scholarly journals PA1 Different Levels of Care for Follow-up of Adults with Congenital Heart Disease

2020 ◽  
Vol 23 ◽  
pp. S408
Author(s):  
R. Willems ◽  
F. Ombelet ◽  
E. Goossens ◽  
K. De Groote ◽  
W. Budts ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Levels Of Care ◽  
Different Levels

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 Discontinuity of Cardiac Follow‐Up in Young People With Congenital Heart Disease Transitioning to Adulthood: A Systematic Review and Meta‐Analysis

2021 ◽  
Author(s):  
Philip Moons ◽  
Sandra Skogby ◽  
Ewa‐Lena Bratt ◽  
Liesl Zühlke ◽  
Ariane Marelli ◽  
...  
Keyword(s):  
United States ◽  
Systematic Review ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Heart Defects ◽  
Meta Analysis ◽  
Transition Programs ◽  
The United States

Background The majority of people born with congenital heart disease require lifelong cardiac follow‐up. However, discontinuity of care is a recognized problem and appears to increase around the transition to adulthood. We performed a systematic review and meta‐analysis to estimate the proportion of adolescents and emerging adults with congenital heart disease discontinuing cardiac follow‐up. In pooled data, we investigated regional differences, disparities by disease complexity, and the impact of transition programs on the discontinuity of care. Methods and Results Searches were performed in PubMed, Embase, Cinahl, and Web of Science. We identified 17 studies, which enrolled 6847 patients. A random effects meta‐analysis of single proportions was performed according to the DerSimonian‐Laird method. Moderator effects were computed to explore sources for heterogeneity. Discontinuity proportions ranged from 3.6% to 62.7%, with a pooled estimated proportion of 26.1% (95% CI, 19.2%–34.6%). A trend toward more discontinuity was observed in simple heart defects (33.7%; 95% CI, 15.6%–58.3%), compared with moderate (25.7%; 95% CI, 15.2%–40.1%) or complex congenital heart disease (22.3%; 95% CI, 16.5%–29.4%) ( P =0.2372). Studies from the United States (34.0%; 95% CI, 24.3%–45.4%), Canada (25.7%; 95% CI, 17.0%–36.7%), and Europe (6.5%; 95% CI, 5.3%–7.9%) differed significantly ( P =0.0004). Transition programs were shown to have the potential to reduce discontinuity of care (12.7%; 95% CI, 2.8%–42.3%) compared with usual care (36.2%; 95% CI, 22.8%–52.2%) ( P =0.1119). Conclusions This meta‐analysis showed that there is a high proportion of discontinuity of care in young people with congenital heart disease. The highest discontinuity proportions were observed in studies from the United States and in patients with simple heart defects. It is suggested that transition programs have a protective effect. Registration URL: www.crd.york.ac.uk/prospero . Unique identifier: CRD42020182413.

scholarly journals Long‐Term Outcome of Patients With Congenital Heart Disease Undergoing Cardiac Resynchronization Therapy

2021 ◽  
Author(s):  
Peter Kubuš ◽  
Jana Rubáčková Popelová ◽  
Jan Kovanda ◽  
Kamil Sedláček ◽  
Jan Janoušek
Keyword(s):  
New York ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Heart Association ◽  
Cardiac Resynchronization ◽  
New York Heart Association ◽  
Crt Response

Background Cardiac resynchronization therapy (CRT) is rarely used in patients with congenital heart disease, and reported follow‐up is short. We sought to evaluate long‐term impact of CRT in a single‐center cohort of patients with congenital heart disease. Methods and Results Thirty‐two consecutive patients with structural congenital heart disease (N=30) or congenital atrioventricular block (N=2), aged median of 12.9 years at CRT with pacing capability device implantation, were followed up for a median of 8.7 years. CRT response was defined as an increase in systemic ventricular ejection fraction or fractional area of change by >10 units and improved or unchanged New York Heart Association class. Freedom from cardiovascular death, heart failure hospitalization, or new transplant listing was 92.6% and 83.2% at 5 and 10 years, respectively. Freedom from CRT complications, leading to surgical system revision (elective generator replacement excluded) or therapy termination, was 82.7% and 72.2% at 5 and 10 years, respectively. The overall probability of an uneventful therapy continuation was 76.3% and 58.8% at 5 and 10 years, respectively. There was a significant increase in ejection fraction/fractional area of change ( P <0.001) mainly attributable to patients with systemic left ventricle ( P =0.002) and decrease in systemic ventricular end‐diastolic dimensions ( P <0.05) after CRT. New York Heart Association functional class improved from a median 2.0 to 1.25 ( P <0.001). Long‐term CRT response was present in 54.8% of patients at last follow‐up and was more frequent in systemic left ventricle ( P <0.001). Conclusions CRT in patients with congenital heart disease was associated with acceptable survival and long‐term response in ≈50% of patients. Probability of an uneventful CRT continuation was modest.

Stent Implantation to Maintain Patency of a Stenosed Blalock Taussig Shunt

2005 ◽  
Vol 13 (3) ◽  
pp. 274-276 ◽  
Author(s):  
Amjad Kouatli ◽  
Jameel Al-Ata ◽  
M Omar Galal ◽  
Muhammed A Amin ◽  
Arif Hussain
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Oxygen Saturation ◽  
Stent Implantation ◽  
Congenital Heart ◽  
Balloon Dilation ◽  
Complex Congenital Heart Disease ◽  
Blalock Taussig Shunt

A 14-year-old female with complex congenital heart disease underwent a left-sided classical Blalock Taussig (BT) shunt 15 days after birth. Ten years after the operation her oxygen saturation had decreased significantly. An angiography revealed a severely stenosed BT shunt. Balloon dilation including implantation of a 6 × 13 mm stent was performed successfully. Immediately after intervention, oxygen saturation rose from 55% to 80 84% in room air. Follow-up at a year and a half later showed the classical BT shunt was still patent.

Implementation of Transition Programs can Prevent Another Lost Generation of Patients with Congenital Heart Disease

2008 ◽  
Vol 7 (4) ◽  
pp. 259-263 ◽  
Author(s):  
Philip Moons ◽  
Deborah Hilderson ◽  
Kristien Van Deyk
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Birth Defect ◽  
Congenital Heart ◽  
Transition Programs ◽  
Medical System ◽  
Lost Generation ◽  
Specialized Care ◽  
Lost To Follow Up

Congenital heart disease is the most frequently occurring birth defect. To date, more than 90% of the children born with a heart defect reach adulthood. Since many patients are prone to residua and sequelae, lifelong specialized care is required. However, studies indicate that about one-half to three-quarters of the patients are lost to follow-up when they have grown up. This has resulted in a virtual lost generation. Lapse of care is associated with significant morbidity. Therefore, implementation of strategies to prevent patients from failing to continue regular follow-up is critical. It is argued that transition programs that inform patients about the rationale for ongoing follow-up and that teach them how to navigate the medical system can avoid another lost generation.

scholarly journals Long-Term Follow-Up After Ablation of Intra-Atrial Re-Entrant Tachycardia in Patients With Congenital Heart Disease

2018 ◽  
Vol 4 (6) ◽  
pp. 771-780 ◽  
Author(s):  
Ivo Roca-Luque ◽  
Nuria Rivas-Gándara ◽  
Laura Dos Subirà ◽  
Jaume Francisco Pascual ◽  
Antònia Pijuan-Domenech ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Long Term Follow Up

Survival of Patients with High-Risk Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

2021 ◽  
Vol 104 (6) ◽  
pp. 895-901
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
High Risk ◽  
Congenital Heart ◽  
First Year ◽  
Pulmonary Arterial ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Background: Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD) with uncorrected left-to-right shunts. Currently, no consensus guideline exists on the management of PAH-CHD in children, especially those who do not meet operability criteria. Objective: To compare survival between three groups of high-risk PAH-CHD, group 1: total correction including both surgical and percutaneous intervention, group 2: palliative treatment, and group 3: conservative with medical treatment group. Materials and Methods: All pediatric patients with PAH-CHD that underwent cardiac catheterization between January 1, 2008 and December 31, 2017 were retrospectively reviewed. Inclusion criteria were high risk PAH-CHD patients who had pulmonary vascular resistance (PVR) greater than 6 Wood unit·m² and PVR-to-SVR ratio greater than 0.3 evaluated in room air. Exclusion criteria were younger than three months of age, severe left side heart disease with pulmonary capillary wedge pressure greater than 15 mmHg, obstructive total pulmonary venous return, and single ventricle physiology. The Kaplan-Meier analysis was performed from the date of PAH diagnosis to the date of all-cause mortality or to censored date at last follow-up. Results: Seventy-six patients with a median age at diagnosis of 27.5 months (IQR 14.5 to 69.0 months) were included in this study. The patients were divided into three subgroups and included 38 patients (50.0%) in group 1, six patients (7.9%) in group 2, and 32 patients (42.1%) in group 3. The median follow-up time was 554 days (IQR 103 to 2,133 days). The overall mortality was 21.7%. One-year survival in patients with simple lesion in group 1 and 3 were 79.5% and 87.5% and patients with complex lesions in group 1, 2, and 3 were 93.8%, 83.3%, and 73.1%, respectively. The results showed that most mortalities occurred in the first year. There were no statistically significant differences in survival among difference types of treatment (log rank test, p=0.522). Conclusion: The mortality of high-risk PAH-CHD patients were not different among those who underwent corrective surgery, palliative, or conservative treatment. The mortality was high in the first year after PAH diagnosis and remain stable afterward. Management decision for an individual with high-risk PAH-CHD patients requires comprehensive clinical assessment to balance the risks and benefits before making individualized clinical judgment. Keywords: Pulmonary hypertension; Congenital heart disease; High-risk patients

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