Long Term Follow-Up of Patients with Systemic Right Ventricle and Biventricular Physiology: A Single Centre Experience
Background: A progressively increasing prevalence of congenital heart disease (CHD) in adulthood has been noticed in recent decades; CHD cases with a systemic right ventricle have a poorer outcome. Methods: Seventy-three patients with SRV evaluated in an outpatient clinic between 2014 and 2020 were...
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-05-01
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| Series: | Journal of Cardiovascular Development and Disease |
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| Online Access: | https://www.mdpi.com/2308-3425/10/5/219 |
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| author | Cristina Ciuca Anna Balducci Emanuela Angeli Mariateresa Di Dio Gabriele Egidy Assenza Elisabetta Mariucci Luca Ragni Luigi Lovato Fabio Niro Valentina Gesuete Lucio Careddu Ylenia Bartolacelli Ambra Bulgarelli Andrea Donti Gaetano Domenico Gargiulo |
| author_facet | Cristina Ciuca Anna Balducci Emanuela Angeli Mariateresa Di Dio Gabriele Egidy Assenza Elisabetta Mariucci Luca Ragni Luigi Lovato Fabio Niro Valentina Gesuete Lucio Careddu Ylenia Bartolacelli Ambra Bulgarelli Andrea Donti Gaetano Domenico Gargiulo |
| author_sort | Cristina Ciuca |
| collection | DOAJ |
| description | Background: A progressively increasing prevalence of congenital heart disease (CHD) in adulthood has been noticed in recent decades; CHD cases with a systemic right ventricle have a poorer outcome. Methods: Seventy-three patients with SRV evaluated in an outpatient clinic between 2014 and 2020 were enrolled in this study. Thirty-four patients had a transposition of the great arteries treated with an atrial switch operation; 39 patients had a congenitally corrected transposition of the great arteries (ccTGA). Results: Mean age at the first evaluation was 29.6 ± 14.2 years; 48% of the patients were female. The NYHA class at the visit was III or IV in 14% of the cases. Thirteen patients had at least one previous pregnancy. In 25% of the cases, complications occurred during pregnancy. Survival free from adverse events was 98.6% at one year and 90% at 6-year follow-up without any difference between the two groups. Two patients died and one received heart transplantation during follow-up. The most common adverse event during follow-up was the presence of arrhythmia requiring hospitalization (27.1%), followed by heart failure (12.3%). The presence of LGE together with lower exercise capacity, higher NYHA class and more dilated and/or hypokinetic RV predicted a poorer outcome. Quality of life was similar to the QoL of the Italian population. Conclusions: Long-term follow-up of patients with a systemic right ventricle is characterized by a high incidence of clinical events, prevalently arrhythmias and heart failure, which cause most of the unscheduled hospitalizations. |
| first_indexed | 2024-03-11T03:37:22Z |
| format | Article |
| id | doaj.art-511751638c4a45068a73e0964df12f39 |
| institution | Directory Open Access Journal |
| issn | 2308-3425 |
| language | English |
| last_indexed | 2024-03-11T03:37:22Z |
| publishDate | 2023-05-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Cardiovascular Development and Disease |
| spelling | doaj.art-511751638c4a45068a73e0964df12f392023-11-18T01:51:20ZengMDPI AGJournal of Cardiovascular Development and Disease2308-34252023-05-0110521910.3390/jcdd10050219Long Term Follow-Up of Patients with Systemic Right Ventricle and Biventricular Physiology: A Single Centre ExperienceCristina Ciuca0Anna Balducci1Emanuela Angeli2Mariateresa Di Dio3Gabriele Egidy Assenza4Elisabetta Mariucci5Luca Ragni6Luigi Lovato7Fabio Niro8Valentina Gesuete9Lucio Careddu10Ylenia Bartolacelli11Ambra Bulgarelli12Andrea Donti13Gaetano Domenico Gargiulo14Pediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyPediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyPediatric Cardiac Surgery and Adult Congenital Heart Disease, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyPediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyPediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyPediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyPediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyRadiology Unit, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyRadiology Unit, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyPediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyPediatric Cardiac Surgery and Adult Congenital Heart Disease, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyPediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyPediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyPediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyPediatric Cardiac Surgery and Adult Congenital Heart Disease, Department of Cardio-Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyBackground: A progressively increasing prevalence of congenital heart disease (CHD) in adulthood has been noticed in recent decades; CHD cases with a systemic right ventricle have a poorer outcome. Methods: Seventy-three patients with SRV evaluated in an outpatient clinic between 2014 and 2020 were enrolled in this study. Thirty-four patients had a transposition of the great arteries treated with an atrial switch operation; 39 patients had a congenitally corrected transposition of the great arteries (ccTGA). Results: Mean age at the first evaluation was 29.6 ± 14.2 years; 48% of the patients were female. The NYHA class at the visit was III or IV in 14% of the cases. Thirteen patients had at least one previous pregnancy. In 25% of the cases, complications occurred during pregnancy. Survival free from adverse events was 98.6% at one year and 90% at 6-year follow-up without any difference between the two groups. Two patients died and one received heart transplantation during follow-up. The most common adverse event during follow-up was the presence of arrhythmia requiring hospitalization (27.1%), followed by heart failure (12.3%). The presence of LGE together with lower exercise capacity, higher NYHA class and more dilated and/or hypokinetic RV predicted a poorer outcome. Quality of life was similar to the QoL of the Italian population. Conclusions: Long-term follow-up of patients with a systemic right ventricle is characterized by a high incidence of clinical events, prevalently arrhythmias and heart failure, which cause most of the unscheduled hospitalizations.https://www.mdpi.com/2308-3425/10/5/219systemic right ventricleadults with congenital heart diseaseatrial switch operationcongenitally corrected transposition of the great arteriesMustard operationSenning operation |
| spellingShingle | Cristina Ciuca Anna Balducci Emanuela Angeli Mariateresa Di Dio Gabriele Egidy Assenza Elisabetta Mariucci Luca Ragni Luigi Lovato Fabio Niro Valentina Gesuete Lucio Careddu Ylenia Bartolacelli Ambra Bulgarelli Andrea Donti Gaetano Domenico Gargiulo Long Term Follow-Up of Patients with Systemic Right Ventricle and Biventricular Physiology: A Single Centre Experience Journal of Cardiovascular Development and Disease systemic right ventricle adults with congenital heart disease atrial switch operation congenitally corrected transposition of the great arteries Mustard operation Senning operation |
| title | Long Term Follow-Up of Patients with Systemic Right Ventricle and Biventricular Physiology: A Single Centre Experience |
| title_full | Long Term Follow-Up of Patients with Systemic Right Ventricle and Biventricular Physiology: A Single Centre Experience |
| title_fullStr | Long Term Follow-Up of Patients with Systemic Right Ventricle and Biventricular Physiology: A Single Centre Experience |
| title_full_unstemmed | Long Term Follow-Up of Patients with Systemic Right Ventricle and Biventricular Physiology: A Single Centre Experience |
| title_short | Long Term Follow-Up of Patients with Systemic Right Ventricle and Biventricular Physiology: A Single Centre Experience |
| title_sort | long term follow up of patients with systemic right ventricle and biventricular physiology a single centre experience |
| topic | systemic right ventricle adults with congenital heart disease atrial switch operation congenitally corrected transposition of the great arteries Mustard operation Senning operation |
| url | https://www.mdpi.com/2308-3425/10/5/219 |
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