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The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease was established in 2005 with the goal of providing the infrastructure, spanning geographical and subspecialty boundaries, for collaboration between health care professionals interested in the analysis of outcomes of treatments provided to patients with congenital cardiac disease, with the ultimate aim of improvement in the quality of care provided to these patients. The purpose of these collaborative efforts is to promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with an emphasis on excellence in teaching, research and community service. This manuscript provides the Introduction to the 2008 Supplement to Cardiology in the Young titled: "Databases and The Assessment of Complications associated with the Treatment of Patients with Congenital Cardiac Disease". This Supplement was prepared by The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease. The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease offers the following definition of the term "Complication": "A complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention. Operative and procedural complications include both intraoperative/intraprocedural complications and postoperative/postprocedural complications in this time interval." The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease offers the following definition of the term "Adverse Event": "An adverse event is a complication that is associated with a healthcare intervention and is associated with suboptimal outcome. Adverse events represent a subset of complications. Not all medical errors result in an adverse event; the administration of an incorrect dose of a medication is a medical error, but it does not always result in an adverse event. Similarly, not all adverse events are the result of medical error. A child may develop pneumonia after an atrial septal defect repair despite intra- and peri-operative management that is free of error. Complications of the underlying disease state, which are not related to a medical intervention, are not adverse events. For example, a patient who presents for medical care with metastatic lung cancer has already developed a complication (Metastatic spread) of the primary lung cancer without any healthcare intervention. Furthermore, complications not associated with suboptimal outcome or harm are not adverse events and are known as no harm events. The patient who receives an incorrect dose of a medication without harm has experienced a no harm event, but not an adverse event." Based on the above definitions, it is apparent that The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease has taken an inclusive approach to defining the universe of complications. Complications may or may not be associated with healthcare intervention and may or may not be associated with suboptimal outcome. Meanwhile, adverse events must be associated with healthcare intervention and must be associated with suboptimal outcome.
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PMID:Introduction--databases and the assessment of complications associated with the treatment of patients with congenital cardiac disease. 1906 74

A complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention. Operative and procedural complications include both intraoperative/intraprocedural complications and postoperative/postprocedural complications in this time interval. The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has set forth a comprehensive list of complications associated with the treatment of patients with congenital cardiac disease, related to cardiac, pulmonary, renal, haematological, infectious, neurological, gastrointestinal, and endocrinal systems, as well as those related to the management of anaesthesia and perfusion, and the transplantation of thoracic organs. The objective of this manuscript is to examine the definitions of operative morbidity as they relate specifically to the renal system. These specific definitions and terms will be used to track morbidity associated with surgical and transcatheter interventions and other forms of therapy in a common language across many separate databases. Although renal dysfunction and renal failure are known risks of congenital heart surgery, accurate estimates of the incidences of these complications are limited. This lack of knowledge is in part due to the lack of uniform definitions of these postoperative complications. The purpose of this effort is to propose consensus definitions for renal complications following congenital cardiac surgery so that collection of such data can be standardized. Clinicians caring for patients with congenital heart disease will be able to use this list for databases, quality improvement initiatives, reporting of complications, and comparing strategies for treatment.
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PMID:Renal complications associated with the treatment of patients with congenital cardiac disease: consensus definitions from the Multi-Societal Database Committee for Pediatric and Congenital Heart Disease. 1906 95

The congenital heart disease 5 (CHD5)/tryptophan rich basic protein (WRB) is a protein containing a tryptophan-rich carboxy-terminal region, which was discovered in the human fetal heart. In humans, this CHD5/WRB is located between the markers ACTL5-D21S268 within the Down syndrome (DS) Region-2 at chromosome 21. Congenital heart disease is commonly linked to DS patients. The functions of this gene product are unknown. To identify the functions of CHD5/WRB in heart formation during embryogenesis, the medaka CHD5 cDNA (mCHD5) was isolated and its gene expression pattern and the localization of its gene product were investigated. The obtained mCHD5 belongs to the CHD5 superfamily, whose members include coiled-coil proteins. The mCHD5 gene was found to be expressed in the developing heart after stage 28 at which the chamber (ventricle and atrium) differentiation in the heart tube is initiated in the embryo. Its gene product was also detected in the developing heart at embryonic stage 28 and 35. Knocking-down of mCHD5 function caused severe cardiac disorder, including abnormal chamber differentiation, abnormal looping and ocular abnormality such as Cyclops. Our results provide the mCHD5 gene expression pattern as well as its physiological role during heart formation in a vertebrate model system.
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PMID:Expression of the congenital heart disease 5/tryptophan rich basic protein homologue gene during heart development in medaka fish, Oryzias latipes. 1920 81

Heart disease is the primary cause of nonobstetric mortality in pregnancy, occurring in 1%-3% of pregnancies and accounting for 10%-15% of maternal deaths. Congenital heart disease has become more prevalent in women of childbearing age, representing an increasing percentage (up to 75%) of heart disease in pregnancy. Untreated maternal heart disease also places the fetus at risk. Independent predictors of neonatal complications include a maternal New York Heart Association heart failure classification >2, anticoagulation use during pregnancy, smoking, multiple gestation, and left heart obstruction. Because cardiac surgical morbidity and mortality in the parturient is higher than nonpregnant patients, most parturients with cardiac disease are first managed medically, with cardiac surgery being reserved when medical management fails. Risk factors for maternal mortality during cardiac surgery include the use of vasoactive drugs, age, type of surgery, reoperation, and maternal functional class. Risk factors for fetal mortality include maternal age >35 yr, functional class, reoperation, emergency surgery, type of myocardial protection, and anoxic time. Nonetheless, acceptable maternal and fetal perioperative mortality rates may be achieved through such measures as early preoperative detection of maternal cardiovascular decompensation, use of fetal monitoring, delivery of a viable fetus before the operation and scheduling surgery on an elective basis during the second trimester. Additionally, fetal morbidity may be reduced during cardiopulmonary bypass by optimizing maternal oxygen-carrying capacity and uterine blood flow. Current maternal bypass recommendations include: 1) maintaining the pump flow rate >2.5 L x min(-1) x m(-2) and perfusion pressure >70 mm Hg; 2) maintaining the hematocrit > 28%; 3) using normothermic perfusion when feasible; 4) using pulsatile flow; and 5) using alpha-stat pH management.
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PMID:Cardiac surgery in the parturient. 1922 82

Congenital heart disease is the most common birth defect in the United States, with an estimated frequency of approximately 12-14 of 1000 live births per year. Neonates with congenital heart disease often need palliative or corrective surgery requiring cardiopulmonary bypass during the first weeks of life. The neonate undergoing cardiopulmonary bypass surgery experiences a more profound metabolic response to stress than that seen in older children and adults undergoing surgery. However, compared with older children and adults, the neonate has less metabolic reserves and is extremely vulnerable to the negative metabolic impact induced by stress, which can lead to suboptimal wound healing and growth failure. There are complications associated with the metabolic derangements of neonatal surgery requiring cardiopulmonary bypass, including but not limited to acute renal failure, chylothorax, and neurological dysfunction. This article discusses the importance of nutrition and metabolic support for the neonate undergoing cardiopulmonary bypass and the immediate postoperative nutrition needs of such a patient. Also, this article uses a case study to examine the feeding methodology used at one particular institution after neonatal cardiac surgery. The purpose of the case study is to provide an illustration of the many factors and obstacles that clinicians often face in the provision and timing of nutrition support.
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PMID:Nutrition support after neonatal cardiac surgery. 1932 98

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.
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PMID:The World Society for Pediatric and Congenital Heart Surgery: its mission and history. 1934 8

The objective of this study was to explore the current practice and attitudes of pediatric cardiologists in the United States and Europe on the transfer and transition of children with congenital heart disease (CHD). A survey among pediatric cardiology programs in the United States and Europe was undertaken. Sixty-nine centers completed and returned the 61-item questionnaire that was specifically devised for this survey. Of 69 participating centers, 74% reported that they transfer their patients to adult-focused care. When a center transfers its patients, 80% transfer them to a formalized Adult Congenital Heart Disease Program. The median age of transfer is 18 years. Comorbidities, pregnancy, and patient/family request to leave pediatric cardiology were identified as initiators for transfer. Complexity of the heart defect was relatively less important when deciding whether to transfer patients. Only one-third of the centers that transfer their patients provide a structured preparation for patients and family. Development of a formal transition program is planned at 59% of the centers that transfer patients. In conclusion, timely transfer and a structured transition process of children with CHD are not implemented in all pediatric cardiology programs. Health-care providers working in pediatric cardiology should make their transfer policies explicit and transition programs ought to be developed.
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PMID:Attitude toward and current practice of transfer and transition of adolescents with congenital heart disease in the United States of America and Europe. 1936 51

Congenital heart disease can predispose individuals to pulmonary vascular remodeling as a result of the abnormality in pulmonary blood flow and pressure that accompanies the specific congenital defect being considered. Pulmonary arterial hypertension associated with congenital heart defects is an important determinant of functional capacity and survival, especially when the Eisenmenger's state of reversed shunt is present. The likelihood of right ventricular dysfunction and failure increases with the degree of pulmonary artery pressure. Thus, the aim of disease management in this patient population should be to prevent or improve right heart failure. Current therapies that modify the progression of pulmonary vascular disease-including endothelin-1 receptor antagonists, phosphodiesterase-5 inhibitors, and prostanoids-should be considered carefully in patients with congenital heart disease-associated pulmonary hypertension. The risks and benefits of altering the balance of pulmonary vascular resistance to systemic vascular resistance must be weighed for each patient.
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PMID:Pulmonary hypertension complicating congenital heart disease. 1956 32

Congenital heart disease is the most frequent neonatal malformation, with an estimated prevalence between 0.8% and 1%. Echocardiography is an important tool for diagnosis and follow-up in cardiology, because it is easy to use, inexpensive, and noninvasive. The study of fetal heart by means of echocardiography allows early diagnosis and treatment of fetal heart anomalies and dysfunction, and such a technique is increasingly used. This exam is often required after morphological fetal echography, when a congenital heart disease is suspected or when there is an increased risk of congenital heart disease. Fetal echocardiography allows to evaluate the cardiac anatomy and function or cardiac rhythm. The purpose of this review is to summarize the main parameters and echocardiographic techniques for the assessment of heart rhythm and function: two-dimensional echocardiography, Doppler, tissue Doppler, and two-dimensional strain.
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PMID:[Evaluation of ventricular function in the fetus]. 1977 46

Anomalies of the cardinal vein system (CVS) are uncommon but if unidentified can lead to life-threatening complications. We report a case with a novel malformation of the CVS. Autopsy with in situ dissection of heart and large vessels in a 25-day-old infant was performed. The infant was diagnosed with congenital heart disease, and systemic venous malformations were suspected by imaging. Correlation between premortem imaging and postmortem anatomy was performed. The superior and inferior left venous systems developed abnormally. A persistent left superior vena cava (PLSVC) drained into the right atrium via the coronary sinus. A persistent left inferior vena cava (PLIVC) continued with the hemiazygos vein (HV), which drained into the PLSVC. The innominate vein was absent. The left renal vein was connected to the HV. Two common iliac veins were identified. The left drained into the PLIVC and the right into the right inferior vena cava (IVC). Perinatal echocardiography identified only the dilated HV draining to an LSVC and a small IVC. Congenital heart disease included hypoplastic left ventricle with hypoplastic aortic arch and subaortic stenosis, which were diagnosed by fetal ultrasound. Remodeling of components of CVS takes place during development, and unknown mechanisms guide this process. Defects of this process can lead to variable malformations, as demonstrated by this case. To our knowledge, the combination of complex malformations of both superior and IVC systems that extends to the common iliac veins has not been reported. We recommend identifying vascular anomalies in situ during autopsy before anatomic relationships are altered.
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PMID:The anatomy of a novel malformation of the cardinal vein system. 1986 44


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