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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., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://cdt.amegroups.com/article/view/10.21037/cdt-22-281/coif). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part V” was commissioned by the editorial office without any funding or sponsorship. HK served as the unpaid Guest Editor of the series. The authors have no other conflicts of interest to declare., (2022 Cardiovascular Diagnosis and Therapy. All rights reserved.)

Background: Pulmonary arterial hypertension (PAH), a common complication in adults with congenital heart disease (CHD), leads to significant morbidity and mortality. Targeted PAH medication is available, but PAH-CHD patient data are limited. Several questions regarding indication, treatment escalation, and combination therapy remain unanswered. The aim of this study was therefore to evaluate PAH-specific treatment in adults with PAH-CHD to better understand PAH-specific therapy management., Methods: In this cross-sectional study we retrospectively examined clinical, demographic, and cardiac-catheterization data and medical management for PAH-CHD, and analyzed clinical course and midterm outcome., Results: Over up to 14 years (median, 6.2 years), 103 PAH-CHD patients (66% female) receiving targeted PAH-therapy for pre-tricuspid-shunt (15.5%), post-tricuspid-shunt (32.0%), and complex CHD (52.4%) were followed. Based on modified clinical European Society of Cardiology (ESC) classification, patients were assigned to the following subgroups: Eisenmenger syndrome (ES) (45.6%), severe pulmonary vascular disease (PVD) in complex CHD (20.4%), post-repair patients (19.4%), prevalent systemic-to-pulmonary shunt (3.9%), coincidental/small defects (0%), and Fontan circulation (10.7%). Changes in targeted PAH therapy were observed 249 times, with up to 6 (median, 2) therapy changes over a median period of 1.3 years. Over the study course, the medical treatment strategy changed towards combination therapy (baseline, 13.6%; study-end, 41%), resulting mostly in stabilized functional class or even improvement in cases of prevalent systemic-to-pulmonary shunt, ES, and patients with repaired CHD. Functional class deterioration, however, was seen in patients with severe PVD due to complex CHD, and Fontan patients. Of the 103 patients in the study, 25 died (24.3%). Patients with repaired CHD and patients with systemic-to-pulmonary shunt or ES showed the best survival rates. Mortality was remarkably higher in patients with severe PVD in complex CHD and Fontan patients., Conclusions: Many patients with PAH-CHD benefited from targeted PAH therapy over a median period of 6.2 years. Treatment decisions after targeted PAH-medication initiation were based mainly on clinical assessment. To counteract disease progression, an escalation towards combination therapy was observed during the study course. We consider survival rates under targeted PAH medication to be favorable, particularly in the ES subgroup. Nevertheless, further research is needed to optimize the use of PAH medication, especially in patients with complex CHD., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://cdt.amegroups.com/article/view/10.21037/cdt-22-266/coif). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part V” was commissioned by the editorial office without any funding or sponsorship. HK served as the unpaid Guest Editor of the series, and reports sponsorship/honoraria received from Actelion/Janssen, Bristol-Myers Squibb. HK is on steering board for COMPERA International Steering Board, received research grant/support from patient organizations: Deutsche Herzstiftung and Herzkind e.V, and received consulting fees from Janssen Pharmaceuticals. The authors have no other conflicts of interest to declare., (2022 Cardiovascular Diagnosis and Therapy. All rights reserved.)