Background: As a result of improved surgical and therapeutical management, more than 90% of patients with congenital heart disease (CHD) reach adulthood. However, the natural course of CHD is complicated by noncardiac medical problems. Aim of the study was to evaluate noncardiac comorbidities in a contemporary cohort of adults with CHD (ACHD). Methods: In a tertiary care center for ACHD, 821 consecutive patients, admitted to the outpatient clinic, were evaluated for clinically relevant noncardiac comorbidities. Results: The consecutively included patients (age: range, 15–80 years; 56% female) represent all types and severity grades of acyanotic and cyanotic CHD. A considerable proportion of ACHD had significant noncardiac comorbidities, which have the potential to profoundly influence the natural course of the underlying disease. In 95.5%, relevant non-cardiac comorbidities were apparent, that could be related to 16 special medical fields as endocrinologic/metabolic disease, gastroenterology/hepatology, gynecology/obstetrics, angiology, orthopedics, neurology/psychiatry and others. Most frequently seen comorbidities were endocrine and metabolic disorders (43.97%). Conclusions: Non-cardiac comorbidities are increasingly common in ACHD. The data revealed non-cardiac comorbidities as they were presented in the cohort of ACHD seen in a tertiary center. The study proves that ACHD with significant non-cardiac comorbidities need multidisciplinary care by medical organ specialists, aside the congenital cardiologist, with a deep knowledge about congenital heart defects, the special effects of the organ disease on the particular heart defect and, how the heart defect may affect the course of the particular organ disease. The study may create the basis for the development of screening programs for comorbidities in ACHD as well as a multidisciplinary concept for diagnosis and treatment of concomitant disorders or for disease prevention. Particularly disease prevention may improve quality of life as well as the further fate of the affected patients.

Introduction: Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension. Objective: To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data. Methods and results: COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is a prospective, international PH registry comprising, at the time of data analysis, >8200 patients with various forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to CHD, who were included between 2007 and 2018 in 49 specialized centers for PH and/or CHD located in 11 European countries. At enrollment, the patients’ median age was 44 years (67% female), and patients had either pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital left heart or aortic disease, or miscellaneous other types of CHD. Upon inclusion, targeted therapies for pulmonary arterial hypertension (PAH) included endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin analogues, and soluble guanylate cyclase stimulators. Eighty patients with Eisenmenger syndrome were treatment-naïve. While at inclusion the primary PAH treatment for the cohort was monotherapy (70% of patients), with 30% of the patients on combination therapy, after a median observation time of 45.3 months, the number of patients on combination therapy had increased significantly, to 50%. The use of oral anticoagulants or antiplatelets was dependent on the underlying diagnosis or comorbidities. In the entire COMPERA-CHD cohort, after follow-up and receiving targeted PAH therapy (n = 511), 91 patients died over the course of a 5-year follow up. The 5-year Kaplan–Meier survival estimate for CHD associated PH was significantly better than that for idiopathic PAH (76% vs. 54%; p < 0.001). Within the CHD associated PH group, survival estimates differed particularly depending on the underlying diagnosis and treatment status. Conclusions: In COMPERA-CHD, the overall survival of patients with CHD associated PH was dependent on the underlying diagnosis and treatment status, but was significantly better as than that for idiopathic PAH. Nevertheless, overall survival of patients with PAH due to CHD was still markedly reduced compared with survival of patients with other types of CHD, despite an increasing number of patients on PAH-targeted combination therapy.

Background: Due to advances in the diagnosis and treatment of congenital heart disease (CHD), the number of adults who are surviving with congenital heart disease (ACHD) is constantly growing. Until recently, the psychological effects of CHD had been widely neglected. Current research provides evidence for an increased risk of emotional distress in ACHD. The concept of illness identity attempts to explain how patients experience and integrate their CHD into their identities. The present study investigated illness identity in relation to clinical parameters and psychological functioning. Psychometric properties of the German version of the Illness Identity Questionnaire (IIQD) were examined. Methods: Self-reported measures on illness identity and psychological functioning (HADS-D) were assessed in a representative sample of 229 ACHD (38 ± 12.5 (18−73) years; 45% female) at the German Heart Center Munich. Descriptive analyses and multiple regression models were conducted. Confirmatory factor analysis was performed to validate the IIQD. Results: The IIQD demonstrated good reliability. The originally-postulated four-factor structure could not be replicated. Anatomic disease complexity and functional status significantly influenced illness identity. Illness identity accounted for unique variances in depression and anxiety: Maladaptive illness identity states (i.e., , engulfment and rejection) were associated with higher emotional distress, whereas adaptive illness (i.e., , acceptance and enrichment) identity states were linked to lower emotional distress. Conclusions: Illness Identity emerged as a predictor of emotional distress in ACHD. Findings raise the possibility that interventions designed to target a patient’s illness identity may improve psychological well-being and cardiac outcomes in ACHD.

Background: Due to the increase in survival rates for congenital heart disease (CHD) in the last decades, over 90% of patients today reach adulthood. Currently, there are more than 300,000 adults with CHD (ACHD) living in Germany. They have an increased need for specialized medical care, since almost all ACHD have chronic heart disease and suffer from specific chronic symptoms, risks, and sequelae. Primary care physicians (PCPs) play a crucial role in referring patients to ACHD specialists or specialized institutions. This cross-sectional study is intended to clarify the real-world care of ACHD from the PCP’s perspective. Methods: This analysis, initiated by the German Heart Centre Munich, was based on a 27-item questionnaire on actual ACHD health care practice in Germany from the PCP’s perspective. Results: In total, 767 questionnaires were considered valid for inclusion. The majority of the PCPs were general practitioners (95.9%), and 84.1% had cared for ACHD during the past year. A majority (69.2%) of the PCPs had cared for patients with simple CHD, while 50.6% and 33.4% had cared for patients with moderate and severe CHD, respectively, in all age groups. PCPs treated almost all typical residual symptoms and sequelae, and advised patients regarding difficult questions, including exercise capacity, pregnancy, genetics, and insurance matters. However, 33.8% of the PCPs did not even know about the existence of certified ACHD specialists or centers. Only 23.9% involved an ACHD-specialized physician in their treatment. In cases of severe cardiac issues, 70.8% of the PCPs referred patients to ACHD-certified centers. Although 52.5% of the PCPs were not sufficiently informed about existing structures, 64.2% rated the current care situation as either “very good” or “good”. Only 26.3% (n = 190) of the responding physicians were aware of patient organizations for ACHD. Conclusions: The present study showed that the majority of PCPs are not informed about the ACHD care structures available in Germany. The need for specialized ACHD follow-up care is largely underestimated, with an urgent need for optimization to reduce morbidity and mortality. For the future, solutions must be developed to integrate PCPs more intensively into the ACHD care network.

Background: In patients with congenital heart disease (CHD), there is little data on the diagnostic and prognostic relevance of hand grip strength (HGS) for clinical assignment, while in the general population the loss of muscle strength and mass is an important risk factor in cardiovascular disease which is conversely associated with morbidity and all-cause mortality. This study aimed to assess the degree of muscle dysfunction using HGS as a biomarker in a large group of patients with CHD who often develop muscle dysfunction. Methods: In total, 385 patients (27.6±13.1 years, 43% female) were included and assigned to 5 diagnostic groups: complex anomalies (n=131), left heart anomalies/aortopathies (n=107), right heart/pulmonary artery anomalies (n=92), primary left to-right-shunts (n=42) and miscellaneous CHD (n=13). Patients with Fontan circulation, chronic cyanosis, morphologic right systemic ventricle, arterial switch operation, or Ebstein’s anomaly were analyzed separately. A control group (CG) consisted of 124 healthy individuals (30.1±12.1 years, 42% female). HGS was measured with a Jamar Hydraulic Hand Dynamometer. Results: HGS was reduced in CHD patients compared to controls (35.2±14.6 versus 43.7±14.4 kg). Most impairments were present in females (26.1±7.6 kg). Patients with cyanosis had lower HGS values compared to acyanotic CHD patients (P=0.03). Patients with left heart lesions had the highest HSG values (40.7±14.7 kg), while patients with primary left-to-right shunt lesions had the lowest HSG values (30.9±11.3 kg). Within specific groups of cardiac anomalies, patients with Fontan circulation showed the lowest (P=0.033) and patients with a morphologic right system ventricle showed higher results (P=0.004). The late mid-term survival was favorable, and 7 patients (1.8%) died in a median interval of 422 days (range, 206–1,824 days) after HGS-testing. Conclusions: This study provides the most comprehensive data on the use of HGS in CHD to date. Grip strength is an easily applicable, repeatedly usable and a cost-effective diagnostic tool to gain a quick, quantifiable assessment of the patient’s current muscle function as an expression of cardiac fitness. Considering the low number of patients who died in the observation period, HGS may not be a suitable tool for survival assessment or identification of patients at risk. However, HGS is well suited to determine muscle function and strength and thereby to identify and to follow-up patients who have an increased cardiovascular risk.

Oral bacteria and odontogenic oral infections are responsible for a high portion of cases with infective endocarditis. Hence, oral health in patients with congenital heart disease (CHD) gains particular importance. This case-control study compared the oral health status in 112 adults with CHD and 168 healthy control subjects. In addition, the patient group was stratified according to the complexity of the heart defect and the recommendation for antibiotic prophylaxis during invasive dental procedures. Considering caries experience, a significantly lower mean DMFT (decayed missing filled teeth) score (7.91 ± 6.63 vs. 13.6 ± 8.15; p < 0.0001) was found in patients with CHD compared to healthy controls. Healthy controls had a higher average number of decayed teeth (0.33 ± 0.76 vs. 1.76 ± 2.61; p < 0.0001). In female subjects a significant lower relative amount of teeth with apical periodontitis was found among CHD patients (3.4% ± 0.9%) as compared to healthy controls (5.6% ± 1.9%) (p = 0.053). Regarding periodontal health, patients with CHD had lower rate of sulcus bleeding (0.32 ± 0.65 vs. 0.71 ± 0.60; p < 0.0001) and less alveolar bone loss than heart healthy individuals (% root length: multi rooted teeth: 8.97 ± 10.64 vs. 23.22 ± 20.70; p < 0.0001; single rooted teeth: 5.59 ± 6.25 vs. 17.30 ± 17.17; p = 0.003). On the contrary, CHD patients presented with higher amount of plaque in comparison to healthy controls (Quigley & Hein index: 2.22 ± 0.67 vs. 1.25 ± 0.72; p < 0.0001). Based on the current results, it can be concluded that adults with CHD have better oral health than heart healthy individuals.