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Showing posts with label congenital heart diseases. Show all posts
Showing posts with label congenital heart diseases. Show all posts

Sunday, December 23, 2018

Tetralogy of Fallot

Key Points
Question  What are the patient and surgical characteristics affecting long-term transplant-free survival following surgical tetralogy of Fallot repair?
Findings  In this cohort study of 3283 patients with tetralogy of Fallot, survival following complete repair was 98.6%, 97.8%, 97.1%, 95.5%, and 94.5% for 1-year, 5-year, 10-year, 20-year, and 25-year survival, respectively, with an early peaking hazard of mortality shortly after repair. Statistically significant associations with decreased long-term survival included staged repair, non–valve-sparing operation, repair in earlier surgical era, and presence of a genetic abnormality.
Meaning  Overall long-term transplant-free survival in repaired tetralogy of Fallot is excellent, with several factors affecting survival, some of which may be modifiable such as planning of the surgical strategy.

Saturday, June 16, 2018

Cirurgia Cardíaca Pediátrica

O Hospital Moinhos de Vento, Renato Abdala Karam Kalil, Luis Nasi e Carisi A. Polanczick estão de parabéns!
A Inauguração do Centro de Cirurgia Cardíaca e Cardiologia Pediátrica no dia de ontem me fez lembrar: o Ambulatório Cardiopulmonar da Enfermaria 38 da Santa Casa (na segunda metade da décadqa de 50) do qual fui diretor; do Serviço do Prof. Eduardo FaracoFlávio Freitas, Adão do Valle Mattos, do Instituto Sábato D' Angelo, Hugo Felipozzi e Adauto Barbosa Lima, da Equipe de Cirurgia Cardíaca daqui, liderada pelo Cid Nogueira, do João Batista Pereira da Enfermaria 30, das cirurgias de crianças de baixo peso (pioneirismo no país), do Berçário da Maternidade Mario Totta, e da Enfermaria de Pediatria, da Maria Clara Mariano da Rocha e da Estela Budiansky, Décio Martins Costa e Carlos Hoffmeiste; do Hospital da Criança Santo Antônio e todos seus pediatras quando lá iniciei um Serviço de Cardiologia Pediátrica que depois repassei para Céu Paranhos de Lima, Joyce Bertoletti e Nestor Daudt, do Ambulatório de Cardiologia Pediátrica do Instituto de Cardiologia; da Cirurgia Cardíaca no mesmo Instituto; do tempo como "Cardiology-affiliate da American Academy of Paediatrics"; do Hospital da Criança Conceição; do Serviço de Cardiologia do Hospital Universitário da PUCRS; do Ambulatório de Cardiologia Preventiva no Centro de Saúde No. 2, do Programa de Prevenção da Febre Reumática na Secretaria da Saúde e do Meio Ambiente do Estado, das atividades conjuntas com a Secretaria de Educação e Cultura e SSMA com escolares de Porto Alegre e de todo o Estado, da Criação do Departamento de Cardiologia Pediátrica da Sociedade Brasileira de Cardiologia (1972) juntamente com a saudosa Rachel Snitkowsy; do Programa Nacional de Prevenção da Febre Reumática, das ações junto à Organização Pan-Americana da Saúde que se iniciaram a pretexto do controle da Febre Reumática na comunidade e cuja primeira Reunião nas Américas aconteceu aqui em Porto Alegre (1975) e da qual fui o coordenador; do Jorge Litvak, do Hector Boffi, do Luis Ruis, Simón Muñoz  e tantos outros do Comité Pan-Americano da Prevenção da Febre Reumática e dos encontros inter-países com a mesma finalidade, do Ad-Hoc Committee of Rheumatic Fever and Rheumatic Heart Diseases do qual fui Chairman, suscedendo a Edward Kaplan nos anos noventa até o início deste século (passando-o para a categoria de Council e introduzindo o Australiano Jonathan Carapetis), da Comissão Científica da International Society and Federation of Cardiology - depois World Heart Federation, da Marianne Burle de Figueiredo, do World Award for Cardiology com que me honraram em 2002 em Sidney, das Ações em conjunto com a Organização Mundial da Saúde, do Porfírio Nordet, dos Projetos em conjunto com a UNESCO e com o apoio da Comunidade Europeia, e da Rainha da Espanha através do Antonio Bayés de Luna - muito embora a parte do projeto a ser implantado em BanglaDesh tenha ficado como uma frustração - Lembrei  do Mário Mancini e Mário Maranhão. Da  Lúcia Pellanda, hoje Reitora da FUCSPA, da homenagem do Departamento de Cardiopatias Congênitas e Cardiologia Pediátrica que recebi em 2012 em Foz do Iguaçu; do Professor Décourt, Adib Jatene, Sergio Almeida Oliveira, Ivo Nesralah, Fernando Lucchesi, Rhadi Macruz e tantos outros...
Lembrei-me de tantos pacientes, alguns recém nascidos, alguns que não sobreviveram, mas outros tantos hoje adultos, já com filhos e até netos...
Comecei a citar nomes, numa tarefa impossível de concluir pois se iniciou há mais de sessenta anos e minha memória já vai falhando...
Algumas histórias que cabem nesse contexto já tenho escrito por aí, mas tenho ainda intenção de reorganizar minha memória como testemunha ocular e protagonista.
clique aqui para um link de slide-show em pdf
Enviado por Hospital Moinhos de Vento
Rua Ramiro Barcelos 910 / Moinhos de Vento / Porto Alegre / RS / Brasil
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Tuesday, February 07, 2017

Congenital HD survival


Question  Has survivorship in young children with congenital heart disease increased in Sweden?
Finding  A registry-based, prospective, matched-cohort study showed that, among children younger than 5 years, survivorship was increased in those born at the beginning of 1990 compared with children born in the 1970s; however, the overall mortality risk was higher, depending on the complexity of the disease, than in matched controls.
Meaning  Absolute and relative survivorship increased substantially, but mortality remains high in young patients with congenital heart disease compared with matched controls, particularly in subsets of children with complex issues.
Abstract
Importance  Mortality in patients with congenital heart disease (CHD) has markedly decreased during recent decades because of advancement in pediatric care. However, there are limited data on survival trends in children and young adults with CHD compared with the general population.
Objective  To determine survivorship in children and young adults with CHD compared with matched controls.
Design, Setting, and Participants  A registry-based, prospective, matched-cohort study was conducted in Sweden. Data from the national patient and cause of death registers were linked to identify individuals with CHD born between January 1, 1970, and December 31, 1993, who were registered at or after birth. Follow-up and comorbidity data were collected until December 31, 2011. Survival analyses were performed with the Cox proportional hazards model; these analyses were performed from January 1, 1970, to December 31, 2011. A total of 21 982 patients with CHD in Sweden were identified. The mean (SD) follow-up time was 27.0 (8.86) years. Children serving as controls (n = 219 816) (10 for each patient), matched for birth year, sex, and county, were randomly selected from the general population.
Main Outcomes and Measures  Survivorship in young patients with CHD and controls.
Results  Of the 21 982 patients who were born between 1970 and 1993 and were registered with the diagnosis of CHD, 10 650 were female (48.4%). Median age at index registration was 4.22 years (interquartile range, 17.07 years). Survivorship among children younger than 5 years was increased from 96% in those born in 1970-1979 to 98% in those born in 1990-1993. Hazard ratios (HRs) of death in relation to that in control individuals decreased from 225.84 (95% CI, 136.84-372.70) to 33.47 (95% CI, 22.54-49.70). A substantial, but less pronounced, absolute and relative increase in survivorship was found in older patients (HRs ranged from 24.52; 95% CI, 11.72-51.26, at 5-9 years to 4.27; 95% CI, 2.29-7.95, at 18-29 years). According to a hierarchical CHD classification, the group of patients with the most severe complex defects (ie, common arterial trunk, transposition of the great vessels, double inlet ventricle, hypoplastic left heart syndrome, tetralogy of Fallot, and atrioventricular septal defect) had the highest risk for death (HR, 64.07; 95% CI, 53.39-76.89).
Conclusions and Relevance  Despite substantially increasing absolute and relative survivorship in children and young adults with CHD, the mortality risk remains high compared with the risk in matched controls. Further research on reducing the death rate in this vulnerable group is required.

Sunday, December 25, 2016

Infants with major Congenital Anomalies: Mortality of thei motheres

Key Points
Question  Do mothers who give birth to an infant with a major congenital anomaly have an increased risk of mortality?
Findings  In this Danish population-based cohort study of 455 250 women, mothers of infants born with a major congenital anomaly had a significantly increased mortality risk compared with women without an affected infant (absolute mortality rate difference, 0.33 per 1000 person-years; hazard ratio, 1.27). This elevated risk was noted both during the first 10 years after the child’s birth, when the mother was likely caring for a dependent child with substantial health needs, and after longer follow-up, and no single cause of death explained this association.
Meaning  Having a child with a major congenital anomaly was associated with a small but significantly increased risk of mortality in the mother.
Abstract
Importance  Giving birth to a child with a major birth defect is a serious life event for a woman, yet little is known about the long-term health consequences for the mother.
Objective  To assess whether birth of an infant born with a major congenital anomaly was associated with higher maternal risk of mortality.
Design, Setting, and Participants  This population-based cohort study (n = 455 250 women) used individual-level linked Danish registry data for mothers who gave birth to an infant with a major congenital anomaly (41 508) between 1979 and 2010, with follow-up until December 31, 2014. A comparison cohort (413 742) was constructed by randomly sampling, for each mother with an affected infant, up to 10 mothers matched on maternal age, parity, and year of infant’s birth.
Exposure  Live birth of an infant with a major congenital anomaly as defined by the European Surveillance of Congenital Anomalies classification system.
Main Outcomes and Measures  Primary outcome was all-cause mortality. Secondary outcomes included cause-specific mortality. Hazard ratios (HRs) were adjusted for marital status, immigration status, income quartile (since 1980), educational level (since 1981), diabetes mellitus, modified Charlson comorbidity index score, hypertension, depression, history of alcohol-related disease, previous spontaneous abortion, pregnancy complications, smoking (since 1991), and body mass index (since 2004).
Results  Mothers in both groups were a mean (SD) age of 28.9 (5.1) years at delivery. After a median (IQR) follow-up of 21 (12-28) years, there were 1275 deaths (1.60 per 1000 person-years) among 41 508 mothers of a child with a major congenital anomaly vs 10 112 deaths (1.27 per 1000 person-years) among 413 742 mothers in the comparison cohort, corresponding to an absolute mortality rate difference of 0.33 per 1000 person-years (95% CI, 0.24-0.42), an unadjusted HR of 1.27 (95% CI, 1.20-1.35), and an adjusted HR of 1.22 (95% CI, 1.15-1.29). Mothers with affected infants were more likely to die of cardiovascular disease (rate difference, 0.05 per 1000 person-years [95% CI, 0.02-0.08]; adjusted HR, 1.26 [95% CI, 1.04-1.53]), respiratory disease (rate difference, 0.02 per 1000 person-years [95% CI, 0.00-0.04]; adjusted HR, 1.45 [95% CI, 1.01-2.08]), and other natural causes (rate difference, 0.11 per 1000 person-years [95% CI, 0.07-0.15]; adjusted HR, 1.50 [95% CI, 1.27-1.76]).
Conclusions and Relevance  In Denmark, having a child with a major congenital anomaly was associated with a small but statistically significantly increased mortality risk in the mother compared with women without an affected child. However, the clinical importance of this association is uncertain.
 

Friday, December 16, 2016

Childhood heart disease

Congenital heart disease is the most common of all birth defects, occurring in about nine per 1000 livebirths globally.1 The cause of most cases of congenital heart disease is unknown and the rate of disease is fairly stable across countries and populations, making the burden heaviest on low-income countries with high fertility rates. Although overall child mortality has decreased by half globally, death and disability due to congenital heart disease has consistently increased in low-income and middle-income countries over the past two decades.2 Access to care for children with heart disease has not kept pace. Of the 1·35 million children born each year with congenital heart disease,1 90% live in places that do not have adequate access to diagnostics or care.3 Furthermore, individuals with congenital heart defects need lifelong care and follow-up from primary care specialists, special attention to dental care, and, in many cases, more surgical interventions from trained health professionals.4 An estimated 58% of congenital heart disease burden could be averted if surgical practices of high-income countries were brought to scale in low-income and middle-income countries (LMICs).5
Data on childhood heart disease in LMICs are not systematically collected in child health or cause of death surveillance programmes, therefore the true burden is probably underestimated. Many of the signs and symptoms of paediatric heart disease (eg, lethargy, poor growth, shortness of breath) can be misdiagnosed; the presenting complaint may be treated, but the underlying condition remains to threaten life and livelihood. Reliable data on the burden of congenital heart disease would better allow countries and the global health community to allocate resources to the child health needs in their communities. As countries develop economically, the burden of poverty-related diseases, especially infectious diseases and nutritional deficiencies, in children younger than 5 years diminishes.6 In their place are the chronic and often complex care needs of conditions such as paediatric heart disease and health systems must be ready to respond accordingly. Today, congenital anomalies, of which heart disease represents nearly half, are the fourth leading cause of neonatal death.7
Caring for children with heart disease is not a dilemma of investing in tertiary versus primary health care. Paediatric cardiac success is possible in low-resource settings when incorporated in broader health systems strengthening efforts, notably surgical scale-up efforts as called for by the Lancet Commission on Global Surgery.8 Results from 27 centres in LMICs representing all continents, except for Africa, that participated in the International Quality Improvement Collaborative for Congenital Heart Surgery in Developing World Countries (IQIC) show that when international investments and training partnerships support such efforts, substantial reductions in infection and mortality rates can be achieved.9 Developing local expertise for treating children with heart disease has ripple effects to other health services. Paediatric cardiac surgery interfaces with many other specialties in a hospital. Investments in paediatric cardiac surgery can, therefore, lead to parallel improvements elsewhere in a hospital.10
Children's HeartLink, a US-based non-profit humanitarian organisation, facilitates long-term relationships between local hospitals and leading international medical teaching institutions. Our model to develop sustainable paediatric cardiac care prioritises integrated health systems, quality training programmes, surveillance, research, and protection against financial hardship. Over the years of our experience, we have seen hundreds of children on waiting lists in Vietnam, India, or China—countries where paediatric cardiac care exists but is distributed unequally, lacks quality, or is stifled by underinvestment. The outcomes for these children are often poor. Some of the lucky ones who undergo surgery have to struggle with health systems ill prepared for children growing up with chronic and sometimes complex medical needs. In the past decade, more than 100 000 children in Brazil, China, India, Malaysia, Ukraine, and Vietnam have received cardiac care at our partner sites.11Four of Children's HeartLink's 13 partner hospitals have become self-sustaining centres of excellence in paediatric cardiac care, and now serve as training partners in their region. Children's HeartLink aims to develop 50 centres of excellence and reach 1 million children with heart disease by 2030.12
In our four-part series of reports The Invisible Child,11 Children's HeartLink brought to light the burden of childhood heart disease and the tremendous inequity in access to paediatric cardiac care. The concluding paper, A Voice for the Invisible Child,13 calls on leaders in health and development to acknowledge paediatric heart disease within the global health agenda (panel).

Panel
Call to action—A Voice for the Invisible Child13

  • Investments in increasing capacity at all levels of the health-care delivery system to screen, diagnose, and treat children with heart disease.
  • Building accredited paediatric cardiac training programmes in all regions globally to assure systematic recognition of the basic signs and symptoms of congenital and rheumatic heart disease.
  • Improve surveillance through systematic data collection on paediatric heart disease in national health surveys and include in burden of disease and cause of child death statistics.
  • Assuring paediatric cardiac care will be included in benefits packages in universal health coverage and social protection platforms, and patients will be protected from catastrophic expenses related to their care.
If the global health community is serious about achieving the UN Sustainable Development Goal 3 target of “end[ing] preventable deaths of newborns and children under 5 years of age”,14 leaders in global health development and local policy makers must partner to increase investments for sustainable and equitable access to paediatric cardiac care across the world.
BZ is the Vice President of International Programs at Children's HeartLink. JBA is the former Chair of Children's HeartLink International Advisory Board. We declare no other competing interests.

References

  1. van der Linde, D, Konings, E, and Slager, M. Birth prevalence of congenital heart disease worldwide. J Am Coll Cardiol2011582241–2247
  2. Institute for Health Metrics and Evaluation. Global Burden of Disease 2015.http://ghdx.healthdata.org/gbd-results-tool2016. ((accessed Nov 14, 2016).)
  3. Tchervenkov, C, Jacobs, J, Bernier, P et al. 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. Cardiol Young20081863
  4. Oster, M and Gurvitz, M. Why lifelong care for patients with congenital heart defects is important.AAP News2015368
  5. Debas, H, Donkor, P, Gawande, A, Jamison, D, Kruk, M, and Mock, C. Disease control priorities, third edition: volume 1. Essential surgeryWorld BankWashington, DC2015
  6. GBD 2015 Child Mortality Collaborators. Global, regional, national, and selected subnational levels of stillbirths, neonatal, infant, and under-5 mortality, 1980–2015: a systematic analysis for the Global Burden of Disease Study 2015. Lancet20163881725–1774
  7. Liu, L, Oza, S, Hogan, D et al. Global, regional, and national causes of under-5 mortality in 2000–15: an updated systematic analysis with implications for the Sustainable Development Goals. Lancet2016(published online Nov 10.)http://dx.doi.org/10.1016/S0140-6736(16)31593-8.
  8. Meara, JG, Leather, AJM, Hagander, L et al. Global surgery 2030: evidence and solutions for achieving health, welfare, and economic development. Lancet2015386569–624
  9. Jenkins, K, Castañeda, A, Cherian, K et al. Reducing mortality and infections after congenital heart surgery in the developing world. Pediatrics2014134e1422–e1430
  10. Dearani, J, Neirotti, R, Kohnke, E et al. Improving pediatric cardiac surgical care in developing countries: matching resources to needs. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu2010;1335–43
  11. Children's HeartLink. Treating the invisible child: childhood heart disease and the global health agendaChildren's HeartLinkMinneapolis, MN2016http://theinvisiblechild.childrensheartlink.org/The-Invisible-Child-Brief-Three.pdf. ((accessed Nov 14, 2016).)
  12. Children's HeartLink. 2015 annual reportChildren's HeartLinkMinneapolis, MN2016http://www.childrensheartlink.org/media/childrens-heartlink-2015-annual-report.pdf. ((accessed Nov 14, 2016).)
  13. Children's HeartLink. A voice for the invisible child: Childhood heart disease and the global health agendaChildren's HeartLinkMinneapolis, MN2016http://theinvisiblechild.childrensheartlink.org/The-Invisible-Child-Brief-Four.pdf. ((accessed Nov 14, 2016).)
  14. United Nations. Sustainable Development Goals, 3.2.https://sustainabledevelopment.un.org/sdg3. ((accessed Oct 14, 2016).)

Wednesday, August 12, 2015

Há mais de quarenta anos...

Mario Antônio Mascarenhas e a Equipe de produção de mídia liderada pela Laura Andrade estiveram hoje em nosso consultório para gravar uma entrevista. Deram-me uma surpresa: durante a simulação de um exame clínico, insistiram que eu examinasse o coração sem tirar a camisa. havia um pequeno auto-falante fixado no tórax e cada vez que eu aproximada o estetoscópio, ouvia uma mensagem: "muito obrigado doutor por ter cuidado de meu coração todos estes anos..."
Acompanho o Mário desde os 7,5 meses, há mais de 43 anos. Na época encaminhei-o ao Dr. Danton Coolei no Texas, onde foi operado com sucesso.
Mais do que um cliente, consegui uma família amiga.

Friday, October 26, 2012

Congenital HD US


Newborn Screening for Critical Congenital Heart Disease: Potential Roles of Birth Defects Surveillance Programs — United States, 2010–2011

Weekly

October 26, 2012 / 61(42);849-853

In September 2011, the Secretary of the U.S. Department of Health and Human Services (HHS) approved the Secretary's Advisory Committee on Heritable Disorders in Newborns and Children (SACHDNC) 2010 recommendation that all newborns be screened for critical congenital heart disease (CCHD) using pulse oximetry, a noninvasive test of blood oxygenation, to prevent mortality and morbidity (1). CDC partnered with the National Birth Defects Prevention Network (NBDPN) to conduct a survey designed to assess state birth defect surveillance programs' potential roles, capabilities, and readiness to assist with newborn screening activities for CCHD. States were surveyed in November 2010, after the initial SACHDNC recommendation, and again in November 2011, after the Secretary's approval. From 2010 to 2011, the number of birth defects surveillance programs involved in CCHD screening increased from one to 10. Barriers exist, such as the lack of legislative authority, staffing, funding, and informatics infrastructure. Sixty-seven percent of programs take an average of more than 12 months to collect complete data on birth defect cases, including congenital heart defects. An assessment of state birth defects programs' existing data and capability to lead the evaluation of screening for CCHD is warranted./.../