Abstract 20041: Single Ventricle Outcome Following Neonatal Palliation of Severe Ebstein’s Anomaly With Modified Starnes Procedure

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
S. Ram Kumar ◽  
Nathan Noh ◽  
Novell Castillo ◽  
Brian Fagan ◽  
Grace Kung ◽  
...  

Background: We have previously shown that neonates in profound cardiogenic shock due to severe Ebstein’s anomaly can be successfully salvaged with fenestrated right ventricular (RV) exclusion and systemic to pulmonary shunt (modified Starnes procedure). The long-term outcome of single ventricle management in these patients is not known. Methods: We retrospectively reviewed the records of 26 patients who underwent neonatal Starnes procedure between 1989 and 2011. Patient demographics, clinical variables and outcome data were collected. Data is presented as mean ± standard errors or median (interquartile ranges). Results: 26 patients (12, 46% boys) underwent Starnes procedure at 7 (5-9) days of life. All were intubated and on prostacyclin infusion, 24 (92%) were inotrope-dependent and 23 (88%) had no antegrade flow from the RV. Two patients had had prior intervention (one tricuspid annuloplasty and one shunt alone). Three patients underwent non-fenestrated RV exclusion, two (67%) of whom died. Of the remaining 23, 3 (13%) died during the same hospitalization. The 21 neonatal survivors have been followed for 7 (6-8) years. One patient died after Glenn. The remaining 20 have successfully undergone Fontan completion with an indexed pulmonary resistance of 1.8 (1.2-2.3) W/m2 and mean pulmonary pressure of 12 (9-18) mm Hg. At last follow-up, all patients have normal left ventricular function, and all but one patient are in NYHA Class I symptoms. Two patients have required pacemaker implantation, while the rest are in sinus rhythm. Survival at 1, 5 and 10 years are 81±4%, 77±3% and 77±3%, respectively. Conclusion: Long-term single ventricle outcomes amongst neonatal survivors of modified Starnes procedure are excellent. There is reliable remodeling of the excluded RV and excellent function of the left ventricle.

2012 ◽  
Vol 23 (1) ◽  
pp. 27-34 ◽  
Author(s):  
Angela Oxenius ◽  
Christine H. Attenhofer Jost ◽  
René Prêtre ◽  
Hitendu Dave ◽  
Urs Bauersfeld ◽  
...  

AbstractObjectivesTo assess clinical presentation, treatment, and outcome of children with Ebstein's anomaly.BackgroundData on long-term outcome of children with Ebstein's anomaly are scarce.MethodsRetrospective analysis of all children with Ebstein's anomaly treated between February, 1979 and January, 2009 in a single tertiary institution. Primary outcomes included patient survival and need for intervention, either cardiac surgery or catheter intervention.ResultsA total of 42 patients were diagnosed with Ebstein's anomaly at a median age of 5 days ranging from 1 day to 11.7 years. Symptoms included cyanosis, heart murmur, and/or dyspnoea. Associated cardiac anomalies occurred in 90% of the patients. Average follow-up was 9.5 plus or minus 7.0 years. The overall mortality rate was 14%. Of the six patients, three died postnatally before treatment. Cardiac surgery and/or catheter-guided interventions were required in 33 patients (79%). Cardiac surgery was performed in 21 (50%) patients at a median age of 9.1 years (range 0.1–16.5 years), including biventricular repair in 13 (62%), one-and-a-half chamber repair in seven (33%), and a staged single-ventricle repair in one. Peri-operative mortality was 4%. Catheter-guided interventions consisted of device closure of an atrial septal defect in three cases and radiofrequency ablation of accessory pathways in nine patients. The estimated 10-year survival was 85.3 plus or minus 5.6%.ConclusionIn children, Ebstein's anomaly is usually diagnosed in the first year of age. Even though children with Ebstein's anomaly often require an intervention, their peri-operative mortality is low and long-term survival is good. Symptomatic newborns requiring an intervention may have a worse outcome.


Circulation ◽  
1992 ◽  
Vol 86 (4) ◽  
pp. 1147-1155 ◽  
Author(s):  
J C Pressley ◽  
J M Wharton ◽  
A S Tang ◽  
J E Lowe ◽  
J J Gallagher ◽  
...  

2020 ◽  
Vol 51 (4) ◽  
pp. 336-342
Author(s):  
Héctor Herrera-Bello ◽  
Nydia Ávila-Vanzzini ◽  
Juan Francisco Fritche-Salazar ◽  
Jorge Kuri-Alfaro ◽  
Manuel B.A. Gaxiola-Macias ◽  
...  

2008 ◽  
Vol 168 (6) ◽  
pp. 685-690 ◽  
Author(s):  
Ya-Mei Chang ◽  
Jou-Kou Wang ◽  
Sheunn-Nan Chiu ◽  
Ming-Tai Lin ◽  
En-Ting Wu ◽  
...  

2011 ◽  
Vol 139 (9-10) ◽  
pp. 591-598 ◽  
Author(s):  
Nebojsa Mujovic ◽  
Miodrag Grujic ◽  
Stevan Mrdja ◽  
Aleksandar Kocijancic ◽  
Goran Milasinovic ◽  
...  

Introduction. Atrioventricular (AV) junction ablation coupled with pacemaker implantation is an effective therapeutic option for rate control in atrial fibrillation (AF) and heart failure (HF). However, there is controversy regarding the long-term outcome of the procedure, since right ventricular stimulation can lead to left ventricular remodelling and HF. Objective. The aim of the study was to determine a 5-year outcome of the procedure on survival, HF control and myocardial function in patients with HF and uncontrolled AF. Methods. All patients with AF and HF who underwent AV-junction ablation with pacemaker implantation in our institution were followed after the procedure. HF diagnosis was established if ?2 of the following criteria were present: 1) ejection fraction (EF) ?45%; 2) previous episode of congestive HF (CHF); 3) NYHA-class ?2; and 4) use of drug-therapy for HF. Results. Study included 32 patients (25 males; 53.4?9.6 years). The mean heart rate was 121?25 bpm before and 75?10 bpm after ablation (p=0.001). Over the follow-up of 5.0?4.0 years nine patients (28.1%) died (five died suddenly, three of terminal CHF and one of stroke). After the procedure, CHF occurrence was reduced (p=0.001), as well as the annual number of hospitalizations (p=0.001) and the number of drugs for CHF (p=0.028). In addition, NYHA-class and EF were improved, from 3.3?0.7 to 1.6?0.8 (p<0.001) and from 39?11% to 51?10% (p<0.001), respectively. Conclusion. In HF patients with uncontrolled AF, 5-year mortality after AV-junction ablation and pacemaker implantation was 28%. In the majority of these patients good rate of AF and HF control were achieved, as well as the improvement of functional status and myocardial contractility.


2010 ◽  
Vol 37 (1) ◽  
pp. 186-192 ◽  
Author(s):  
Catalin Constantin Badiu ◽  
Christian Schreiber ◽  
Jürgen Hörer ◽  
Daniel Junichiro Ruzicka ◽  
Michael Wottke ◽  
...  

2021 ◽  
Vol 10 (Supplement_1) ◽  
Author(s):  
J Grand ◽  
K Miger ◽  
A Sajadieh ◽  
L Kober ◽  
C Torp-Pedersen ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): The Danish Heart Foundation Background In acute heart failure (AHF), low systolic blood pressure (SBP) has been associated with poor outcome. Less is known of the risk related to normal versus elevated SBP and interaction with left ventricular ejection fraction. Purpose The aim of the present study was to assess the association between baseline SBP and short- and long-term outcome in a large cohort of AHF-patients. Methods A pooled cohort of four randomized controlled trials investigating the vasodilator serelaxin versus placebo in patients admitted with AHF and an SBP from 125 to 180 mmHg. Endpoints were 180-day all-cause mortality and a short-term composite endpoint (worsening heart failure, all-cause mortality or hospital readmission for HF through Day 14). Left ventricular ejection fraction (LVEF) was categorized into HFrEF (&lt;40%) and HFpEF (= &gt;40%). Multivariable Cox regression was used and adjusted for age, sex, baseline body mass index, HFrEF, serum estimated glomerular filtration rate, allocated treatment (placebo/serelaxin), diabetes mellitus, ischemic heart disease, and atrial fibrillation/flutter. Measurements and Main Results A total of 10.533 patients with a mean age of 73 (±12) years and median SBP of 140 (130-150) mmHg were included within mean 8.2 hours from admission. LVEF was assessed in 8493 (81%), and of these, 4294 (51%) had HFrEF. Increasing SBP as a continuous variable was inversely associated with 180-day mortality (HRadjusted: 0.93 [0.88-0.98], p = 0.004 per 10 mmHg increase) and with the composite endpoint (HRadjusted: 0.90 [0.85-0.95], p &lt; 0.0001 per 10 mmHg increase). A significant interaction was observed regarding LVEF, revealing that SBP was not associated with mortality in patients with HFpEF  (HRadjusted: 1.01 [0.94-1.09], p = 0.83 per 10 mmHg increase), but SBP was associated with increased mortality in HFrEF (HRadjusted: 0.80 [0.73-0.88], p &lt; 0.001 per 10 mmHg increase) (Figure). Conclusions Elevated SBP is independently associated with favorable short- and long-term outcome in AHF-patients. The association between SBP and mortality was, however, not present in patients with preserved LVEF. Abstract Figure. Survival plots by SBP and LVEF


2020 ◽  
Vol 128 (10) ◽  
pp. 687-692 ◽  
Author(s):  
Joachim Feldkamp ◽  
F. Grünwald ◽  
Markus Luster ◽  
Kerstin Lorenz ◽  
Christian Vorländer ◽  
...  

AbstractThyroid nodules and cysts are frequently diagnosed in Germany with a prevalence of about 20% in young adults reaching up to 70% in older adults. Surgery is the standard treatment of symptomatic nodules, nodules with suspicion of malignancy and thyroid cancer. Radioiodine treatment is applied for autonomously functioning nodules. During the last years new non-surgical and non-radioiodine techniques have been introduced to treat thyroid nodules. These techniques include ethanol/polidocanol treatment, radiofrequency, microwave, and laser ablation, and high frequency ultrasound ablation. A significant reduction in nodule size could be documented for these techniques in several studies, but long-term outcome data are missing. Until now, there is no general consensus regarding the appropriate indications for these methods. For this reason, the Thyroid Section (German Society for Endocrinology), the Thyroid Working Committee (German Society for Nuclear Medicine), and the German Association of Endocrine Surgeons (CAEK) for the German Society of General and Visceral Surgery (DGAV) reviewed the respective literature, discussed the pro and cons and developed a consensus statement and recommendation to help physicians and patients in their decision making.


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