In a recent study a team of experts reported that the treatment of some patients newborns with disorder of single ventricle with digoxin during a critical phase it can improve their survival and lead to better outcomes.
The results of research were published on Journal of the American Heart Association.
Digoxin: new hope for newborns with heart problems
A strong and rhythmic pulse is a lifelong companion of those with a healthy heart. But for about 1% of children born each year in the United Statesdevelopmental heart problems affect regular heartbeat.
A group of severe developmental anomalies determines the physiology of a single ventricle, or a single functioning pumping chamber in the heart. These are extremely rare, affecting approximately 5 in 100,000 newborns, but pose a serious danger to newborns when they enter the world.
Reshma Reddy, MD, a pediatric cardiac critical care physician at the Medical University of South Carolina, said that children born with single ventricle physiology are likely the highest-risk patient group treated by pediatric cardiologists.
Single ventricle disorders occur when there is a developmental problem that leads to a smaller, undeveloped lower heart chamber or a missing valve and include, among others, hypoplastic left heart syndrome, tricuspid atresia and the double-inlet left ventricle.
When a heart must struggle to have only one functional pumping chamber instead of the usual two, it must work harder and less efficiently to send oxygenated blood to the body and recover deoxygenated blood from the lungs. The body may not receive an adequate supply of oxygen, and a whole host of medical problems can overwhelm the child.
To survive being born with single ventricle physiology, babies must undergo a series of surgeries that are not curative but can lead to more stable circulation that supports heart function. They often have their first surgery between 7 and 14 days of age, then a second procedure is performed when they are older, usually around 3 to 4 months.
The time between surgeries is called the interstage period, and during this stage patients are seriously ill and at high risk. But in their study, Reddy and his team found that digoxin was effective in reducing this risk for patients undergoing an approach called a hybrid procedure.
The hybrid procedure is an initial palliative surgery that can be used as an alternative to the classic Norwood procedure, a highly complex surgery that requires a long period of support with a heart-lung bypass machine and leaves patients extremely vulnerable. With the hybrid procedure, children undergo both catheterization and surgery, but usually do not need to spend time on the bypass machine. Removing this variable reduces stress on this already vulnerable population.
In the study, researchers examined data collected from 2008 to 2021 and reported to the National Pediatric Cardiology Quality Improvement Collaborative, a network of more than 60 pediatric cardiology treatment centers that care for more than 1,400 patients with single ventricle disease.
The study looked at 259 children who underwent the hybrid procedure at 45 different treatment centers. Half of these children were treated with digoxin between phase 1 and 2 surgeries, and the other half did not receive digoxin.
The results showed that patients taking digoxin had significantly lower mortality than those not receiving it and were significantly less likely to need a transplant during the interstage period. The same results were previously reported for the Norwood procedure, and the current study confirms the parallel for patients undergoing the hybrid procedure.
Because there is much variation between treatment centers – many centers recommend the hybrid procedure for patients with certain risk factors and use the Norwood method for other patients – it was important to confirm that the use of digoxin is likely beneficial for these patients as long as they tolerate the drug is good.
Reddy estimates that approximately 50% of centers prescribe digoxin for this purpose and hopes that this published confirmation will encourage a change in practice in centers that have been waiting for more certainty in this population.
Researchers and healthcare professionals are always looking for innovative strategies to help this patient population,” he said. “And anything we can do to increase the survival of our sickest and most vulnerable patients in their most vulnerable time of life makes a huge difference.”
Single ventricle disorder in adults: heart transplant is safer
UCLA-led research finds that among adult congenital heart disease (CHD) transplant patients, single ventricle physiology correlates with greater short-term mortality.
Conditional 10-year survival was similar for patients with biventricular coronary artery disease and for most patients with single ventricle coronary artery disease, and significantly better for patients with biventricular coronary artery disease than for disease-free heart transplant patients coronary.
Coronary heart disease is a heterogeneous group of structural abnormalities that can be considered
as a spectrum ranging from very severe lesions that require multiple surgeries to others that are not so high risk. Historically, single-ventricle CHD subtypes were all considered higher risk than their biventricular counterparts, which would lead some transplant centers to be hesitant to perform a heart transplant in these patients.
The researchers analyzed National (National) Network datasets for hospital samples and procurement and transplantation for the period 2005-2020. The study is published in the Journal of the American College of Cardiology.
Of 382 adult heart transplant patients with congenital heart disease (CHD), 185 (48%) had single ventricle physiology. Compared with patients with biventricular coronary artery disease, those with single ventricle coronary artery disease showed significantly reduced survival at one year (80% versus 91%) and at 10 years (54% versus 71%).
Among patients who survived the first year post-transplant, patients with biventricular coronary artery disease had similar 10-year survival to that of single-ventricle patients, except for those with hypoplastic left heart syndrome (79% versus 71%). . Furthermore, biventricular CHD transplant patients showed significantly better 10-year conditional survival compared to their non-CHD counterparts (79% versus 68%).
Limitations of the study include the lack of granularity in the datasets that would have allowed analysis of all aspects involved in transplantation. Additionally, there were no common patient identifiers between the two datasets, so the researchers had to rely on a new probability linking methodology to match the records.
The findings have significant implications for patient selection and listing strategies, alleviate concerns regarding heart transplantation in adults with coronary heart disease, and destigmatize most subtypes of single ventricle coronary heart disease.
“To date, there have been no large-scale national studies examining survival outcomes in single ventricle patients, despite the widespread availability of transplant data dating back to 1987,” said lead author Dr. Syed Shahyan Bakhtiyar, visiting researcher at the University of Washington. UCLA Division of Cardiac Surgery and general surgery resident at the University of Colorado.
“As a result, not only have patients and their families lacked essential prognostic information, but surgeons and transplant teams have also been limited in their ability to make fully informed decisions about these patients' practice listing and transplantation. Our findings not only alleviate concerns associated with heart transplant problems in adult patients with coronary heart disease as a whole, but also work to destigmatize most subtypes of single ventricle coronary heart disease.”
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