The current and future role of imaging of thoracic aortic disease: a North American society for cardiovascular imaging commentary on the 2022 AHA/ACC guidelines for the diagnosis and management of aortic disease.

The 2022 AHA/ACC Guidelines for the Diagnosis and Management of Aortic Disease introduced important updates for managing thoracic aorta aortic disease (TAD). In particular, the Guidelines underscore multimodality imaging's role in diagnosis, risk assessment, and monitoring of patients with TAD. This commentary aims to distill key imaging aspects from the Guidelines to provide a concise reference for the cardiovascular imaging community. Primary areas of focus include: (1) The importance of imagers in the multidisciplinary TAD care team, (2) Appropriate imaging techniques along with their strengths and weaknesses, (3) Aortic measurement methods and how aortic size and growth should contribute to TAD risk assessment, (4) Imaging evaluation of acute aortic syndrome. We have also highlighted several areas of ongoing uncertainty and confusion, specifically related to aortic measurement techniques and descriptive terminology. Finally, a perspective on the future of TAD imaging is discussed with a focus on advanced imaging tools and techniques as well as the potential role of artificial intelligence.

Advances in the Prenatal Management of Fetal Cardiac Disease.

Advances in the field have improved the prenatal management of cardiovascular diseases over the past few decades; however, there remains considerable challenges in the approach towards patient selection as well as the applicability of available therapies. This review aims to discuss the current knowledge, outcomes and challenges for prenatal intervention for congenital heart disease.

Deep Learning for Improved Risk Prediction in Surgical Outcomes.

The Norwood surgical procedure restores functional systemic circulation in neonatal patients with single ventricle congenital heart defects, but this complex procedure carries a high mortality rate. In this study we address the need to provide an accurate patient specific risk prediction for one-year postoperative mortality or cardiac transplantation and prolonged length of hospital stay with the purpose of assisting clinicians and patients' families in the preoperative decision making process. Currently available risk prediction models either do not provide patient specific risk factors or only predict in-hospital mortality rates. We apply machine learning models to predict and calculate individual patient risk for mortality and prolonged length of stay using the Pediatric Heart Network Single Ventricle Reconstruction trial dataset. We applied a Markov Chain Monte-Carlo simulation method to impute missing data and then fed the selected variables to multiple machine learning models. The individual risk of mortality or cardiac transplantation calculation produced by our deep neural network model demonstrated 89 ± 4% accuracy and 0.95 ± 0.02 area under the receiver operating characteristic curve (AUROC). The C-statistics results for prediction of prolonged length of stay were 85 ± 3% accuracy and AUROC 0.94 ± 0.04. These predictive models and calculator may help to inform clinical and organizational decision making.

Prediction of One-Year Transplant-Free Survival after Norwood Procedure Based on the Pre-Operative Data.

This paper discusses computational modeling of predictive risk factors for neonates undergoing a Norwood surgical procedure, a multi-stage cardiac procedure that restores functional systemic circulation in patients such as neonates with Hypoplastic Left Heart Syndrome (HLHS). In this model, we apply machine learning based binary classication to 549 cases reported by the Pediatric Heart Networks Single Ventricle Reconstruction Trial. We use neural networks classier to predict risk factors for individual patients undergoing a Norwood procedure for the repair of HLHS. Results indicate that independent risk can be calculated with 85% accuracy and 0.94 area under the receiver operating characteristics curve. This model may help physicians provide counseling for families and medically optimize patients prior to surgery by modifying individual risk factors.

Trans-aortic His bundle ablation with permanent ventricular pacing via the coronary sinus in L-transposition of great arteries with classic Fontan procedure.

We report findings in a patient with 1-transposition of great arteries with single ventricle and classic Fontan procedure who required both His bundle ablation for palliation of refractory atrial tachycardia and placement of a transvenous dual-chamber atrio-ventricular permanent pacemaker for hemodynamic support. A method for retrograde trans-aortic His bundle ablation in this congenital anomaly and subsequent placement of permanent dual chamber pacemaker using the right atrium and coronary sinus as an access to the ventricle is described.

Recent advances in pediatric cardiology--electrophysiology, transcatheter and surgical advances.

Whereas the medical advances were reviewed in the previous paper, electrophysiological, transcatheter and surgical advances will be the focus in this review. Greater understanding of the arrhythmias, development of non-pharmacological treatment, namely catheter ablation and internal cardioverter-defibrillator (ICD) and miniaturization pacemakers and IDs have occurred in the last decade so that the methods could be applied to smaller and more complex patient population. Surgery has been the traditional treatment option for palliation and correction of congenital and acquired heart defects in infants and children. During the last one to one and one-half decades, a remarkable number of transcatheter methods were developed and refined. These developments during the last decade were reviewed and include long-term results of balloon dilatation procedures, transcatheter closure of atrial septal defects, patent ductus arteriosus, and ventricular septal defect, percutaneous valve replacement, intravascular stents to manage vascular obstructive lesions that can not be satisfactorily balloon-dilated, catheter completion of Fontan procedure, myocardial reduction in hypertrophic cardiomyopathy and other miscellaneous procedures. Recent advances in the transcatheter modes of therapy have added a new dimension to the management of neonates, infants and children with heart disease. They should now be added to the armamentarium available to the Pediatrician and Pediatric Cardiologist in the management of cardiac problems in the pediatric patient. Surgical methods and concepts have been greatly refined such that surgery can be undertaken even in the sickest and most complex patient. The majority of congenital heart defects can be corrected by open heart surgery; some require prior palliation and others can be operated without prior palliation. Recent advances in various defects were reviewed.

What is new in pediatric cardiology.

Enormous advances in the diagnosis and management of heart disease in pediatric patient have taken place during the last-four decades. In this review the authors will concentrate on the developments within the last five to ten years. It will deal with what may be called medical advances. Recent advances in molecular genetics and defining the familial patterns have led to finding that certain genetic and molecular factors are linked to congenital heart disease and arrythmia, thus providing opportunity for improved genetic counseling and future gene therapy. Medical treatment of congenital heart disease targets not only the augmentation of ventricular contractility (positive inotropy) but also addresses the neuro-humoral derangement associated with it. The ultrasound technology for the evaluation of the heart has come a long way from the early A-mode and M-mode capabilities to color Doppler, 2-dimentional and 3-dimentional capabilities.

Neonatal coarctation repair with a long isthmus: the isthmus patch.

This case report illustrates a ductal-dependent coarctation repair in a neonate whose long isthmus was believed to make conventional end-to-end repair problematic. The isthmus and left subclavian artery were isolated and augmented with a homograft while flow to the descending aorta was preserved through the ductus. After patch augmentation of the isthmus, ductal tissue was resected and an end-to-end anastomosis was performed using the length of the augmented isthmus. Angiography 18 months later showed excellent growth of the arch despite homograft tissue comprising the majority of the isthmus at the time of repair.

Presence of prolonged dispersion of qt intervals in late survivors of childhood anthracycline therapy.

To determine the incidence of abnormal dispersion of QT intervals on an ECG the authors measured these intervals in long-term survivors after anthracycline treatment for childhood malignancies and compared the incidence to the traditional markers, prolonged QT and QTc intervals. Prolonged QT dispersion (QTd) and corrected QT dispersion (QTcd) have been associated with serious arrhythmias and sudden death in many forms of heart disease. The above intervals and shortening fraction (SF) on echocardiogram were determined in 64 late survivors who were 4-22 years post anthracycline therapy. They were divided into 2 groups: group I, 39 patients with normal function with SF >or= 29%, and group II, 15 patients with SF < 29%. An additional 10 patients had died and had SF < 29% at the time of death. They were divided into 2 groups: group III, 5 patients who died with heart failure after a mean of 12 years from of therapy, and group IV, 5 patients who had a sudden cardiac death after a mean of 9 years from end of therapy. In group I patients, the incidence of prolonged QT and QTc intervals was < 6%, whereas that of QTd and QTcd was > 38%. In group II patients, the presence of prolonged QT and QTc intervals was < 16%, whereas that of QTd and QTcd was > 40%. The QTc (p =.01), QTd (p =.02), and QTcd (p =.01) were significantly higher and SF (<.01) significantly lower in group IV compared to those alive (group I and II patients) with no significant difference in age, number of years of follow-up, precordial radiation dose, or anthracycline dose between the two. The combination of QTcd > 110 ms and diminished SF < 29% was found to be associated with sudden death. In summary, the long-term survivors of anthracycline therapy had a high incidence (>38%) of abnormally prolonged QT dispersion intervals, QTd and QTcd, even in those with normal QT and QTc on an ECG and normal shortening fraction on an echocardiogram.

Late cardiotoxicity after bolus versus infusion anthracycline therapy for childhood cancers.

OBJECTIVE: To compare the long-term myocardial function of patients who had been treated with infusion anthracycline therapy (administered continuously over >24 hr, IG) versus bolus therapy (administered over <30 min, BG). METHODS: We selected 25 patients (BG) and 19 patients (IG) who had three or more years of disease free survival. We evaluated the echocardiograms for left ventricular shortening fraction (SF) obtained at baseline, within one year after the end of therapy (early follow-up), and on long-term follow-up. RESULTS: The mean anthracycline dose in the BG was 385 mg/m(2) and in the IG was 345 mg/m(2) (P = 0.07). During therapy, one patient in BG and none in IG developed diminished SF. During early follow-up, five of the 22 patients in BG and one of the 17 patients in IG developed diminished SF (P = 0.2). Of these five patients with diminished SF, three patients in BG and none in IG continued to have abnormally low SF long-term. At mean of 7 years, five of the 25 patients in BG and two of the 19 in IG had diminished SF on (P = 0.7). Late left ventricular dilatation was seen in 8% in BG and 5% in IG (P = 1.0). CONCLUSIONS: At mean of 7 years after end of therapy, diminished cardiac function was seen in 20% of the patient who had received bolus anthracycline compared to 11% of patients who had received it via infusion. This difference did not prove to be statistically significant.

Elevated IgA and IgM anticardiolipin antibodies in acute Kawasaki disease.

There is marked activation of the endothelium and immune system in Kawasaki disease. Anticardiolipin antibodies (aCL) can cause activation of the endothelium. We measured aCL levels in acute Kawasaki disease patients and compared them to other febrile patients to see whether their aCL responses were different. Twenty-one patients with acute Kawasaki disease and 16 patients with an acute febrile illness were recruited. The aCL levels were measured in the sera of febrile patients and in Kawasaki disease patients prior to immunoglobulin therapy. There was no significant difference between the IgG aCL levels (p = 0.87) between the Kawasaki disease and febrile patients. However, the IgM (p = 0.01) and IgA (p = 0.03) aCL were significantly higher in patients with acute Kawasaki disease than in febrile children. Elevation of IgA aCL has been reported in association with other vasculitides and IgA-secreting plasma cells have been demonstrated in the vascular tissue in Kawasaki disease.