpatients with HF with reduced ejection fraction (HFrEF) are managed in Europe, in light of this novel treatment option. Methods ARIADNE was a prospective registry of patients with HFrEF treated by office-based cardiologists (OBC) or selected primary care physicians (recognized as HF specialists; PCP) in a real world setting. HFrEF patients were included prospectively, independently of whether treatment had been changed recently or not. 9069 patients were recruited from 687 centres in 17 European countries. Results The mean age of all patients was 68.1 years (S/V: 67.3 years, Non-S/V: 68.9 years). The majority of patients were in NYHA class II (61.3%), or NYHA class III (37.1%) overall, while more patients in the S/V group showed NYHA class III (S/V: 42.8%, Non-S/V: 30.9%). Mean LVEF was slightly lower in the S/V group than in the Non-S/V group (S/V: 32.7%, Non-S/V: 35.4%, overall 34.0%). The most frequently observed signs of HF were dyspnoea upon effort, followed by fatigue, palpitations on exertion at baseline. More patients tend to have more severe symptoms in the S/V groups (e.g. for dyspnoea on effort, Non-S/V: moderate 40.8%, severe 8.6%; S/V: moderate 46.4%, severe 14.1%). 44.0% of patients from the S/V group and 39.3% of non-S/V patients reported at least one hospitalization within 12 months prior to baseline, of which 73.3% in S/V and 69.9% in non-S/V patients were due to HF., At baseline, 44.7% of the patients used a CV device, of which most were implantable cardioverter defibrillator (ICD: Non-S/V 54.2%, S/V: 52.8%), implantable cardioverter defibrillator (CRT-ICD:Non-S/V 21.9%, S/V: 27.0%), and pacemaker (Non-S/V: 13.4%, S/V: 10.5%). The mean KCCQ overall summary score was 62.6 in the S/V group and 69.5 in the Non-S/V group at baseline. 83.9% of patients were treated with ARB or ACEi in Non-S/V group, (ACEi 57.3%, ARB 26.9%). The most frequently taken drug combinations in either group were ACEi/ ARB or S/V with β -blockers (Non-S/V 69.3%, S/V 67.3%). 40.2% in the Non-S/V group and 42.9% in S/V groups used a combination of ACEi/ARB or S/V, β-blocker and MRA. Conclusions The ARIADNE prospective registry provides insights and reflects variations in HF treatment practices in outpatients in Europe and the way S/V was introduced by OBCs and specialized PCPs in a real-world setting. In the observed population, S/V is more often prescribed to slightly younger patients with slightly lower LVEF, there was a greater observed percentage of S/V patients NYHA class III, with lower quality of life measurements and with more severe symptoms and recent hospitalizations for heart failure. Funding Acknowledgement Type of funding source: Private company. Main funding source(s): Novartis Pharma AG


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