Impaired Myocardial Work in Children with Hypertrophic Cardiomyopathy and Left Ventricular Fibrosis on Cardiac Magnetic Resonance Imaging.

Echocardiography is pivotal for diagnosis and monitoring of hypertrophic cardiomyopathy (HCM) and can evaluate myocardial function using myocardial work (MW) calculations. Echocardiography is often supplemented by cardiovascular magnetic resonance (CMR) imaging, which can detect myocardial fibrosis using late gadolinium enhancement (LGE). We sought to study the relationship between baseline LGE and MW at baseline and during follow-up in pediatric HCM patients. During the study period (2008-2023), 75 patients were followed up for HCM. In 14 patients (age 14.2 ± 2.8 years, 50.0% male, 6.4 ± 2.9 years follow-up), both LGE-CMR and echocardiography were performed. Global work index (GWI), global constructive work (GCW), global wasted work, and global work efficiency (GWE) were measured, and myocardial fibrosis was estimated by qualitative assessment of LGE. Patients with LGE (n = 7) exhibited significantly impaired baseline MW, including GWI (mean difference, MD - 487.4 mmHg %, 95% CI [- 866.8 mmHg % to - 108.3 mmHg %], p = 0.027), GCW (MD - 536.8 mmHg %, 95% CI [- 929.8 mmHg % to - 144.4 mmHg %], p = 0.020), and GWE (MD - 4.4%, 95% CI [- 8.1% to - 0.7%], p = 0.039). Regional analysis revealed impaired MW indices in segments with LGE, notably basal and mid septal segments. GWI demonstrated high diagnostic performance for LGE presence (sensitivity 93%, specificity 88%, and area under receiver operating characteristic curve 0.85). Baseline LGE presence had no significant impact on MW deterioration during follow-up. MW is significantly impaired in HCM patients with myocardial fibrosis, highlighting potential utility of echocardiography-derived MW analysis as a valuable tool.

Time course of hypertension and myocardial dysfunction following anthracycline chemotherapy in pediatric patients.

Background: Anthracyclines are associated with cardiac dysfunction. Little is known about the interplay of pre-existing hypertension and treatment response. We aimed to investigate the relationship between hypertension and the development of cancer therapy-related cardiac dysfunction (CTRCD) in pediatric patients treated with anthracycline chemotherapy. Methods: Pediatric patients with cancer who received anthracycline chemotherapy from 2013 to 2021 were retrospectively included. Serial cardiac assessments were conducted during and after chemotherapy. The primary outcome was the development of CTRCD, classified as mild, moderate, or severe according to contemporary definitions. Results: Among 190 patients undergoing anthracycline chemotherapy, 34 patients (17.9 %) had hypertension (24 patients Stage 1, and 10 patients Stage 2) at baseline evaluation. Patients underwent chemotherapy for a median of 234.4 days (interquartile range 127.8-690.3 days) and were subsequently followed up. Hypertension was frequent during follow-up 31.3 % (0-3 months), 15.8 % (3-6 months), 21.9 % (0.5-1 years), 24.7 % (1-2 years), 31.1 % (2-4 years) and 35.8 % (beyond 4 years) (P for trend < 0.001). Freedom from mild CTRCD at 5 years was 45.0 %, freedom from moderate CTRCD was 87.8 % at 5 years. Baseline hypertension did not increase the risk of mild (HR 0.77, 95 % CI: 0.41-1.42, P = 0.385) or moderate CTRCD (HR 0.62, 95 % CI: 0.14-2.72, P = 0.504). Patients with baseline hypertension showed different global longitudinal strain (P < 0.001) and LVEF (P < 0.001) patterns during follow-up. Conclusions: Pediatric patients often develop CTRCD post-anthracycline chemotherapy. Those with pre-existing hypertension show a unique treatment response, despite no increased CTRCD risk, warranting further investigation.

A Valved Expanded Polytetrafluoroethylene Conduit can Acquire Endothelium After Implantation.

We demonstrate histopathology, neointimal proliferation, and neo-endothelialization in an explanted valved expanded polytetrafluoroethylene (ePTFE) conduit 40 months postimplantation that was void of calcification and inflammation, confirmed by CD-31 positivity on immunohistochemistry. Grossly, there was no distortion with preserved leaflets and lack of calcification. Good biocompatibility, nonreactivity, and low antigenicity, combined with neointimal and endothelial layer generation within the conduit might explain the low infection rates and minimal thrombogenicity. These findings support the use of handmade, valved ePTFE conduits as an economically viable option as a right ventricle to pulmonary artery conduit.

Harlequin effect and central veno-arterial extracorporeal life support.

"Harlequin effect" may be observed in the watershed region of a patient with pulmonary dysfunction, receiving peripheral veno-arterial extracorporeal membrane oxygenation via the femoral vessels. In such cases, retrograde oxygenated blood from the peripheral inflow cannula converges with the antegrade deoxygenated blood ejected from the left ventricle. This occurs when the left ventricle is ejecting significantly but the recovery of pulmonary function lags behind. Herein, we describe the occurrence of "Harlequin effect" in the setting of central veno-arterial extracorporeal membrane oxygenation that ensues due to the persistence of right ventricular dysfunction in the presence of an interatrial communication. This results in right to left shunting at the atrial level while weaning the patient from extracorporeal life support.

Preparatory stage of interventional extracardiac Fontan.

The extracardiac Fontan can be completed as an interventional procedure when the preparatory stage for Fontan completion is performed at the time of the bidirectional Glenn operation. In this video tutorial, we present the technique for the preparatory stage of an interventional extracardiac Fontan. The interventional aspect offers the advantage of avoiding a redo sternotomy, which involves the risk of cardiac injury, injury to the mediastinal collaterals due to the single-ventricle physiology, the acquired deranged coagulation that may incur morbidity associated with sternal re-entry, and the problems pertinent to cardiopulmonary bypass and/or cardioplegic arrest in this subset of patients.

Completion of an interventional extracardiac Fontan.

The extracardiac Fontan can be completed via transcatheter perforation of the pericardial membrane created during the preparatory stage, thus establishing continuity between the inferior vena cava and the pulmonary artery. This step is followed by deployment of a covered stent to isolate the systemic and pulmonary circuits. The procedure avoids the morbidity associated with cardiac reoperation and is a safe option for patients who present late with prohibitive pulmonary artery pressures in whom primary Fontan completion may not be feasible.

Right ventricular outflow tract histology post-stenting and in-stent stenosis.

The right ventricular outflow tract (RVOT) stenting is used for the treatment of Fallot-type lesions when not amenable to complete correction or when surgical palliation carries a substantial risk. Despite the increasing clinical application, there is a lack of data that describe the RVOT morphology after stenting. This article elucidates post-RVOT stenting and in-stent stenosis, which is thought to be a zonal phenomenon, in this case, predominantly occurring proximally, in the portion of the stent apposing the RVOT infundibulum.

Myocardial Parametric Mapping by Cardiac Magnetic Resonance Imaging in Pediatric Cardiology and Congenital Heart Disease.

Parametric mapping, that is, a pixel-wise map of magnetic relaxation parameters, expands the diagnostic potential of cardiac magnetic resonance by enabling quantification of myocardial tissue-specific magnetic relaxation on an absolute scale. Parametric mapping includes T1 mapping (native and postcontrast), T2 and T2* mapping, and extracellular volume measurements. The myocardial composition is altered in various disease states affecting its inherent magnetic properties and thus the myocardial relaxation times that can be directly quantified using parametric mapping. Parametric mapping helps in the diagnosis of nonfocal disease states and allows for longitudinal disease monitoring, evaluating therapeutic response (as in Thalassemia patients with iron overload undergoing chelation), and risk-stratification of certain diseases. In this review article, we describe various mapping techniques and their clinical utility in congenital heart disease. We will also review the available literature on normative values in children, the strengths, and weaknesses of these techniques. This review provides a starting point for pediatric cardiologists to understand and implement parametric mapping in their practice.

Abnormally proximal aortic origin of the brachiocephalic artery: Surgical implications.

Abnormal proximal aortic origin of the brachiocephalic artery is a very rare condition. It can occur in isolation or associated with complex congenital heart disease affecting the right ventricular outflow tract. Its recognition carries relevant surgical implications for the safe conduct of cardiopulmonary bypass and for any surgical procedures that directly involve the proximal ascending aorta and its branches.

Restriction of congenital portosystemic shunt using the modified microvascular plug.

Congenital portosystemic shunts (CPSS) may produce a variety of severe, clinically detrimental presentations. When indicated, closure is recommended; however, if the intrahepatic portal venous system (IPVS) is underdeveloped complete closure may not be possible and may result in severe acute portal hypertension. Staged restriction of CPSS flow by both surgical and complex transcatheter interventions has been successful in augmenting development of the IPVS such that complete occlusion of the CPSS can be performed. We report use of a modified microvascular plug to restrict CPSS flow with subsequent IPVS development and safe complete occlusion of CPSS.

Selective Coronary Artery Angiography in Hypoplastic Left Heart Syndrome.

Coronary artery disease in palliated hypoplastic left heart syndrome is uncommon. Myocardial infarction from a coronary thrombus, serving as a substrate for ventricular arrhythmia in Fontan physiology, is under-reported despite known hypercoagulopathic state. Traditional risk factors for coronary artery occlusion include intracardiac thrombi, hyperlipidemia, and hypertension. Baffle leaks and abnormal ventriculocoronary fistulae found in these patients are contributing factors. We sought to assess and describe coronary artery involvement in this complex patient population. Our research highlights both the need to assess distal coronary vasculature and to thoroughly evaluate hemodynamics and biventricular function with new-onset troponin leak or ventricular arrhythmias.

Pulse oximetry screening for detection of congenital heart defects at 1646 m in Albuquerque, New Mexico.

AIM: To determine the false-positive rate of pulse oximetry screening at moderate altitude, presumed to be elevated compared with sea level values and assess change in false-positive rate with time. METHODS: We retrospectively analysed 3548 infants in the newborn nursery in Albuquerque, New Mexico, (elevation 5400 ft) from July 2012 to October 2013. Universal pulse oximetry screening guidelines were employed after 24 hours of life but before discharge. Newborn babies between 36 and 36 6/7 weeks of gestation, weighing >2 kg and babies >37 weeks weighing >1.7 kg were included in the study. Log-binomial regression was used to assess change in the probability of false positives over time. RESULTS: Of the 3548 patients analysed, there was one true positive with a posteriorly-malaligned ventricular septal defect and an interrupted aortic arch. Of the 93 false positives, the mean pre- and post-ductal saturations were lower, 92 and 90%, respectively. The false-positive rate before April 2013 was 3.5% and after April 2013, decreased to 1.5%. There was a significant decrease in false-positive rate (p = 0.003, slope coefficient = -0.082, standard error of coefficient = 0.023) with the relative risk of a false positive decreasing at 0.92 (95% CI 0.88-0.97) per month. CONCLUSION: This is the first study in Albuquerque, New Mexico, reporting a high false-positive rate of 1.5% at moderate altitude at the end of the study in comparison to the false-positive rate of 0.035% at sea level. Implementation of the nationally recommended universal pulse oximetry screening was associated with a high false-positive rate in the initial period, thought to be from the combination of both learning curve and altitude. After the initial decline, it remained steadily elevated above sea level, indicating the dominant effect of moderate altitude.

Failure of Cellularization of Ventriculotomy Patch Leading to Right Ventricular Pseudoaneurysm.

Enlargement of the bulboventricular foramen (BVF) in double-inlet left ventricle or the ventricular septal defect (VSD) in tricuspid atresia with transposition of the great arteries is one approach for prevention or treatment of systemic ventricular outflow obstruction. Most often, BVF/VSD restriction is bypassed preemptively or addressed directly at the time of Glenn/Fontan procedures as part of staged univentricular palliation. We describe a patient who underwent enlargement of a restrictive VSD during Fontan completion and subsequently presented with an asymptomatic pseudoaneurysm of the right ventricle at the ventriculotomy site.

Tetralogy of Fallot with absent pulmonary valve-When the ductus is present: A case of isolated branch pulmonary artery and review of literature.

Tetralogy of Fallot/Absent Pulmonary Valve (TOF/APV) has been classically associated with the absence of a patent ductus arteriosus (PDA). We present a rare case of APV in TOF with a discontinuous left pulmonary artery (LPA) that was suspected during fetal echocardiogram. Postnatal echocardiogram confirmed the origin of a hypoplastic LPA from the PDA. Despite an aneurysmal (right pulmonary artery) (RPA), axial imaging demonstrated widely patent tracheobronchial system with no evidence of bronchial compression. Clinically, the child required only minimal respiratory support. Genetic testing was positive for 22 q11deletion, commonly associated with this lesion. Surgery consisted of unifocalization of the discontinuous LPA with placement of a valved pulmonary homograft during complete repair of this lesion. Our case highlights the importance of prenatal detection, to aid in the prompt initiation of prostaglandins so as to ensure early rehabilitation of the left lung. Inability to visualize one of the branch pulmonary arteries (PA's) and a PDA on fetal echocardiogram in TOF/APV must raise suspicion for an eccentric branch PA with ductal origin.

Cor triatriatum dexter with right ventricular hypoplasia: Role of multimodality imaging in decision making.

Cor Triatriatum Dexter (CTD) is a rare congenital anomaly involving the systemic venous valves. Failure of regression of the right-sided sinus venosus valve leads to abnormal septation of the right atrium and a variety of right atrial and tricuspid valve obstructive lesions. The presentation can be varied ranging from asymptomatic to persistent neonatal cyanosis. We describe a late diagnosis of CTD in a 10-month-old male with persistent hypoxia despite balloon valvuloplasty for mild pulmonic valve stenosis with a large secundum atrial septal defect and a mildly hypoplastic right ventricle.

Clinico-Pathological Characteristics of Triple Negative and Non Triple Negative High Grade Breast Carcinomas with and Without Basal Marker (CK5/6 and EGFR) Expression at a Rural Tertiary Hospital in India.

UNLABELLED: Aims of the study were to evaluate the expression Cytokeratin 5/6(CK5/6) and Epidermal Growth Factor Receptor (EGFR) among triple negative breast cancers and high grade infiltrating duct carcinomas. Further to probe if triple negative phenotype can be a surrogate marker for basal phenotype and to correlate the expression of basal markers with disease free survivals among triple negative phenotype and high grade infiltrating duct carcinomas. METHODS: Expression of CK5/6 and EGFR were studied by Immunohistochemistry (IHC) in 31 triple negative and 19 non-triple negative high grade breast carcinomas. RESULTS: 21 of the 31 triple negative phenotype (67.7%) breast carcinomas and 7 out of 19 non-triple negative (36.8%) breast carcinomas showed expression of basal markers (CK5/6 and/or over-expression of EGFR). There were statistically significant associations of all the basal-like tumors with negative hormonal status. The basal markers positive phenotype subjects had a shorter disease free interval as compared to basal markers negative phenotype subjects. CONCLUSION: Basal-like breast carcinomas constitute a unique clinical and pathological entity, characterized by high tumor grade and a propensity for lack of ER, PR and HER2 expression. Basal phenotypes have a more aggressive course than non-basal phenotype. "Triple negative" status cannot be used as a surrogate for "basal marker expression".