Abstract 17017: Risk Prediction of Death and Failure in Patients With Atrio-pulmonary Connections: 40 Years Experience in Australia and New Zealand

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Chin L Poh ◽  
Diana Zannino ◽  
Robert G Weintraub ◽  
David S Winlaw ◽  
Dorothy J Radford ◽  
...  

Objective: To identify parameters predictive of death and failure in patients with an atrio-pulmonary connection Fontan. Methods: Retrospective analysis of long-term follow-up data using the Australia and New Zealand Fontan Registry Results: There were 245 patients surviving hospital discharge after an atriopulmonary Fontan from 1975 to 1994. At latest follow-up, 54 pts died, 29 underwent heart transplantation and 93 had Fontan failure (death, transplantation, Fontan conversion, protein losing enteropathy, severe left ventricular dysfunction on echocardiography or NYHA3). Twenty-eight years survival, freedom from death and transplantation and freedom from Fontan failure were respectively 62% (95%CI 55-71%), 58% (95% CI 50-67%) and 40% (95% CI 33-50%). Predictors of death and transplantation were dextrocardia (HR 1.8;p= 0.05), longer Fontan bypass time (HR= 1.01, p=0.05) and moderate or more late atrioventricular valve regurgitation (HR 3.97; p=0.02). One hundred and seven patients developed atrial arrhythmias, with first documented event at 15.7 years (IQR 10.5-19.3) post Fontan completion. Development of arrhythmia increased the likelihood of death or transplantation (HR 3.05, p<0.001) and Fontan failure (HR 4.7, p<0.001). Thromboembolic events occurred in 42 patients, 30 (71%) of whom were on warfarin but less than half (12/30) within therapeutic range. Thirty-nine patients underwent Fontan conversion. Threshold for conversion was lower in one center in which patients were operated earlier with better functional status, shorter history of arrhythmias and less antiarrhythmic medications. Conversion resulted in a reduced risk of Fontan failure (HR 0.48) in patients cared for by the center with lower conversion threshold but an increased risk in the remaining centres (HR 5.65) (p=0.023). Conclusions: Arrhythmias is an early sign predicting failure of the Fontan circulation of those with an atrio-pulmonary Fontan. Fontan conversion is only associated with improved outcomes when timely performed.

Heart ◽  
2020 ◽  
pp. heartjnl-2020-317619
Author(s):  
David W Baker ◽  
Mark R Dennis ◽  
Diana Zannino ◽  
Chris Schilling ◽  
Patricia D Moreno ◽  
...  

ObjectiveA high risk of morbidity and mortality is well documented in adults with a Fontan circulation. The difference in outcomes between those with and without significant morbidity at the time of transition to adult care has not been well characterised.MethodsWe analysed clinical outcomes in patients enrolled in the Australian and New Zealand Fontan Registry ≥16 years of age. Low risk (LR) Fontan patients were defined as those without history of sustained arrhythmia, thromboembolic event, transplantation, Fontan conversion, protein-losing enteropathy, plastic bronchitis, New York Heart Association class III/IV and/or moderate/severe atrioventricular valve regurgitation or ventricular dysfunction. Increased risk (IR) patients had one or more risk factor.ResultsInclusion criteria were met in 822 patients; mean age 26±8 years, median follow-up from age 16 was 9 years, 203 had atriopulmonary connection (APC) and 619 had total cavopulmonary connection (TCPC). Survival at 30 years was higher in the LR versus IR; 94% versus 82% (p=0.005), 89% versus 77% (p=0.07) for APC and 96% versus 89% (p=0.05) for TCPC. LR patients experienced less Fontan failure (HR 0.34, 95% CI 0.23 to 0.49, p<0.001) and ventricular dysfunction (HR 0.46, 95% CI 0.29 to 0.71, p=0.001) compared with IR patients. For LR TCPC patients, modelled survival projections at 60 years were 49%–67%.ConclusionsClinical outcomes for adolescents LR at transition to adult care are markedly superior to those who have established risk factors for Fontan failure, which is an important consideration when formulating individualised long-term risk estimates and counselling patients.


2015 ◽  
Vol 17 (6) ◽  
pp. 285
Author(s):  
Lucian Florin Dorobantu ◽  
Ovidiu Chioncel ◽  
Alexandra Pasare ◽  
Dorin Lucian Usurelu ◽  
Ioan Serban Bubenek-Turconi ◽  
...  

Myxomas comprise 50% of all benign cardiac tumors in adults, with the right atrium as their second most frequent site of origin. Surgical resection is the only effective therapeutic option for patients with these tumors. The association between right atrial myxomas and severe left ventricular systolic dysfunction is extremely rare and makes treatment even more challenging. This was the case for our patient, a 47-year-old male with a right atrial mass and a severely impaired left ventricular function, with a 20% ejection fraction. Global enlargement of the heart was also noted, with moderate right ventricular dysfunction. The tumor was successfully excised using the on-pump beating heart technique, with an immediate postoperative improvement of the left ventricular ejection fraction to 35%. The technique proved useful, with no increased risk to the patient.


2021 ◽  
Vol 10 (11) ◽  
pp. 2440
Author(s):  
Anja Linde ◽  
Eva Gerdts ◽  
Kåre Steinar Tveit ◽  
Ester Kringeland ◽  
Helga Midtbø

We explored the association between subclinical cardiac organ damage (OD) with comorbidities and psoriasis severity in 53 psoriasis patients on infliximab treatment (age 47 ± 15 years, 30% women) and 99 controls without psoriasis (age 47 ± 11 years, 28% women). Cardiac OD was assessed by echocardiography as the presence of increased left ventricular (LV) relative wall thickness (RWT), LV hypertrophy or dilated left atrium. Psoriasis severity was graded using the psoriasis area and severity index (PASI). The prevalence of hypertension was 66% in psoriasis vs. 61% in controls (p = 0.54) and cardiac OD seen in 51 and 73%, respectively (p = 0.007). Psoriasis was associated with a lower prevalence of cardiac OD (odds ratio (OR) 0.32, 95% confidence interval (CI) 0.13–0.77, p = 0.01) independent of age, sex, smoking, body mass index, and hypertension. Among psoriasis patients, hypertension was associated with increased risk of subclinical cardiac OD (OR 6.88, 95% CI 1.32–35.98, p = 0.02) independent of age, sex, and body mass index. PASI at treatment initiation was associated with a higher RWT at follow-up, independent of sex, age, and hypertension (β 0.36, p = 0.006) while no association with current PASI was found. In conclusion, cardiac OD was less prevalent in psoriasis patients on infliximab treatment than controls. Hypertension was the major covariable for subclinical cardiac OD in psoriasis.


2021 ◽  
pp. 1-7
Author(s):  
Carlos Castillo-Pinto ◽  
Jessica L. Carpenter ◽  
Mary T. Donofrio ◽  
Anqing Zhang ◽  
Gil Wernovsky ◽  
...  

Abstract Objective: Children with CHD may be at increased risk for epilepsy. While the incidence of perioperative seizures after surgical repair of CHD has been well-described, the incidence of epilepsy is less well-defined. We aim to determine the incidence and predictors of epilepsy in patients with CHD. Methods: Retrospective cohort study of patients with CHD who underwent cardiopulmonary bypass at <2 years of age between January, 2012 and December, 2013 and had at least 2 years of follow-up. Clinical variables were extracted from a cardiac surgery database and hospital records. Seizures were defined as acute if they occurred within 7 days after an inciting event. Epilepsy was defined based on the International League Against Epilepsy criteria. Results: Two-hundred and twenty-one patients were identified, 157 of whom were included in our analysis. Five patients (3.2%) developed epilepsy. Acute seizures occurred in 12 (7.7%) patients, only one of whom developed epilepsy. Predictors of epilepsy included an earlier gestational age, a lower birth weight, a greater number of cardiac surgeries, a need for extracorporeal membrane oxygenation or a left ventricular assist device, arterial ischaemic stroke, and a longer hospital length of stay. Conclusions: Epilepsy in children with CHD is rare. The mechanism of epileptogenesis in these patients may be the result of a complex interaction of patient-specific factors, some of which may be present even before surgery. Larger long-term follow-up studies are needed to identify risk factors associated with epilepsy in these patients.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Ethan J Rowin ◽  
Barry J Maron ◽  
Tammy S Haas ◽  
John R Lesser ◽  
Mark S Link ◽  
...  

Background: Increasing penetration of high spatial resolution cardiovascular magnetic resonance (CMR) imaging into routine cardiovascular practice has resulted in more frequent identification of a subset of hypertrophic cardiomyopathy (HCM) patients with thin-walled, scarred left ventricular (LV) apical aneurysms. Prior experience involved relatively small numbers of patients with short follow-up and therefore the risk associated with this subgroup remains incompletely defined. Therefore, we assembled a large HCM cohort with LV apical aneurysms and long-term follow-up in order to clarify clinical course and prognosis. Methods and Results: Of 2,400 HCM patients, 60 (2.5%) were identified by CMR with LV apical aneurysm, 24 to 86 years of age, including 19 (32%) <45 years old; 70% male, and followed for 5.6 ± 3.5 years. Over the follow-up period, 24 patients experienced 31 adverse disease-related complications including: appropriate implantable cardioverter-defibrillator discharge for VT/VF (n=11), received or listed for heart transplant (n=6), heart failure death (n=5), nonfatal thromboembolic events (n=4), resuscitated out-of-hospital cardiac arrest (n=3), and sudden death (n=2). In addition, an intracavitary thrombus was identified in the apical aneurysm in 9 patients without a thromboembolic history. Combined HCM-related death and aborted life threatening event rate was 8.6% per year, nearly 6-fold greater than the 1.5% annual mortality rate reported in the general HCM population. Conclusions: Patients with LV apical aneurysms represent a high-risk subgroup within the diverse HCM spectrum, associated with substantial increased risk for disease-related morbidity and mortality, including advanced heart failure, thromboembolic stroke and sudden death. Identification of this unique HCM phenotype should prompt consideration for primary prevention ICD, and anticoagulation for stroke prophylaxis.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Zi Ye ◽  
Maurice Enriquez-Sarano ◽  
Joseph Malouf ◽  
Hector I Michelena ◽  
Allan S Jaffe ◽  
...  

Introduction: Left ventricular longitudinal strain (LV-LS) 1) predicts mortality in patients with aortic stenosis (AS) and 2) is highly correlated to type-B natriuretic peptide (BNP) values. The BNP ratio (measured BNP/maximal expected BNP value specific for age and sex) is a powerful independent predictor of death in patients with AS. Hypothesis: we hypothesize that BNP activation (i.e. BNP ratio >1) affects the association between LV-LS and mortality in patients with asymptomatic AS and preserved LV ejection fraction (EF ≥50%). Methods: 315 patients (age 74±12 years, 56% men and mean aortic valve area = 1.02±0.15cm2) underwent simultaneous Doppler echocardiographic and BNP measurements. LV-LS was calculated as the average of 12 LV segments from apical 2- and 4-chamber views using Velocity Vector Imaging. Results: Mean LV-LS was -16.8±3.2%, LV EF 66±7%, median BNP level 121 (interquartile 48-320) pg/ml. 58% of patients had BNP activation. Better LV-LS was associated with lower log BNPratio (regression coefficient 0.10, p<0.001). After a median follow-up of 6.5 yrs (interquartile: 3.6-8.2), 119 deaths occurred. After adjustment for age, sex, Charlson score index, hemoglobin level, aortic valve replacement (as a time dependent variable), LV-LS and log BNPratio were separately associated with increased risk for death (all p<0.01). Further adjustment for predictors of mortality, LV-LS and log-BNP ratio remained associated with increased risk for death (hazard ratio HR [95%CI]: 1.09 [1.03-1.15]; p=0.003 and 1.82 [1.52-2.19]; p<0.0001 respectively). In patients without BNP activation (i.e. normal BNP), LV-LS was associated with mortality (HR: 1.22 [1.04-1.43]; p=0.01) while it was not in patients with BNP activation (p=0.22). Conclusions: In patients with asymptomatic AS, without clinically obvious myocardial impairment (i.e. normal LVEF), a notable proportion of patients present with myocardial alterations detected by an elevated BNPratio or reduced LV-LS. These signs of myocardial alterations were predictive of mortality after diagnosis. Thus both BNP and LV-LS should be assessed in the clinical setting to provide complementary information on prognosis in patients with asymptomatic AS and preserved LV EF.


Cardiology ◽  
2019 ◽  
Vol 142 (1) ◽  
pp. 7-13
Author(s):  
Gabriele Di Gesaro ◽  
Giuseppa Caccamo ◽  
Diego Bellavia ◽  
Calogero Falletta ◽  
Chiara Minà ◽  
...  

Heart failure (HF) with reduced ejection fraction (HFrEF) has a well-known epidemic relevance in western countries. It affects up to 1–2% of patients > 60 years and reaches a prevalence of 12% in octogenarian patients. The role of N-terminal pro-B-type natriuretic peptide (NT-proBNP) and high-sensitive troponin I (hsTnI) in risk stratifying HFrEF patients has been established; at present, evidence is exclusively based on one-time assessments, and the prognostic usefulness of serial biochemical assessments in this population still remains to be determined. We prospectively recruited 226 patients with chronic HFrEF, who were all referred to the Outpatient Clinic of our institution from November 2011 through September 2014. Recruited patients underwent full clinical evaluation with complete history taking and physical examination as well as ECG, biochemical assessment, and standard 2D and Doppler flow echocardiography at the first visit, and then again at each visit during the follow-up, repeated every 6 months. During the follow-up period, cardiovascular (CV) death, which occurred in 16 patients, was not statistically correlated with gender (p = 0.088) or age (p = 0.1636); however, baseline serum levels of NT-proBNP, which were 3 times higher in deceased patients, were significantly related to this clinical event (p = 0.001). We found that NT-proBNP represents a strong and independent predictor of CV outcome; serum levels of hsTnI, which are significantly related to an increased risk of hospitalization, cannot properly predict the relative risk of CV mortality. Our study validates, eventually, the multimarker strategy, which reflects the complexity of the HF pathophysiology.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
G Casas ◽  
G Oristrell ◽  
J Limeres ◽  
R Barriales ◽  
J R Gimeno ◽  
...  

Abstract BACKGROUND Left ventricular noncompaction (LVNC) is associated with an increased risk of systemic embolisms (SE). However, incidence and risk factors are not well established. PURPOSE To evaluate the rate of SE in LVNC and describe risk factors. METHODS LNVC patients were included in a multicentric registry. Those with SE were considered for the analysis. RESULTS 514 patients with LVNC from 10 Spanish centres were recruited from 2000 to 2018. During a median follow-up of 4.2 years (IQR 1.9-7.1), 23 patients (4.5%) had a SE. Patients with SE (Table 1) were older at diagnosis, with no differences in gender and had similar cardiovascular risk factors. They were more frequently under oral anticoagulation (OAC). Besides, they had a more reduced LVEF, and more dilated LV and left atrium (LA). Late gadolinium enhancement (LGE) was more frequent, altogether suggesting a more severe phenotype. Patients with SE had non-significantly higher rates of hospitalization for heart failure (33% Vs 24%, p = 0.31) and atrial fibrillation (35% Vs 19%, p = 0.10). In multivariate analysis, only LA diameter was an independent predictor of SE (OR 1.04, p = 0.04). A LA diameter &gt; 45 mm had an independent 3 fold increased risk of SE (OR 3.04, p = 0.02) (Image 1). CONCLUSIONS LVNC carries a moderate mid-term risk of SE, which appears to be irrespective of atrial fibrillation and associated with age, LV dilatation and systolic dysfunction and mainly LA dilatation. This subgroup of patients should be considered for oral anticoagulation in primary prevention. Table 1 Systemic embolisms (n = 23) No systemic embolisms (n = 491) p Men, n (%) 15 (65) 289 (56) 0.52 Median age at diagnosis (IQR) - yr 60 (48-76) 48 (30-64) 0.02 Median follow up (IQR) - yr 5.9 (3.1-7.8) 4.2 (1.8-7.1) 0.18 OAC, n (%) 19 (83) 118 (24) 0.01 LVEF (SD) - % 37 (15) 48 (17) 0.01 LVEDD (SD) - mm 58 (11) 54 (10) 0.04 LA diameter (SD) - mm 46 (9) 39 (9) 0.01 Characteristics of patients with and without systemic embolisms Abstract P1441 Figure. Image 1


2013 ◽  
Vol 31 (26_suppl) ◽  
pp. 167-167
Author(s):  
Neil M. Iyengar ◽  
Patrick Glyn Morris ◽  
Sujata Patil ◽  
Carol Chen ◽  
Alyson Abbruzzi ◽  
...  

167 Background: The addition of H to chemotherapy has improved outcomes in HER2-positive early BC. This approach is associated with (w/) an increased risk (<4%) of congestive heart failure (CHF). Dose-dense (every 2 weeks) anthracycline-taxane therapy (Rx) improves survival compared to the every 3 week schedule and can be combined w/ anti-HER2 Rx w/ no increased risk of cardiotoxicity up to 36 months. Here we report the incidence of NYHA Class III/IV CHF in 2 phase II studies with longer follow-up. Methods: We conducted a retrospective review of pts w/ HER2 + early stage BC treated at MSKCC and DFCI on two trials: In trial A - pts received dd AC (60/600 mg/m2) x 4 → T (175mg/m2) x 4 (w/ pegfilgrastim) w/ H x 1 year. Trial B differed w/ use of weekly T (80mg/m2) x 12 and the addition of L (1000mg orally daily) x 1 year. Left ventricular ejection fraction (LVEF) was prospectively assessed by a multi-gated acquisition scan serially throughout Rx. Results: Trial A enrolled 70 pts and Trial B enrolled 95 pts w/ the median age of 46 years (range 27-73 years). Overall, the 5-year distant disease-free survival (DDFS) for trials A and B is 92% (95%Cl; 83-97%) and 89% (95%CI; 81-94%), respectively. The baseline median LVEF was 68% (range 52-81%). In total, 28 of 165 (17%) pts had pre-existing hypertension. Now at a median follow-up of 84 and 57 months respectively, only one (1.4%, 95%CI; 1.36-7.7%) and 4 (4.2%, 95%CI; 4.2-10.4%) pts developed CHF. Since our earlier report, 1 additional CHF event occurred (Trial B) at month 44. Conclusions: Longer follow-up of these 2 studies demonstrate that dd AC → TH with or without L is associated w/ a low risk of CHF. This is consistent w/ the long-term cardiac toxicity reported from the randomized phase III studies of H w/ conventionally scheduled anthracycline-based regimens (with or without taxanes). DDFS outcomes are also encouraging. Clinical trial information: NCT00591851 and NCT00482391.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Nader Makki ◽  
Olurotimi Mesubi ◽  
Curtis Steyers ◽  
Brian Olshansky

Background: Ventricular arrhythmias (VAs) are among the most commonly reported adverse effects associated with left ventricular assist devices (LVADs). However, prevalence of VAs post-LVAD implantation, and their relation to all-cause mortality, remains to be elucidated. We conducted a meta-analysis and systematic review of observational studies with the primary objective of evaluating the risk of VAs after LVAD implantation and the risk of all-cause mortality in patients with LVADs who had VAs. Methods: We searched Medline, Embase and Cochrane Central from 2001 to 2014. Two reviewers independently searched,selected and assessed quality of included studies with differences resolved by consensus. Data were collected and analyzed using random and fixed-effect model, as appropriate, with inverse variance weighting. Results: Of 2,393 studies identified, 15 observational studies were eligible including 1,517 patients with a mean follow up of 201 days. An LVAD was associated with an increased risk of VA after implantation (OR = 2.21, 95% confidence interval [CI] 1.37-3.59, p<0.001).There was an increased risk of all-cause mortality in LVAD patients who had post-LVAD VA (OR = 1.91, 95% CI 1.18-3.11, p<0.001). Using meta-regression and sensitivity analyses to account for risk factors such as etiology of cardiomyopathy, duration of follow-up, destination LVAD versus bridge therapy and presence of an implantable cardioverter defibrillator at time of LVAD implantation did not change the results of our main analysis. Conclusions: LVADs are associated with an increased risk of VA and presence of VAs post LVAD implantation is associated with increased risk of all-cause mortality.


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