ABSTRACT
BACKGROUND: Pediatric heart transplant candidates on the waitlist have the highest mortality rate among all solid organ transplants. A risk score incorporating a candidate's individual risk factors may better predict mortality on the waitlist and optimize organ allocation to the sickest of those awaiting transplant. METHODS: Using the United Network for Organ Sharing (UNOS) database, we evaluated a total of 5542 patients aged 0-18 years old on the waitlist for a single, first time, heart transplant from January 2010 to June 2019. We performed a univariate analysis on two-thirds (N = 3705) of these patients to derive the factors most associated with waitlist mortality or delisting secondary to deterioration within 1 year. Those with a p <0.2 underwent a multivariate analysis and the resulting factors were used to build a prediction model using the Fine-Grey model analysis. This predictive scoring model was then validated on the remaining one-third of the patients (N = 1852). RESULTS: The Pediatric Risk to OHT (PRO) scoring model utilizes the following unique patient variables: blood type, diagnosis of congenital heart disease, weight, presence of ventilator support, presence of inotropic support, extracorporeal membrane oxygenation (ecmo) status, creatinine level, and region. A higher score indicates an increased risk of mortality. The PRO score had a predictive strength of 0.762 as measured by area under the ROC curve at 1 year. CONCLUSION: The PRO score is an improved predictive model with the potential to better assess mortality for patients awaiting heart transplant.
Subject(s)
Heart Defects, Congenital , Heart Transplantation , Liver Transplantation , Tissue and Organ Procurement , Humans , Child , Infant, Newborn , Infant , Child, Preschool , Adolescent , Risk Factors , Waiting Lists , Retrospective StudiesABSTRACT
Pulmonary atresia with intact ventricular septum is a complex cyanotic congenital heart lesion with the potential for myocardial ischaemia due to the presence of coronary artery anomalies. We present a case of a two-day-old baby with postnatal diagnosis of pulmonary atresia with intact ventricular septum in whom non-invasive ferumoxytol-enhanced 4D cardiac magnetic resonance angiography was used for the assessment of coronary artery anatomy.
ABSTRACT
A major aortopulmonary collateral artery is a rare and easily missed diagnosis that is usually associated with Tetralogy of Fallot or pulmonary atresia. We present two cases of major aortopulmonary collaterals associated with trisomy 21 and atrioventricular septal defect with balanced ventricles in which the diagnosis went undetected until after initial cardiac repair.
Subject(s)
Down Syndrome , Heart Defects, Congenital , Heart Septal Defects , Pulmonary Atresia , Tetralogy of Fallot , Humans , Infant , Down Syndrome/complications , Down Syndrome/diagnosis , Pulmonary Artery/abnormalities , Heart Septal Defects/complications , Heart Septal Defects/diagnosis , Heart Septal Defects/surgery , Pulmonary Atresia/complications , Pulmonary Atresia/diagnosis , Pulmonary Atresia/surgery , Heart Defects, Congenital/complications , Collateral Circulation , Tetralogy of Fallot/complications , Tetralogy of Fallot/diagnosisABSTRACT
We present a pilot case using an innovative fusion of echocardiogram and MRI achieved with a MATLAB-based imaging programme to explore the feasibility of this imaging strategy in the functional and anatomic assessment of a patient with repaired tetralogy of Fallot requiring pulmonary valve intervention. Echocardiogram and MRI neutralises the disadvantages and limitations of each individual imaging modality and yields important anatomic and haemodynamic information crucial to the treatment decision-making process. Future image fusion strategies can apply to three-dimensional images and image-directed therapy for CHD.
