Julien Ivor Ellis Hoffman

Journal of the Royal Society of Medicine, 1979

Pediatric Cardiology

, aged 99. His entire career was marked by imagination, creativity, and a devotion to the care of children with heart disease. Dr. Rudolph was born in 1924 in South Africa. He earned his medical degree at the University of the Witwatersrand, Johannesburg, where he graduated summa cum laude, winning most of the prizes awarded to medical students. After receiving his MB, BCh degree in 1946, he completed his residency at the Transvaal Memorial Hospital for Children, having decided to specialize in pediatrics. After a brief sojourn in the United Kingdom, during which he passed the examinations for membership of the Royal College of Physicians of London and the Royal College of Physicians of Edinburgh, he returned to South Africa, continuing to work as a pediatrician. At that time, he wrote a thesis on hemophilia that earned him an MD degree. After a great deal of correspondence, he accepted a position with Dr. Alexander Nadas of the Boston Children's Hospital and emigrated with his family to the United States in 1951. Dr. Nadas had just started a pediatric cardiology department at Harvard, and appointed Dr. Rudolph, who soon became head of the nascent pediatric cardiac catheterization laboratory. He set up an experimental animal laboratory, working on measuring cardiac output and trying to develop a chronic in-utero animal model. After struggling to find catheters that did not clot, he found a suitable silastic catheter that allowed development of a chronically instrumented fetal preparation in sheep. The trajectory of his research was set. During his first few years in Boston Dr. Rudolph participated in several important clinical studies, now classics in the field. The studies focused on common congenital heart

Congenital Heart Disease, 2018

European Heart Journal, 2014

Adults with congenital heart disease must be managed by a new generation of subspecialist cardiologists, says John Deanfield Five decades ago children with congenital heart disease were not expected to survive into adulthood but now .90% do. This success has been achieved by medical and surgical breakthroughs, step wise improvements in care and organizational changes. The result is a new subspecialty of adult cardiology, namely adult congenital heart disease (ACHD). 'Congenital heart disease is no longer a paediatric specialty', says Prof. John Deanfield (London, UK). 'Adult cardiologists have to organise services to manage this growing challenging group of patients'. Medical systems across Europe are at various stages of reorganizing care to address the needs of ACHD patients. The first principle is that adults with congenital heart disease need to be looked after in an adult environment with adult facilities. 'There should be no such thing as a paediatric cardiology centre', says Deanfield. 'Every centre should treat patients with congenital heart disease over their whole life'. Established paediatric cardiology specialist centres should have firm links with an adult institution to which patients can be seamlessly transferred. Currently, there are very few physicians, surgeons, and support staff (nurses, technicians, etc) who are experts in ACHD. In most countries, care is being organized in a hub and spoke model with a small number of specialized centres and a network of care in the community. This model of care is designed to ensure that all patients have access to expert care appropriate to their condition. This has been supported by the European Society of Cardiology guidelines on the management of grown up congenital heart disease, first published in 2003 and updated in 2010. The system then needs to be populated with properly trained people. In most European countries, the majority of experts in congenital heart disease has a background in paediatric cardiology. Adult cardiologists, however, are now also entering the field through formal training programmes that enable them to subspecialize in ACHD as part of their adult training. Deanfield says it is' reasonable for specialists to come through the paediatric cardiology or adult cardiology route, provided they address gaps in their training. Adult cardiologists should have exposure to paediatric cardiology to really understand ACHD. Similarly, paediatric cardiologists must undergo training in adult medicine. Research in ACHD is facing a number of issues. First is that the spectrum of cases in congenital heart disease is diverse, with relatively small numbers of different diagnoses at a large number of centres. This contrasts to coronary disease, which can be investigated in

Pediatric Cardiology, 2003

To analyze complex and noncomplex cardiac malformations regarding prevalence and in relation to demographic variables, we pooled data on infants (age 1 year or younger) with congenital cardiovascular defects from three large birth defect registries in California, Sweden, and France. Altogether, 12,932 infants had one or more congenital heart defects out of 4.4 million live births and stillbirths. The registries in Sweden and France obtained data through reporting from various sources; in California, medical records were reviewed. As expected, definitions and ascertained conditions differed among each of the registries. The total rates for severe defects were similar (1.43 per 1,000), but differed for specific defects. Clear differences in epidemiological characteristics existed for specific defects; for example, severe cardiac defects sex ratios were significantly high for hypoplastic left heart syndrome, dtransposition of great vessels, double outlet right ventricle, total anoralous pulmonary venous return, tetralogy of Fallot, and significantly low for pulmonary atresia without ventricular septal defect and endocardial cushion defect. Few defects were similar for several epidemiological characteristics, but, for example, the combination of ventricular and atrial septal defects appeared equivalent with endocardial cushion defect under some circumstances, yet behaved differently with regard to associated noncardiovascular defects.

Circulation, 1968

It is difficult to make prognoses and decisions on therapy of individuals with congenital heart disease (CHD) and plan efficiently for medical services and training programs because studies on the incidence and relative frequency of different types of CHD at birth, mortality rates, times of death, and prevalence rates in children and adults are incomplete or unrepresentative. The present study assesses the merits of previous surveys and attempts to indicate the best information about these rates. Specific attention has been directed to data on ventricular septal defect (VSD), with an estimated incidence of at least 20/10,000 live births; about 10% of these children died before 15 years of age. There should thus be about 18/10,000 surviving school children with VSD, yet the most intensive school surveys show only about half this number. This supports the suggestion that there is a high incidence (perhaps 50%) of spontaneous closure of VSD during childhood. Spontaneous closure may also occur frequently after childhood, but this conclusion needs to be confirmed by better surveys.

Arquivos Brasileiros de Cardiologia, 2001

Open Journal of Pediatrics, 2012

Aim: To assess basic trends in epidemiology of congenital heart defects (CHDs). Method: Population based prospective observational study. Material: CHDs in infants born alive in a Norwegian county 1982-2011. Results: In 828/71 217 infants (12 per 1000) a CHD was diagnosed. The prevalence increased from 8 to 12 per 1000 after introduction of early echocardiography in newborns with suspected CHD from 1986 (p = 0.0001). Ventricular septal defect (VSD) was the dominating CHD (474; 57%). In 222 (27%) the CHDs were missed and diagnosed after discharge from hospital after birth. Twelve critical CHDs (causing death or requiring invasive procedures before 28 days of life) were missed. Prenatal diagnosis of critical CHDs increased from 4/67 (6%) born 1997-2006 to 4/11 (36%) born 2007-11 (p = 0.01). In 177 (21%) a syndrome or extracardial defect occurred. The occurrence of CHDs associated with chromosomal disorders (60/73 (82%) trisomies) more than doubled from the cohort born in the first 10-year period 1982-91 (0.6 per 1000) to the last 2002-11 (1.4 per 1000) (p < 0.0001) in parallel with increasing births in women ≥ 35 years old in the population. 237 (29%) underwent therapeutic procedures (203 (86%) surgery, of whom 16 after initial catheter intervention, and 34 (14%) catheter intervention alone). 39/237 (16%) died, 101 (43%) were repaired and 97 (41%) had some minor residual defect. The death rate declined significantly from 65/532 (12%) born 1982-2001 to 11/296 (4%) born 2002-11 (p = 0.0001). 37/76 (49%) deaths occurred within 28 days after birth. Conclusions: The rate of detection of CHDs increased substantially after introduction of echocardiography in newborns with suspected CHD, especially VSDs. Some critical CHDs were overlooked. The prenatal detection rate of such defects increased. The prevalence of CHDs with associated chromosomal disorders increased in parallel with increasing maternal age in the population. Most deaths occurred during the neonatal period. Increasing survival increases the load on long-term follow-up programmes.

Revista română de pediatrie, 2020

Introduction. A congenital heart disease is a defect in the structure of the heart or great vessels that is present at birth. Signs and symptoms depend on the specific type of defect. The cause of a congenital heart defect is often unknown but remain the most common congenital malformations in newborns and infants with a major role in early and late neonatal morbidity and mortality. Material and methods. The study was carried out in the Department of Neonatology and Premature in the collaboration with the Department of Pediatric Cardiology, from the Clinical Emergency Hospital for Children Louis Țurcanu Timișoara, in a period of 3 years (2015-2018). There were 70 patients included in the study, who presented congenital heart malformation diagnosed clinically, by ultrasound and Angio-CT. Results. The incidence of cardiac malformations in our unit was 2.11%. The most common clinical signs encountered in patients with congenital heart disease were dyspnea associated with cyanosis, systolic murmur and oxygen saturation oscillations (SaO2). Associated with pregnancy, the presence of teratogenic factors with possible implication in congenital heart malformations was also detected. There were 12 cases involved genetic syndromes, Down's syndrome having the highest prevalence. Due to the complexity of the cardiac malformation in the studied group, 11 patients (15.71%) died. Conclusions. Congenital cardiovascular malformations are a problem of public health. In the assessment of a newborn with cardiac pathology, there should be involved obstetricians, neonatologists, anesthetists, pediatric cardiologists, pediatric cardiovascular surgeons, geneticists, with the common goal of preventing, diagnosing, monitoring and treating congenital heart abnormalities.