The hypoplastic left heart syndrome (HLHS) is arguably one of the most complex forms of congenital heart disease. It is probably best thought of as a wide spectrum of cardiac malformations characterized by varying degrees of underdevelopment of the mitral valve, left ventricle, aortic valve, ascending aorta and the aortic arch. It is universally fatal without intervention in the neonatal period and perhaps no other congenital cardiovascular condition poses as formidable a challenge when it comes to management. There is no cure and the most common strategy for survival involves a series of palliative surgical interventions resulting in a single right ventricle providing systemic output through a reconstructed aortic outflow, while a total cavopulmonary connection provides passive pulmonary perfusion.
Since the first successful Norwood procedure in 1981, significant strides have been made in improving diagnosis and preoperative care, refining surgical techniques, and advancing postoperative management, with outcome data now available for nearly four decades. These show that neonatal survival has improved significantly, reaching close to 90% in more experienced centers, and that survival after the Fontan procedure, including reasonable quality of life, is possible for up to 20 years in as many as 80% of patients. Still, at least a third of children with HLHS don’t survive to the second decade of life, while the prospects of circulatory failure, Fontan associated liver disease, plastic bronchitis, protein-losing enteropathy and heart transplant await the survivors. Yet, we continue undeterred in our pursuit to improve the quality and duration of life in these patients, and we have witnessed impressive new advances in medical and surgical management since the new millennium. Prenatal diagnosis is allowing thorough prenatal counselling and fetal interventions, “hybrid” procedures and staged left ventricular recruitment strategies are offering new and promising treatment options, while new and improved imaging techniques stand to meliorate the diagnosis and management of long-term complications.
This Research Topic will cover the current and evolving management strategies for the spectrum of the HLHS, addressing the following specific themes:
1. Prenatal diagnosis and counselling
2. Fetal interventions and prospects of maternal hyperoxygenation
3. Stage I palliation strategies for HLHS: Norwood with BT shunt vs. Sano shunt vs. “Hybrid” approach
4. Post-operative management after stage I: considerations and pitfalls
5. Interstage mortality and surveillance
6. The borderline left heart: staged LV recruitment strategies
7. Outcomes and complications after biventricular conversion
8. Echocardiographic evaluation of systolic and diastolic function at different stages of palliation
9. Pre-Fontan evaluation: cath, MRI or both?
10. Comprehensive MRI evaluation of the Fontan circulation
11. Management of heart failure after Fontan surgery
12. Interventional strategies in the failing Fontan circulation
13. Arrhythmias in the failing Fontan: diagnosis and treatment strategies
14. Thrombosis and anticoagulation at different stages of palliation
15. Fontan associated liver disease: surveillance and management
16. Diagnosis and management of lymphatic insufficiency
17. Late outcome and prognosis of HLHS
18. Heart transplantation and prospects of neonatal xenotransplantation
19. Neurodevelopmental outcomes
The types of manuscripts sought will be review articles.
The hypoplastic left heart syndrome (HLHS) is arguably one of the most complex forms of congenital heart disease. It is probably best thought of as a wide spectrum of cardiac malformations characterized by varying degrees of underdevelopment of the mitral valve, left ventricle, aortic valve, ascending aorta and the aortic arch. It is universally fatal without intervention in the neonatal period and perhaps no other congenital cardiovascular condition poses as formidable a challenge when it comes to management. There is no cure and the most common strategy for survival involves a series of palliative surgical interventions resulting in a single right ventricle providing systemic output through a reconstructed aortic outflow, while a total cavopulmonary connection provides passive pulmonary perfusion.
Since the first successful Norwood procedure in 1981, significant strides have been made in improving diagnosis and preoperative care, refining surgical techniques, and advancing postoperative management, with outcome data now available for nearly four decades. These show that neonatal survival has improved significantly, reaching close to 90% in more experienced centers, and that survival after the Fontan procedure, including reasonable quality of life, is possible for up to 20 years in as many as 80% of patients. Still, at least a third of children with HLHS don’t survive to the second decade of life, while the prospects of circulatory failure, Fontan associated liver disease, plastic bronchitis, protein-losing enteropathy and heart transplant await the survivors. Yet, we continue undeterred in our pursuit to improve the quality and duration of life in these patients, and we have witnessed impressive new advances in medical and surgical management since the new millennium. Prenatal diagnosis is allowing thorough prenatal counselling and fetal interventions, “hybrid” procedures and staged left ventricular recruitment strategies are offering new and promising treatment options, while new and improved imaging techniques stand to meliorate the diagnosis and management of long-term complications.
This Research Topic will cover the current and evolving management strategies for the spectrum of the HLHS, addressing the following specific themes:
1. Prenatal diagnosis and counselling
2. Fetal interventions and prospects of maternal hyperoxygenation
3. Stage I palliation strategies for HLHS: Norwood with BT shunt vs. Sano shunt vs. “Hybrid” approach
4. Post-operative management after stage I: considerations and pitfalls
5. Interstage mortality and surveillance
6. The borderline left heart: staged LV recruitment strategies
7. Outcomes and complications after biventricular conversion
8. Echocardiographic evaluation of systolic and diastolic function at different stages of palliation
9. Pre-Fontan evaluation: cath, MRI or both?
10. Comprehensive MRI evaluation of the Fontan circulation
11. Management of heart failure after Fontan surgery
12. Interventional strategies in the failing Fontan circulation
13. Arrhythmias in the failing Fontan: diagnosis and treatment strategies
14. Thrombosis and anticoagulation at different stages of palliation
15. Fontan associated liver disease: surveillance and management
16. Diagnosis and management of lymphatic insufficiency
17. Late outcome and prognosis of HLHS
18. Heart transplantation and prospects of neonatal xenotransplantation
19. Neurodevelopmental outcomes
The types of manuscripts sought will be review articles.
