Management Of Congenital Heart Disease Research Articles

Update on the challenges facing the adult with congenital heart disease community: for both the patient and provider.

The success in the management of congenital heart disease has resulted in a growing population now expected to reach adulthood. The declining mortality during childhood results in adults with decades of cardiovascular disease burden. The purpose of this review is to provide an update on some of the common issues encountered when caring for the adult with congenital heart disease through a review of recently published articles. This review focuses on addressing the lack of information, gaps in clinical care, single ventricle complications, and heart failure in the adults with congenital heart disease population. The advancing age of the congenital heart population comes with a substantial life cardiovascular risk. Despite this, they typically experience and report a good quality of life. Providing care to adults with congenital heart disease requires a comprehensive team to address the many complications that are known to arise. This comprehensive team should be available to all.

Congenital Heart Disease and Pulmonary Arterial Hypertension in South America (2013 Grover Conference Series)

South America is a territory of 17,819,100 km(2), where ∼388 million people live in 13 countries. In the region, access to medical assistance (e.g., for treatment of cardiovascular disorders) is relatively easy in metropolitan areas but difficult in remote places such as the Andes and the Amazon. Altitudes up to ∼6,700 m influence the prevalence of congenital heart disease (CHD) and pulmonary arterial hypertension (PAH). In tertiary centers, CHD is now treated earlier in life but remains an important etiology of PAH. In adolescents and adults with PAH assisted at institutions devoted to treatment of cardiovascular disorders, the relative frequency of PAH-CHD (∼50%-60%) is even higher than that of idiopathic PAH. In one big tertiary center in São Paulo, Brazil, the prevalence of advanced PAH in children and adults with CHD is 1.2% and 4.2%, respectively. In young patients with cardiac septal defects (aged up to 2 years), pulmonary vascular abnormalities are a matter of concern in the decision about operability in 4.9% of cases. Access to specific PAH drugs is not uniform in South America, being unrealistic in remote places. In big cities, there are real possibilities for management of complex CHD, neonatal disorders, and even cardiac transplantation. Research activities have been implemented at clinical, translational, and basic levels. However, because of social and economic inequalities and political issues, access to best standards of medical care remains a problem in the region as a whole.

Right ventricular systolic strain evolution during peri-operative management of congenital heart diseases

RV systolic strain evolution during peri-operative management of congenital heart diseases (CHD) is unknown. In this prospective study, RV peak systolic strain (PSS) was measured using 2D speckle tracking echocardiography (Qlab10.0 software, Philips) in 39 children undergoing surgery of a CHD (Median age: 17 months, min. 6 days old, max. 14.3 years old). Three measures were performed the day before surgery, few hours after the surgery and before discharge and compared to conventional echocardiographic parameters of RV and left ventricular (LV) function. The relationships between the evolution of RV-PSS, peri-operative parameters and the type of CHD were assessed. Mean RV-PSS at baseline was –19.5 ± 4.8. RV-PSS was moderately correlated with the heart rate ( r = 0.49), the LV Tmad ( r = –0.48), the TAPSE ( r = –0.54) and the tricuspid S’ wave ( r = –0.44) (all P < 0.05). RV-PSS was decreased in cyanotic CHD ( P < 0.05), in children with congestive symptoms ( P = 0.01) and increased in ASD ( P = 0.02). RV-PSS was higher in RV volume increased condition such as ASD than in RV pressure increased condition such as Fallot tetralogy ( P = 0.006). RV-PSS decreased after surgery ( P < 0.0001). Mean difference between pre- and post-operative RV-PSS was 7.5 ± 4.4. The difference was correlated with initial RV-PSS ( r = –0.80), the weight ( r = 0.54), the ultrafiltration rate ( r = 0.43)(all P < 0.05) but not with the duration of aortic clamp, the duration of extracorporeal circulation, the troponin peak level nor the lactates peak level. A higher difference was associated with a shorter duration of mechanical ventilation ( P = 0.04) and a shorter stay in intensive care unit ( P = 0.03). RV-PSS was better at discharge (median 6 days, P = 0.0009) but remained lesser than at the initial exam ( P < 0.0001). RV-PSS decrease after surgery of CHD. This decrease seems mainly related to loading condition rather than to RV contractility given its relationship with a faster post-operative evolution.

Increasing mortality burden among adults with complex congenital heart disease.

Progress in management of congenital heart disease has shifted mortality largely to adulthood. However, adult survivors with complex congenital heart disease are not cured and remain at risk of premature death as young adults. Thus, our aim was to describe the evolution and mortality risk of adult patient cohorts with complex congenital heart disease. Among 12,644 adults with congenital heart disease followed at a single center from 1980 to 2009, 176 had Eisenmenger syndrome, 76 had unrepaired cyanotic defects, 221 had atrial switch operations for transposition of the great arteries, 158 had congenitally corrected transposition of the great arteries, 227 had Fontan palliation, and 789 had repaired tetralogy of Fallot. We depict the 30-year evolution of these 6 patient cohorts, analyze survival probabilities in adulthood, and predict future number of deaths through 2029. Since 1980, there has been a steady increase in numbers of patients followed, except in cohorts with Eisenmenger syndrome and unrepaired cyanotic defects. Between 1980 and 2009, 308 patients in the study cohorts (19%) died. At the end of 2009, 85% of survivors were younger than 50 years. Survival estimates for all cohorts were markedly lower than for the general population, with important differences between cohorts. Over the upcoming two decades, we predict a substantial increase in numbers of deaths among young adults with subaortic right ventricles, Fontan palliation, and repaired tetralogy of Fallot. Anticipatory action is needed to prepare clinical services for increasing numbers of young adults at risk of dying from complex congenital heart disease.

Adult congenital heart disease and pregnancy

Adults with congenital heart disease now form the largest group of women with cardiac disease becoming pregnant in the developed world. This is both a mark of impressive steps forward in the management of congenital heart disease and also a challenge to the medical community to develop systems of care that will best serve these women and their babies. Each woman with congenital heart disease presents a unique pattern of challenges for the cardiologist, obstetrician, and anesthesiologist, and their care should be tailored to deal with their individual circumstances. As this population of patients continues to grow, we must continue to learn and improve our diagnostic tools and management strategies to refine their care. This review intends to focus on reviewing the outcomes in this set of patients and also an approach to the assessment and the management of these patients, primarily for an audience of obstetricians, pediatricians, and anesthesiologists.

Management of Congenital Heart Disease in Adults

Management of Congenital Heart Disease in Adults

Echocardiographic Nomograms for Ventricular, Valvular and Arterial Dimensions in Caucasian Children with a Special Focus on Neonates, Infants and Toddlers

A quantitative echocardiographic assessment is often essential for the management of congenital heart disease, especially in the first months of life. Despite this, pediatric echocardiographic nomograms are limited and heterogeneous, particularly for neonates and infants. The aim of this study was to establish reliable echocardiographic nomograms in a broad population of healthy Caucasian children. Two-dimensional and M-mode measurements of 22 cardiovascular structures were performed.Models using linear, logarithmic, exponential, and square root relationships were tested. Heteroscedasticity was tested using the White and Breusch-Pagan tests. Age, weight, height, and body surface area(BSA; calculated using seven different formulas) were used as the independent variables in different analyses to predict the mean value of each echocardiographic measurement. Structured Z scores were then computed. A total of 445 consecutive Caucasian Italian healthy subjects (age range, 0 days to 36 months; 49%female subjects) with BSAs ranging from 0.12 to 0.67 m(2) were prospectively enrolled. The calculation of BSA using the Haycock formula provided the best results, while other formulas either underestimated (DuBois, Mosteller, Dreyer, and Meban) or overestimated (Boyd and Gehan) BSA. The Haycock formula has been used when presenting data as predicted values (mean ± 2 SDs) for a given BSA and within equations relating echocardiographic measurements to BSA. For all the measurements, there was no significant intraobserver or interobserver variability. The investigators report new, reliable echocardiographic Z scores derived from a large population of Caucasian neonates, infants, and toddlers calculated using a rigorous statistical design. These nomograms represent a valid diagnostic tool for echocardiographic quantification in this age group.

Embryonic Heart Progenitors and Cardiogenesis

The mammalian heart is a highly specialized organ, comprised of many different cell types arising from distinct embryonic progenitor populations during cardiogenesis. Three precursor populations have been identified to contribute to different myocytic and nonmyocytic cell lineages of the heart: cardiogenic mesoderm cells (CMC), the proepicardium (PE), and cardiac neural crest cells (CNCCs). This review will focus on molecular cues necessary for proper induction, expansion, and lineage-specific differentiation of these progenitor populations during cardiac development in vivo. Moreover, we will briefly discuss how the knowledge gained on embryonic heart progenitor biology can be used to develop novel therapeutic strategies for the management of congenital heart disease as well as for improvement of cardiac function in ischemic heart disease.

The Effects of Perioperative Inhaled Iloprost on Pulmonary Hypertension with Congenital Heart Disease

Objectives: The treatment of choice for congenital heart disease (CHD) with pulmonary arterial hypertension (PAH) is still controversial. We assessed the efficacy and safety of perioperative inhaled iloprost therapy in CHD with PAH. Methods: Among 45 patients with a ventricular septal defect and/or an atrial septal defect with PAH, 28 patients were treated with inhaled iloprost before and after surgery. Perioperative clinical parameters and plasma B-type natriuretic peptide (BNP) were evaluated. Results: No statistical difference in the estimated right ventricular systolic pressure (e-RVP), the e-RVP-to-systemic pressure ratio, and preoperative BNP levels between the iloprost group and the control group were found. Among the iloprost group, oxygen saturation was increased significantly after iloprost inhalation therapy (p = 0.0052). The iloprost group was also significantly correlated with less use of inhaled nitric oxide in the immediate postoperative period compared to the control group (p = 0.021). The durations of mechanical ventilation (p = 0.018), ICU stay (p = 0.005), and chest tube use (p = 0.039) were significantly shorter in the iloprost group compared to the control group. The plasma BNP, checked on 7th day of postoperatively, was lower in the iloprost group than in the control group (p = 0.008). Conclusion: Perioperative inhaled iloprost therapy showed the benefit of cardiac functional improvement and early weaning of postoperative supportive care in the management of CHD with PAH.

Apport de l’échographie volumique dans l’évaluation et la prise en charge prénatale anténatale des anomalies cardiovasculaires fœtales

To highlight the value of 3D ultrasound in the prenatal assessment of fetal cardiovascular abnormalities. A retrospective offline analysis of volume datasets of fetuses diagnosed with cardiovascular anomalies by 2D ultrasound was performed. Thirty-four fetuses with 38 cardiac malformations were evaluated. Mean gestational age at diagnosis was 26 weeks. Isolated cardiovascular malformations were detected in 23 fetuses. Extracardiac abnormalities were identified in eight fetuses. Ten terminations of pregnancy were performed. Offline analysis of cardiovascular anomalies conferred significant diagnostic advantages over 2D ultrasound. 3D ultrasound is a valuable tool for the prenatal diagnosis and the management of congenital heart diseases.

The Management of the Older Adult Patient with Anomalous Left Coronary Artery from the Pulmonary Artery Syndrome: A Presentation of Two Cases and Review of the Literature

ALCAPA (anomalous left coronary artery from pulmonary artery) syndrome is a rare congenital abnormality that involves an anomalous insertion of the left coronary artery into the pulmonary artery. Ninety percent of patients present in the first year of life with signs and symptoms of heart failure or sudden cardiac death secondary to chronic myocardial ischemia. There have been an increasing number of reports of ALCAPA patients surviving to adulthood. There seems, however, to be a tendency to die suddenly in the third decade of life. Adult survivors are either asymptomatic or present with mitral regurgitation, cardiomyopathy, myocardial ischemia, or malignant arrhythmias. The management of the older patient presenting with symptoms resulting from ischemia and progressive left ventricular dysfunction remains a challenge. Treatment is largely based on guidelines for adult congenital heart disease management and an extrapolation of evidence from heart failure practice. Currently, surgical reimplantation of the anomalous coronary onto the aorta is the mainstay of treatment. The management of heart failure, sudden cardiac death, and ventricular arrhythmia present problems that are not addressed by reimplantation of the anomalous vessel alone. In this report, we present two cases with different modes of presentation and discuss treatment options.

Advances in Fetal Echocardiography: Early Imaging, Three/Four Dimensional Imaging, and Role of Fetal Echocardiography in Guiding Early Postnatal Management of Congenital Heart Disease

In this article, we review a number of topics that we believe reflect new and exciting aspects of fetal echocardiography. These new advances include early fetal cardiovascular imaging around 14 weeks, the utility of three/four dimensional imaging technology for the fetus, and finally the utility of fetal echocardiography for antenatal and perinatal care of congenital heart diseases to improve and optimize outcome. Finally, we briefly discussed future directions in fetal cardiac intervention.

Congenital Heart Disease in Mexico

Consistent with the mission of the World Society for Pediatric and Congenital Heart Surgery to promote health care for children with congenital heart disease all around the world, a Mexican Association of Specialists in Congenital Heart Disease (abbreviated in Spanish as AMECC) was created in Mexico in 2008. Our efforts were coordinated with those of the National Health Secretary with the objective being implementation of a national plan for regionalization of care for patients with congenital heart disease. To improve our knowledge related to technologic and human resources for management of congenital heart disease, we developed a national survey. Finally, a national database was created for collecting all Mexican centers' information related to congenital heart disease care in order to quantify the advances related to the proposed plans. The database utilized international consensus nomenclature. The aim of this article is to show the sequence of our actions in relation to direct accomplishments and the current status of congenital heart disease care in Mexico. This article emphasizes the main aspects of these actions: regionalization project implementation, national survey results, and cardiovascular pediatric surgical database creation. Knowledge of outcomes related to successful actions would be useful for those countries that face similar challenges and may lead them to consider adoption of similar measures with the respective adjustments to their own reality.

Usefulness of Cystatin C in the Postoperative Management of Pediatric Patients With Congenital Heart Disease

The characteristics of the renal marker cystatin C (Cys-C) in association with the postoperative management of children with congenital heart disease (CHD) remain unclear. Serum Cys-C and creatinine (Cr) levels were measured preoperatively and on the third postoperative day in 53 consecutive CHD patients (age, 1 day-11 years). On the third postoperative day, the patients were divided into 2 groups: the clinically severe group, requiring continuous infusion of diuretic drugs or peritoneal dialysis; and the non-severe group, composed of those without such needs. Preoperative Cys-C level decreased with age (by month) during the first year of life and remained almost constant thereafter, while Cr level increased with age. The Cys-C ratio (Cys-C level on the third postoperative day/preoperative level) was positively correlated with Cr ratio (R=0.57, P<0.001). Both Cys-C and Cr levels increased in correlation with the clinical severity of renal impairment. Receiver operating characteristic curve analysis failed to demonstrate an advantage of Cys-C over Cr in detecting severity. Cys-C may be a useful marker of renal function in terms of hemodynamic status in the postoperative management of CHD, although its superiority over Cr could not be confirmed. Future studies should clarify the role of Cys-C in clinical decision-making and evaluate the relationship of Cys-C with factors that may affect its levels.

3D ultrasound and Doppler angiography for evaluation of fetal cardiovascular anomalies

ObjectiveTo highlight the value of 3D ultrasound in the prenatal assessment of fetal cardiovascular anomalies through offline diagnosis and/or second opinion (e.g. via internet link). MethodsA retrospective offline analysis of volume datasets of fetuses diagnosed with cardiovascular anomalies by 2D ultrasound was conducted. ResultsThirty-three fetuses with 38 cardiac malformations were evaluated. Mean gestational age at diagnosis was 26weeks (range, 20–34weeks). Isolated cardiovascular malformations were detected in 23 fetuses. Extracardiac abnormalities were identified in 8 fetuses, of which 2 had trisomy 21 and 1 had trisomy 18. One fetus exhibited 22q11 microdeletion. Ten pregnancy terminations were performed. ConclusionOffline analysis of cardiovascular anomalies conferred significant diagnostic advantages over 2D ultrasound. 3D ultrasound is invaluable for the prenatal diagnosis and management of congenital heart diseases. It may be used to facilitate scientific cooperation between high- and low-income countries.

Book Review: Surgical Management of Congenital Heart Disease

Book Review: Surgical Management of Congenital Heart Disease

Clinical Effect of Tolvaptan in Acute Heart Failure Affected by Neurohumoral Factors

Background: Along with the advancement of the management of congenital heart disease, the number of patients with adult congenital heart disease (ACHD) is increasing. Thus, it is required to establish the ACHD care system to treat such patients in Japan. The present study aimed to examine the current status of ACHD patients in our Registries, named as the Chronic Heart Failure Analysis and Registry in the Tohoku District 1 and 2 (CHART-1, 2) Studies. Method: We analyzed the data of HF patients in the CHART Studies to examine the prevalence and characteristics of ACHD. Results: In the CHART-1 (stage C/D of CHF) study, conducted in 20002005, 23 (2.0%) out of 1,175 patients with CHF had ACHD. In the CHART-2 study (stage B/C/D, 2006-2010), there were 185 (1.8%) of ACHD out of 10,219 patients with CHF, where there were 76 (1.9%) out of 4,103 patients with stage C/D. In the CHART-2 study, ACHD patients with stage C/D tended to have worse prognosis than those with stage B (P50.08). Furthermore, 48% and 59% of ACHD patients were treated in hospitals without ACHD care in CHART-1 and CHART-2 Studies, respectively. Conclusions: These results indicate that ACHD care is not sufficient yet and that it is important to establish ACHD care system that covers Japan.

Low-dose Tolvaptan Treatment Reduces Pulmonary Vascular Resistance Index in Patients with Chronic Heart Failure

Background: Along with the advancement of the management of congenital heart disease, the number of patients with adult congenital heart disease (ACHD) is increasing. Thus, it is required to establish the ACHD care system to treat such patients in Japan. The present study aimed to examine the current status of ACHD patients in our Registries, named as the Chronic Heart Failure Analysis and Registry in the Tohoku District 1 and 2 (CHART-1, 2) Studies. Method: We analyzed the data of HF patients in the CHART Studies to examine the prevalence and characteristics of ACHD. Results: In the CHART-1 (stage C/D of CHF) study, conducted in 20002005, 23 (2.0%) out of 1,175 patients with CHF had ACHD. In the CHART-2 study (stage B/C/D, 2006-2010), there were 185 (1.8%) of ACHD out of 10,219 patients with CHF, where there were 76 (1.9%) out of 4,103 patients with stage C/D. In the CHART-2 study, ACHD patients with stage C/D tended to have worse prognosis than those with stage B (P50.08). Furthermore, 48% and 59% of ACHD patients were treated in hospitals without ACHD care in CHART-1 and CHART-2 Studies, respectively. Conclusions: These results indicate that ACHD care is not sufficient yet and that it is important to establish ACHD care system that covers Japan.

450 What do Children with Congenital Heart Diseases do when they Grow Up

BackgroundAdvances in the diagnosis and interventional-surgical management of congenital heart diseases had as a result a considerable number of patients reaching the adolescent and young adult life.AimsThe aim of this...

Medium and Long-Term Outcomes After Bilateral Pulmonary Artery Stenting in Children and Adults With Congenital Heart Disease

Percutaneous stenting of branch pulmonary arteries (PAs) has become an important interventional therapy in the complete management of congenital heart disease. Few data exist on long-term outcomes for patients requiring placement of bilateral stents. This study aimed to determine the medium- to long-term outcomes after bilateral stenting of branch PAs in patients with congenital heart disease. A retrospective study analyzed all procedural and follow-up data on patients undergoing bilateral PA stenting during a single procedure. From October 2001 to May 2012, 26 patients underwent bilateral PA stenting (total of 62 stents). The mean age of these patients was 9.5 ± 12 years, and their mean weight was 27 ± 19 kg. The mean procedural time was 191 ± 67 min, and the mean fluoroscopy time was 50 ± 29 min. The mean gradient across the right pulmonary artery decreased from 35 ± 22 to 11 ± 12 mmHg (p < 0.001). The left pulmonary artery gradient decreased from 32 ± 17 to 10 ± 12 mmHg (p < 0.001), and the right ventricle to descending aorta pressure ratio decreased from 71 ± 18 % to 46 ± 13 % (p < 0.001). Intraprocedural adverse events were encountered in four patients (1 small PA dissection, 2 intraprocedural arrhythmias, and 1 first-degree burn on the thigh). The mean follow-up period was 41 ± 23 months. Two deaths occurred. Further interventions were required for 12 patients. Bilateral PA stenting is effective and safe, with an instantaneous significant decrease in pressure gradients. The need for reintervention is common, so continued follow-up assessment is warranted.