Dataset Information

Epidemiology of adult congenital heart disease among the general population in Kuwait.

ABSTRACT:

Background

Adult congenital heart disease (ACHD) is a highly underrepresented entity in medical literature, especially in the middle-eastern region.

Hypothesis

This study is the first to assess the prevalence of adult congenital heart disease among the population of Kuwait.

Methods

After a retrospective register review of patients in Kuwait being followed up in the chest diseases hospital was conducted, patients who fit the inclusion criteria were enrolled in the study. Using the American College of Cardiology Task Force 1 of the 32nd Bethesda conference classification of the severity of ACHD, the patients were classified into those with simple, moderate, and complex congenital heart diseases. The age and gender of the patients, as well as the type repair performed, and the residual cardiac findings were recorded to assess the association between the complexity and residuals. Associations were assessed using STATA 15.

Results

A total of 611 patients were evaluated over a period of 18 months. The youngest participant was 20 years of age, and the oldest participant was 88 years old. Male participants with moderate congenital heart disease class were more common in our study population. Patients with complex congenital heart disease have more residual cardiac lesion than the moderate or simple groups. Almost (70%) of patients with complex cardiac anomalies have undergone either partial or complete repair. The most prevalent cardiac defect was atrial septal defect (21.5%). Tetralogy of Fallot was the most prevalent defect in the moderate group, representing (13%) of the group. The most prevalent anomaly in the complex group was double outlet right ventricle (DORV) representing (15.38%).

Conclusion

Adult Congenital heart disease is a growing entity of heart disease due to advanced repair techniques. This population requires registries to document cases and assign specialists for the management and care of this special group of patients.

Highlights

First database of adult congenital heart disease in Kuwait. The most prevalent heart defect was ASD in Kuwait. TOF was the most prevalent defect in the moderate group; and DORV was the most prevalent in the complex group. Patients with moderate ACHD tended to have a more complete repair than those in the complex group.

SUBMITTER: Ashkanani H

PROVIDER: S-EPMC8027582 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:Background and objectiveAs more children with congenital heart disease survive to adulthood, adult congenital heart disease (ACHD) prevalence will increase (currently ~1 million US patients). Heart failure (HF) accounts for 26-42% of ACHD deaths. The rate of ACHD heart transplantations (ACHD HTx) is also increasing. We describe the ACHD HTx recipient/candidate cohort, analyze ACHD HTx outcomes, identify ACHD HTx specific challenges, and discuss opportunities to better serve more patients with ACHD HF.MethodsPubMed literature search including articles published from 2010-2023. Reviewed 89 studies, 67 included. Our search focused on the challenges of ACHD HTx and potential solutions.Key content and findingsACHD HTx recipients are young [median age 35 years, interquartile range (IQR): 24-46 years]. 87-95% of ACHD HTx recipients had prior cardiac surgery. The most common underlying diagnoses include transposition of the great arteries (31%) and Fontan/Glenn circulation (28%). 63% of listed ACHD HTx candidates received a transplant within one year of listing. Post-transplant 1-year survival is 80%, 5-year survival 74%, and 10-year survival 59%. There are 4 unique ACHD HTx challenges: (I) difficulty in assessing pulmonary hypertension, resulting in some centers selecting oversized donor hearts. However, selecting oversized hearts does not improve post-operative mortality and could prolong waitlist time. (II) Increased immunologic sensitization, increasing rejection risk. Desensitization therapy has enabled sensitized HTx recipients to enjoy outcomes similar to non-sensitized recipients. (III) Procedural complexity with ~30% of cases requiring additional surgical reconstruction. Detailed multidisciplinary planning, extensive imaging, and transferring the patient into the operating room early can help manage the complexities and reduce organ ischemic time. (IV) Increased intraoperative bleeding due to patients' surgical histories and circulatory collaterals. Preoperative collateral coil embolization and select utilization of hypothermic circulatory arrest can help reduce bleeding. Additional Fontan specific challenges include extensive great artery repair, liver failure, plastic bronchitis, and protein loss enteropathy. Finally, given limited donor heart availability, mechanical circulatory support is a promising technology for patients with ACHD HF.ConclusionsThe prevalence of ACHD HTx is slowly but steadily increasing. The operational complexity of ACHD HTx can be managed, and the majority of recipients have excellent outcomes (59% 10-year survival).