CONGENITAL HEART DISEASE PATIENTS WITH MULTIPLE STERNOTOMIES ARE AT INCREASED RISK OF A DECREASED FVC AND POOR EXERCISE CAPACITY

2019 ◽  
Vol 73 (9) ◽  
pp. 606
Author(s):  
Imran R. Masood ◽  
Jon Detterich ◽  
Daniel Cerrone ◽  
Roberta Kato ◽  
Arash Sabati
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Exercise Capacity ◽  
Congenital Heart ◽  
Increased Risk

scholarly journals Contemporary use of Selexipag in pulmonary arterial hypertension associated with congenital heart disease: a case series

2020 ◽  
Author(s):  
Sarah Blissett ◽  
David Blusztein ◽  
Vaikom S Mahadevan
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Side Effects ◽  
Arterial Hypertension ◽  
Exercise Capacity ◽  
Congenital Heart ◽  
Pulmonary Veins ◽  
Case Series ◽  
Pulmonary Arterial

Abstract Background There are significant risks of parenteral prostacyclin use in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), which may limit their use. Selexipag is an oral, selective prostacyclin analogue that has been shown to reduce disease progression and improve exercise capacity in patients with PAH-CHD. Administering Selexipag in patients with PAH-CHD could potentially overcome some of the risks of parenteral therapy while improving clinical outcomes. Case summary We report five cases highlighting the clinical uses of Selexipag in patients with PAH-CHD. In the first two cases, Selexipag was initiated as part of a Treat-to-close strategy. In the third case, initiation of Selexipag improved symptoms and objective exercise capacity in a patient with Eisenmenger syndrome. In the fourth and fifth cases, rapid cross-titration protocols were used to transition from parenteral prostacyclins to Selexipag. In the fourth case, Selexipag was initiated in the context of significant side effects limiting parenteral prostacyclin use. In the fifth case, Selexipag was used to down-titrate from parenteral prostacyclins following closure of a sinus venosus atrial septal defect and redirection of anomalous pulmonary veins. Discussion Selexipag is a promising oral therapy for patients with at various stages of the spectrum of PAH-CHD to improve symptoms, exercise capacity and, in some cases, haemodynamics. Our cases also highlight practical aspects of Selexipag use including targeting the individualized maximally tolerated dose for each patient, managing side effects and managing dose interruptions.

scholarly journals LVNC – A review

2021 ◽  
Vol 2 (4) ◽  
Author(s):  
Lisa Brandon ◽  
◽  
Brian Kerr ◽  
Ken McDonald ◽  
◽  
...  
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Gold Standard ◽  
Congenital Heart ◽  
Imaging Techniques ◽  
Disease Modifying Therapies ◽  
Increased Risk ◽  
Disease Modifying ◽  
No Gold Standard

LVNC is a relatively new clinical entity, with a significant increase in awareness and diagnosis in recent years. Currently the aetiology and pathogenesis of LVNC remains uncertain, alongside prevalence, however the diagnosis of LVNC appears to be increasing with improving imaging techniques. For educational purposes involving a rare clinical condition, we present the case of a 52 year old gentleman who was diagnosed with LV non compaction via ECHO and CMR. Interestingly it was noted two of his children had congenital heart disease, one daughter had Tetralogy of Fallot, and a second daughter had both an ASD and VSD. Challenges facing LVNC involve difficulty of diagnosis with no gold standard yet available, uncertainty of benefit with standard disease modifying therapies for HF-REF, and apparent increased risk of arrhythmias suggesting early ICD placement may be warranted for patients. Keywords: Hr-Ref; heart failure; lv non compaction; arrhythmias; lcd Risk.

scholarly journals Nutritional assessment and associated factors in children with congenital heart disease in Ethiopia.

2021 ◽  
Author(s):  
Temesgen Tsega Desta
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Associated Factors ◽  
Congenital Heart ◽  
Nutritional Assessment ◽  
Failure To Thrive ◽  
Cross Sectional ◽  
Care Givers ◽  
Increased Risk

ABSTRACT Infants and children with congenital heart disease exhibit a range of delays in weight gain and growth. In some instances, the delay can be relatively mild, whereas in other cases, cause the failure to thrive. OBJECTIVES To determine the nutritional status and associated factors of pediatric patients with congenital heart disease. MATERIAL AND METHODS A cross sectional analytical study was done over a period of 6months (Feb to Jul 2020). A total of 228 subjects with congenital heart disease that come to the cardiac center during the study period where included until the calculated sample size was attained. Data was collected from patient card and care givers of the children included in the study after obtaining their informed consent using data inquiry sheet. RESULTS A total of 228 children from age 3month to 17yrs. Most of the subjects had acyanotic heart disease accounting for 87.7%. The overall prevalence of wasting, underweight and stunting were 41.3%, 49.1% and 43% respectively. Among this children with congenital heart disease those with PAH were found more likely have wasting compared to those without PAH with an odds of 1.9 (95% CI: 1.0-3.4) and also greater chance of being stunted with an odds of 1.9 (95% CI: 1.0-3.4). children above 5years of age were 2.3 times more likely to be underweight. CONCLUSION Malnutrition is a major problem of patients with CHD. Pulmonary hypertension and older age are associated with increased risk of undernutrition. KEYWORDS: Acyanotic, cyanotic, Pulmonary hypertension, underweight, wasting and stunting.

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.

scholarly journals Mismatch between self-estimated and objectively assessed exercise capacity in patients with congenital heart disease varies in regard to complexity of cardiac defects

2020 ◽  
pp. 1-7
Author(s):  
Julius M. Woile ◽  
Stefan Dirks ◽  
Friederike Danne ◽  
Felix Berger ◽  
Stanislav Ovroutski
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Oxygen Uptake ◽  
Exercise Capacity ◽  
Congenital Heart ◽  
Cardiopulmonary Exercise Testing ◽  
Peak Oxygen Uptake ◽  
Subjective Rating ◽  
Cardiac Defects ◽  
Cardiopulmonary Exercise

Abstract Aim: Regular evaluation of physical capacity takes a crucial part in long-term follow-up in patients with congenital heart disease (CHD). This study aims to examine the accuracy of self-estimated exercise capacity compared to objective assessments by cardiopulmonary exercise testing in patients with CHD of various complexity. Methods: We conducted a single centre, cross-sectional study with retrospective analysis on 382 patients aged 8–68 years with various CHD who completed cardiopulmonary exercise tests. Peak oxygen uptake was measured. Additionally, questionnaires covering self-estimation of exercise capacity were completed. Peak oxygen uptake was compared to patient’s self-estimated exercise capacity with focus on differences between complex and non-complex defects. Results: Peak oxygen uptake was 25.5 ± 7.9 ml/minute/kg, corresponding to 75.1 ± 18.8% of age- and sex-specific reference values. Higher values of peak oxygen uptake were seen in patients with higher subjective rating of exercise capacity. However, oxygen uptake in patients rating their exercise capacity as good (mean oxygen uptake 78.5 ± 1.6%) or very good (mean oxygen uptake 84.8 ± 4.8%) was on average still reduced compared to normal. In patients with non-complex cardiac defects, we saw a significant correlation between peak oxygen uptake and self-estimated exercise capacity (spearman-rho −0.30, p < 0.001), whereas in patients with complex cardiac defects, no correlation was found (spearman-rho −0.11, p < 0.255). Conclusion: The mismatch between self-estimated and objectively assessed exercise capacity is most prominent in patients with complex CHD. Registration number at Charité Universitätsmedizin Berlin Ethics Committee: EA2/106/14.

Sign in / Sign up

Export Citation Format

Share Document