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. Atrial septostomy is an under recognized option in symptomatic patients on maximal PAH therapy. However, creating a sustainable and restrictive atrial communication is challenging with existing devices. We describe emergency use of the Occlutech® Atrial Flow Regulator, a novel device, in a 35-year-old female with PAH supported on veno-arterial extracorporeal membrane oxygenation after postpartum decompensation.

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. Of the 142 interventional cardiologist respondents, 95 (66.9%) reside in North America and 47 (33.1%) worldwide. We elected to exclude the data from interventionalists outside North America in this analysis as it was not representative of worldwide practice. Hypoxemia was considered to be the most common trigger for development of APCs by 56 (58.9%) respondents. After completion of total cavopulmonary connection, 30 (31.6%) respondents reported the APC burden stays the same while 31 (32.6%) feel it decreases. In evaluating the burden of APC flow, only 4 (4.2%) reported measuring oxygen saturation at different pulmonary artery segments, 21 (22.1%) perform segmental aortic angiograms, and 18 (19%) perform selective bilateral subclavian artery angiograms. A majority of respondents, 71 (74.7%), occlude the feeder vessel at different locations, while 10 (10.5%) occlude only the origin of the vessel. Our study demonstrates significant variation in the understanding of the cause and prognosis of APCs in patients with single ventricle palliation. Furthermore, there is variation in the approach for diagnosis and management among interventional cardiologists. Further studies are required to improve understanding of APCs and develop universal management guidelines.

Trends in the off-label use of ß-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. Approximately 73% of off-label drugs currently prescribed for various conditions do not have sufficient scientific evidence for safety and efficacy. For example, ß-blockers are a class of drugs with FDA-approval for very few indications in pediatrics but are commonly used for various off-label indications. Interestingly, the proportion of off-label use of ß-blockers in adults is at about 52% (66.2 million) of the total number of ß-blockers prescribed. The frequency of off-label use of ß-blockers in children is also high with limited data on the indications as well as safety and efficacy. We present trends in off-label use of ß-blockers in children to discuss drug safety and efficacy and include recommendations for pediatric providers.

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. The anatomical details of the 3D printed models were instrumental in planning surgical techniques especially in determining between single ventricle, 1.5 ventricle, and biventricular repair.

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. A retrospective chart review was performed of SV patients who underwent cardiac catheterization before and after Fontan procedure between February 2007 and 2017. Data were collected from two tertiary care centers in the Midwest. Of the 304 SV patients, 62 (20.4%) underwent embolization of one or more IMAs, whereas 242 (79.6%) did not. The rate of embolization of IMA was 40.5% in one center and 14.5% in the second center. Among patients who received IMA embolization, left internal mammary artery (LIMA) embolization was seen in 6 (9.7%) patients. Majority of patients underwent either right internal mammary artery (RIMA) embolization (n = 25; 40.3%) or RIMA and LIMA embolization (n = 27; 43.5%). IMA embolization in SV patients is common. Embolizing IMAs early in life will likely eliminate a valuable graft option for coronary artery bypass grafting should it be required in the future care of these patients. Multi-center, prospective, nation-wide studies are warranted to examine coronary artery disease in the SV population and true frequency of IMA embolization. Delineation of which IMAs were embolized is a necessary in surgical and cardiac intervention national data, such as Society of Thoracic Surgeons (STS) database. All measures should be taken to preserve IMAs patency, if deemed feasible and safe.

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). Rapid tumor regression was observed on echocardiography, but it was unlikely that it was confounded by the natural disease course of regression. The presented case suggests that mTOR inhibitors may play a significant role in the treatment of large cardiac rhabdomyomas in critically ill neonates.

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. The integration of the strengths of two or more imaging modalities has the potential to enhance visualization of cardiac pathomorphology. We describe the feasibility of hybrid 3D printing from two imaging modalities in a patient with congenitally corrected transposition of the great arteries (L-TGA). Hybrid 3D printing may be useful as an additional tool for cardiologists and cardiothoracic surgeons in planning interventions in children and adults with CHD.

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. The feasibility of deriving three-dimensional printing from ultrasound provides an additional cost-effective and patient-centered option for interventional cardiologists and surgeons for the management and care of congenital heart disease patients.

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. Pediatric research and clinical nurses are a resource to help children and parents understand the procedure. Important topics include donor screening, patient assessment before, during, and after treatment; routes of administration and positioning; preparation for discharge and followup evaluation.

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.

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. RESULTS: Of the 267 participants (n = 157 males, 59%) with an average age of 35 ± 13 years, the majority (n = 204, 76%) were employed. Patients with complex CHD (n = 103, 39%) were less likely to have enrolled in college or completed a graduate degree (P = 0.0115), and more likely to have an annual income of < $50,000 (P = 0.0056) and lower WAI scores (P = 0.0026) than patients with simple and moderate CHD. Unemployed patients (n = 63, 24%) with complex CHD (n = 27, 43%) were more likely to have higher PHQ-9 scores (P = 0.0242) indicating mild, moderate, or severe depression (P = 0.0482) than unemployed patients with simple and moderate CHD. Patients with complex CHD had lower self-perception of work ability compared with patients with simple and moderate CHD (P = 0.0007). Finally, patients in NYHA Functional Class I had higher WAI scores than NYHA Class III-IV (P < 0.0001). CONCLUSIONS: This study demonstrates that employed patients with complex CHD have lower education level, income, and work ability. Unemployed patients are more likely to exhibit symptoms of depression and have low self-perception of work ability. Occupational health programs focusing on promoting general health perception, increasing exercise capacity, and improving psychosocial health must be considered to improve work ability in patients with adult CHD to maintain livelihood.

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. DESIGN: Electronic survey study of ACHD providers. SETTING: Canada and United States. PARTICIPANTS: Three hundred eighty-three ACHD specialists listed on the Adult Congenital Heart Association directory between February and April 2017. OUTCOME MEASURES: Burnout was measured using the Maslach Burnout Inventory (MBI) to understand factors contributing to work life and EHR satisfaction. Chi-square and Wilcoxon Rank Sum tests were used for statistical analysis. RESULTS: Of the 383 invited participants, 110 (28.7%) completed surveys with the majority (n = 88, 80.7%) reporting from an academic medical center. Burnout was defined as high scores on the emotional exhaustion and/or depersonalization MBI subscales. When comparing the 40% (n = 44) that met criteria for burnout with those that did not, there was strong disagreement that a reasonable amount of time is spent on clerical tasks related to direct (P = .0043) or indirect (P = .0004) patient care. There was strong disagreement that EHRs increased efficiency (P = .006) or the patient portal improved patient care (P = .0215). Finally, physicians who met criteria for burnout had lower personal accomplishment scores (P = .0355). CONCLUSIONS: Our results suggest time spent on EHRs creates clerical burden exacerbating ACHD physician burnout. The high levels of emotional exhaustion may decrease quality of ACHD care by directing focus away from physician-patient interaction. Health care systems must develop best practice for EHR design and implementation to optimize patient advocacy and care, and decrease physician burnout.

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. A retrospective chart review was also performed at a large regional referral center in the Midwest on subjects 0-18 years old who underwent CPB from 2005-2015. Information obtained included a unique identifier, date of birth, age, procedure performed, CPB time, date of surgery and date and type of Thyroid Function Test (TFT) ordered. RESULTS: 1,153 individuals from 64 congenital heart centers were contacted via email to participate in the electronic survey. In the 3-month response window, 129 completed surveys were received from cardiologists (55%), intensivists (17%), surgeons (15%), "other" (8%), and advanced practice providers (5%). This yielded a response rate of 11.2%. Of the 129 respondents, only 10 providers routinely order TFTs prior to (n=7) and after (n=1) CPB or when clinically indicated (n=2). All 10 providers order thyroid stimulating hormone test, 7 order thyroxine, and 3 order triiodothyronine. Only 1 provider routinely treats children with prophylactic thyroid hormone replacement therapy after CPB. Our retrospective review included 502 CPB events with 442 unique patients. Of the events, 20 patients received preoperative TFT testing while 11 received postoperative testing. CONCLUSIONS: There is a general lack of uniformity in the evaluation, diagnosis, and treatment of transient secondary hypothyroidism in pediatric postoperative CPB patients.

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. Recognition of characteristic symptoms of PDA in a timely manner is essential for optimal management and outcomes. Understanding the science behind treatment options is also imperative for pediatric nurses to provide the best care and effectively educate parents. Pediatric nurses are a significant resource in managing extremely premature neonates through comprehensive assessment, effective parent education, and high-quality patient-centered care.

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. Arrhythmias are a common adverse event, particularly in patients with an atriopulmonary connection, which may lead to acute decompensation and early death. We describe a 30-year-old woman who underwent successful BVR for pulmonary atresia with intact ventricular septum and demonstrate that where favorable anatomy exists with a failing Fontan, BVR should be considered.

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. We describe LMCAA revascularization with a LIMA graft and mitral valve repair in a 7-week-old neonate with successful outcome 1 year postoperatively.

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. The integration of multiple imaging modalities for hybrid 3D printing has also been shown to create accurate printed heart models, which may prove to be beneficial for interventional cardiologists, cardiothoracic surgeons, and as an educational tool. Further advancements in the integration of different imaging modalities into a single platform for hybrid 3D printing and virtual 3D models will drive the future of personalized cardiac medicine.

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. We describe the successful use of an atrial flow regulator, a novel implantable atrial communication device, in a 54-year-old female with severe irreversible PAH.