Experiences and Attitudes Toward Telemedicine in an Adult Congenital Heart Disease Clinic: Lessons Learned from the COVID-19 Pandemic.

The coronavirus disease 2019 (COVID-19) pandemic has driven a broader adoption of telemedicine (TM). We aim to describe adult congenital heart disease (ACHD) patient experiences with TM and explore factors associated with positive attitude toward future TM visits. This is a cross-sectional, single-center study in an outpatient ACHD clinic from February to June, 2022.

Combined ventricular dysfunction and atrioventricular valve regurgitation after the Norwood procedure are associated with attrition prior to superior cavopulmonary connection.

Background: Infants with hypoplastic left heart syndrome (HLHS) or a variant are at risk of ventricular dysfunction (VD) and atrioventricular valve regurgitation (AVVR) prior to superior cavopulmonary connection (SCPC). Although the impact of these complications in isolation has been described, their effect in combination on attrition is poorly defined.

Methods: A retrospective observational study of patients with HLHS or variants undergoing a Norwood procedure between 2008 and 2020 at a single center was performed.

Feasibility of Digital Stethoscopes in Telecardiology Visits for Interstage Monitoring in Infants with Palliated Congenital Heart Disease.

Infants with staged surgical palliation for congenital heart disease are at high-risk for interstage morbidity and mortality. Interstage telecardiology visits (TCV) have been effective in identifying clinical concerns and preventing unnecessary emergency department visits in this high-risk population. We aimed to assess the feasibility of implementing auscultation with digital stethoscopes (DSs) during TCV and the potential impact on interstage care in our Infant Single Ventricle Monitoring & Management Program.

Association of Home Monitoring and Unanticipated Interstage Readmissions in Infants With Hypoplastic Left Heart Syndrome.

Background The impact of home monitoring on unanticipated interstage readmissions in infants with hypoplastic left heart syndrome has not been previously studied. We sought to examine the association of our institution's Infant Single Ventricle Management and Monitoring Program (ISVMP) with readmission frequency, cumulative readmission days, and readmission illness severity and to identify patient-level risk factors for readmission. Methods and Results We performed a retrospective single-center cohort study comparing infants with hypoplastic left heart syndrome enrolled in ISVMP (December 2010-December 2019) to historical controls (January 2007-November 2010).

Initial Experience with Telemedicine for Interstage Monitoring in Infants with Palliated Congenital Heart Disease.

Infants with staged surgical palliation for congenital heart disease are at high-risk for interstage morbidity and mortality; home monitoring programs have mitigated these risks. In 2019, we instituted telemedicine (TM) in our established Infant Single Ventricle Monitoring Program. All consecutive patients discharged following neonatal operation/intervention were monitored until subsequent stage 2 surgical palliation.

Telemedicine in pediatric cardiology: pros and cons.

Purpose Of Review: The aim of this article is to review recent uses of telemedicine in pediatric cardiology, highlighting merits, challenges, and future directions.

Recent Findings: The COVID-19 pandemic accelerated telemedicine use, which has had a positive impact with respect to providers, patients, and their caregivers. Recent data have demonstrated the feasibility and effectiveness of telemedicine through expediting needed care and reducing healthcare utilization, including unnecessary emergency department visits, transports, and hospitalizations.

Relationship Between Serum Brain-Type Natriuretic Peptide and Biomarkers of Growth in Infants With Shunt-Dependent Single Cardiac Ventricle.

For infants with shunt-dependent or ductal-dependent single ventricle heart disease, poor growth is common and associated with morbidity and impaired neurodevelopmental outcomes. Although attention has focused on nutrition to promote weight gain, little is known about the relation between heart failure and growth factors. A prospective observational pilot study was performed to assess the relation between heart failure, assessed by brain natriuretic peptide (BNP), and growth factors (insulin-like growth factor 1 [IGF-1] and insulin-like growth factor-binding protein 3) at 3 visits: (1) before discharge from neonatal intervention with the establishment of stable pulmonary blood flow, (2) immediately before superior cavopulmonary connection, and (3) before discharge after superior cavopulmonary connection operation.

Obesity trends in children, adolescents, and young adults with congenital heart disease.

Objectives: To determine the prevalence, age of onset, and risk factors for overweight and obesity in children with congenital heart disease (CHD).

Study Design: Children with CHD who were seen at our institution from 1996 to 2017 were studied. Patients were full-time residents of the United States and were receiving all cardiac care at our institution.

The Harada score in the US population of children with Kawasaki disease.

Objective: To describe and quantify the presentations of Kawasaki disease (KD) in a children's hospital over 10 years to assess the Harada score in a US population.

Methods: A retrospective chart review from 2001 to 2011 of children discharged from Cleveland Clinic with the diagnosis of KD. Demographic and clinical data were collected and Harada scores were derived to evaluate efficacy in predicting risk for coronary artery aneurysms (CAAs).

Percutaneous PFO closure for paradoxical stroke in 8-kg twins.

Patent foramen ovale (PFO) is a known risk factor for paradoxical embolus, especially in the presence of other risk factors such as hypercoagulable states or central lines. A PFO is a common incidental finding in infants and children. However, paradoxical emboli are not common in infants, and so PFO closure is rarely indicated in this age group.

Prenatal diagnosis and neonatal surgical management of a giant proximal right coronary artery to right ventricular fistula.

We report a case of prenatal diagnosis and early neonatal surgical repair of a large proximal right coronary artery to right ventricular fistula. The surgical findings and technical details of the reparative operation are discussed in the context of the differential diagnosis, which, in addition to coronary-cameral fistula, also includes aortoventricular tunnel and ruptured sinus of Valsalva aneurysm. Timely and appropriate diagnosis and surgical management resulted in preserved patency of the right coronary artery and restoration of normal right ventricular function.

Melbourne shunt promotes growth of diminutive central pulmonary arteries in patients with pulmonary atresia, ventricular septal defect, and systemic-to-pulmonary collateral arteries.

Background: We manage patients with pulmonary atresia, ventricular septal defect, major systemic-to-pulmonary collateral arteries, and diminutive central pulmonary arteries with a staged approach. The first procedure is a central end-to-side aortopulmonary shunt (Melbourne shunt) intended to cause growth and development of the central pulmonary arteries. We subsequently measured central pulmonary artery growth after Melbourne shunt.

Use of a straight, side-hole delivery sheath for improved delivery of Amplatzer ASD occluder.

The Amplatzer ASD occluder may be difficult to position in some patients with a large atrial septal defect (ASD) or deficiency of one or more atrial septal rims. We developed a method to modify a Mullins transseptal sheath to enhance delivery. The resulting sheath is straight and has an exit orifice essential in the side of the distal portion of the sheath-a straight, side-hole (SSH) delivery sheath.

Intracardiac echocardiography during transcatheter interventions for congenital heart disease.

Although transesophageal echocardiography is often used for guidance during transcatheter interventions, few data exist regarding the use of the newer modality of intracardiac echocardiography. This brief report summarizes our single center experience using intracardiac echocardiographic guidance during transcatheter interventional procedures for congenital heart disease.

Prospective diagnosis of alveolar capillary dysplasia in infants with congenital heart disease.

Alveolar capillary dysplasia (ACD) is a lethal pulmonary disorder found in newborns that is characterized by severe pulmonary hypertension and hypoxemia. We report on the clinical behavior of this disorder in a series of patients and its association with congenital heart disease, especially left heart obstructive disease; we also report a prospective diagnosis of ACD by lung biopsy in a newborn with congenital heart disease, which prevented futile and prolonged medical intervention.

Sustained local delivery of heparin to the rabbit arterial wall with an electroporation catheter.

Current methods of local drug delivery frequently fail to achieve a prolonged therapeutically effective tissue drug level without producing vascular trauma. A novel double-balloon catheter system, incorporating electroporation technology, has been designed and tested to deliver heparin into rabbit carotid arteries in an overstretch balloon injury model in vivo. Following arterial injury, fluoresceinated heparin was delivered into the volume between the two inflated balloons, and the artery was subjected to an electrical pulse.

Isolated peripheral pulmonary artery stenoses in the adult.

Background: Isolated peripheral pulmonary artery stenosis (PPS) in the adult is rare and frequently unsuspected. We review in this article our experience with 12 adult patients with isolated PPS, half of whom had been previously diagnosed with chronic pulmonary thromboembolic disease.

Methods And Results: The presentation, evolution, and management of 12 adults with isolated PPS, 17 to 51 years of age (mean, 36.

Association of systemic to pulmonary collateral arteries with tetralogy of Fallot and absent pulmonary valve syndrome.

Two patients with tetralogy of Fallot and absent pulmonary valve syndrome with significant systemic to pulmonary collateral arteries are described. Search for these rarely reported vessels should be included in the workup of these complex patients.

Tetralogy of Fallot, absent pulmonary valve, partial anomalous pulmonary venous return and coarctation of the aorta.

We describe a unique combination of tetralogy of Fallot, absent pulmonary valve, aortic coarctation with partial anomalous pulmonary venous connection and a retroaortic innominate vein. Surgical repair was successfully accomplished in two stages: coarctation repair preceded correction of the intracardiac anomalies.

Hepatic venous blood and the development of pulmonary arteriovenous malformations in congenital heart disease.

Background: Pulmonary arteriovenous malformations (PAVMs) are a known complication after some types of cavopulmonary anastomoses (CVPAs). Their cause is unknown, but they may be related to the absence of pulsatile flow or the presence or absence of circulating factors. These PAVMs are diffuse and are presumed to be progressive and irreversible.

Traumatic aortopulmonary window as a complication of pulmonary artery balloon angioplasty: transcatheter occlusion with a covered stent. A case report.

We describe a case in which pulmonary artery balloon angioplasty was complicated by formation of an aortopulmonary window, a previously unreported complication associated with this technique. A novel transcatheter approach for occluding the defect, a covered stent, was utilized. Deployment of the covered stent significantly reduced the shunt, allowed for clinical stabilization, and averted the need for emergency surgical intervention.

"Intramural" residual interventricular defects after repair of conotruncal malformations.

Background: We report an unusual type of residual interventricular communication in patients with conotruncal malformations in which the aorta is completely or partly aligned with the right ventricle (RV). Interventricular communications after surgical repair usually result from additional defects, patch dehiscence, or incomplete closure and lie in the septal plane. However, after a right ventricular aorta is baffled to the left ventricle, the ventricular septal defect (VSD) patch and RV free wall form part of the systemic outflow tract.

Generalized arteriopathy in Williams syndrome: an intravascular ultrasound study.

Objectives: We used intraluminal ultrasound imaging to provide additional information about the vascular pathology in Williams syndrome.

Background: The cardiovascular pathology of Williams syndrome consists of medial hypertrophy in both systemic and pulmonary arteries, which results in lumen narrowing.

Methods: Systemic and pulmonary arteries were examined in vivo using intravascular ultrasound imaging (5F, 30-MHz catheter) in three children with Williams syndrome.

Sensitivity and specificity of blood cultures obtained through intravascular catheters.

The reliability of blood cultures obtained through indwelling intravascular catheters is controversial. In this study, the results of 200 catheter blood cultures were compared with those of an equal number of peripheral blood cultures drawn at the same time. Catheter blood cultures were found to be 96% sensitive (95% confidence interval, 89% to 100%) and 98% specific (95% confidence interval, 96% to 100%) for the detection of septicemia.

Infants with apparent life threatening events. Cardiac rhythm and conduction.

The role of cardiac dysrhythmias in the pathogenesis of Sudden Infant Death Syndrome (SIDS) is uncertain, but there have been several reports of infants with Apparent Life Threatening Events (ALTE) due to significant dysrhythmias. To further characterize the cardiac rhythm and conduction of these "at-risk for SIDS" infants, we performed 24-hour continuous (Holter) electrocardiograms on 100 full term, healthy infants with ALTE. Sixty-two patients (62%) had one or more dysrhythmias on Holter monitor.