Complex Congenital Heart Lesions Research Articles

Right ventricle-dependent coronary circulation diagnosed by non-invasive ferumoxytol-enhanced 4D cardiac magnetic resonance angiography in pulmonary atresia with intact ventricular septum.

Pulmonary atresia with intact ventricular septum is a complex cyanotic congenital heart lesion with the potential for myocardial ischaemia due to the presence of coronary artery anomalies. We present a case of a two-day-old baby with postnatal diagnosis of pulmonary atresia with intact ventricular septum in whom non-invasive ferumoxytol-enhanced 4D cardiac magnetic resonance angiography was used for the assessment of coronary artery anatomy.

Trajectory of Left Ventricular Remodeling in Children With Valvar Aortic Stenosis Following Balloon Aortic Valvuloplasty

Aortic valve stenosis is the most common type of congenital left ventricular (LV) outflow tract obstruction. Balloon aortic valvuloplasty (BAV) has become the first-line treatment pathway in many centers. Our aim was to assess the trajectory of LV remodeling following BAV in children and its relationship to residual aortic stenosis (AS) and insufficiency (AI). Children <18 years of age who underwent BAV for isolated aortic stenosis from 2004 to 2012 were eligible for inclusion. Those with AI before BAV, other complex congenital heart lesions, or <2 accessible follow-up echocardiograms were excluded. Baseline and serial echocardiographic data pertaining to aortic valve and LV size and function were retrospectively collected through December 2017 or the first reintervention. Longitudinal data was assessed using per-patient time profiles with superimposed trend lines using locally estimated scatterplot smoothing. Associations with reintervention or death were also evaluated. Among the 98 enrolled children, the median (interquartile range) age at BAV was 2.8 months (0.2-75). The median (interquartile range) follow-up was 6.8 years (1.9-9.0). Children with predominantly residual AI (n=11) demonstrated progressive increases in their LV end-diastolic dimension Z score within the first 3 years after the BAV, followed by a plateau (P<0.001). Their mean LV circumferential and longitudinal strain values remained within the normal range but lower than in the non-AI group (P<0.001 and P=0.001, respectively). Children with predominantly residual aortic stenosis (n=44) had no changes in LV dimensions but had a rapid early increase in mean LV circumferential and longitudinal strain. The cumulative proportion (95% CI) of reintervention at 5 years following BAV was 33.7% (23.6%-42.4%). Our study demonstrates that LV remodeling occurs mainly during the first 3 years in children with predominantly residual AI after BAV, with no subsequent significant functional changes over the medium term. These data improve our understanding of expected patient trajectories and thus may inform decisions on the timing of reintervention.

C94. The characteristics and outcome of pediatric patients with congenital heart disease (CHD) infected by coronavirus disease 2019 (COVID-19) between march 2020 to february 2021 at sanglah hospital: a case series of 12 patients

BackgroundCoronavirus disease 2019 (COVID-19) has a complex connection to cardiovascular system. COVID-19 infection has been proven to cause significant dysfunction in lung, heart, and endothelial cells. Therefore, patients with complex congenital heart lesions have a high risk of developing severe complications and worse outcomes. This paper aims to determine the characteristics and outcome of pediatric congenital heart disease (CHD) patients infected by COVID-19 at Sanglah hospital.Case SummaryWithin the period of March 2020 to February 2021, there were 12 cases of pediatric CHD accompanied by COVID-19 infection, which consists of 8 females and 4 males within the age of 6 days to 18 years. Based upon symptomatic CHD classification, there were 4 cyanotic CHD cases and 1 acyanotic case, respiratory problems were reported in all 5 cases. These are pneumonia in 4 cases and respiratory distress caused by hyaline membrane disease (HMD) in 1 case. 2 cases require mechanical ventilation. The outcome of all inpatient cases were favorable.DiscussionTo date, there are only few scientific evidence detailing how COVID-19 affects CHD. We were unable to calculate the correlation analysis since the population sample was too small. The most severe cases that requires mechanical ventilation had comorbidities in the form of malnutrition and down syndrome. From the perspective of pathophysiology, one of the most important factor determining outcome was the severity of CHD itself. Other contributing factors were COVID-19’s destructive effect on heart tissue and co-infection by other microorganism.

C94. The characteristics and outcome of pediatric patients with congenital heart disease (CHD) infected by coronavirus disease 2019 (COVID-19) between march 2020 to february 2021 at sanglah hospital: a case series of 12 patients

BackgroundCoronavirus disease 2019 (COVID-19) has a complex connection to cardiovascular system. COVID-19 infection has been proven to cause significant dysfunction in lung, heart, and endothelial cells. Therefore, patients with complex congenital heart lesions have a high risk of developing severe complications and worse outcomes. This paper aims to determine the characteristics and outcome of pediatric congenital heart disease (CHD) patients infected by COVID-19 at Sanglah hospital.Case SummaryWithin the period of March 2020 to February 2021, there were 12 cases of pediatric CHD accompanied by COVID-19 infection, which consists of 8 females and 4 males within the age of 6 days to 18 years. Based upon symptomatic CHD classification, there were 4 cyanotic CHD cases and 1 acyanotic case, respiratory problems were reported in all 5 cases. These are pneumonia in 4 cases and respiratory distress caused by hyaline membrane disease (HMD) in 1 case. 2 cases require mechanical ventilation. The outcome of all inpatient cases were favorable.DiscussionTo date, there are only few scientific evidence detailing how COVID-19 affects CHD. We were unable to calculate the correlation analysis since the population sample was too small. The most severe cases that requires mechanical ventilation had comorbidities in the form of malnutrition and down syndrome. From the perspective of pathophysiology, one of the most important factor determining outcome was the severity of CHD itself. Other contributing factors were COVID-19’s destructive effect on heart tissue and co-infection by other microorganism.

Gradually Progressive Dyspnea and Exercise Intolerance in an Otherwise Active Middle-Aged Woman: Why Was the Presentation of Congenital Heart Disease Delayed?

Patients with congenital heart disease (CHD) increasingly are surviving into adulthood. In the United States alone, there are more than one million adult patients living with CHD with the number increasing about 5% each year. With more than 85% of infants with CHD surviving into adulthood with their disease, encounters with these patients in the operating room for cardiac and noncardiac operative procedures is becoming more commonplace. Most of these patients receive corrective surgery early in life, although some may live with uncorrected CHD with no-to-relatively mild symptoms and present at a later time in life with symptoms of heart failure or pulmonary hypertension. The authors present an adult patient with uncorrected CHD presenting with late onset of heart failure symptoms. The authors also review the patient's complex congenital heart lesion, transesophageal echocardiography findings, and intraoperative management.

Diagnosis and treatment of adults with congenital heart disease.

Approximately 50million adults worldwide have known congenital heart disease (CHD). Among the most common types of CHD defects in adults are atrial septal defects and ventricular septal defects followed by complex congenital heart lesions such as tetralogy of Fallot. Adults with CHDs are more likely to have hypertension, cerebral vascular disease, diabetes and chronic kidney disease than age-matched controls without CHD. Moreover, by the age of 50, adults with CHD are at a greater than 10% risk of experiencing cardiac dysrhythmias and approximately 4% experience sudden death. Consequently, adults with CHD require healthcare that is two- to four-times greater than adults without CHD. This paper discusses the diagnosis and treatment of adults with atrial septal defects, ventricular septal defects and tetralogy of Fallot.

1161 Baseline echocardiographic parameters associated with reintervention in children with aortic valve stenosis following balloon aortic valvuloplasty

Abstract Introduction Aortic valve (AV) stenosis is the most common type of congenital left ventricular outflow tract obstruction. Short-term outcomes following balloon aortic valvuloplasty (BAV) including residual aortic stenosis, aortic insufficiency, and procedural complications have been established. The impact of pre-intervention AV characteristics on long-term outcomes has not been well studied. Purpose The aim of this study was to determine the relationship between the initial parameters on baseline echocardiogram and the time to reintervention in children with AV stenosis following BAV. Methods Children from the newborn period to 18 years of age with AV stenosis who underwent BAV from 2004-2012 were included. Patients with aortic insufficiency prior to BAV, complex congenital heart lesions, or less than two accessible follow-up echocardiograms were excluded. Baseline and serial echocardiographic data pertaining to aortic valve and LV size and function was retrospectively collected until December 2017 or until the first reintervention. Time to reintervention or death was evaluated. Results Among the 98 enrolled patients, the median [IQR] age at BAV was 2.8 months [0.2-75]. The median [IQR] duration of follow-up was 6.8 [1.9-9.0] years. Eighty-nine (83%) patients had bicuspid valve morphology and the median [IQR] peak-to-peak catheterization gradient prior to BAV was 49 [34-65] mmHg. The cumulative proportion [95% CI] of reintervention at 5 years following BAV was 33.7% [23.6%, 42.4%]. Primary indications for reintervention were aortic stenosis (57%), aortic insufficiency (14%), or mixed valve disease (30%). Reinterventions included repeat BAV (49%), AV repair (15%), and AV replacement (36%). Increased LVEF at baseline as well as increased mean LV circumferential strain at baseline were associated with decreased risk of reintervention (HR [95% CI] (1 unit increments): 0.974 [0.959-0.989], p &amp;lt; 0.001; 0.939 [0.884-0.997], p = 0.041 respectively). Increased AV annulus z-score was also associated with decreased risk of reintervention (HR [95% CI] (1 unit increments): 0.806 [0.698-0.93], p = 0.003). Conclusions Our results demonstrate that better left ventricular function at baseline, measured by LVEF and mean LV circumferential strain, is associated with a decreased risk of reintervention in neonates and children following BAV. We have also shown that a bigger AV annulus prior to BAV is associated with a decreased risk of reintervention.

Recent advances in cardiac catheterization for congenital heart disease.

The field of pediatric and adult congenital cardiac catheterization has evolved rapidly in recent years. This review will focus on some of the newer endovascular technological and management strategies now being applied in the pediatric interventional laboratory. Emerging imaging techniques such as three-dimensional (3D) rotational angiography, multi-modal image fusion, 3D printing, and holographic imaging have the potential to enhance our understanding of complex congenital heart lesions for diagnostic or interventional purposes. While fluoroscopy and standard angiography remain procedural cornerstones, improved equipment design has allowed for effective radiation exposure reduction strategies. Innovations in device design and implantation techniques have enabled the application of percutaneous therapies in a wider range of patients, especially those with prohibitive surgical risk. For example, there is growing experience in transcatheter duct occlusion in symptomatic low-weight or premature infants and stent implantation into the right ventricular outflow tract or arterial duct in cyanotic neonates with duct-dependent pulmonary circulations. The application of percutaneous pulmonary valve implantation has been extended to a broader patient population with dysfunctional ‘native’ right ventricular outflow tracts and has spurred the development of novel techniques and devices to solve associated anatomic challenges. Finally, hybrid strategies, combining cardiosurgical and interventional approaches, have enhanced our capabilities to provide care for those with the most complex of lesions while optimizing efficacy and safety.

Right Ventricular Outflow Tract Obstruction: Pulmonary Atresia With Intact Ventricular Septum, Pulmonary Stenosis, and Ebstein's Malformation.

The objectives of this review are to discuss the anatomy, pathophysiology, clinical course, and current treatment strategies for pulmonary atresia with intact ventricular septum, pulmonary stenosis, and Ebstein's anomaly. MEDLINE and PubMed. Considerable advances have been made in management strategies for these complex congenital heart lesions, which have led to improved outcomes.

Trends in Congenital Heart Disease

Congenital heart disease (CHD) is the most common class of major congenital malformations. Although there is slight variation between many population-based studies, CHD occurs in ≈1% of live births, with similar prevalence throughout world,1–3 and in 10% of aborted fetuses.4 It is also the leading cause of mortality from birth defects.5 Approximately one-quarter of the 40 000 children born with CHD annually in the United States require intervention in the first year of life. In the 60 years since the first successful repair of a congenital heart defect using cardiopulmonary bypass in 1953, the accurate diagnosis and effective treatment of even the most complex congenital heart lesions have become standard practice. Critical CHD is often lethal in the absence of treatment, and effective surgical, catheter, and medical therapies have dramatically extended life expectancy. With advances in the survival of patients with CHD, there have been major demographic shifts, so that adult patients with CHD now outnumber children even with complex forms of CHD.6 The expected surgical outcomes for patients with simpler congenital cardiovascular lesions (eg, typical forms of septal defects) have continually improved7 and have long since reached a very high level of excellence. These procedures can now be reasonably viewed as a commodity, which is widely available in the developed world. In treatment of simpler lesions, research is increasingly focused on methods to increase the safety, efficiency, and value of therapeutic procedures, and to provide high-quality alternatives to children in nations with resource-constrained healthcare infrastructure and budgets, as well. On the other end of the spectrum of disease severity, outcomes continue to improve in patients with more complex lesions (Figure 1).8 Survival through childhood is now common even in the most complex and lethal malformations, such as hypoplastic left heart syndrome. …

Cardiac Magnetic Resonance Imaging and Computed Tomography in Newborns With Congenital Heart Disease

Congenital heart disease continues to be a leading cause of neonatal morbidity; however, advancements in diagnostic technology and surgical techniques have markedly improved patient survival. In particular, in-depth noninvasive imaging with computed tomography angiography (CTA) and cardiac magnetic resonance imaging (CMRI) has had a tremendous effect on the amount of information available to the surgeon or interventionalist before any procedure. CTA and CMRI have their own advantages and disadvantages, and this review provides an overview of these technologies, highlighting clinical indications. This article also provides images and videotapes to better highlight our understanding of various complex congenital heart lesions.

Insights gained from three-dimensional imaging modalities for closure of ventricular septal defects.

Ventricular septal defects (VSDs) are the commonest group of congenital heart lesions, accounting for ≈30% of congenital heart defects in liveborn infants.1 Defects in the ventricular septum are classified according to their position when the ventricular septum is viewed from the right ventricle and these may vary in their location, size, and number.2,3 VSDs can occur as isolated lesions or as part of more complex disease, for example, transposition of the great arteries or double outlet right ventricle, where the location and size of such defects assume great importance in planning the optimal mode of surgical repair.4 In most cases, surgical closure remains the mainstay treatment for VSDs, but selected lesions may be amenable to catheter closure.5,6 Cross-sectional echocardiography is the most commonly used noninvasive diagnostic method to delineate the position of a VSD before surgery.7 Multiple sonographic cuts and cross-sectional sweeps of the region of interest are used to build a virtual reconstruction of the three-dimensional (3D) anatomy in the mind of the echocardiographer. However, for the surgeon who deals solely with 3D images there can remain an element of uncertainty when called to operate on the patient. The number, size, location of VSDs, and their relationship to adjacent structures are central for selecting whether the defects might be best closed via the tricuspid valve, via the semilunar valves, or via a ventriculotomy or whether these might not be readily approachable and a palliative pulmonary artery banding is desirable.8,9 In this review, we present key 3D echocardiographic projections and examples of different types of VSDs where the images have a direct effect on the approach taken to VSD closure. Comparison of 3D echocardiography (3DE) with other 3D imaging modalities is also described. Images are presented in an …

3D echocardiography in congenital heart disease: a valuable tool for the surgeon.

Real-time 3D echocardiography has been used increasingly in the assessment of patients with congenital heart disease. A number of studies have confirmed that this modality can be used as a complementary method to delineate morphology and spatial relationships of simple and more complex congenital heart lesions during surgical planning. Communication between the echocardiographer and surgeon can be simplified as simulation of surgical views can be achieved, thus minimizing the potential for error related to mental reconstruction. This review summarizes the available evidence for the role of real-time 3D echocardiography in congenital heart disease as an imaging modality to assist surgeons.

Neurodevelopmental outcomes after congenital heart surgery and strategies for improvement

The present review focuses on neurodevelopmental and neuropsychologic outcomes following neonatal and infant heart surgery for complex congenital heart lesions. The data include recent reports on perioperative MRI and recent results of randomized clinical trials addressing perioperative variables. Advancements in magnetic resonance techniques have reinforced earlier data that newborns with complex congenital heart lesions are frequently born with brain immaturity. Randomized clinical trials have looked at several important perioperative candidate predictors as potential independent risk factors for worsened neurodevelopmental outcomes: no difference was found between regional cerebral perfusion and deep hypothermic circulatory arrest on 1-year outcomes; no difference was found between the modified Blalock-Taussig shunt and the right ventricular to pulmonary artery shunt as part of the Norwood procedure on 14-month outcomes; at 16-year testing for individuals with transposition of the great arteries following the arterial switch operation, no significant difference was found between low-flow cardiopulmonary bypass and deep hypothermic circulatory arrest. Randomized clinical, cross-sectional, and prospective trials have explored robust analyses looking for independent risk factors for worsened neurodevelopmental outcomes. Most of these risk factors are patient-related or socioeconomic, with only a few potentially modifiable.

Successful Hybrid Rescue of Occluded Pulmonary Artery in Pulmonary Atresia

Hybrid cardiac procedures are a result of synergistic cooperation between the cardiologist and surgeon. They are a combination of surgical repair and intraoperative catheter-based interventions, and may provide an effective option for the repair of complex congenital heart lesions. We present a 30-year-old man with pulmonary atresia and a ventricular septal defect. He previously had a Waterston shunt placed at 10 days of age and a right pulmonary artery shunt at 4 years of age. He had ventricular septal defect closure with a 23-mm homograft conduit connecting the right ventricular outflow tract to the main pulmonary artery confluence at 9 years of age. The patient presented to us 21 years later with pulmonary conduit obstruction, right pulmonary artery stenosis, and an occluded left pulmonary artery (LPA; Figures 1, 2A, and 2B). The catheterization demonstrated right ventricular pressure (70% of systemic arterial pressure) at 89/14 mm Hg. There was a 28-mm Hg gradient to the right pulmonary artery, and the LPA was occluded. A 7-mm distal LPA was demonstrated by left pulmonary vein wedge angiogram. Given the long occlusion between the conduit and the lower LPA, we were concerned that the LPA would not be accessible for direct patch augmentation, and hence decided to perform a hybrid procedure; …

Anesthetic management of a consecutive cohort of women with heart disease for labor and delivery

Background The cardiovascular changes of pregnancy may place additional stress upon women with pre-existing heart disease, increasing peripartum morbidity and mortality. The purpose of this descriptive study was to report the anesthetic management of a large cohort of pregnant women with heart disease. Methods The medical records of 522 consecutive parturients (657 pregnancies) with heart disease who delivered at Toronto General Hospital or Mount Sinai Hospital in Toronto, Ontario, Canada between 1986 and 2004 were reviewed. Obstetric, medical and anesthetic management data were collected and the women were stratified by New York Heart Association (NYHA) functional status at delivery. The main outcome of interest was the method of analgesia or anesthesia administered during labor and delivery. Univariate and multivariate analysis was performed to identify risk factors associated with the administration of general anesthesia. Results Of 657 pregnant women, 602 were NYHA 1/2 and 55 were NYHA 3/4 at time of delivery. Epidural analgesia was administered to 84% of NYH 1/2 women and 83% of NYH 3/4. The cesarean section rates were 29% and 31% respectively. The rate of general anesthesia for the entire cohort was 9%. Factors associated with the use of general anesthesia for operative delivery included cesarean delivery (adjusted O.R. 74; 95% CI 9.5, 573), delivering at Toronto General Hospital site (adjusted O.R. 5.5; 95% CI 2.3, 13.3), presence of complex congenital heart lesion (adjusted O.R. 2.3; 95% CI 1.0, 5.4) and each week of premature delivery (adjusted O.R. 1.3; 95% CI 1.1, 1.5). Three percent suffered intrapartum cardiac complications; there was one death. Conclusions Pregnant women with heart disease managed within an organized program may undergo labor and delivery with acceptable rates of complications. Cesarean section, epidural analgesia/anesthesia and general anesthesia rates are similar to those in the general obstetric population.

Magnetic Resonance Imaging in Congenital Heart Disease

Magnetic resonance imaging (MRI) is increasingly being applied to critically ill newborns with congenital heart disease (CHD) with the hope of understanding mechanisms accounting for adverse neurodevelopmental outcome. This powerful technique has opened an unprecedented window into normal development and pathogenesis during fetal life and surrounding surgery. Much as the technological advances of cardiopulmonary bypass, cardiac intensive care, and transcatheter and surgical techniques have translated into improved survival for neonates with complex congenital heart lesions, MRI promises to elucidate mechanisms and measure outcomes in a way that will inform treatment and establish neurointensive care to lessen neurologic morbidity. Many agree that improving neurodevelopmental outcome in CHD is the preeminent challenge to the field.1 MRI studies in CHD have made the important and surprising observations that (1) brain injury is present before surgery,2 (2) injury is most commonly focal affecting the white matter,3 and (3) brain development is delayed in term infants with CHD.4 Despite its promise as a sensitive early outcome variable that could be used to structure interventional studies with novel trial design,5 success to date has largely been descriptive, identifying clinical risk factors in limited case series. A particular challenge applying these findings to clinical practice is that the incidence of injury and associated risk factors are often unique to each form of CHD.6 For this reason, the approach has not substantially altered clinical care to date. However, several themes have emerged from multiple studies, providing confidence that the identified risk factors are valid and potent. These include the significance of postoperative hypotension,3,6 hypoxemia3 and diminished regional cerebral oxygen saturation.6,7 Although multiple studies have noted the high frequency of preoperative brain injury, particularly in neonates with transposition of the great arteries (TGA),2,6,8 few risk factors have been identified …

Sudden cardiac death caused by native aortic valve thrombosis in an adult patient with a complex congenital cyanotic heart disease

Cyanotic congenital heart disease is associated with an increased risk of thromboembolism. We describe the case of sudden cardiac death caused by native aortic valve thrombosis in adult cyanotic patient. Such embolic complication was not previously described in cyanotic patient. It is also the first report of sudden cardiac death as an initial presentation of the native aortic valve thrombosis. Cyanotic congenital heart disease (CCHD) is associated with an increased risk of thromboembolism due to different haemathologic abnormalities associated with hypoxemia and secondary polycythemia [H. Kajimoto, M. Nakazawa, K. Murasaki, et al. Increased thrombogenesity in patients with cyanotic congenital heart disease. Circ J Jun 2007;71(6):948–953]. Cerebrovascular events, venous and arterial thrombosis have been described in patients with CCHD [N. Ammash, C.A. Warnes. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J Am Coll Cardiol Sep 1996;28(3):768–772]. We present a case of the native aortic valve thrombosis in an adult patient with a complex cyanotic congenital heart lesion which led to sudden death.

Case report: Retroperitoneoscopic pheochromocytoma removal in an adult with Eisenmenger’s syndrome

Patients with uncorrected or palliated, complex congenital heart lesions requiring surgery can benefit from laparoscopic techniques, but retroperitoneal insufflation may render them hemodynamically unstable. Alterations in cardiopulmonary physiology during retroperitoneal insufflation have been studied, yet there are no cases detailing this approach in patients with congenital heart lesions. We present a case of a pheochromocytoma removal via retroperitoneoscopy in a patient with a palliated, complex heart lesion. A 28-yr-old woman was admitted for removal of a pheochromocytoma through retroperitoneoscopy. The main feature of her heart disease was a complete atrioventricular canal defect. She eventually developed Eisenmenger's syndrome and became chronically cyanotic. Retroperitoneal insufflation with CO2 gas did not change hemodynamic variables. Significant increases in peak airway pressures were encountered, possibly due to the distending effects of insufflation, or due to increasing the minute ventilation to reduce exogenous CO2. Arterial CO2 remained stable, but a significant increase between end-tidal and arterial levels became apparent with insufflation. Tumour manipulation led to systemic (and possibly pulmonary) hypertension, which exacerbated ventricular dysfunction. This condition resulted in atrioventricular valve regurgitation, as seen on transesophageal echocardiography, and diminished pulmonary blood flow with subsequent desaturation. These changes resolved with antihypertensive medications. The patient's trachea was extubated four hours postoperatively, and she recovered uneventfully. Patients with altered cardiopulmonary physiology may tolerate retroperitoneoscopic insufflation with relative hemodynamic stability. Appropriate use of short-acting, vasoactive drugs and aggressive monitoring of PaCO2 and hemodynamic variables is required.

The Role of Concomitant Arrhythmia Surgery in Patients Undergoing Repair of Congenital Heart Disease

Long-term survival after repair of complex congenital heart lesions is associated with the late development of arrhythmias as well as residual hemodynamic abnormalities. Understanding arrhythmias as electromechanical problems provides the basis for surgical intervention to correct the arrhythmia as well as anatomical disturbances. Operative techniques are highly effective in treating atrial reentry tachycardia and atrial fibrillation. Surgery for ventricular tachycardia is less effective: the arrhythmia may be reduced by improving hemodynamics, but a defibrillator may be required. Integration of device therapy into surgery may improve outcomes by preventing bradycardia as a precursor to tachycardia, and optimizing ventricular synchrony.