Outcome of surgery for congenital heart disease – A perspective from Malaysia

Congenital heart disease (CHD) is a common malformation in children, occurring in 6–9/1000 live births. It can manifest as a severe or critical lesion that necessitates immediate intervention and poses a high risk of morbidity and mortality if not detected or treated promptly. Furthermore, CHD can occur in isolation or with other anomalies, potentially leading to complications or death. In addition to CHD severity and related medical conditions, mortality and survival rates for CHD are affected by the economic status of the country, with high-income countries (HICs) having lower mortality and higher survival rates than lower- and middle-income countries (LMICs). Furthermore, the Global Burden of Disease Study 2017 showed a significant global disparity in CHD mortality rates.[1] HICs have shown a steady decline in CHD mortality rates, while LMICs have not made the same progress. Many of the LMICs do not have robust data to compare the outcomes of congenital heart surgery due to the lack of a national registry or database. Furthermore, the burden of CHD is increasing in India and other LMICs as the diagnosis rate has improved. However, due to a lack of infrastructure and manpower issues, there is a huge discrepancy in supply and demand for treatment for CHD. In most LMICs, the children undergoing surgery have major comorbidities such as small for gestational age, serious infections, prolonged cyanosis, pulmonary vascular disease, and malnutrition. The specific indicators for quality of care for CHD have been established from various national databases from the HICs. However, there are no comparable data from any large prospective studies in the LMICs. There are some global initiatives to investigate the outcomes of congenital heart surgery through the domain of the International Quality Improvement Collaborative (IQIC) and the recently introduced World Database from the World Society of Pediatric and Congenital Heart Surgeons. These databases could shed some light to gauge the outcomes of congenital heart surgery nationally, though it may not be the true representation of the whole nation. Kadiyani et al. have performed a systematic review and meta-analysis looking into the outcome of surgery for CHD in India. The analysis included 135 studies with 30,587 patients aged between 1 day and 65 years. The pooled mortality for surgical outcomes for CHD in India was 5.63% compared to Western data of 2.65%. Furthermore, the estimated mortality risk was higher than Western databases for all subcategories studied except for surgeries in the grown-ups with CHD population and coarctation repair.[2] The results from the pooled data mirror those of most other parts of the developing world. For instance, one would like to compare them with neighboring regions such as Southeast Asia. Malaysia, a middle-income country, exhibits characteristics typical of economies within this income bracket. From a state-based estimate, the birth prevalence for CHD is 6.7/1000 births.[3] With the estimated 600,000 births, the estimated new cases of CHD are over 4000 patients. An estimate of about 2000 will need some surgical intervention in a year. Two of five CHDs are expected to be either severe or significantly associated with syndromes and extracardiac defects. Furthermore, there was a significant increase in the detection of severe lesions in recent years, leading to an increased burden on a middle-income country’s already limited-resources healthcare system. In a study on the diagnosis of critical CHD, the median age of diagnosis was 4 days, with only 61 (9.1%) detected prenatally and 342 (51.2%) detected late.[4] The highest rate of late diagnosis was observed in coarctation of the aorta with a rate of 74%. Trend analysis shows a statistically significant reduction in late diagnosis and a significant increase in prenatal detection. However, Cox regression analysis shows the timing of diagnosis does not affect the outcome of CHD. There is some limited data available on the outcomes of congenital heart surgery. On analysis of results from the Pediatric Cardiology Clinical information system, the overall mortality rate was 15%, with a median age of death of 3.7 months (interquartile range: 0.9–9.8 months). Preoperative/intervention death was observed in 300 (11%), and 68 (3.2%) children died within 30 days of surgery or intervention. The overall estimated survival at 1, 5, 10, and 15 years was 88%, 85%, 84%, and 83%, respectively. The independent factors associated with mortality were male gender, associated syndrome or extra-cardiac defect, pulmonary hypertension, antenatal diagnosis, and severe lesions.[5] Only one center actively reports its results to international multicentric registries. The results showed most cases were performed beyond infancy. Nearly 70% of the operated cases fall in RACHS 2 and 3. The 30-mortality rate was 2.5%. The mortality rate based on the RACHS score showed a mortality of 1.2% for RACHS 2, 2.1% for RACHS 3, and 7.6% for the RACHS 4 category. These results mirror the results available from similar data from the various Indian studies. This important work by Kadiyani et al. has shed some light on some of the limitations in obtaining data to assess the outcomes for CHD in India and other LMICs.[2] The pooled data may not be the true representation of the outcomes described as the number of centers involved in the various studies is limited. Furthermore, there exists heterogeneity in the outcomes among the centers in India. Most of the pooled data reported in various publications were from international databases such as the IQIC. International databases may be restricted by several limitations in analyzing the data and may be unable to meet all expectations of the individual centers and national requirements. Hence, it is imperative to establish a national database on congenital heart surgery using national resources and facilities. A national database will allow all centers within the country to access their statistics and results and compare them. The users will also be able to reach the number of participating centers, the total number of patients, and the overall results. Furthermore, the database will be able to offer data analysis reports of selected most common index procedures. One of the important benefits of the database will be the benchmarking of the results among centers compared to national or even international outcomes. Such benchmarking may be particularly important in new programs, especially in the developing world. Participating in such databases and receiving feedback information benchmarked to national data may in and on itself stimulate efforts in participating sites directed toward improving performances. Besides, evaluating and sharing information transparently on differences in care and practices across sites, along with training local teams in quality improvement initiatives, to drive improvements in the quality-of-care outcomes. With the help of the national database, further collaboration can be established to develop a global platform for exchanging experience and knowledge in caring for children and babies with CHD. One such global platform is the World database from the World Society for Pediatric and Congenital Heart Surgery.[6] It is the first organization to create a platform for exchanging knowledge and experience, regardless of the socioeconomic status of a particular program or country. The effort has made an important opportunity for developing countries to analyze and improve outcomes following the repair of congenital heart defects.