Drs. Kochilas and Oster Awarded $1.6 Million DOD Grant
This project focuses on the long-term outcomes of congenital heart diseases (CHD), which is one of the Fiscal Year 2018 Peer Reviewed Medical Research Programs Topic Areas. It specifically encourages a population-based and outcomes-based research project aiming to assess the health outcomes of individuals with congenital heart disease across their life spans.
Congenital heart disease is the most common type of structural birth defect, affecting almost 1% of all children born in the US each year. In the past, monitoring the health of people with various CHD revealed important information about the course of these individuals living with unrepaired CHD. This knowledge, along with tremendous advancements in cardiology and surgery, have led to a growing number of CHD survivors with complex issues who have never entered adulthood before. With age, these patients are continuously exposed to additional cardiovascular and non-cardiovascular conditions with the general population. However, risk from these conditions is likely higher than the general population as the CHD survivors are typically frailer. Understanding the long-term outcomes for the patients with CHD is important for identifying opportunities for intervention and planning for health care services in this concerning population. Nevertheless, in the absence of longitudinal registries for CHDs in the US, assessment of long-term outcomes after congenital heart surgery remains incomplete and the number of survivors with an operated CHD is only approximated by data from other countries.
Our project aims to fill important gaps related to the long-term outcomes for patients with CHD by linking the Pediatric Cardiac Care Consortium (PCCC), a large US-based registry for interventions of CHDs, with the US national death registry (National Death Index - NDI) and the organ transplant registry (Organ Procurement and Transplantation Network - OPTN). This linkage, currently available through 2014, captures organ failure and mortality patterns in a large cohort of patients after interventions for CHD. As part of this work, we plan to use this unique linked dataset of PCCC-NDI-OPTN and extend it through 2020 to provide reliable survival estimates for patients with operated CHDs over a maximum period of 38 years of follow-up. With this tool, our study aims to provide:
i) overall survival estimates for patients operated for a CHD at the time of surgery, and
ii) survival estimates beyond a certain time point or after reaching a specific milestone in the course of a specific CHD (conditional survival).
Overall estimates include information for people who have died within the first few years after their initial operation, resulting from residual abnormalities or complications of the procedures that saved their life in the first place. This information is important for patients and families entering their journey in the treatment of CHD and can also provide the framework to estimate the number of people living with an operated CHD in the US. However, this information, frequently provides a bleak prognosis that is not relevant to the millions that live today with an operated CHD. A far more important set of questions for these individuals may be: “Now that I have reached 10 years of age (or that I have survived my first year after a certain procedure), what are my chances to reach 40 years of age without needing a heart transplant?”. Answers to these questions are critical for CHD survivors, their families, care takers, third party payers and future employers and can be answered by the “conditional survival” analysis suggested in this study. Such information may be helpful to decrease the anxiety that often surrounds the lives of these patients and may also identify groups of patients and time points on their life course that higher surveillance is required to prevent serious events.