scholarly journals Development of heart failure in young patients with congenital heart disease: a nation-wide cohort study

Open Heart ◽  
2019 ◽  
Vol 6 (1) ◽  
pp. e000858 ◽  
Author(s):  
Thomas Gilljam ◽  
Zacharias Mandalenakis ◽  
Mikael Dellborg ◽  
Georgios Lappas ◽  
Peter Eriksson ◽  
...  
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Young Patients ◽  
Cumulative Probability ◽  
Congenital Heart Malformations ◽  
National Patient ◽  
Probability Of Death

ObjectiveHeart failure (HF) is a common cause of hospitalisation and death in adults with congenital heart disease (CHD). However, the risk of HF in young patients with CHD has not been determined.MethodsBy linkage of national patient registers in Sweden, we identified 21 982 patients with CHD born between 1970 and 1993, and compared these with 10 controls per case. Follow-up data were collected from birth until 2011 or death.ResultsOver a mean follow-up of 26.6 years in patients with CHD and 28.5 years in controls, 729 (3.3%) and 75 (0.03%) developed HF, respectively. The cumulative incidence of HF in all CHD was 6.5% and in complex CHD 14.8% up to age 42 years. Thus, one patient in 15 with CHD runs the risk of developing HF before age 42 years, a risk that is 105.7 times higher (95 % CI 83.2 to 134.8) compared with controls. For patients with complex CHD (such as conotruncal defects, univentricular hearts, endocardial cushion defects), one in seven will develop HF, a HR of 401.5; 95% CI 298 to 601 as compared with controls. The cumulative probability of death in patients with CHD, after HF diagnosis, was 63.4% (95% CI 57.5 to 69.3).ConclusionsAn extremely high risk of developing HF (more than 100-fold) was found in patients with CHD, compared with matched controls, up to the age of 42 years. Patients with complex congenital heart malformations carried the highest risk and have to be considered as the main risk group for developing HF.

Abstract 19314: Impact of Body Mass Index on Clinical Outcomes in Complex Adult Congenital Heart Disease

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Norihisa Toh ◽  
Ines Uribe Morales ◽  
Zakariya Albinmousa ◽  
Tariq Saifullah ◽  
Rachael Hatton ◽  
...  
Keyword(s):  
Heart Failure ◽  
Body Mass Index ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Adult Congenital Heart Disease ◽  
Cardiac Events ◽  
Increased Risk ◽  
Adult Congenital

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.

P1804Cancer in young patients with congenital heart disease: A registry-based, matched, prospective cohort study

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
Z Mandalenakis ◽  
C K Karazisi ◽  
K S Skoglund ◽  
A R Rosengren ◽  
G L Lappas ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Young Patients ◽  
Systematic Screening ◽  
Incidence Of Cancer ◽  
Increased Risk ◽  
Using Data ◽  
Risk Of Cancer

Abstract Background Adult patients with congenital heart disease (CHD) have an increased incidence of cancer presumably due to repeated radiation exposure, genetic predisposition or a continued stress factor during heart interventions. Purpose We aimed to investigate the risk to be diagnosed with cancer from birth up to the age of 42 years in patients with CHD and compared to matched controls. Methods Using data from the Patient and Cause of Death Registers in Sweden, we identified successive cohorts of patients with CHD born 1970–79, 1980–89 and 1990–93. Each CHD patient was matched for birth year, sex and county with 10 controls without CHD from the general population with follow-up data and comorbidities collected from birth until 2011. Results We identified 21,982 patients with CHD and 219,816 matched controls of whom 428 (1.95%) and 2,072 (0.94%), respectively, were diagnosed with cancer over a mean follow-up of 26.2 and 27.5 years. The overall hazard ratio (HR) for cancer was 2.24 times (95% confidence interval [CI], 2.01–2.48) in children and young adults with CHD compared to controls; higher in men (HR 2.41 (95% CI, 2.08–2.79)) and among patients with isolated atrial or ventricular defects, as well as in the large group of miscellaneous anomalies. The cumulative incidence of cancer in patients with CHD increased by birth cohort with almost 2% among those born 1990–93 been diagnosed with cancer on reaching adulthood. Conclusions Young patients with CHD had more than 2 timmes increased risk to develop cancer compared to matched controls. At the age of 42, 1 out of 50 patients with CHD developed cancer and the risk was significantly higher in men with CHD. The patient groups with atrial and ventricular septal defects had a significantly increased risk of cancer and a systematic screening for cancer could be considered to this vulnerable group of patients. Acknowledgement/Funding By grants from the Swedish state under the agreement concerning research and education of doctors, the Swedish Research Council.

scholarly journals Growth differentiation factor 15 as a useful biomarker of heart failure in young patients with unrepaired congenital heart disease of left to right shunt

2020 ◽  
Vol 75 (6) ◽  
pp. 697-701
Author(s):  
Yoshiyuki Kagiyama ◽  
Shuichi Yatsuga ◽  
Masahiro Kinoshita ◽  
Yusuke Koteda ◽  
Shintaro Kishimoto ◽  
...  
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Young Patients ◽  
Growth Differentiation Factor 15 ◽  
Differentiation Factor

scholarly journals Burden of Ischemic Stroke in Patients With Congenital Heart Disease: A Nationwide, Case‐Control Study

2021 ◽  
Author(s):  
Kok Wai Giang ◽  
Maria Fedchenko ◽  
Mikael Dellborg ◽  
Peter Eriksson ◽  
Zacharias Mandalenakis
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Ischemic Stroke ◽  
Congenital Heart ◽  
Increased Risk ◽  
All Cause Mortality ◽  
National Patient ◽  
Study Population

Background Patients with congenital heart disease (CHD) are at increased risk of developing ischemic stroke (IS) compared with controls without CHD. However, the long‐term outcomes after IS, including IS recurrence and mortality risk, remain unclear. Methods and Results We identified all patients with CHD in Sweden who were born between 1930 and 2017 using the Swedish National Patient Register and the Cause of Death Register. Ten controls without CHD were randomly selected from the general population and matched for birth year and sex for each patient with CHD. The follow‐up of the study population was performed between January 1970 and December 2017. In total, 88 700 patients with CHD (50.6% men) and 890 450 matched controls (51.0%) were included in this study. During a mean follow‐up of 25.1±22.0 years, patients with CHD had a 5‐fold higher risk of developing an index IS (hazard ratio [HR], 5.01; 95% CI, 4.81–5.22) compared with controls. However, the risk of developing a recurrent IS was lower in patients with CHD compared with controls (HR, 0.66; 95% CI, 0.56–0.78), an observation that persisted after adjustment for cardiovascular risk factors and comorbidities. Patients with CHD were also at a significantly lower risk of all‐cause mortality after index IS than controls (HR, 0.53; 95% CI, 0.49–0.58). Conclusions Patients with CHD had a 5‐fold higher risk of developing index IS compared with matched controls. However, the risk of recurrent IS stroke and all‐cause mortality were 34% and 47% lower, respectively, in patients with CHD compared with controls.

Risk of ischemic stroke in adult patients with congenital heart disease

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
K.W Giang ◽  
M Fedchenko ◽  
M Dellborg ◽  
P Eriksson ◽  
A Rosengren ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Heart Failure ◽  
Atrial Fibrillation ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Ischemic Stroke ◽  
General Population ◽  
Congenital Heart ◽  
Funding Source

Abstract Introduction With an increasing proportion of adults with congenital heart disease (CHD) surviving into middle age and beyond, CHD patients will be at increased risk of acquired cardiovascular conditions, such as ischemic stroke. Compared to controls, patients with CHD have a higher prevalence of arrhythmias, persistent shunts enabling paradoxical embolization, heart failure, mechanical valves as well as potentially hypercoagulable states, all of which can further increase the risk of stroke. Purpose The aim of our study was to investigate the risk of developing ischemic stroke in adults with CHD in Sweden compared to controls from the general population. Methods We used data from the Swedish National Patient and Cause of Death registries to identify all CHD patients ≥18 years of age, born during the period 1930–1998, with a first time diagnosis of ischemic stroke. Follow-up started in January 1970 and went on until December 2017. Approximately ten controls matched for age and sex were randomly selected from the general population for each patient with CHD. CHD diagnoses were classified into six lesion groups according to a previously published hierarchical classification system. Results In total, 43,110 patients with CHD and 474,267 controls were included in the study (51.4% men) and mean follow up time was 25.4±18.4 years. Patients with CHD had a 6 times higher risk of developing an ischemic stroke compared with controls (hazard ratio 6.0, 95% confidence interval 5.8–6.2, p≤0.001), with altogether 8.8% (n=3785) of CHD patients developing ischemic stroke compared with 1.6% (n=7516) of controls. Ischemic stroke was more common in all CHD lesion groups; however, patients with atrial septal defects/patent foramen ovale had the highest incidence rate of ischemic stroke with an incidence rate of 76.1 events/10,000 patient years compared with 8.7 in controls. Patients with CHD and ischemic stroke had markedly less hypertension, diabetes mellitus and hypercholesterolemia, compared with controls (7.1%, 2.0%, 2.9% respectively in CHD patients, compared with 19.6%, 6.6%, 5.3%, in controls, p≤0.001 for all). In addition, atrial fibrillation and heart failure were only slightly more common in CHD patients with ischemic stroke compared to controls (atrial fibrillation: 12.0% in CHD vs 10.4% in controls, p=0.01; heart failure: 8.7% in CHD vs 7.3% in controls, p=0.009). Conclusion In this large nationwide study, we found that the risk of ischemic stroke in adult patients with CHD was six times higher than in controls, despite a lower prevalence of common risk factors for stroke such as hypertension, diabetes mellitus and hypercholesterolemia. In addition, atrial fibrillation and heart failure were only slightly more common in CHD patients compared with controls. This implies that the etiology of ischemic stroke might be different in CHD patients compared with controls. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): This work was funded by the Swedish state under an agreement between the Swedish government and county councils, the ALF agreement (Grant number: 236611) and the Swedish Heart-Lung Foundation (Grant Number: 20090724).

Abstract 15875: Waitlist Outcomes for Adults With Congenital Heart Disease Listed for Heart Transplantation in the United States

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Laith Alshawabkeh ◽  
Nan Hu ◽  
Knute D Carter ◽  
KellyAnn Light-McGroary ◽  
Joseph E Cavanaugh ◽  
...  
Keyword(s):  
United States ◽  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Heart Transplantation ◽  
Congenital Heart ◽  
Adult Survivors ◽  
The United States ◽  
Probability Of Death ◽  
Clinical Worsening

Background: Heart failure represents a final common pathway for many adult survivors with congenital heart disease (ACHD). Data assessing severity and management of heart failure are limited, and often heart transplantation is the only viable treatment option. The criteria used to determine priority status at the time of transplant listing, however, does not account for ACHD physiology. We investigated the differences in death or delisting due to clinical worsening in ACHD vs. non-ACHD candidates for heart transplantation. Methods: We conducted a retrospective study of all patients listed for heart transplantation in the United States between 1999 and 2014 using the Scientific Registry of Transplant Recipients. Patients were censored at the time of transplant or delisting due to improvement. Results: Among the 1,290 ACHD and 35,559 non-ACHD patients listed for heart transplantation, median age was 35 vs. 56 years and 62% vs. 76% were male, respectively. Waitlist outcomes for the full follow up time per initial listing status are shown in Figure 1. Of the patients who died or worsened, 57% were initially listed at the lowest priority status for ACHD compared to 40% for non-ACHD. In patients initially listed at status 1A, the 180-day actuarial probability of death or delisting due to worsening was 41% for ACHD vs. 29% for non-ACHD, p < 0.01; and for death alone was 29% vs. 21%, p = 0.05; respectively, Figure 2. Conclusion: ACHD candidates for heart transplantation in the United States are more frequently listed at the lowest priority status, and when listed at the highest priority status are more likely to die or be delisted due to clinical worsening compared to other candidates.

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