Congenital Cardiac Lesions Research Articles

Abstract TP231: Incidence of Stroke and Transient Ischemic Attack in Adults With Congenital Heart Disease: A Systematic Review and Meta-Analysis

Introduction: Congenital cardiac lesions result in abnormal flow patterns that increase risk of cerebrovascular events. We investigated the incidence of stroke and transient ischemic attack (TIA) in adults with congenital heart disease (ACHD). Methods: A systematic review was performed per PRISMA guidelines to identify all studies reporting the incidence of stroke and TIA in ACHD. Two independent reviewers screened studies, which were included if patients were age 16 and older with congenital heart disease, and if the outcome was stroke or TIA. Random-effects meta-analysis was conducted to estimate the pooled incidence rate of stroke and TIA with 95% confidence intervals. The Newcastle-Ottawa Scale for risk of bias was applied. This systematic review is registered (CRD42022322144). Results: Of 11,028 identified abstracts, 27 studies met inclusion criteria. 22 studies reported mean or median age, which was less than 60 years in 18 studies. 39 estimates of incidence rates from 24 studies were entered in the meta-analysis. The pooled incidence rate estimate of stroke and TIA was 0.58 per 100 person years, with significant heterogeneity between studies (95% CI 0.39-0.86, I2 97.8%, p<0.001). Secondary analyses were performed stratified by CHD and outcome subtype. The pooled incidence rate of ischemic stroke was estimated at 0.59 per 100 person years (95% CI 0.36-0.95, I2 98%, p=0.01). Conclusions: This meta-analysis found that the incidence rate of stroke and TIA is higher in ACHD than age-adjusted global estimates, and similar to the American Heart Association reported global ischemic stroke incidence rate for individuals >60 years (0.52 per 100 person years).

3D Printed Cardiac Models as an Adjunct to Traditional Teaching of Anatomy in Congenital Heart Disease—A Randomised Controlled Study

Three-dimensional (3D) printed cardiac models are increasingly being used for medical education, simulation and training, communication, surgical planning and research. Given the complexities of congenital cardiac anatomy, 3D printing is well suited as an adjunct to traditional teaching methods. This study aims to explore the influence of 3D printed cardiac models as a teaching aid for nurses and paediatric trainees. We hypothesise that using 3D models as an adjunct to didactic teaching methods improves knowledge and confidence levels of participants, regardless of their cardiology experience. A prospective randomised study was performed recruiting paediatric nurses and doctors at a tertiary paediatric hospital. All participants undertook traditional congenital cardiac teaching describing normal cardiac anatomy and seven congenital lesions of increasing complexity (atrial septal defect, ventricular septal defect, vascular ring, partial anomalous pulmonary venous return, tetralogy of Fallot, transposition of the great arteries, and double outlet right ventricle). The intervention group received an additional recorded demonstration while handling 3D printed models of a normal heart and the same lesions. Pre- and post-intervention assessments were completed using a subjective Likert-scale questionnaire and objective multiple-choice examination. A total of 73 health practitioners (30 cardiac nurses and 43 paediatric trainees) were included. Subjective knowledge and confidence levels substantially improved in the intervention group (both p<0.001), with no differences observed in the control group. Greater improvement in both subjective and objective post-test scores was observed in the intervention group. A pronounced difference between pre- and post-teaching objective examination scores was found in both groups (p=0.002), with larger improvements observed in the intervention group. The mean score in the intervention group after teaching increased by 4.27 (21.4% improvement), as opposed to 2.28 (11.4% improvement) in the control group. There was no difference in pre-test score or post-test improvement based on previous cardiology experience. Three-dimensional (3D) printed cardiac models, when used as an adjunct to traditional teaching methods, substantially improve knowledge and confidence levels of health professionals on a range of congenital cardiac lesions. These models enhance the learners' educational experience and understanding of cardiac anatomy by overcoming the limitation of two-dimensional representations of 3D structures.

Morphogenetic processes in the development and evolution of the arteries of the pharyngeal arches: their relations to congenital cardiovascular malformations.

The heart and aortic arch arteries in amniotes form a double circulation, taking oxygenated blood from the heart to the body and deoxygenated blood to the lungs. These major vessels are formed in embryonic development from a series of paired and symmetrical arteries that undergo a complex remodelling process to form the asymmetric arch arteries in the adult. These embryonic arteries form in the pharyngeal arches, which are symmetrical bulges on the lateral surface of the head. The pharyngeal arches, and their associated arteries, are found in all classes of vertebrates, but the number varies, typically with the number of arches reducing through evolution. For example, jawed vertebrates have six pairs of pharyngeal arch arteries but amniotes, a clade of tetrapod vertebrates, have five pairs. This had led to the unusual numbering system attributed to each of the pharyngeal arch arteries in amniotes (1, 2, 3, 4, and 6). We, therefore, propose that these instead be given names to reflect the vessel: mandibular (1st), hyoid (2nd), carotid (3rd), aortic (4th) and pulmonary (most caudal). Aberrant arch artery formation or remodelling leads to life-threatening congenital cardiovascular malformations, such as interruption of the aortic arch, cervical origin of arteries, and vascular rings. We discuss why an alleged fifth arch artery has erroneously been used to interpret congenital cardiac lesions, which are better explained as abnormal collateral channels, or remodelling of the aortic sac.

A revised terminology for the pharyngeal arches and the arch arteries.

The pharyngeal arches are a series of bulges found on the lateral surface of the head of vertebrate embryos. In humans, and other amniotes, there are five pharyngeal arches and traditionally these have been labelled from cranial to caudal-1, 2, 3, 4 and 6. This numbering is odd-there is no '5'. Two reasons have been given for this. One is that during development, a 'fifth' arch forms transiently but is not fully realised. The second is that this numbering fits with the evolutionary history of the pharyngeal arches. Recent studies, however, have shown that neither of these justifications have basis. The traditional labelling is problematic as it causes confusion to those trying to understand the development of the pharyngeal arches. In particular, it creates difficulties in the field of congenital cardiac malformations, where it is common to find congenital cardiac lesions interpreted on the basis of persistence of the postulated arteries of the fifth arch. To resolve these problems and to take account of the recent studies that have clarified pharyngeal arch development, we propose a new terminology for the pharyngeal arches. In this revised scheme, the pharyngeal arches are to be labelled as follows-the first, most cranial, the mandibular (M), the second, the hyoid (H), the third, the carotid (C), the fourth, the aortic (A) and the last, most caudal, the pulmonary (P).

Postcardiotomy extracorporeal membrane oxygenation in patients with congenital heart disease; the effect of place of initiation.

Postcardiotomy extracorporeal membrane oxygenation (ECMO) in pediatric patients can be affected by the place of initiation, either in the operating room (OR) or the pediatric cardiac intensive care unit (PCICU). This study aimed to characterize and compare patients who had postcardiotomy ECMO initiation in the OR or PCICU and evaluate risk factors for hospital mortality. This retrospective study included 103 patients who required postcardiotomy ECMO support after the repair of congenital cardiac lesions from 2010 to 2022. Patients were grouped according to the place of ECMO insertion into two groups. Group 1 (n = 69) had ECMO insertion in the OR, and Group 2 (n = 34) had ECMO insertion in the PCICU. Cardiac arrest occurred significantly more often in patients with ECMO insertion in the PCICU (21 (61.76%) vs. 13 (18.84%); p < 0.001). Pre-ECMO lactate levels, pH, VIS, base deficit, and PaO2 did not differ between the groups. Re-exploration for bleeding was significantly higher in Group 1 (32 (46.38%) vs. 8 (23.53%); p = 0.03). Cannula repositioning (4 (11.76%) v. 2 (2.90%); p = 0.09) and mechanical ventilation time were nonsignificantly higher in Group 2 (19.5 (10-31) vs. 11 (5-25) days; p = 0.07). No difference in mortality was found between groups (42 (60.87%) vs. 23 (67.65%), p = 0.50). By multivariable analysis, elevated lactate on ECMO and low pH before ECMO were associated with mortality. ECMO insertion in the OR has a comparable mortality rate to PCICU insertion. Pre-ECMO low pH and high lactate during ECMO could predict mortality.

A Biomimetic Approach Utilizing Pulsatile Perfusion Generates Contractile Vascular Grafts.

Surgical implantation of decellularized cadaveric arteries is routinely used to treat right-sided congenital cardiac lesions. These acellular conduits lack the capacity for somatic growth and are prone to stenosis and calcification, necessitating multiple operations throughout childhood. Islet-1+ cardiovascular progenitor cells (CPCs) have demonstrated the capacity for differentiation into all cell types of the heart and outflow tracts. We hypothesize that CPC seeding of decellularized pulmonary arteries and bioreactor culture under physiologic flow conditions will drive vascular differentiation of CPCs and result in a conduit more suitable for implantation and long-term growth. We began by decellularizing ovine pulmonary arteries and characterizing the composition of the extracellular matrix (ECM). Hemodynamic testing of decellularized vessels in a custom bioreactor was used to define the scaffold mechanical properties over a range of pressures and flow rates. Next, our expanded ovine CPCs were suspended in growth media and injected intramurally into decellularized pulmonary arteries that were subsequently cultured in either static or pulsatile cultures. A combination of immunohistochemistry, real-time polymerase chain reaction (PCR), and tissue bath contraction studies were used to evaluate the bioengineered arteries before transplantation. Pulmonary artery patches from the most favorable culture conditions were then implanted into juvenile sheep to provide proof of concept. Hematoxylin and eosin staining indicated complete removal of cell nuclei (n = 9), whereas double-stranded DNA isolation from tissue homogenates showed 99.1% DNA removal (p < 0.01, n = 4). Furthermore, trichrome and elastin staining verified maintenance of collagen and elastin. Immunohistochemistry and PCR analyses (n = 4 per group) confirmed contractile smooth muscle presence on only our 3-week pulsatile scaffolds via presence of calponin 1 and myosin heavy chain 11. Tissue bath studies demonstrated that smooth muscle contraction generated by our 3-week pulsatile scaffolds (2.23 ± 0.19 g, n = 4) is comparable with native tissue contraction strength (2.78 ± 0.06 g, n = 4). Ovine transplantation confirmed that our graft can be safely implanted, retains contractile smooth muscle cells, and recruits native endothelium. Longer duration of physiologic pulsatile culture drives differentiation of CPCs seeded on ECM conduits toward a mature, contractile phenotype that is maintained for several weeks in vivo. Longer term studies to assess somatic growth potential are needed. Impact statement The current field of vascular transplantation relies on cadaveric and synthetic grafts to treat right-sided congenital cardiac lesions. These grafts do not grow somatically with our patients. This results in multiple reoperations throughout childhood to increase the size of the graft. Our bioengineered alternative demonstrates successful implantation, contractile smooth muscle cells, and a native endothelial layer. This research demonstrates a pilot study confirming the viability of a bioengineered alternative to the current standard of care in the field of vascular transplantation.

Inhaled Nitric Oxide and Higher Necrotizing Enterocolitis Rates in Congenital Heart Disease Patients

IntroductionCertain congenital cardiac lesions are at increased risk for the development of necrotizing enterocolitis (NEC). These patients are often reliant on pulmonary and systemic vasomodulators to maintain adequate perfusion and oxygenation. This study sought to determine whether pulmonary or systemic vasodilator treatment is protective against the development of NEC in this population. MethodsWe utilized International Classification of Diseases (ICD) codes to identify high risk congenital cardiac disease patients ≤6 mo of age, cared for at a tertiary children's hospital between January 2011 and January 2021. Cardiac anomalies were stratified into ductal dependent (pulmonary DD-P or systemic DD-S) or independent lesions. The rate of NEC development in those who received vasodilators (inhaled nitric oxide [iNO], pulmonary vasodilators, systemic vasodilators) was compared to controls in a multivariate analysis. ResultsOf the 352 patients, who met inclusion criteria, 77.6% had ductal dependent lesions (DD-S 41.9%, DD-P 35.7%), 19.5% received iNO, and 37.5% received other vasodilatory drugs. The overall NEC rate was 15.1%. On univariate analysis, DD-S, iNO use, and systemic vasodilators was associated with a significantly higher risk of NEC, while DD-P was associated with lower NEC risk. On multivariate analysis, only iNO (odds ratio 2.725, confidence interval [1.36-5.44]) and DD-S (odds ratio 2.279, confidence interval [1.02-5.11]) were independent risk factors for NEC. ConclusionsIn patients with at-risk congenital cardiac disease lesions, a ductus dependent systemic circulation or iNO treatment is associated with an increased risk of developing NEC. The presence of iNO or DD-S should be utilized as markers of increased risk both in the prevention and workup of suspected NEC.

Right ventricular cardiac resynchronization therapy in patients with right ventricular conduction delay and heart failure

Right ventricular cardiac resynchronization therapy in patients with right ventricular conduction delay and heart failure

Spectrum of Grown up Congenital Heart (GUCH) at Armed Forces Institute of Cardiology/ National Institute of Heart Diease: 10 years Experience

Objective: To determine the spectrum of Grown up Congenital Heart (GUCH) at Armed Forces Institute of Cardiology/National Institute of Heart Dieases.&#x0D; Study Design: Descriptive cross sectional study.&#x0D; Place and Duration of Study: Paediatric Cardiac Surgery and Obstetric department of Armed Force Institute of Cardiology/National Institute of Heart Diease, Rawalpindi Pakistan, from 2011 to 2021.&#x0D; Methodology: It was a descriptive cross sectional study conducted at Paediatric Cardiology, Paediatric Cardiac Surgery and Obstetric department of AFIC/NIHD. After taking informed consent, a total of 1344 patients fulfilling inclusion and exclusion criteria with non probability consecutive sampling were enrolled in study from 2011 to 2021. Diagnosis, transthoracic echocardiography findings, procedural details (cardiac catheterization/cardiac surgery/obstetrical intervention) and outcome of all patients were noted.&#x0D; Results: Out of 1344 patients, 700 patients had cardiac catheterization. Among patients who underwent cardiac cath, 304(43%)were males and 396(56.6%) were females. 279(39%) diagnostic and 421(61%) cardiac interventional procedures were done.Most common procedure done was ASD device closure in 227(32%) patients. Complications were seen in 29(4%) patients and mortality in 3(0.4%) patients. There were a total of 188 patients who underwent congenital cardiac surgery. Out of patients who underwent congenital cardiac surgery procedures, 96(51%) were male and 92(49%) were female patients. Major complications were observed in 51(25%) patients and the mortality was 11(5%).There were total of 456 patients in obstetrics department. Out of 456 patients, 54(11%) were with congenital cardiac lesions .The most common defect was VSD 27(6%)patients and dilated cardiomyopathy (DCM) 27(6%) patients followed by ASD and TOF. The maternal and fetal mortality was12(2.7%) and 22(5.5%) respectively.&#x0D; Conclusion:With immense advancement in diagnosis and management of congenital cardiac diseases, a significant number of patients remain undiagnosed and untreated till the time they present as GUCH. Most common presentation is ASD which is mostly manageable by transcatheter intervention, however late presentation carries a risk of complications like pulmonary hypertension.

Abstract 14923: A Special Type Aortic Left Ventricular Tunnel With Bicuspid Aortic Valve: A Case Report

Introduction: Aortic-left ventricular tunnel (ALVT) is an extremely rare congenital cardiac lesion that is an abnormal passage connecting the ascending aorta to the left ventricle. ALVT can be diagnosed by the use of real-time two-dimensional and continuous-wave Doppler echocardiography. Case presentation: A two-year and seven-month-old boy with recently diagnosed influenza presented to the hospital because of severe tachypnea and fatigue after running. Physical examination revealed a grade 3/6 systolic and diastolic murmur at the second and third left intercostal space. Transthoracic echocardiography (GE E95 with a 5-MHz scanning head) demonstrated that the aortic valve was a bicuspid aortic valve (BAV) with mild-moderate insufficiency during diastole, the peak systolic velocity (PSV) and gradient of aortic valve were 1.9m/s and 14mmHg, respectively; The aortic sinotubular junction (ASJ) was stenotic, the PSV and gradient of ASJ were 2.7m/s and 29mmHg, respectively; A tubular-like structure originated in the outlet septum beneath the right coronary cusp and terminated in the ascending aorta superior to the right coronary aortic sinus (Fig. A, B), CDFI and continuous-wave Doppler showed unobstructed to-and-fro (systolic and diastolic) flow in the tunnel with a high-velocity color signal that aliased during systole (Fig. C-E). Clinical considerations for congenital heart disease: ALVT combines BAV with aortic insufficiency and supravalvar aortic stenosis. The patient was referred for possible surgical correction. Discussion: In this patient, the type of ALVT does not belong to any one of Hovaguimian’s classifications. Two-dimensional echocardiography can display the tunnel-like structure of ALVT in real-time, and CDFI can identify that two-stage bidirectional shunted blood flow, systolic ventricular ejection occurs through both the tunnel and the semilunar valve, and the diastolic flow reverses from the aorta to ventricle via ALVT.

Managing uncertainty in decision-making of common congenital cardiac defects.

Decision-making in congenital cardiac care, although sometimes appearing simple, may prove challenging due to lack of data, uncertainty about outcomes, underlying heuristics, and potential biases in how we reach decisions. We report on the decision-making complexities and uncertainty in management of five commonly encountered congenital cardiac problems: indications for and timing of treatment of subaortic stenosis, closure or observation of small ventricular septal defects, management of new-onset aortic regurgitation in ventricular septal defect, management of anomalous aortic origin of a coronary artery in an asymptomatic patient, and indications for operating on a single anomalously draining pulmonary vein. The strategy underpinning each lesion and the indications for and against intervention are outlined. Areas of uncertainty are clearly delineated. Even in the presence of "simple" congenital cardiac lesions, uncertainty exists in decision-making. Awareness and acceptance of uncertainty is first required to facilitate efforts at mitigation. Strategies to circumvent uncertainty in these scenarios include greater availability of evidence-based medicine, larger datasets, standardised clinical assessment and management protocols, and potentially the incorporation of artificial intelligence into the decision-making process.

Inhaled Nitric Oxide Treatment Is Associated with Higher Rates of Necrotizing Enterocolitis in At-risk Congenital Cardiac Disease Patients

INTRODUCTION: Certain congenital cardiac lesions are at increased risk for the development of necrotizing enterocolitis (NEC). These patients with these lesions are often reliant on pulmonary and systemic vasomodulators to maintain adequate perfusion and oxygenation. This study sought to determine whether pulmonary or systemic vasodilator treatment is protective against the development of NEC in this population. METHODS: We used ICD codes to identify high-risk congenital cardiac disease patients ≤6 months of age, cared for at a tertiary children’s hospital between January 2011 and January 2021. Cardiac anomalies were stratified into ductal-dependent (pulmonary DD-P or systemic DD-S) or independent lesions. The rate of NEC development in those who received vasodilators (inhaled nitric oxide [iNO], pulmonary vasodilators, systemic vasodilators) was compared with controls in a multivariate analysis. RESULTS: Of the 353 patients who met inclusion criteria, 77.6% had ductal-dependent lesions (DD-S 41.9%, DD-P 35.7%), 19.5% received iNO, and 51.8% received other vasodilatory drugs. The overall NEC rate was 15.3%. On univariate analysis, DD-S, iNO use, and systemic vasodilators had a significantly higher risk of NEC, and DD-P was associated with lower NEC risk (Figure). On multivariate analysis, only iNO (odds ratio 2.725 [CI 1.36 to 5.44]) and DD-S (odds ratio 2.279 [CI 1.02 to 5.11]) were independent risk factors for NEC.FigureCONCLUSION: In patients with at-risk congenital cardiac disease lesions, a ductus-dependent systemic circulation and/or iNO treatment increases the risk of developing NEC. The presence of iNO or DD-S should be used as a marker of increased risk both in the prevention and workup of suspected NEC.

Do we really need a new classification for cor triatriatum sinister?

Current classifications of cor triatriatum sinister (CTS) do not address the associated heart defects or single ventricle pathology. Therefore, these classifications are not prognostic classifications and only describe the anatomy and the pulmonary venous drainage. The proposed classification considered the associated congenital cardiac lesions and the single ventricle pathology, therefore, it could have prognostic value. Future multicenter studies are required to measure the performance of this classification and its prognostic value in patients with CTS.

Treatment of advanced heart failure in adults with congenital heart disease: a narrative review and clinical cases.

The number of adults with congenital heart disease (ACHD) is increasing worldwide. Almost all congenital cardiac lesions can be successfully treated due to the progress in neonatal surgery and pediatric cardiology with a high likelihood of surviving until adulthood. However, ACHD frequently develop sequelae related to the initial cardiac anomaly. Heart failure (HF) is one of the most common complications associated with a high morbidity and mortality. The authors did search the PubMed database regarding relevant content covering publications up to March 2022. Relevant manuscripts were classified according to the impact factor of the journal, being a guideline manuscript, a position paper by a society or a comprehensive review of the current literature. Optimal HF treatment remains an unmet need in ACHD. In particular, advanced HF therapy with cardiac resynchronization therapy, ventricular assist devices or organ transplantation is still very different and more specific in ACHD compared to non-ACHD. This review aims to compile international views and evidence from the literatures on the treatment of advanced HF in ACHD. Current challenges, but also the success of different treatment strategies in ACHD are illustrated by clinical cases. The main finding of the review is that data is still scarce regarding ACHD with advanced HF and international efforts to collect data regarding these patients needed to improve the current standard of care.

Transcatheter interventions in patients with a Fontan circulation: Current practice and future developments.

The Fontan operation represents the last of multiple steps that are offered a wide range of congenital cardiac lesions with a single ventricle (SV) physiology. Nowadays this surgical program consists of a total cavopulmonary connection (TCPC), by anastomosing systemic veins to the pulmonary arteries (PAs), excluding the right-sided circulation from the heart. As a result of imaging, surgical, percutaneous, and critical care improvements, survival in this population has steadily increased. However, the Fontan physiology chronically increases systemic venous pressure causing systemic venous congestion and decreased cardiac output, exposing patients to the failure of the Fontan circulation (FC), which is associated with a wide variety of clinical complications such as liver disease, cyanosis, thromboembolism, protein-losing enteropathy (PLE), plastic bronchitis (PB), and renal dysfunction, ultimately resulting in an increased risk of exercise intolerance, arrhythmias, and premature death. The pathophysiology of the failing Fontan is complex and multifactorial; i.e., caused by the single ventricle dysfunction (diastolic/systolic failure, arrhythmias, AV valve regurgitation, etc.) or caused by the specific circulation (conduits, pulmonary vessels, etc.). The treatment is still challenging and may include multiple options and tools. Among the possible options, today, interventional catheterization is a reliable option, through which different procedures can target various failing elements of the FC. In this review, we aim to provide an overview of indications, techniques, and results of transcatheter options to treat cavopulmonary stenosis, collaterals, impaired lymphatic drainage, and the management of the fenestration, as well as to explore the recent advancements and clinical applications of transcatheter cavopulmonary connections, percutaneous valvular treatments, and to discuss the future perspectives of percutaneous therapies in the Fontan population.

Noonan syndrome associated with hypoplastic left heart syndrome.

Noonan syndrome is an inherited disorder caused by alterations in the RAS-MAPK pathway. There have been several identified genotype-phenotype associations made with respect to congenital cardiac lesions and Noonan syndrome variants, but limited data exist regarding single ventricle disease in this population. Here, we report two patients with PTPN11-related Noonan syndrome and hypoplastic left heart syndrome variants.

Current development of bovine jugular vein conduit for right ventricular outflow tract reconstruction.

Right ventricular outflow tract (RVOT) reconstruction is a common surgical method to treat congenital cardiac lesions, and bovine jugular vein conduit (BJVC) has become a prevalent candidate of prosthetic material for this procedure since 1999. Although many clinical studies have shown encouraging results on BJVCs, complications such as stenosis, aneurysmal dilatation, valve insufficiency, and infective endocarditis revealed in other clinical outcomes still remain problematic. This review describes the underlying mechanisms causing respective complications, and summarizes the current technological development that may address those causative factors. Novel crosslinking agents, decellularization techniques, conduit coatings, and physical reinforcement materials have improved the performances of BJVCs. The authors expect that the breakthroughs in the clinical application of BJVC may come from new genetic research findings and advanced characterization apparatuses and bioreactors, and are optimistic that the BJVC will in the future provide sophisticated therapies for next-generation RVOT reconstruction.

The ductus arteriosus in neonates with critical congenital heart disease.

The ductus arteriosus (DA) has a paradoxical biological role in neonates with congenital heart disease (CHD) and can present with significant management challenges. Critical congenital cardiac lesions rely on the patency of the DA to provide either systemic or pulmonary blood flow. A patent DA (PDA) that remains open can also have adverse consequences depending on the degree of systemic to-pulmonary shunting and volume of ductal steal. As such, the presence of a PDA may pose a challenge in the medical management and timing of surgical repair. In this perspective article, we provide an understanding of the role of the DA in the circulatory system in neonates with CHD and discuss traditional and emerging approaches to support the pulmonary and systemic circulations with manipulation of the DA.

Ductus arteriosus: more than just the patent ductus arteriosus.

The ductus arteriosus is important to fetal circulation. Failure to close at birth is a common event. In this educational pictorial essay, we illustrate the association of the ductus arteriosus with a variety of congenital cardiac, vascular and pulmonary lesions. These lesions can impact the systemic circulation, the pulmonary circulation or the airway and include coarctation of the aorta, ductal origin of the pulmonary artery and vascular rings.

Considerations in Critical-Care and Anesthetic Management of Adult Patients Living With Fontan Circulation

The Fontan procedure is a staged palliation for various complex congenital cardiac lesions, including tricuspid atresia, pulmonary atresia, hypoplastic left heart syndrome, and double-inlet left ventricle, all of which involve a functional single-ventricle physiology. The complexity of the patients' original anatomy combined with the anatomic and physiologic consequences of the Fontan circulation creates challenges. Teens and adults living with Fontan palliation will need perioperative support for noncardiac surgery, peripartum management for labour and delivery, interventions related to their structural heart disease, electrophysiology procedures, pacemakers, cardioversions, cardiac surgery, transplantation, and advanced mechanical support. This review focuses on the anesthetic and intensive care unit (ICU) management of these patients during their perioperative journey, with an emphasis on the continuity of preintervention planning, referral pathways, and postintervention ICU management. Requests for recipes and doses of medications are frequent; however, as in normal anesthesia and ICU practice, the method of anesthesia and dosing are dependent on the presenting medical/surgical conditions and the underlying anatomy and physiologic reserve. A patient with Fontan palliation in their early 20s attending school full-time with a cavopulmonary connection is likely to have more reserve than a patient in their late 40s with an atriopulmonary Fontan at home waiting for a heart transplant. Each case will require an anesthetic and critical care plan tailored to the situation. The critical care environment is a natural extension of the anesthetic management of a patient, with complex considerations for a patient with Fontan palliation.