Adult congenital heart disease with special reference to the data on long-term follow-up of patients surviving to adulthood with or without surgical correction

1992 ◽  
Vol 13 (suppl H) ◽  
pp. 111-116 ◽  
Author(s):  
J. E. Deanfield
Heart ◽  
2014 ◽  
Vol 100 (Suppl 3) ◽  
pp. A3.2-A4 ◽  
Author(s):  
Sandhya Santharam ◽  
Maria Theodosiou ◽  
Sara Thorne ◽  
Paul Clift ◽  
Lucy Hudsmith ◽  
...  

2018 ◽  
Vol 4 (6) ◽  
pp. 771-780 ◽  
Author(s):  
Ivo Roca-Luque ◽  
Nuria Rivas-Gándara ◽  
Laura Dos Subirà ◽  
Jaume Francisco Pascual ◽  
Antònia Pijuan-Domenech ◽  
...  

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Norihisa Toh ◽  
Ines Uribe Morales ◽  
Zakariya Albinmousa ◽  
Tariq Saifullah ◽  
Rachael Hatton ◽  
...  

Background: Obesity can adversely affect most organ systems and increases the risk of comorbidities likely to be of consequence for patients with complex adult congenital heart disease (ACHD). Conversely, several studies have demonstrated that low body mass index (BMI) is a risk factor for heart failure and adverse outcomes after cardiac surgery. However, there are currently no data regarding the impact of BMI in ACHD. Methods: We examined the charts of 87 randomly selected, complex ACHD patients whose first visit to our institution was at 18-22 years old. Patients were categorized according to BMI at initial visit: underweight (BMI < 18.5 kg/m 2 ), normal (BMI 18.5 - 24.9 kg/m 2 ), overweight/obese (BMI ≥ 25 kg/m 2 ). Events occurring during follow-up were recorded. Data was censured on 1/1/2014. Cardiac events were defined as a composite of cardiac death, heart transplantation or admission for heart failure. Results: The cohort included patients with the following diagnoses: tetralogy of Fallot n=31, Mustard n=28, Fontan n=17, ccTGA n=9 and aortic coarctation n=2. The median (IQR) duration of follow-up was 8.7 (4.2 - 1.8) years. See table for distribution and outcomes by BMI category. Cardiac events occurred in 17/87 patients. After adjustment for age, sex, and underlying disease, the underweight group had increased risk of cardiac events (HR=12.9, 95% CI: 2.8-61.5, p < 0.05). Kaplan-Meier curves demonstrate the poorer prognosis of underweight patients (Figure). Conclusions: Underweight was associated with increased risk of late cardiac events in ACHD patients. We were unable to demonstrate significant overweight/obesity impact.


2019 ◽  
Vol 14 (4) ◽  
pp. 525-533 ◽  
Author(s):  
Madalena Coutinho Cruz ◽  
André Viveiros Monteiro ◽  
Guilherme Portugal ◽  
Sérgio Laranjo ◽  
Ana Lousinha ◽  
...  

2020 ◽  
Author(s):  
Ingrid Schusterova ◽  
Alzbeta Banovcinova ◽  
Marianna Vachalcova ◽  
Marta Jakubova ◽  
Panagiotis Artemiou

Abstract Background: Primary and secondary aortopathy are frequently encountered in patients with congenital heart disease. The aim of this study is to present our experience and the incidence of primary and secondary adult CHD-associated aortopathy.Methods. The cohort is comprised of adult patients with congenital heart disease from the registry of the Eastern Slovakia Institute of Cardiovascular Diseases. Data from the last follow-up examinations are included in this study. In the primary and secondary aortopathy group were 35 and 12 patients respectively. As a control group were selected 64 patients with non aortopathy associated congenital heart disease (atrial and ventricular septal defect).Results: Patients with primary and secondary aortopathy had larger ascending aorta/aortic root diameters than the control group (36.28 (26-49) mm vs 30.25 (21-41) mm p=0.000113, 33.82 27-49) mm vs 29.03 (19-38)mm p=0.000366 and 42.1 (30-50) mm vs 30.25 (21-41) mm, p=0.000106, 35.67 (27-48) mm vs 29.03 (19-38) mm, p=0.000119 respectively). Moreover, patients with secondary aortopathy had statistically significant larger ascending aorta diameter compared to the patients with primary aortopathy ( 42.1 (30-50) mm vs 36.28 ( 26-49) mm p=0.030). During the follow-up period, were performed only in 2 patients (one from each group) operations on the aortic root and the ascending aorta due to aortic root or ascending aorta dilatation.Conclusion: More patients with secondary aortopathy had dilated ascending aorta/ aortic root, as well as larger aortic diameters compare to the patients with primary aortopathy. Routine follow-up of these patients with attention to aortic diameter is necessary.


2020 ◽  
Vol 41 (43) ◽  
pp. 4168-4177 ◽  
Author(s):  
Eva Freisinger ◽  
Joachim Gerß ◽  
Lena Makowski ◽  
Ursula Marschall ◽  
Holger Reinecke ◽  
...  

Abstract Aims  To evaluate the use of novel oral anticoagulants (NOACs) compared with vitamin K antagonists (VKAs) in adult congenital heart disease (ACHD) and assess outcome in a nationwide analysis. Methods and results  Using data from one of Germany’s largest Health Insurers, all ACHD patients treated with VKAs or NOACs were identified and changes in prescription patterns were assessed. Furthermore, the association between anticoagulation regimen and complications including mortality was studied. Between 2005 and 2018, the use of oral anticoagulants in ACHD increased from 6.3% to 12.4%. Since NOACs became available their utilization increased constantly, accounting for 45% of prescribed anticoagulants in ACHD in 2018. Adult congenital heart disease patients on NOACs had higher thromboembolic (3.8% vs. 2.8%), MACE (7.8% vs. 6.0%), bleeding rates (11.7% vs. 9.0%), and all-cause mortality (4.0% vs. 2.8%; all P &lt; 0.05) after 1 year of therapy compared with VKAs. After comprehensive adjustment for patient characteristics, NOACs were still associated with increased risk of MACE (hazard rate—HR 1.22; 95% CI 1.09–1.36) and increased all-cause mortality (HR 1.43; 95% CI 1.24–1.65; both P &lt; 0.001), but also bleeding (HR 1.16; 95% CI 1.04–1.29; P = 0.007) during long-term follow-up. Conclusion  Despite the lack of prospective studies in ACHD, NOACs are increasingly replacing VKAs and now account for almost half of all oral anticoagulant prescriptions. Particularly, NOACs were associated with excess long-term risk of MACE, and mortality in this nationwide analysis, emphasizing the need for prospective studies before solid recommendations for their use in ACHD can be provided.


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