scholarly journals Adult patients with congenital heart disease (GUCH): lights and shadows

2019 ◽  
Vol 13 (4) ◽  
pp. 189-199
Author(s):  
Luciano Daliento ◽  
Elettra Pomiato ◽  
Giovanni Vescovo ◽  
Massimo Padalino ◽  
Giovannella Russo
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Fetal Echocardiography ◽  
First Year ◽  
Term Survival ◽  
Surgery Outcome ◽  
The Sixties

More than 60 years have passed since Lillehei “The father of open surgery” started the era of correction of congenital heart diseases1. At the end of the sixties, only the minority of the patients with complex congenital heart diseases survived the first year after birth2; nowadays on the contrary, the grown-up congenital heart population exceeds the pediatric one3–5. The reasons of such a change are different: fetal echocardiography has made prenatal diagnosis of heart anomalies possible, thus causing the raise of interruptions of pregnancy. On the other hand, the high diagnostical accuracy and the brilliant surgery outcome along with an improved medical management allow higher survival rate6–8. Early during seventies Perloff foretold: “…it is a simple matter of time before a population of adult with congenital heart disease would emerge […]; we are obliged to look beyond the present and define our ultimate goal: the quality of life of long-term survival “9.

Congenital heart disease in the adult

2010 ◽  
pp. 2842-2872
Author(s):  
S. A. Thorne
Keyword(s):  
Congenital Heart Disease ◽  
United Kingdom ◽  
Heart Disease ◽  
Congenital Heart ◽  
Specialist Care ◽  
Term Survival ◽  
Long Term Survival ◽  
The United Kingdom ◽  
Growing Population

Adults with congenital heart disease are a growing population, and now outnumber children with congenital heart disease in the United Kingdom. Many patients with repaired hearts can now, with specialist care, expect to live a normal or near normal lifespan. Other survivors have complex, surgically altered hearts and circulations that reflect the surgical and interventional practices of the preceding two decades. Their long-term outlook is unknown and they remain at lifelong risk of complications that may require further intervention. The organization of services to provide specialist care is key to their long-term survival....

scholarly journals Accuracy of Prenatal Diagnosis of Congenital Cardiac Malformations

2018 ◽  
Vol 41 (01) ◽  
pp. 011-016 ◽  
Author(s):  
Diesa Pinheiro ◽  
Bruna Varisco ◽  
Marcelo Silva ◽  
Rafaela Duarte ◽  
Graciele Deliberali ◽  
...  
Keyword(s):  
Risk Factors ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Fetal Heart ◽  
Heart Diseases ◽  
Fetal Echocardiography ◽  
Congenital Heart Diseases ◽  
Obstetric Ultrasound ◽  
Morphological Evaluation

Abstract Objective To evaluate the accuracy of the diagnosis of fetal heart diseases obtained through ultrasound examinations performed during the prenatal period compared with the postnatal evaluation. Methods A retrospective cohort study with 96 pregnant women who were attended at the Echocardiography Service and whose deliveries occurred at the Complexo Hospitalar Santa Casa de Porto Alegre, in the state of Rio Grande do Sul, Brazil. Risk factor assessment plus sensitivity and specificity analysis were used, comparing the accuracy of the screening for congenital heart disease by means of obstetrical ultrasound and morphological evaluation and fetal echocardiography, considering p < 0.05 as significant. The present study was approved by the Research Ethics Committee of the Institution. Results The analysis of risk factors shows that 31.3% of the fetuses with congenital heart disease could be identified by anamnesis. The antepartum echocardiography demonstrated a sensitivity of 97.7%, a specificity of 88.9%, and accuracy of 93% in the diagnosis of congenital heart disease. A sensitivity of 29.3% was found for the obstetric ultrasound, of 54.3% for the morphological ultrasound, and of 97.7% for the fetal echocardiography. The fetal echocardiography detected fetal heart disease in 67.7% of the cases, the morphological ultrasound in 16.7%, and the obstetric ultrasound in 11.5% of the cases. Conclusion There is a high proportion of congenital heart disease in pregnancies with no risk factors for this outcome. Faced with the disappointing results of obstetric ultrasound for the detection of congenital heart diseases and the current unfeasibility of universal screening of congenital heart diseases through fetal echocardiography, the importance of the fetal morphological ultrasound and its performance by qualified professionals is reinforced for a more appropriate management of these pregnancies.

scholarly journals Influence of congenital heart disease-caused hypoxia on intrathymic development of t-lymphocytes in children of first year of life

2019 ◽  
Vol 36 (4) ◽  
pp. 46-54
Author(s):  
N. P. Loginova ◽  
V. A. Chetvertnykh ◽  
G. A. Khromtsova ◽  
R. M. Shekhmametyev ◽  
L. A. Chetvertnykh
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
T Lymphocytes ◽  
T Lymphocyte ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Immunological Methods ◽  
First Year ◽  
First Year Of Life ◽  
Degree Of Severity

Aim. To study the influence of hypoxia, caused by congenital heart disease, on the intrathymic development of T-lymphocytes and provision of the organism with T-cellular resource in children with cardiac pathology of different degree of severity. Materials and methods. The paper presents the results of complex immunomorphological study of the thymus in children of the first year of life (n = 129) with congenital heart diseases. Hystochemical and immunological methods were used to assess the influence of congenital heart disease-caused hypoxia on the functional status of thymus regarding provision of a child with T-lymphocytes. Results. It was established that the degree of severity of congenital heart disease correlated with the degree of suppression of T-lymphocyte pool formation. During the first year of life, proliferative activity of thymocytes (assessed by Ki-67 expression) in the thymus falls, being the reason of decrease in the number of CD3+ thymocytes. In conditions of marked hypoxia (blue type of defect) the process of T-lymphocyte differentiation is inhibited that causes preservation of less mature forms of thymocytes. As a result, hypoxia is the reason of proliferation suppression and thymocyte differentiation, influencing subpopulation composition of the cells. Thus, insufficient quantity of thymic migrants enters the peripheral blood that confirms low functional activity of thymus regarding provision of the organism with T-cellular resource. Conclusions. No doubt, it can be the cause of immunodeficient state and reduced formation of adaptive immunity in this category of children.

Surgery for Congenital Heart Disease: Improvements in Outcomes

2018 ◽  
Vol 35 (06) ◽  
pp. 557-560 ◽  
Author(s):  
Richard Jonas ◽  
Gerard Martin
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Complex Congenital Heart Disease ◽  
Term Outcome ◽  
Term Survival ◽  
Younger Age ◽  
Care Outcomes ◽  
Moderate Complexity

AbstractCardiac surgery for congenital heart disease has changed dramatically since the first surgery in 1938. During the early era, children underwent surgery at older ages often with palliative procedures before their corrective operation. Not surprisingly, in the early era, there was considerably higher early and late mortality, including the additive risks of having more than one procedure and a long period of living with an unphysiological palliated circulation. Over time with advances in noninvasive diagnosis, surgical approach, cardiopulmonary bypass techniques, and team-based care, outcomes have improved. Children now undergo corrective surgery at a younger age and have fewer palliative procedures. Short-term outcome as measured by the commonly used metric “procedural early mortality” (i.e., death before hospital discharge or less than 30 days following a surgical procedure) is now as low as 1 or 2% for many low-to-moderate complexity procedures. Late outcomes have also improved with long-term survival of hospital survivors for simple lesions being close to population controls. Late outcomes for more complex defects still show diminishing survival relative to a control population. Examination of the causes of death provides insights into areas in which clinical improvements may further improve the outlook for children with complex congenital heart disease.

Abstract 2132: Late Functional Outcomes after Operation for Ebstein’s Anomaly

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Morgan Brown ◽  
Joseph A Dearani ◽  
Gordon K Danielson ◽  
Frank Cetta ◽  
Heidi M Connolly ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Functional Outcomes ◽  
Congenital Heart ◽  
Pulmonary Atresia ◽  
Complex Congenital Heart Disease ◽  
Ebstein Anomaly ◽  
Term Survival ◽  
Cardiac Medication

Objective To review the long-term functional outcomes of patients with Ebstein anomaly who had cardiac surgery at our institution. Methods From April 1, 1972 to January 1, 2006, 539 patients with Ebstein anomaly had 604 cardiac operations at Mayo Clinic Rochester. The 506 late survivors form the basis of this report. Patient records were reviewed and all patients were mailed a medical questionnaire or contacted by telephone. Patients who had associated complex congenital heart disease were excluded (pulmonary atresia, conotruncal abnormalities, and atrioventricular discordance and ventriculoarterial discordance). Results At the initial operation at our institution, the mean age was 24 years (range 8 days to 79 years) and 53% were female. Survival at 5, 10, 15, and 20 years was 94%, 90%, 86%, and 76%. Survival free of late reoperation was 86%, 74%, 62%, and 46% at 5, 10, 15, and 20 years respectively. Surveys were returned by 285 patients. Two hundred and thirty-seven (83%) of patients were in NYHA class I or II and 34% were taking no cardiac medication. Five patients (2%) reported having endocarditis, 1 patient (<1%) reported a stroke, and 103 patients (36%) had atrial fibrillation or flutter. There were a total of 202 pregnancies among 82 women. Recurrence of congenital heart disease was low. Only 9/232 (0.04%) of liveborn children had congenital heart disease. Conclusions Patients have good long term survival and functional outcomes, after operation for Ebstein anomaly. Atrial arrhythmias are relatively common both before and after operation. Pregnancy appears to be well tolerated with low recurrence risk of congenital heart disease.

Prenatal detection of congenital heart disease with fetal echocardiography in Saudi Arabia: Systemic Review

2018 ◽  
pp. 1-4
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Fetal Echocardiography ◽  
Color Doppler ◽  
High Volume ◽  
Systemic Review ◽  
Clinical Value

Objectives: To evaluate the clinical value of color doppler echocardiography for the diagnosis of fetal congenital heart disease (CHD) included in routine obstetric care which provides relevant information for decision making, detailed anatomic survey performed at skilled, high‐volume obstetric centers, and to investigate the technical limitations of fetal echocardiography in this patient population. Methods: This was a retrospective descriptive review of fetal echocardiograms performed at Children’s Hospital Riyadh from (2010-2017). All women referred during the second trimester for fetal echocardiography because of maternal diabetes were included. Those with severe heart disease suspected on obstetric ultrasound examination were excluded. Results: There were 313 initial fetal echocardiograms. Forty three patients were diagnosed with multiple heart diseases, 32 patients were diagnosed with suspected mild heart disease (such as small ventricular septal defect), of which 8 patients had normal follow up study. Of 150 patients who had normal follow‐up fetal evaluation, 33 patients had severe CHD, 2 patients had IDDM (diabetes). Most of these pregnancies did not have a postnatal cardiac evaluation. Forty‐seven fetuses had benign cardiac findings. Fifty patients were asked to return for at least one follow‐up visit, most due to the inability to complete the exam at the initial visit. Conclusions: In an environment with access to high‐volume, skilled comprehensive ultrasound services, fetal echocardiography by a pediatric cardiology program adds little to the care of women with diabetes and high risk women. Poor acoustic windows may indicate frequent multiple visits.

Congenital heart disease in the adult

2020 ◽  
pp. 3559-3595
Author(s):  
S.A. Thorne
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Specialist Care ◽  
Term Survival ◽  
The United Kingdom ◽  
Developing Heart ◽  
Growing Population

Adults with congenital heart disease are a growing population, and now outnumber children with congenital heart disease in the United Kingdom. Many patients with repaired hearts can now, with specialist care, expect to live a normal or near normal lifespan. Other survivors have complex, surgically altered hearts and circulations that reflect the surgical and interventional practices of the preceding two decades. Their long-term outlook is unknown and they remain at lifelong risk of complications that may require further intervention. The organization of services to provide specialist care is key to their long-term survival. As the population of adults with congenital heart disease ages, so the number developing heart failure increases. Services caring for patients need to develop a robust end-of-life pathway that focuses on symptoms and quality of life, and runs in parallel with other therapies.

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