Clinical Profile and Quality of Life of Adult Patients After the Fontan Procedure.

Increasingly, more patients with univentricular heart reach adulthood. Therefore, long-term psychological features are an important concern. The aim of this study was to evaluate the clinical and psychological profile of post-Fontan adult patients and to identify the most significant determinants of quality of life. In this retrospective cross-sectional study, we reviewed the surgical and medical history of post-Fontan adult patients. Patients underwent a 24-h electrocardiogram, echocardiography and exercise testing. Self-report questionnaires were used to assess the Work Ability Index, quality of life (Satisfaction with Life Scale), perceived health status (SF-36 questionnaire), coping strategies (Brief Cope questionnaire) and presence of mood disorders (Hospital Anxiety and Depression Scale). Thirty-nine patients aged between 18 and 48 years (mean 27.5 years) were enrolled. The mean follow-up was 21.5 years. Most patients were unmarried (82.9 %), had a high school diploma (62.9 %) and were employed (62.9 %). Twenty-nine patients (82.3 %) had at least one long-term complication. The median single ventricle ejection fraction was 57 %, and the median maximal oxygen consumption was 26.8 ml/min/kg. This population tended to be anxious and to use adaptive coping strategies. Quality of life was perceived as excellent or good in 57.2 % of cases and was not related to either cardiac function or exercise capacity. Both quality of life and SF-36 domains were related to the Work Ability Index. This cohort of post-Fontan adult patients enjoyed a good quality of life irrespective of disease severity.

Serial cardiopulmonary exercise testing in young patients after one-and-half ventricle repair and Fontan procedure: a comparative study.

BACKGROUND: The Fontan procedure is the palliative surgical treatment for different congenital heart diseases (CHD) with a univentricular heart, but it has been associated with decreased exercise capacity, cardiovascular morbidity, and premature mortality. The one-and-half ventricle repair (1.5VR) was introduced as an alternative to the Fontan procedure, specifically for selected patients with borderline hypoplastic right ventricle (HRV), aiming for a more physiological circulation. Despite these efforts, the benefit of 1.5VR over Fontan circulation comparison on clinical and functional outcomes remains unclear. The aim of this study was to investigate and compare young patients with HRV after 1.5VR with those with functional single right or left ventricles (FSRV or FSLV) after Fontan palliation over a 10-year follow-up period. METHODS: In this retrospective observational study, serial cardiopulmonary exercise tests (CPETs) performed in patients with 1.5VR and Fontan circulation between September 2002 and March 2024 have been analyzed. Only patients with at least 10 years of follow-up were considered. RESULTS: A total of 41 patients were included (age at baseline 8.6 ± 2.6 years): 21 with FSLV, 12 with FSRV, and 10 with 1.5VR. No differences in cardiorespiratory fitness and efficiency were shown at the first CPET assessment among the three groups. At 10-year follow-up, 1.5VR had higher cardiorespiratory fitness and efficiency compared to FSLV and FSRV patients. CONCLUSIONS: These findings suggest that the 1.5VR may provide superior long-term functional outcomes than the Fontan procedure in patients with borderline HRV. Further studies are needed to evaluate the impact on hard clinical endpoints.

Therapeutic Management of Anomalous Coronary Arteries Originating From the Opposite Sinus of Valsalva: Current Evidence, Proposed Approach, and the Unknowing.

Anomalous coronary arteries originating from the opposite sinus of Valsalva (ACAOS) are a challenge because of their various anatomic and clinical presentation. Although the prevalence is low, the absolute numbers of detected ACAOS are increasing because of the growing use of noninvasive anatomical imaging for ruling out coronary artery disease. As evidence-based guidelines are lacking, treating physicians are left in uncertainty for the optimal management of such patients. The sole presence of ACAOS does not justify surgical correction, and therefore a thorough anatomic and hemodynamic assessment is warranted. Invasive and noninvasive multimodality imaging provides information to the clinical question whether the presence of ACAOS is an innocent coincidental finding, is responsible for the patient's symptoms, or even might be a risk for sudden cardiac death. Based on recent clinical data, focusing on the pathophysiology of patients with ACAOS, myocardial ischemia is dependent on both the extent of fixed and dynamic components, represented by anatomic high-risk features. These varying combinations should be considered individually in the decision making for the different therapeutic options. This state-of-the-art review focuses on the advantages and limitations of the common contemporary surgical, interventional, and medical therapy with regard to the anatomy and pathophysiology of ACAOS. Further, we propose a therapeutic management algorithm based on current evidence on multimodality invasive and noninvasive imaging findings and highlight remaining gaps of knowledge.

Surgical repair of incomplete cleft sternum and cardiac anomalies in early infancy.

Congenital cleft sternum is a rare congenital anomaly due to lack of fusion of the lateral mesodermal plates. We report two cases of incomplete superior cleft sternum associated with cardiac anomalies that underwent successful complete surgical correction.