Emergency Use of Occlutech® Atrial Flow Regulator Device Facilitates Weaning From Veno-Arterial Extracorporeal Membrane Oxygenation in a Patient With Severe Pulmonary Arterial Hypertension.

Pulmonary arterial hypertension (PAH) is a severe and progressive disease. Treatment options include anti-PAH medications, continuous intravenous therapies, and diuretics. Lung transplant is required in many cases.

Aortopulmonary Collaterals in Single Ventricle Physiology: Variation in Understanding Occlusion Practice Among Interventional Cardiologists.

Although aortopulmonary collaterals (APCs) frequently develop in patients with single ventricle palliation, there is a lack of understanding of pathophysiology, natural history, and outcomes with no universal guidelines for management and interventional practice. We conducted a study to assess the views held by interventional congenital cardiologists regarding the hemodynamic impact of APCs in patients with single ventricle palliation, and their embolization practice. An electronic survey using the Pediatric Interventional Cardiology Symposium (PICS) mailing list was conducted between February and March 2019 with one reminder sent 2 weeks after initial invitation for participation.

Complex Determinants of Work Ability in Adults With Congenital Heart Disease and Implications for Clinical Practice.

Background: "Work ability" is the employees' capacity to meet the demands of their job. As more patients with complex congenital heart disease (CHD) are now reaching adulthood, we assessed work ability and factors impacting livelihood in adult CHD.

Methods: The work ability index (WAI) questionnaire and patient health questionnaire-9 (PHQ-9) were administered at 2 Midwest adult CHD centres from February 2017 to 2018.

Moving beyond two-dimensional screens to interactive three-dimensional visualization in congenital heart disease.

Beginning with the discovery of X-rays to the development of three-dimensional (3D) imaging, improvements in acquisition, post-processing, and visualization have provided clinicians with detailed information for increasingly accurate medical diagnosis and clinical management. This paper highlights advances in imaging technologies for congenital heart disease (CHD), medical adoption, and future developments required to improve pre-procedural and intra-procedural guidance.

Trends in the off-label use of β-blockers in pediatric patients.

The use of US Food and Drug Administration (FDA)-approved drugs for the treatment of an unapproved indication or in an unapproved age group, or at doses or route of administration not indicated on the label is known as off-label use. Off-label use may be beneficial in circumstances when the standard-of-care treatment has failed, and/or no other FDA-approved medications are available for a particular condition. In pediatric patients, off-label use may increase the risk of adverse events as pharmacokinetic and pharmacodynamic data are limited in children.

Three-dimensional printing for surgical planning in complex congenital heart disease.

Surgical management of complex congenital heart disease (CHD) is challenging. Three-dimensional (3D) printing can improve multidisciplinary team decision-making, patient and family understanding, and education of medical professionals. We describe 3D printing for surgical management of five patients with complex CHD.

Electronic health record associated stress: A survey study of adult congenital heart disease specialists.

Background: Physician burnout has many undesirable consequences, including negative impact on patient care delivery and physician career satisfaction. Electronic health records (EHRs) may exacerbate burnout by increasing physician workload.

Objective: To determine burnout in adult congenital heart disease (ACHD) specialists by assessing stress associated with EHRs.

Determination of the Frequency of Right and Left Internal Mammary Artery Embolization in Single Ventricle Patients: A Two-Center Study.

Embolization of systemic to pulmonary artery collaterals to regulate pulmonary arterial flow or pressure of the cavopulmonary circulation in patients with single ventricle is a common practice. The relative incidence and impact of this practice on future interventions like coronary artery bypass grafting is poorly understood. This study aims to evaluate the frequency and implications of internal mammary artery (IMA) embolization in the single ventricle (SV) population.

Transient Secondary Hypothyroidism and Thyroid Hormone Replacement Therapy in Pediatric Postoperative Cardiopulmonary Bypass.

Background: To develop an understanding of current practices in the management of transient secondary hypothyroidism in pediatric postoperative cardiopulmonary bypass (CPB) patients.

Methods: Electronic survey comprising a 10-item questionnaire was sent to sixty-four high volume pediatric heart centers in the United States and United Kingdom. Survey participants included cardiologists, intensivists, cardiothoracic surgeons, and advanced practice providers.

Nursing Assessment, Education, and Care of Extremely Premature Neonates with Patent Ductus Arteriosus.

The care of extremely premature neonates with suspected or confirmed diagnosis of patent ductus arteriosus (PDA) is a frequent challenge for pediatric nurses. It is important for nurses to have adequate knowledge of the normal postnatal changes in cardiovascular and pulmonary function to recognize any adverse symptoms. Nurses caring for these vulnerable neonates must have a thorough understanding of the pathophysiology of a PDA in order to assess, plan, and implement patient-centered care.

Integration of Computed Tomography and Three-Dimensional Echocardiography for Hybrid Three-Dimensional Printing in Congenital Heart Disease.

Three-dimensional (3D) printing is an emerging technology aiding diagnostics, education, and interventional, and surgical planning in congenital heart disease (CHD). Three-dimensional printing has been derived from computed tomography, cardiac magnetic resonance, and 3D echocardiography. However, individually the imaging modalities may not provide adequate visualization of complex CHD.

Biventricular Repair of Pulmonary Atresia After Fontan Palliation.

Fontan palliation is used when biventricular repair (BVR) is not possible. Early outcomes are acceptable; however, the long-term sequelae include protein-losing enteropathy, declining functional status, increased pulmonary vascular resistance, heart failure, and hepatic and renal dysfunction. These adverse events are characteristic of persistent venous hypertension and may be avoided if restoring biventricular circulation is possible.

Regression of massive cardiac rhabdomyoma on everolimus therapy.

Cardiac rhabdomyoma is the primary feature of the genetic disease, tuberous sclerosis complex, the most common cardiac tumor diagnosed in neonates and infants. Spontaneous regression is observed in most cases, but these tumors may cause hemodynamic instability, arrhythmias or other complications. We describe the case of a critically ill neonate, resuscitated after cardiac arrest secondary to massive locally invasive cardiac rhabdomyoma, who was successfully treated with everolimus (mammalian target of rapamycin [mTOR] inhibitor).

Surgical Treatment of Neonate With Congenital Left Main Coronary Artery Atresia.

Left main coronary artery atresia (LMCAA) is a rare congenital malformation with a nonspecific and varied clinical presentation. Ventricular dysfunction and mitral insufficiency are expected ischemic consequences in the neonatal period. Left internal mammary artery (LIMA) bypass grafting (CABG) is uncommon because of the technical difficulties in performing this procedure in neonates.

Implantable atrial flow regulator for severe, irreversible pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a severe, progressive and fatal disease. The creation of an interatrial right-to-left shunt in patients with PAH may enhance systemic ventricular output at the expense of desaturation. However, creating sustainable restricted interatrial communication is challenging.

Hybrid 3D printing: a game-changer in personalized cardiac medicine?

Three-dimensional (3D) printing in congenital heart disease has the potential to increase procedural efficiency and patient safety by improving interventional and surgical planning and reducing radiation exposure. Cardiac magnetic resonance imaging and computed tomography are usually the source datasets to derive 3D printing. More recently, 3D echocardiography has been demonstrated to derive 3D-printed models.

Transcatheter intervention in cor triatriatum sinister.

Cor triatriatum sinister is a rare condition caused by a membrane in the left atrium, resulting in left ventricular inflow obstruction. This developmental anomaly is usually diagnosed in childhood. However, a rare presentation during adulthood is observed when the membrane is incomplete.

Pulmonary Arteriovenous Malformations in Dyskeratosis Congenita.

Pulmonary arteriovenous malformations (PAVMs) are rare lesions known to cause cyanosis due to abnormal communication between the pulmonary arteries and veins. They are commonly seen in association with hereditary hemorrhagic telangiectasia, congenital heart disease, hepatopulmonary syndrome, and portopulmonary shunting, but rarely in patients with dyskeratosis congenita (DC). We describe a patient previously diagnosed with DC confirmed to have microscopic PAVMs after bone marrow transplantation and discuss possible pathogenic mechanisms.

Ultrasound-Derived Three-Dimensional Printing in Congenital Heart Disease.

Three-dimensional printing technology has significant clinical implications for the management of congenital heart disease. Computed tomography and magnetic resonance imaging have been established as imaging tools for the creation of physical three-dimensional models. The potential use of non-invasive bedside imaging techniques such as three-dimensional echocardiography to derive three-dimensional printed models can revolutionize the planning of interventions for complex congenital malformations.

What nurses need to know about fecal microbiota transplantation: education, assessment, and care for children and young adults.

Fecal microbiota transplantation (FMT) is an emerging experimental therapy for treatment of recurrent Clostridium difficile infection. In the future, FMT has the potential to be a treatment modality in other diseases that involve gut dysbiosis. As use of FMT is likely to expand, pediatric nurses need a clear understanding of FMT to provide appropriate education, assessment, and care for these patients.