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 4th 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. METHODS: Transcatheter fenestrated atrial septal defect closures (Occlutech GmbH, Jena, Germany) were performed on a compassionate-use basis in 5 consecutive adult patients with atrial septal defect-associated pulmonary arterial hypertension and severe heart failure with prohibitive surgical mortality risks. Change in systemic oxygen saturation, 6-minute walk test, NYHA class, echocardiographic and haemodynamic parameters were used as parameters of outcome. RESULTS: All patients were female, mean age 48.8 ± 13.5 years, followed up for a median of 29 months (max 64 months). Significant improvements observed in the 6-minute walk test, and oxygen saturation comparing day 0 time point to all other follow-up time points data (B = 1.32, SE = 0.28, t (22.7) = -4.77, p = 0.0001); and in the haemodynamic data (including pulmonary vascular resistance and pulmonary pressure) (B = -0.60, SE = 0.22, t (40.2) = 2.74, p = .009). All patients showed improved right ventricular size and function along with NYHA class. There were no procedure-related complications. CONCLUSION: Fenestrated atrial septal defect closure is feasible in adults with decompensated heart failure and atrial septal defect-associated pulmonary arterial hypertension. It results in sustained haemodynamic and functional improvement.

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. RECENT FINDINGS: The understanding of functional morphology of AVSDs has improved significantly with detailed 3D echocardiographic evaluation of the atrioventricular junction and valve morphology. As prenatal detection of AVSDs has increased significantly, it has become the most common fetal cardiac diagnosis enabling antenatal counseling and delivery planning. Advances in diagnosis and perioperative care have resulted in optimal outcomes. The diagnosis and management of AVSDs have improved over the years with enhanced understanding of anatomy and perioperative care resulting in optimal short and long-term outcomes.

Correction of sinus venosus atrial septal defects with the 10 zig covered Cheatham-platinum stent - An international registry.

BACKGROUND: Covered stent correction of sinus venosus ASDs (SVASD) is a relatively new technique. Challenges include anchoring a sufficiently long stent in a nonstenotic superior vena cava (SVC) and expanding the stent at the wider SVC-RA junction without obstructing the anomalous right upper pulmonary vein (RUPV). The 10-zig covered Cheatham-platinum (CCP) stent has the advantage of being available in lengths of 5-11 cm and dilatable to 34 mm in diameter. METHODS: An international registry reviewed the outcomes of 10-zig CCP stents in 75 patients aged 11.4-75.9 years (median 45.4) from March 2016. Additional stents were used to anchor the stent in the SVC or close residual shunts in 33/75. An additional stent was placed in 4/5 (80%) with 5/5.5 cm CCPs, 18/29 (62%) with 6 cm CCPs, 5/18 (28%) with 7 cm CCPs, 5/22 (23%) with 7.5/8 cm CCPs and 0/1 with an 11 cm CCP. A "protective" balloon catheter was inflated in the RUPV in 17. RESULTS: Early stent embolization in two patients required surgical removal and defect repair and tamponade was drained in one patient. The CT at 3 months showed occlusion of the RUPV in one patient. Follow up is from 2 months to 5.1 years (median 1.8 years). QP:QS has reduced from 2.5 ± 0.5 to 1.2 ± 0.36 (p < .001) and RVEDVi from 149.1 ± 35.4 to 95.6 ± 21.43 ml/m2 (p < .001). CONCLUSIONS: Ten-zig CCPs of 7-8 cm appear to provide reliable SVASD closure with a low requirement for additional stents. Careful selection of patients and meticulous attention to detail is required to avoid complications.

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.

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.

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. We describe features of preoperative care, surgical intervention, and postoperative course that enabled this infant to survive.

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.

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. This case is an example of how patients with congenital heart disease can be palliated in creative ways through thoughtful collaboration between surgical and interventional cardiology teams.

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. Within this context, it is unimaginable for even the highly trained professionals to consider saturation as low as 65% as acceptable. However, all available data suggests that newborns can thrive in a hypoxemic environment as they are conditioned to withstand extreme low saturations in the fetal environment. An approach utilizing the benefits of the hypoxic conditioning would prompt the practice of optimal targeted oxygen saturation range in the clinical management of the newborn. Our current understanding of cyanotic congenital heart disease and the physiology of single ventricle circulation, where oxygen saturation in mid 70s is acceptable, is supported by clinical and animal studies. This article argues the need to challenge our current acceptable target oxygen saturation in the newborn and provides the reasoning behind accepting lower target oxygen levels in the critically ill newborn. Challenging the current practice is expected to open a debate paving the way to understand the risks of high target oxygen levels in the newborn compared with the benefits of permissive hypoxia in avoiding the associated morbidity and mortality of oxygen radical injury.

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.

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. The development of pulmonary arteriovenous malformations (PAVMs) in PCD is not an established association. PAVMs as the etiology for hypoxemia may have been overlooked due to the lack of awareness of this rare association. We present a child with diagnosis of PCD with significant hypoxemia in the absence of bronchiectasis, found to have diffuse bilateral PAVMs and discuss possible physiopathologic mechanisms.

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.

Atrial septostomy with a predefined diameter using a novel occlutech atrial flow regulator improves symptoms and cardiac index in patients with severe pulmonary arterial hypertension.

OBJECTIVES: A novel Occlutech atrial flow regulator (AFR) implantation gives an atrial septal predefined predictable fenestration. BACKGROUND: Atrial septostomy relieves syncope in pulmonary arterial hypertension (PAH) by improving left heart filling, cardiac output and systemic oxygen transport despite hypoxia. Symptoms recur when small fenestrations close spontaneously. METHODS: AFR was implanted after informed consent in patients with severe PAH presenting with syncope and right heart failure. Symptoms, hemodynamics, echocardiographic parameters, brain natriuretic peptide (BNP) levels and device patency were serially documented. RESULTS: Twelve patients aged 28.3 ± 8.5 years with severe PAH underwent AFR implantation. All procedures were successful without any major complications. All patients had relief of syncope and 6-min walk distance improved significantly from 377.3 ± 33.2 to 423 ± 31.32 m. The cardiac index (2.36 ± 0.52 to 2.89 ± 0.56 L/min/m2 ) and systemic oxygen transport (367.5 ± 75.5 to 428.0 ± 67.1 ml/min/m2 ) also showed a significant improvement. Inferior caval vein congestion and pericardial effusion reduced due to improvement in heart failure, but other echocardiographic parameters of right ventricular function did not show significant change. The reduction in BNP levels too did not reach statistical significance. The device was patent in all patients at a median follow-up of 189 days (range 10-296 days) resulting in a significant reduction of oxygen saturations from 98 ± 0.18 to 85.26 ± 2.86% after exercise. CONCLUSIONS: AFR implantation was feasible and safe in all patients with PAH. There was a significant improvement of symptoms, six-minute walk distance, cardiac index and systemic oxygen transport. The device maintained patency in short-term follow-up and the resultant hypoxia was tolerated well.