Healthcare status of adults with pulmonary hypertension due to congenital heart disease

Background: In the long-term course of treated and untreated congenital heart defects (CHD), pulmonary hypertension (PH) is one of the most relevant complications. Since PH carries a high risk for mortality and morbidity, it is important to improve the status of healthcare and medical knowledge regarding the affected patients. Therefore, this study aimed to determine the current medical care status, health-related knowledge, and specific counseling needs of adults with various forms of CHD (ACHD) who are at increased risk of developing PH, as well as those with manifest PH. Methods: In this retrospective cross-sectional study, a representative sample of 803 ACHD were analyzed. Patients were split into three groups based on CHD: low risk for PH, at risk for pre- or post-capillary PH, and manifest PH. Data collection took place between September 2017 until February 2018 in a tertiary care center for ACHD. Healthcare status and specific needs for information or advice were analyzed using a questionnaire designed by our group. The state of knowledge of the patients was assessed by comparing this questionnaire and the corresponding medical records. Results: Both patients with manifest PH (n=51) and patients at risk to develop PH (n=629) were insufficiently informed about their health status, specific care structures available to them, and patient organizations. About 50% of the patients had specific counseling needs, especially regarding physical capability and sports, daily stress, rehabilitation measures, and pregnancy. Only 47.8% of patients with manifest PH were aware of suffering from PH (P<0.001). In particular, the patients had large knowledge deficits regarding comorbidities related to their health condition. Conclusions: PH is a quantitatively and qualitatively underestimated residuum or sequela of CHD that significantly affects outcome and prognosis in ACHD. Multidisciplinary, structured, and specific counseling of affected individuals with corresponding risk constellations is urgently needed. A prerequisite for this is closer collaboration between primary care physicians (PCPs), such as general practitioners, family physicians, internists, or general cardiologists, and ACHD specialists. Targeted patient counseling and care could have a positive impact on the level of awareness of those affected and favorably influence their prognosis.