Use of Interactive Visualization and 3D Printing in the Repair of Complex Congenital Heart Disease Presenting in Adult Life.

We report a case of a 25-year-old male with a heterotaxy-like constellation of congenital heart defects consisting of complete atrioventricular septal defect, transposition of the great arteries, subpulmonary stenosis, L-looped ventricles, hypoplastic right ventricle, and a distant aorta arising from the right ventricle. This case demonstrates how 3D printing and interactive 3D visualization may facilitate a unique surgical repair.

Atrial septal defect-associated pulmonary hypertension with decompensated heart failure: outcomes after fenestrated device closure.

Background: Up to 90% of adults with untreated atrial septal defect will be symptomatic by 4 decade, and 30-49% will develop heart failure. 8-10% of these patients have pulmonary arterial hypertension with a female predominance regardless of age. We aimed to demonstrate that fenestrated closure can be safely performed in patients with decompensated heart failure and atrial septal defect-associated pulmonary arterial hypertension with improved outcome.

Atrioventricular Septal Defects: Pathology, Imaging, and Treatment Options.

Purpose Of Review: Atrioventricular septal defects (AVSD) represent a broad spectrum of congenital anomalies from simple to the most complex heart defects including some distinct types. Clinical presentation and timing of intervention differ by morphological subset and functional anatomy. Herein, we review morphological variations and characteristics that determine appropriate intervention and provide insights into functional anatomy based on detailed three-dimensional (3D) assessment of AVSDs.

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.

Catheter-induced acute Blalock-Taussig shunt thrombosis and bailout.

Modified Blalock-Taussig shunt thrombosis is a life-threatening event. We describe an extremely rare catheter-induced shunt thrombosis in an infant with complex CHD and its successful treatment utilising a single low dose of local recombinant tissue plasminogen activator in conjunction with balloon angioplasty.

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.

Management of 760-g Extremely Premature Infant With Dextro-Transposition of the Great Arteries.

Extremely low birth weight neonates with complex congenital heart disease have increased mortality risk. Multi-organ dysfunction, pulmonary disease, fluctuating pulmonary vascular resistance, and complex cardiovascular anatomy create a challenge for effective management. We present the case of a 760-g neonate with dextro-transposition of the great arteries, ventricular septal defect, patent ductus arteriosus, and single coronary artery with proximal intramural segment of the right coronary artery branch.

Hybrid Approach to Right Ventricle Decompression in Muscular Pulmonary Atresia with Intact Ventricular Septum.

Muscular pulmonary atresia with intact ventricular septum (PA/IVS) in neonates is traditionally managed by surgery. We describe hybrid approach to decompress the right ventricle (RV) and establish RV to pulmonary artery connection in a neonate avoiding cardiopulmonary bypass. A 21-gauge access needle was used to perforate the atretic pulmonary valve via periventricular approach followed by stent placement.

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.

Target Oxygen Levels and Critical Care of the Newborn.

Despite our growing experience in the medical care of extremely preterm infants and critically ill neonates, there are serious gaps in the understanding and clinical application of the adaptive physiology of the newborn. Neonatal physiology is often misinterpreted and considered similar to that of adult physiology. The human psyche has been seriously influenced, both from an evolutionary and survival point of view, by the cause and effect of hypoxemia which is considered as a warning sign of impending death.

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.

Left Ventricular Pseudoaneurysm Following Surgical Repair of Ventricular Septal Defect in an Infant.

Left ventricular pseudoaneurysm (LV-PSA) is a rare complication following cardiac surgery, let alone in the pediatric population. Other known causes of LV-PSA are trauma, percutaneous cardiac intervention, and infections. This report describes the development of LV-PSA following surgical repair of ventricular septal defect (VSD) and coarctation of aorta (CoA) in an infant.

Intracoronary recombinant tissue plasminogen activator in an infant with hypoplastic left heart syndrome and complete left main coronary artery thrombosis.

An infant with hypoplastic left heart syndrome (HLHS) presented with complete heart block and severe myocardial dysfunction requiring ECMO support due to complete left main coronary artery (LMCA) thrombosis. Current guidelines for managing coronary artery thrombosis in infants with single ventricle physiology are inadequate. We describe successful LMCA and branch recanalization via intra coronary infusion of recombinant tissue plasminogen activator and discuss management of acute coronary thrombosis in children with single ventricle physiology.

Pulmonary arteriovenous malformations leading to hypoxemia in child with primary ciliary dyskinesia.

Primary ciliary dyskinesia (PCD) is a rare genetic condition characterized by respiratory tract infections, situs inversus or heterotaxy, and male infertility. Chronic respiratory infections begin in childhood and result in complications such as bronchiectasis. As hypoxemia is often attributed to bronchiectasis, other etiologies for desaturation in this setting are not routinely evaluated.

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.

Stenting and Reimplanting Disconnected Pulmonary Artery in Tetralogy of Fallot.

Tetralogy of Fallot with absent pulmonary valve syndrome (TOF/APV) is a rare congenital malformation. Although pulmonary artery (PA) anomalies have been observed in TOF, its association with disconnected PA is extremely rare. We report successful stenting of the disconnected left PA in a 3-year-old boy with TOF/APV followed by surgical reimplantation.

Is the Hepatic Factor a miRNA that Maintains the Integrity of Pulmonary Microvasculature by Inhibiting the Vascular Endothelial Growth Factor?

Background: The "hepatic factor," a molecule or group of molecules present in the hepatic venous blood, essential for the prevention of the development of pulmonary arteriovenous malformations (PAVMs) and right-to-left shunting has been a conceptual enigma in the understanding of many related conditions.

Methods: Patients with various forms of liver diseases including acute hepatic failure, and others with normal hepatic function like hereditary hemorrhagic telangiectasia (HHT), inflammatory and parasitic disorders, cardiogenic hepatopulmonary syndrome (cHPS) and skin disorders like Dyskeratosis congenita are all known to cause PAVMs. Over a period of the last two decades our understanding of the pathogenesis of PAVMs has changed, but the mechanisms are still not clearly understood.

Pathophysiology of Patent Ductus Arteriosus in the Preterm Infant.

The ductus arteriosus is a muscular artery connecting two elastic arteries with different resistances. It is a normal fetal structure that only becomes pathological if it remains patent after birth. A varied clinical impact is observed as some neonates may be asymptomatic, symptoms may be deferred until later in life, or the infant may be overtly symptomatic and present as early as the first days of life.

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.