Congenital heart disease and pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2855-2861
Author(s):  
Jolien Roos-Hesselink ◽  
Annemien van den Bosch
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Functional Class ◽  
Cardiac Events ◽  
Childbearing Age ◽  
Increased Risk ◽  
History Of ◽  
Valvular Function ◽  
Residual Lesions

Improvement in diagnosis and treatment of congenital heart disease has increased the number of women reaching childbearing age, including women with more complex malformations. However, these women face an increased risk of cardiac and obstetric complications. The risk of pregnancy depends not only on the underlying congenital heart disease, but also on additional factors such as residual lesions, ventricular and valvular function, functional class, and cyanosis. Risk assessment before pregnancy in all patients is necessary, and in particular, functional status before pregnancy and history of previous cardiac events are of prognostic value. Therefore, all women should be seen and counselled by well-trained specialists in an expertise centre prior to pregnancy. In addition, an experienced multidisciplinary team should be available to provide care, before, during, and after pregnancy.

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.

Abstract 19082: Cardiac Events and Mortality During Admissions for Delivery in Women with Congenital Heart Disease

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Robert M Hayward ◽  
Elyse Foster ◽  
Zian H Tseng
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Cardiac Arrest ◽  
Heart Disease ◽  
Length Of Stay ◽  
Hospital Mortality ◽  
Congenital Heart ◽  
Cardiac Events ◽  
Live Births ◽  
History Of

Background: Labor, delivery, and the postpartum period are a time of increased arrhythmia and congestive heart failure (CHF) incidence. With improvements in the treatment of congenital heart disease (CHD), more women are reaching childbearing age and may be at increased risk for cardiac events and mortality during pregnancy and delivery. Methods: The Healthcare Cost and Utilization Project was used to identify admissions for vaginal and cesarean delivery in California hospitals between 1/1/2005 and 12/31/2011. We compared length of stay, in-hospital mortality, incident CHF, cardiac arrest, and incident arrhythmias for women without CHD to women with non-complex CHD (NC-CHD) and complex CHD (C-CHD). Results: We identified 2,720,980 deliveries resulting in 2,770,382 live births (74% of live births in the state over this period), which included 3,218 women with NC-CHD and 248 women with C-CHD. History of CHF was more common in women with CHD (8.1% for C-CHD, 2.6% for NC-CHD, and 0.08% for women without CHD, p<0.00005 for NC-CHD compared to no CHD and for C-CHD compared to no CHD). Those with CHD were more likely to undergo cesarean section (Table 1). Length of stay was significantly longer in women with CHD (2.6 ± 2.3 days for women without CHD, 3.4 ± 10.2 days for women with NC-CHD and 5.0 ± 13.3 days for women with C-CHD). In-hospital mortality was not significantly higher in women with CHD (Table 1). Incident heart failure, arrhythmias, and cardiac arrest were uncommon in all groups (Table 1). Conclusions: In this study of 2.7 million women admitted to California hospitals for delivery, women with CHD were more likely to undergo cesarean section and had longer length of stay. Despite more frequent history of CHF in women with CHD, incident CHF and arrhythmias were rare during hospitalization. In-hospital mortality and cardiac arrest were not higher in CHD patients. These results suggest that in pregnant women with CHD, cardiac events and mortality at the time delivery are uncommon.

P5353Pregnancy in congenital heart disease - risk stratificaton

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Pataki ◽  
M Szegedi ◽  
A Temesvari ◽  
L Ablonczy ◽  
P Andreka ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
High Risk ◽  
Premature Birth ◽  
Congenital Heart ◽  
Left Ventricular ◽  
High Risk Pregnancy ◽  
Cardiac Events ◽  
Risk Pregnancy ◽  
Increased Risk

Abstract Introduction Maternal congenital heart disease is a leading cause of peripartum maternal mortality, which is also associated with increased risk of fetal morbidity and mortality. We aimed to provide risk stratification for congenital heart disease patients using the recently introduced CARPREG (Cardiac Disease in Pregnancy) II score at our Institute. Patients and methods We enrolled 191 pregnant women with congenital heart disease (mean age at pregnancy 29.2±5,7 years). We recorded all foetal (premature birth, abortion, congenital heart abnormalities) and maternal (postpartum heart failure, stroke, hypertension) events in 276 pregnancies. High-risk pregnancy was defined as a CARPREG II score of 4≤. Results High-risk pregnancy was detected in 14.1%. We found fetal and maternal events in 13.8% and 14.5% of all pregnancies, respectively, which both were more prevalent in the high-risk group (p<0.001). No maternal death occurred, whereas premature birth and abortion was detected in 7.6 and 2.9%, respectively, both complications were more frequent in the high-risk patient population as compared to the lower risk patients (30.8 vs. 7.2%, p<0.001). In univariate analysis, CARPREG II of 4≤ was significantly associated with fetal (p<0.001, OR: 4.2) and maternal (p<0.001, OR: 5.3) events. Risk factors of the CARPREG II were further analyzed using multivariate logistic regression analysis: prior cardiac events or arrhythmias (p=0.027) and cyanosis (p=0.026) were independent predictors of fetal complications. Left ventricular outflow tract obstruction (p<0.001), cardiac interventions (p=0.024) and prior cardiac events or arrhythmias (p=0.001) demonstrated significant association with maternal events. Conclusion The CARPREG II could help in the identification of high-risk pregnancies and thus aids the selection of patients for strict monitoring and special therapies. In line with previous studies we detected higher rates of fetal and maternal events among patients with CARPREG II of 4≤ score.

Impact of Major Residual Lesions on Outcomes After Surgery for Congenital Heart Disease

2021 ◽  
Vol 77 (19) ◽  
pp. 2382-2394
Author(s):  
Meena Nathan ◽  
Jami C. Levine ◽  
Maria I. Van Rompay ◽  
Linda M. Lambert ◽  
Felicia L. Trachtenberg ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Residual Lesions

scholarly journals Familial congenital heart disease in Bandung, Indonesia

2013 ◽  
Vol 53 (3) ◽  
pp. 173
Author(s):  
Sri Endah Rahayuningsih
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Dilated Cardiomyopathy ◽  
Congenital Heart ◽  
Family Studies ◽  
Recurrence Risk ◽  
Descriptive Study ◽  
Pedigree Analysis ◽  
Tricuspid Atresia ◽  
History Of

Background Congenital heart disease (CHD) may occur inseveral members of a family. Studies have shown that familialgenetic factor play a role in CHD.Objective To identify familial recurrences of CHD in familieswith at least one member treated for CHD in Dr. Hasan SadikinHospital, Bandung Indonesia.Methods In this descriptive study, subjects were CHD patientshospitalized or treated from January 2005 to December 2011. Weconstructed family pedigrees for five families.Results During the study period, there were 1,779 patients withCHD. We found 5 families with 12 familial CHD cases, consistingof 8 boys and 4 girls. Defects observed in these 12 patients weretetralogy of Fallot, transposition of the great arteries, persistentductus arteriosus, ventricular septa! defect, tricuspid atresia,pulmonary stenos is, and dilated cardiomyopathy. Persistent ductusarteriosus was the most frequently observed defect (4 out of 12subjects) . None of the families had a history of consanguinity. Therecurrence risk of CHD among siblings was calculated to be 0.67%,and the recurrence risk ofCHD among cousins was 0.16%.Conclusion Familial CHD may indicate the need for geneticcounseling and further pedigree analysis.

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 Double Inlet Left Ventricle with Eisenmenger Syndrome in an Adult – A Case Report

2017 ◽  
Vol 5 (1) ◽  
pp. 53-56
Author(s):  
Rahul Regi Abraham ◽  
Rahul Regi Abraham
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricle ◽  
Congenital Heart ◽  
Pulmonary Stenosis ◽  
Eisenmenger Syndrome ◽  
Tertiary Center ◽  
History Of ◽  
Double Inlet Left Ventricle

Background: Patient diagnosed with double inlet left ventricle (prevalent in 5 – 10 in 100,000 newborns) complicated with Eisenmenger syndrome had a median survival age of 14 years without corrective surgery. Congenital heart disease such as this is usually treated by multiple surgeries during early childhood. A surgically uncorrected case in adults is not of common occurrence. Further, generalized itching after coming in contact with water (aquagenic pruritis) presented an interesting conundrum to treat. Case: A 29-year-old patient in India presented at a primary health care center with a history of difficulty breathing and discoloration of extremities since birth. He also gave a history of itching which commonly occurred after taking bath, hemoptysis and history of turning blue in color after birth. Patient had received no treatment besides regular phlebotomies. On examination, there was grade IV clubbing and conjunctival congestion. Cardiovascular examination revealed an enlarged heart, heaving apex beat and a pan-systolic murmur. A provisional diagnosis of a congenital cyanotic heart disease was made. Investigations revealed hemoglobin of 16.8g/dl. X–ray and electrocardiogram showed hypertrophy of the ventricles. An echocardiogram showed double inlet left ventricle with L-malposed vessels but without pulmonary stenosis. A final diagnosis of congenital heart disease; double inlet left ventricle, L-malposed vessels without pulmonary stenosis, Eisenmenger Syndrome and absolute erythrocytosis was made. Patient was advised for further management with a cardiologist in a tertiary center but the patient did not follow up. Conclusion: Unlike in high-income countries where most congenital heart diseases are detected and dealt with at birth whereas low-and middle-income nations often have to deal with cases that present much later and should often be included in the differential diagnosis. Inability to follow up cases, centers that are poorly equipped and lack of facilities for investigations, patient’s lack of medical awareness, and financial restrictions are major barriers to providing optimal treatment.

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.

Preconception Assessment of Women of Childbearing Age With Congenital Heart Disease

2019 ◽  
Vol 72 (8) ◽  
pp. 683-684
Author(s):  
Minerva Rodríguez Martín ◽  
Agustín Carlos Martín García ◽  
Luisa García-Cuenllas ◽  
Juan Carlos Castro-Garay ◽  
Beatriz Plata ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Women Of Childbearing Age ◽  
Childbearing Age

Abstract 13468: Effect of Listing Criteria on Transplant Rate and Early Outcomes in Adults With Congenital Heart Disease

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Wagih m Zayed ◽  
Neha Bansal ◽  
Snehal R Patel ◽  
Jacqueline M Lamour ◽  
Daniel J GOLDSTEIN ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Primary Diagnosis ◽  
Organ Allocation ◽  
Transplant Recipients ◽  
Allocation Policy ◽  
Kaplan Meier ◽  
Allocation Criteria ◽  
History Of

Introduction: Heart failure (HF) is the leading cause of death in adults with congenital heart disease (ACHD). Heart transplant (HT) is one of the few options for the treatment of advanced HF in this growing population. In October 2018, the United Network for Organ Sharing (UNOS) implemented a change in organ allocation criteria. The effect of this change on outcomes in ACHD patients (pts) after listing and transplant has not been evaluated. Hypothesis: Change in organ allocation criteria negatively impacts outcomes in ACHD patients. Methods: Data from the Scientific Registry of Transplant Recipients in pts age > 18 years old listed for HT between Oct. 2016 and 0ct. 2019 and followed through March 2020 were analyzed. Pts were grouped by diagnosis (ACHD and non-ACHD) and by the time of listing (pre- and post-change in allocation criteria). Differences in comorbidities, outcomes while listed, and 1-year Kaplan Meier survival post-HT were compared among groups. For comparison, post-change criteria (status 1-6) were equated to pre-change criteria (status 1A, 1B, 2). Results: Over 3 years, 11,931 patients were listed for HT; 459 had a primary diagnosis of ACHD. ACHD was present in 279/7942 pts listed in the 2 years pre-change and 180/3989 pts in the year post-change. ACHD pts listed post-change were less likely to have a history of cardiac surgery (88% vs. 79%, p=0.01) and more likely to have an abnormal BMI (p=0.015) than ACHD pts pre-change. Post-change, ACHD pts were listed at a higher priority status compared to pre-change ACHD. (Figure). The proportion of pts transplanted with ACHD increased slightly pre- and post-change (3.7% vs. 4.1%). There was no difference in 1-year survival in ACHD pts transplanted pre- and post-change (Figure). Conclusions: Recent changes to the UNOS organ allocation policy increased the proportion of ACHD patients transplanted with no change in early post-HT survival.

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