Congenital Cardiac Care Research Articles

Parental Knowledge Regarding Lifelong Congenital Cardiac Care

To assess parental knowledge regarding lifelong congenital cardiac care (LLCCC). National guidelines recommend that nearly 50% of adult survivors with congenital heart disease (CHD) receive LLCCC; the number of adults who receive such care seems far less. Inadequate parental knowledge of LLCCC might contribute to care interruption. In this multicenter study, we administered a questionnaire to parents of children with moderate and complex CHD to assess knowledge of LLCCC. A total of 500 parents participated; the median age of their children was 10 years (range: 2-18 years). Most parents (81%) understood that their child would need LLCCC, but only 44% recognized that their child's cardiology care should be guided by an adult congenital heart specialist in adulthood. More than half (59%) of the parents stated that their current cardiology team had never spoken to them about LLCCC, but 96% wished to learn more. Variables associated with parental LLCCC knowledge included previous discussions regarding LLCCC, underlying cardiac surgical diagnosis, and level of parental education. A substantial number of parents of children with moderate and complex CHD lack knowledge about LLCCC, but almost all of them have a desire to learn more about the care their child will need as an adult.

Risk Factors for Death After Adult Congenital Heart Surgery in Pediatric Hospitals

Despite the central role that pediatric hospitals play in the surgical treatment of congenital heart disease, little is known about outcomes of adult congenital cardiac surgical care in pediatric hospitals. Risk factors for inpatient death, including adult congenital heart (ACH) surgery volume, are poorly described. We obtained inpatient data from 42 free-standing pediatric hospitals using the Pediatric Health Information System data base 2000 to 2008 and selected ACH surgery admissions (ages 18 to 49 years). We examined admission characteristics and hospital surgery volume. Of 97 563 total (pediatric and adult) congenital heart surgery admissions, 3061 (3.1%) were ACH surgery admissions. Median adult age was 22 years and 39% were between ages 25 to 49 years. Most frequent surgical procedures were pulmonary valve replacement, secundum atrial septal defect repair, and aortic valve replacement. Adult mortality rate was 2.2% at discharge. Multivariable analyses identified the following risk factors for death: age 25 to 34 years (adjusted odds ratio [AOR], 2.1; P=0.009), age 35 to 49 years (AOR, 3.2; P=0.001), male sex (AOR, 1.8; P=0.04), government-sponsored insurance (AOR, 1.8; P=0.03), and higher surgical risk categories 4+ (AOR, 21.5; P=0.001). After adjusting for case mix, pediatric hospitals with high ACH surgery volume had reduced odds for death (AOR, 0.4; P=0.003). There was no relationship between total congenital heart surgery volume and ACH inpatient mortality. Older adults, male sex, government-sponsored insurance, and greater surgical case complexity have the highest likelihood of in-hospital death when adult congenital surgery is performed in free-standing pediatric hospitals. After risk-adjustment, pediatric hospitals with high ACH surgery volume have the lowest inpatient mortality.

Innovation in Congenital and Paediatric Cardiac Critical Care

Innovation in Congenital and Paediatric Cardiac Critical Care

Progress in Congenital Cardiac Care for Newborns and Infants: The Emerging Role of “Off-label” Medications

Congenital heart disease accounts for a major percentage of clinically significant birth defects and is the leading cause for infant deaths that are due to a congenital anomaly. Extraordinary advancements during the last three decades in noninvasive diagnosis, surgical repairs, cardiac catheterization interventions, and perioperative pediatric intensive care management have dramatically improved survival for children born with congenital heart disease. Advances in care have included management with Food and Drug Administration–approved medications, as well as many “off-label” medications. An off-label drug has been defined as a drug used for a patient “younger than the Food and Drug Administration (FDA)–approved age range for any indication of that drug” (Arch Pediatr Adolesc Med. 2007; 161: 282-290). This article will review selected off-label medications currently used in the pediatric congenital heart management for treatment of perioperative low cardiac output, congestive heart failure, pulmonary hypertension, sedation, anticoagulation, and pain.

The World Society for Pediatric and Congenital Heart Surgery: Its Mission and History

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) was established in 2006 to assemble pediatric and congenital heart surgeons from all continents and regions of the world and their colleagues from related specialties dealing with pediatric and congenital heart disease. Since its birth, it has held a highly successful inaugural scientific meeting in 2007 in Washington, DC, and a World Summit on Pediatric and Congenital Heart Surgery Services, Education, and Cardiac Care for Children and Adults with Congenital Heart Disease in 2008 in Montreal. It currently has 549 members from 71 countries and in a short period of time has become the largest organization in the world of pediatric and congenital heart surgeons. Its brief history already seems to be a guarantee of a promising future. Projects in the areas of research, training and education, patient care, and community service will allow the Society to reach its goals. By bringing together professionals from every region of the world, the WSPCHS should play a significant role in the improvement of care for children and adults with congenital heart disease around the world.

The improvement of care for paediatric and congenital cardiac disease across the World: a challenge for the World Society for Pediatric and Congenital Heart Surgery

The diagnosis and treatment for paediatric and congenital cardiac disease has undergone remarkable progress over the last 60 years. Unfortunately, this progress has been largely limited to the developed world. Yet every year approximately 90% of the more than 1,000,000 children who are born with congenital cardiac disease across the world receive either suboptimal care or are totally denied care.While in the developed world the focus has changed from an effort to decrease post-operative mortality to now improving quality of life and decreasing morbidity, which is the focus of this Supplement, the rest of the world still needs to develop basic access to congenital cardiac care. The World Society for Pediatric and Congenital Heart Surgery [http://www.wspchs.org/] was established in 2006. The Vision of the World Society is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The Mission of the World Society is to promote the highest quality comprehensive care to all patients with pediatric and/or congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with emphasis on excellence in education, research and community service.We present in this article an overview of the epidemiology of congenital cardiac disease, the current and future challenges to improve care in the developed and developing world, the impact of the globalization of cardiac surgery, and the role that the World Society should play. The World Society for Pediatric and Congenital Heart Surgery is in a unique position to influence and truly improve the global care of children and adults with congenital cardiac disease throughout the world [http://www.wspchs.org/].

Aspirin

Dependence on an expanded polytetrafluoroethylene graft for provision of pulmonary blood flow is a common yet precarious interval through which a population of patients with congenital heart disease must pass. The goals of the systemic-to–pulmonary artery shunt are to relieve cyanosis and to provide time before establishing in-series circulation by either complete repair of 2-ventricle lesions or, in the case of the single-ventricle patient, a bidirectional Glenn shunt. In the case of patients with single-ventricle anatomy, this period of parallel circulation is necessary to permit the lung maturation and the reduction in pulmonary vascular resistance that are necessary for subsequent palliation. For patients who will ultimately achieve a 2-ventricle repair, the goals of a preliminary shunt may include increasing the size of the pulmonary artery size or having a larger, older patient at the time of repair. This period of parallel circulation is tenuous, and the patient remains at increased risk during this period of altered circulation. Article p 293

Forty-First Annual Meeting, The Society of Thoracic Surgeons

Forty-First Annual Meeting, The Society of Thoracic Surgeons

Clinical pathways can be based on acuity, not diagnosis

The standardization of medical practice is gaining acceptance as a technique for controlling length of stay and hospital charges, while maintaining quality. Most clinical pathways address specific diagnoses or procedures, but we have developed a new approach in which pathways for cardiac care are based on acuity. All congenital cardiac surgical care rendered at Columbus Children's Hospital now falls within one of four such clinical pathways. This simplified approach is easy to use and has been well accepted. Our experience in a group of 107 consecutive patients treated in this fashion is described. The results of variance analyses, along with length of stay and charge data, are presented to demonstrate the degree to which resource utilization can be standardized in this widely variable group of patients whose problems were made cohesive by classification according to acuity level. We conclude that the resultant standardization offers considerable advantages for the managed care environment.

Pressure-synchronized cineangiography during experimental cardiopulmonary resuscitation.

Pressure-synchronized cineangiography during experimental cardiopulmonary resuscitation.