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Adult Congenital Heart Disease: An Increasing and Ageing Population: Part...
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Adult Congenital Heart Disease: An Increasing and Ageing Population: Part II

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Cardiology".

Deadline for manuscript submissions: 31 October 2024 | Viewed by 5928

Special Issue Editor

Prof. Dr. Oktay Tutarel

E-Mail Website
Guest Editor
1. Adult Congenital Heart Disease, Technical University of Munich, Munich, Germany
2. Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Munich, Germany
Interests: adult congenital heart disease; pulmonary hypertension; heart failure
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Due to the advances in the diagnosis and treatment of congenital heart disease in newborns and children that took place in recent decades, today, the majority of patients survive to adulthood. However, unfortunately, a cure for congenital heart disease is seldom achieved, and residua and sequela are common, leading to an ever-increasing group of adult patients with congenital heart disease (ACHD). However, the population is not only increasing but also ageing. Consequently, as ACHD patients are getting older, comorbidities are acquired similar to in the general population, and sometimes even with a higher prevalence. Several recent studies have emphasized the importance of these comorbidities for increasing the morbidity and mortality in this patient group in addition to the underlying congenital heart defects. Nonetheless, this ageing ACHD population is less well-studied than children or young adults with congenital heart disease. The present Special Issue aims to fill this gap by focusing on the ageing ACHD population and the impact of comorbidities on the outcome of these patients.

Prof. Dr. Oktay Tutarel
Guest Editor

Manuscript Submission Information

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Keywords

  • adult congenital heart disease
  • comorbidities
  • acquired diseases
  • ageing

Published Papers (5 papers)

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Research

13 pages, 5760 KiB  
Article
Pulmonary Artery Dilatation Due to Pressure or Volume Overload in Congenital Heart Disease
by Monika Kaldararova, Katarina Bobocka, Andrea Kantorova, Erika Drangova, Jana Polakova Mistinova, Filip Klauco, Tereza Hlavata, Adriana Reptova, Tatiana Valkovicova and Iveta Simkova
J. Clin. Med. 2024, 13(6), 1567; https://doi.org/10.3390/jcm13061567 - 09 Mar 2024
Viewed by 529
Abstract
Background: Pulmonary artery dilatation is described mostly in association with pulmonary hypertension. Patients/Methods: Study analysis: 60 patients with pulmonary arterial hypertension in congenital heart disease (PAH-CHD); 64 with repaired tetralogy of Fallot/pulmonary regurgitation (rTOF/PR); and 80 healthy (NORMAL). Measured were: main pulmonary artery [...] Read more.
Background: Pulmonary artery dilatation is described mostly in association with pulmonary hypertension. Patients/Methods: Study analysis: 60 patients with pulmonary arterial hypertension in congenital heart disease (PAH-CHD); 64 with repaired tetralogy of Fallot/pulmonary regurgitation (rTOF/PR); and 80 healthy (NORMAL). Measured were: main pulmonary artery (MPA) diameter and MPA/ascending aorta (Ao asc) ratio, by echocardiography (ECHO) and computer tomography or magnetic resonance imaging (CT/MRI). Results: In MPA diameter, significant differences between PAH-CHD, rTOF/PR, and NORMAL were found (median): 37 vs. 27 vs. 21 mm (p < 0.0001). In MPA/Ao asc ratio, there was a difference between PAH-CHD and NORMAL (median): 1.3 vs. 0.8 (p < 0.0001), but not between rTOF/PR and NORMAL: 0.74 vs. 0.8 (p = 0.3). Significant MPA dilatation (>40 mm) was present: in PAH-CHD, 35% (ECHO) and 76.9% (CT/MRI) of patients, while in rTOF/PR, 3.1% (ECHO) and 7.8% (CT/MRI). Severe MPA dilatation (>50 mm) occurred only in PAH-CHD: 16.7% (ECHO) and 31.4% (CT/MRI), while not in rTOF/PR. There was a significant correlation between ECHO and CT/MRI measurements, but ECHO was underestimated in all parameters. Conclusions: MPA dilatation due to pressure overload is more frequent and more severe; volume overload also leads to MPA dilatation but is less severe. The MPA/Ao asc ratio is not reliable for MPA dilatation estimation in rTOF/PR. Full article
(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population: Part II)
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16 pages, 573 KiB  
Article
Respiratory Muscle Function and Exercise Performance in Adult Patients with Fontan Circulation
by Raquel Ladrón-Abia, Pilar Cejudo Ramos, Israel Valverde Pérez, Rocío Camacho Fernández De Liger, Amadeo-José Wals-Rodríguez, María José Rodríguez Puras, Begoña Manso García and Pastora Gallego
J. Clin. Med. 2023, 12(14), 4593; https://doi.org/10.3390/jcm12144593 - 10 Jul 2023
Viewed by 762
Abstract
At rest, a good Fontan circulation can provide a normal cardiac output (CO). However, as a consequence of its unique hemodynamic nature, the limitations of the Fontan circuit are exposed during exercise. We aimed to provide a comprehensive assessment of the pathophysiology of [...] Read more.
At rest, a good Fontan circulation can provide a normal cardiac output (CO). However, as a consequence of its unique hemodynamic nature, the limitations of the Fontan circuit are exposed during exercise. We aimed to provide a comprehensive assessment of the pathophysiology of exercise in adult Fontan patients (FPs) and identify factors limiting their functional capacity (FC). In a single-center study conducted in 37 FPs aged ≥16 years and 19 healthy-controls (HCs) who underwent CPET on a cycle ergometer in February and March 2022, the mean peakVO2 was 21 ± 5.4 mL/kg/min, which was 55% of the predicted value. Morphologically, the left single ventricle showed a higher peakVO2% predicted value (57.4 ± 14.4% vs. 43.4 ± 8.1%, p = 0.045). The factors associated with low peakVO2 values were an early flattened or descending O2 pulse at maximal exertion (52 ± 14% vs. 62 ± 12.5, p = 0.04 and 47.6 ± 9% vs. 60 ± 14, p = 0.018, respectively) and chronotropic insufficiency (53 ± 12% vs. 69.8 ± 20%, p = 0.008). The OUES was found to be a useful parameter to assess the FC in FPs in maximal and submaximal exercise testing. A strong positive correlation was observed between the %OUES and peakVO2%predicted (r = 0.726, p > 0.001). The lung function was impaired in the FPs, mostly with a mild restrictive pattern (56.8%). The FPs showed lower inspiratory muscle strength compared to the HCs but it was not statistically associated with either the peakVO2 or VE/VCO2 slope. Regular intense physical activity improves one’s FC. Although FPs have inspiratory muscle weakness, its impact on their FC is unclear. The peakVO2% predicted grew progressively higher as the level of physical activity increased (low level 49.5 ± 14%, moderate level 55 ± 12%, intense level 69 ± 20%). Full article
(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population: Part II)
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10 pages, 1255 KiB  
Article
NT-proBNP Is a Predictor of Mortality in Adults with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease
by Susanne J. Maurer, Veronika Habdank, Jürgen Hörer, Peter Ewert and Oktay Tutarel
J. Clin. Med. 2023, 12(9), 3101; https://doi.org/10.3390/jcm12093101 - 24 Apr 2023
Cited by 1 | Viewed by 992
Abstract
Background: About 5–10% of adults with congenital heart disease (ACHD) will develop pulmonary arterial hypertension (PAH), which is associated with significant mortality. Studies on risk factors for poor outcome in a contemporary cohort of these patients with PAH associated with CHD (PAH-CHD) are [...] Read more.
Background: About 5–10% of adults with congenital heart disease (ACHD) will develop pulmonary arterial hypertension (PAH), which is associated with significant mortality. Studies on risk factors for poor outcome in a contemporary cohort of these patients with PAH associated with CHD (PAH-CHD) are rare. Methods: In this retrospective, single-center study, adult patients with the diagnosis PAH-CHD who had at least one contact as an outpatient or inpatient at the German Heart Centre Munich during the period January 2010–September 2019 were included. Patients with PAH without a CHD were excluded. The primary endpoint was all-cause mortality. Results: Altogether, 158 patients (mean age 39.9 ± 15.4 years, female 64.6%) were included in the study. A pre-tricuspid shunt was present in 17.7%, other shunts in 51.3%, PAH associated with complex CHD in 22.8%, and segmental PAH in 8.2%. An NT-proBNP measurement at baseline was available in 95 patients (60.1%). During a median follow-up of 5.37 years [IQR 1.76–8.63], the primary endpoint occurred in 10 patients (6.7%). On univariate analysis, CRP (log) (HR 3.35, 95% CI (1.07–10.48), p = 0.037), NT-proBNP (log) (HR: 7.10, 95% CI: 1.57–32.23, p = 0.011), and uric acid (HR: 1.37, 95% CI: 1.05–1.79, p = 0.020) were predictors of the primary endpoint. On multivariate analysis, only NT-proBNP (log) (HR: 6.91, 95% CI: 1.36–35.02, p = 0.0196) remained as an independent predictor. Conclusion: NT-proBNP is an independent predictor of all-cause mortality in a contemporary cohort of PAH-CHD patients. The role of CRP and uric acid should be further assessed in future studies. Full article
(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population: Part II)
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14 pages, 735 KiB  
Article
Elective Ascending Aortic Aneurysm Surgery in the Elderly
by Feyza Memis, Carlijn G. E. Thijssen, Arjen L. Gökalp, Maximiliaan L. Notenboom, Frederike Meccanici, Mohammad Mostafa Mokhles, Roland R. J. van Kimmenade, Kevin M. Veen, Guillaume S. C. Geuzebroek, Jelena Sjatskig, Franciscus J. ter Woorst, Jos A. Bekkers, Johanna J. M. Takkenberg and Jolien W. Roos-Hesselink
J. Clin. Med. 2023, 12(5), 2015; https://doi.org/10.3390/jcm12052015 - 03 Mar 2023
Viewed by 1668
Abstract
Background. No clear guidelines exist for performing preventive surgery for ascending aortic (AA) aneurysm in elderly patients. This study aims to provide insights by: (1) evaluating patient and procedural characteristics and (2) comparing early outcomes and long-term mortality after surgery between elderly and [...] Read more.
Background. No clear guidelines exist for performing preventive surgery for ascending aortic (AA) aneurysm in elderly patients. This study aims to provide insights by: (1) evaluating patient and procedural characteristics and (2) comparing early outcomes and long-term mortality after surgery between elderly and non-elderly patients. Methods. A multicenter retrospective observational cohort-study was performed. Data was collected on patients who underwent elective AA surgery in three institutions (2006–2017). Clinical presentation, outcomes, and mortality were compared between elderly (≥70 years) and non-elderly patients. Results. In total, 724 non-elderly and 231 elderly patients were operated upon. Elderly patients had larger aortic diameters (57.0 mm (IQR 53–63) vs. 53.0 mm (IQR 49–58), p < 0.001) and more cardiovascular risk factors at the time of surgery than non-elderly patients. Elderly females had significantly larger aortic diameters than elderly males (59.5 mm (55–65) vs. 56.0 mm (51–60), p < 0.001). Short-term mortality was comparable between elderly and non-elderly patients (3.0% vs. 1.5%, p = 0.16). Five-year survival was 93.9% in non-elderly patients and 81.4% in elderly patients (p < 0.001), which are both lower than that of the age-matched general Dutch population. Conclusion. This study showed that in elderly patients, a higher threshold exists to undergo surgery, especially in elderly females. Despite these differences, short-term outcomes were comparable between ‘relatively healthy’ elderly and non-elderly patients. Full article
(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population: Part II)
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10 pages, 717 KiB  
Article
Transcatheter Interventions for Atrioventricular Dysfunction in Patients with Adult Congenital Heart Disease: An International Case Series
by Nili Schamroth Pravda, Hana Vaknin Assa, Lars Sondergaard, Vilhelmas Bajoras, Horst Sievert, Kerstin Piayda, Amos Levi, Guy Witberg, Yaron Shapira, Ashraf Hamdan, Leor Perl, Shahar Vig, Leonard Blieden, Ran Kornowski, Rafael Hirsch and Pablo Codner
J. Clin. Med. 2023, 12(2), 521; https://doi.org/10.3390/jcm12020521 - 09 Jan 2023
Cited by 1 | Viewed by 1523
Abstract
Introduction: A substantial proportion of patients with adult congenital heart disease (ACHD) suffer from worsening valvular dysfunction in adulthood. Transcatheter valve interventions can offer a therapeutic alternative to surgery for those at high surgical risk. There is emerging but limited data on transcatheter [...] Read more.
Introduction: A substantial proportion of patients with adult congenital heart disease (ACHD) suffer from worsening valvular dysfunction in adulthood. Transcatheter valve interventions can offer a therapeutic alternative to surgery for those at high surgical risk. There is emerging but limited data on transcatheter interventions for atrioventricular (AV) valve dysfunction in patients with ACHD. Methods: We compiled an international collaborative multi-center registry focusing on adult patients with congenital heart disease undergoing transcatheter AV valve interventions (repair or replacement). Included were patients from three international centers who underwent procedures between 2016 and 2022. Demographic, clinical, and procedural data were compiled. Results: Nine patients with ACHD underwent AV valve interventions. The median age was 48 years (IQR (37; 56), 55% women). At baseline, seven patients (78%) were in NYHA functional class III and two (22%) were in NYHA functional class II. The diagnosis of ACHD varied. Three valve interventions were performed on the subpulmonary AV valve and six on the systemic AV valve. The primary valvular pathology was regurgitation (six patients, 78%). Five procedures were valve-in-valve interventions, and four procedures were transcatheter edge-to-edge repair procedures. There were no major complications or peri-procedural complications or peri-procedural mortality. One patient developed a suspected non-obstructive thrombus on the valve that was medically treated. One patient did not improve clinically following the procedure and underwent a heart transplant, one patient died 6 months following the procedure due to a cardiovascular implantable electronic device infection. At one year, six patients were in NYHA functional class I, and one patient was in NYHA functional class III. In conclusion, transcatheter AV heart valve interventions are feasible and safe procedures in carefully selected ACHD patients. These procedures can offer an effective treatment option in these younger patients with high surgical risk. Full article
(This article belongs to the Special Issue Adult Congenital Heart Disease: An Increasing and Ageing Population: Part II)
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