Cyanotic congenital heart disease in the Kingdom of Bahrain

Abstract EN

Background and Objectives: Cyanotic congenital heart disease (CCHD) is a high-risk condition in infants with congenital heart disease (CHD) and often requires urgent management.

It is important to have reliable information about the various cyanotic CHDs and their management.

As there is no data on CCHD in the Kingdom of Bahrain, we conducted this study to analyze the different types of CCHD, their management, and outcomes.

Methods: This was a retrospective descriptive study conducted from 2000 to 2019 on 554 patients with confirmed diagnosis of CCHD.

Results: Mean age at presentation was 1.8 years (range 0 day to 56 years) and 108 (19.5%) were diagnosed within 24 hours of life.

The mean duration of follow-up was 7.9 years.

The most common CCHDs included Tetralogy of Fallot (TOF) (44.9%), univentricular heart defects (UVH) (11.4%), and D-transposition of great arteries (D-TGA) (10.5%).The least common CCHD was hypoplastic right heart syndrome (HRHS) (0.4%).

The overall mortality was 15% (n=83).

The mortality rate was highest in patients with hypoplastic left heart syndrome (HLHS), noted in 52% cases.

Conclusions: The most common CCHD was TOF, and the most common mode of presentation was cyanosis.

The prognosis and outcomes were good in patients with biventricular repair but poor in patients with HLHS.

Early diagnosis and timely intervention can reduce the morbidity and mortality associated with CCHD.