RESUMO
BACKGROUND: late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. METHODS AND RESULTS: we conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. CONCLUSIONS: preoperative clinical and morphological features are important risk factors for postoperative PVO and survival.
Assuntos
Procedimentos Cirúrgicos Cardiovasculares , Complicações Pós-Operatórias/epidemiologia , Pneumopatia Veno-Oclusiva/epidemiologia , Feminino , Humanos , Recém-Nascido , Masculino , Veias Pulmonares/anormalidades , Estudos Retrospectivos , Fatores de Risco , Síndrome de Cimitarra/cirurgia , Resultado do TratamentoRESUMO
We report two brothers, their mother and a maternal cousin who had a distinctive facial phenotype, mild brachydactyly and prominence of the interphalangeal joints. One brother and the mother also had multiple ventricular extrasystoles. Six other relatives in four generations were probably affected on the basis of history and family photographs. We also report a further individual from a different family with a similar facial phenotype, Pierre-Robin sequence, tapering fingers and multiple ventricular extrasystoles. These families have some similarities to those reported by Stoll et al. in a single family, showing dominant inheritance. Our patients would seem to have the same or a related condition.
Assuntos
Anormalidades Múltiplas/patologia , Anormalidades Craniofaciais/patologia , Fácies , Falanges dos Dedos da Mão/anormalidades , Complexos Ventriculares Prematuros/congênito , Anormalidades Múltiplas/diagnóstico , Anormalidades Múltiplas/genética , Adolescente , Adulto , Estatura , Criança , Feminino , Falanges dos Dedos da Mão/diagnóstico por imagem , Humanos , Masculino , Micrognatismo/patologia , Linhagem , Radiografia , Síndrome , Complexos Ventriculares Prematuros/fisiopatologiaRESUMO
OBJECTIVE: To assess the impact of early corrective surgery on the short and medium term outcome in tetralogy of Fallot (TOF). MATERIALS AND METHODS: All patients under 12 months of age undergoing correction of isolated TOF between February 1997 and July 2003 were reviewed retrospectively. Outcome data for mortality, post-operative care management, major morbidity and clinical follow-up were analysed. RESULTS: Fifty-two operations were performed. The mean age at surgery was 5 months (range 1-12) of whom 16 (30.8%) were less than 3 months old, including 2 neonates, 22 (42.3%) were 3-6 months old and 14 (26.9%) were 7-12 months old. There was 1 (1.9%) early death caused by a cerebro-vascular accident and 1 (1.9%) late death secondary to acute infective endocarditis. There were no differences in post-operative morbidities attributable to age. Patients under 3 months old required greater duration of post-operative ventilation, ITU stay and in-hospital stay. At a mean follow-up of 4.0 years (range 1.5-8.0), 33 (63.5%) patients had well-tolerated pulmonary regurgitation (PR) and 3 (5.8%) patients required re-operation for right ventricular outflow tract obstruction (RVOTO). All patients had right bundle-branch-block but with QRS < 150 ms. CONCLUSION: Early definitive repair of TOF can be performed safely on patients under 6 months old. Age at surgery does not appear to affect the medium term haemodynamic outcome. However, early surgery does escalate the need for ICU care. This data suggests repair in asymptomatic patients be delayed until 3-6 months of age.
Assuntos
Tetralogia de Fallot/cirurgia , Distribuição por Idade , Fatores Etários , Cuidados Críticos , Métodos Epidemiológicos , Feminino , Humanos , Lactente , Recém-Nascido , Tempo de Internação , Masculino , Cuidados Pós-Operatórios/métodos , Complicações Pós-Operatórias , Insuficiência da Valva Pulmonar/etiologia , Respiração Artificial , Resultado do TratamentoRESUMO
OBJECTIVES: We describe the morphologic variability in pulmonary atresia with intact ventricular septum (PAIVS) within a population-based study. BACKGROUND: An uncommon disease, PAIVS shows considerable morphologic heterogeneity. Clinical reports, based mostly on small samples of patients, may not reflect the true spectrum of pathology of this condition. We have studied the entire range of morphology in a prospective population-based study of patients over a five-year period (1991 to 1995). METHODS; As part of the United Kingdom and Ireland Collaborative Study of PAIVS, all 18 pediatric cardiac centers were visited by a single investigator. Morphologic features of each case were determined by direct review of the echocardiograms and angiocardiograms, from surgical and autopsy reports, and by review of pathology specimens where available. RESULTS: Among 183 live-born infants, atresia was valvar (membranous) in 74.7% and muscular in 25.3%. Muscular obliteration of the apical trabecular cavity, and in some cases its infundibulum, resulted in "bipartite" right ventricle (RV) in 33.6%, and a "unipartite" chamber in 7.7%. The remaining 58.7% had "tripartite" morphology. Coronary arterial abnormalities were identified in 45.8%, including arterial stenoses, interruptions and ectasia in 7.6%. Ebstein's malformation coexisted in 18 patients. Median tricuspid valvar size and RV inlet Z-scores were -5.2 and -5.1, respectively. CONCLUSIONS: This study provides unique data on the diverse pathology of PAIVS in an unselected population. This will help determine if published reports reflect the true spectrum of pathology of the condition.
Assuntos
Comunicação Interventricular/patologia , Atresia Pulmonar/patologia , Feminino , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/patologia , Comunicação Interventricular/diagnóstico , Septos Cardíacos/patologia , Humanos , Recém-Nascido , Irlanda , Masculino , Estudos Prospectivos , Atresia Pulmonar/diagnóstico , Valva Pulmonar/patologia , Reino UnidoRESUMO
OBJECTIVE: The objective of this article is to evaluate the incidence of reoperation and the associated risk and survival after primary repair of congenital heart defects using cardiopulmonary bypass in children. METHODS: We present a retrospective analysis of 1220 consecutive children under 16 years [649 (53%) under 1 year] operated on between 1976 and 2001 by 1 surgeon (J.L.M.). RESULTS: The early (30-day) mortality was 6.9%; 171 patients had 206 reoperations. The early mortality for the first reoperation was 10.4% and for second reoperation, 3.8%. Of the first-time reoperations 63% were inevitable, 15% were planned, and 22% were unexpected. The overall 20-year freedom from reoperation was 83% and survival (including early mortality) was 86%. The 10-year freedom from reoperation and survival, respectively, was as follows: aortic valvotomy, 77% and 92%; pulmonary valvotomy, 90% and 95%; atrial septal defect, 99% and 100%; partial atrioventricular septal defect (AVSD), 81% and 94%; complete AVSD, 74% and 70%; VSD, 95% and 97%; double-outlet right ventricle, 66% and 68%; truncus arteriosus, 54% and 71%; Mustard, 85% and 86%; arterial switch, 78% and 74%; Fontan, 77% and 66%; Fallot, 91% and 93%; and total anomalous pulmonary venous drainage, 89% and 84%. Of those undergoing aortic valvotomy, 53% were infants, but when aortic valve replacement became necessary an adult valve could be inserted. Introduction of the total cavopulmonary connection to the Fontan procedure in 1990 with prior cavopulmonary anastomoses has greatly improved outcome, with only 1 reoperation and no deaths since then. Patients with AVSD required 12 reoperations for mitral regurgitation, mostly through the "cleft," the closure of which in the past 5 years has provided promising results. CONCLUSIONS: The majority of reoperations after repair of congenital heart defects in children are inevitable, and their incidence varies for different types of procedures. These findings will help in informing parents about the possible outcome of surgery, but the introduction of newer techniques may reduce the need for reoperation further. The survival is encouraging.
Assuntos
Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Reoperação , Adolescente , Fatores Etários , Procedimentos Cirúrgicos Cardíacos , Criança , Proteção da Criança , Pré-Escolar , Inglaterra/epidemiologia , Seguimentos , Humanos , Incidência , Lactente , Bem-Estar do Lactente , Recém-Nascido , Recidiva , Estudos Retrospectivos , Análise de Sobrevida , Fatores de Tempo , Resultado do TratamentoRESUMO
OBJECTIVE: The optimal time and approach of repair of tetralogy of Fallot (TOF) remain controversial. The purpose of this study was to evaluate the outcome following repair of TOF in infants with particular regard to the surgical approach used. PATIENTS: One hundred and sixty infants (mean age 195+/-89 days, range 11-364 days) undergoing repair of a simple TOF were studied. Between 1974 and 2000, a transventricular approach (RV) was used in 91 and between 1988 and 2000, a transatrial (RA) approach in 69 infants. Ten of these infants (6.2%) had a previous palliative shunt (four in the RV versus six in the RA group). A transannular patch (TAP) was inserted in 96 (60%) infants (76 versus 20). Follow-up was complete (mean 14.5+/-5.2 versus 6+/-1 years). RESULTS: There were three operative deaths (1.9%), (two in RV versus one in RA group). A re-operation for right ventricular outflow tract obstruction (RVOTO) was performed in 19 patients (3 versus 16). Ten-year freedom from re-operation for RVOTO (+/-standard error of the mean) was 88+/-4% (98+/-2 versus 72+/-6%, P<0.0001). Within the RA group, 5-year freedom from re-operation for RVOTO for those who had a TAP was 79+/-9% and it was 75+/-4% for those having a simple repair. Six patients in the RV group required pulmonary valve replacement (PVR). Ten-year freedom from PVR was 98+/-1% (97+/-2 versus 100%, P=0.3). There were two late deaths, one in each group. Ten-year survival was 97+/-1%. One patient in the RV group developed late recurrent ventricular tachycardia requiring the implantation of a defibrillator. At most recent echocardiography, all but the patient who had the defibrillator had good right and left ventricular function. CONCLUSIONS: Transventricular and transatrial repair of TOF in infancy, are associated with an acceptable operative risk, low incidence of late arrhythmia, good bi-ventricular function and excellent survival. In our experience, however, transatrial repair has a disturbing incidence of early and mid-term residual or recurrent RVOTO, even when a TAP has been used.
Assuntos
Tetralogia de Fallot/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Distribuição de Qui-Quadrado , Ecocardiografia , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Reoperação , Análise de Sobrevida , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/fisiopatologia , Resultado do TratamentoRESUMO
OBJECTIVE: Pulmonary venous obstruction (PVO) is an important cause of late mortality in total anomalous pulmonary venous connection (TAPVC). We aimed to describe current practices for the management of postoperative PVO and the efficacy of the different interventional procedures. METHODS: We conducted a retrospective international collaborative population-based study involving 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. Patients with TAPVC born between January 1, 1998, and December 31, 2004, were identified. Patients with functionally univentricular circulation or atrial isomerism were excluded. All available data and images were reviewed. RESULTS: Of 406 patients undergoing repair of TAPVC, 71 (17.5%) had postoperative PVO. The diagnosis was made within 6 months of surgery in 59 (83%) of the 71 patients. In 12, serial imaging documented change in appearance of the pulmonary veins. Good-sized pulmonary veins can progress to diffusely small veins and rarely atresia. Patients presenting after 6 months had less severe disease; all are alive at most recent follow-up. Fifty-six (13.8%) of 406 patients underwent intervention for postoperative PVO: 44 had surgical treatment and 12 had an initial catheter intervention. One half underwent 1 or more reinterventions. Three-year survival for patients with postoperative PVO was 58.7% (95% confidence intervals, 46.2%-69.2%) with a trend that those having a surgical strategy did better (P = .083). Risk factors for death included earlier presentation after TAPVC repair, diffusely small pulmonary veins at presentation of postoperative PVO, and an increased number of lung segments affected by obstruction. CONCLUSIONS: Postoperative PVO tends to appear in the first 6 months after TAPVC repair and can be progressive. Early intervention for PVO may be indicated before irreversible secondary changes occur.
Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Endovasculares , Pneumopatia Veno-Oclusiva/terapia , Síndrome de Cimitarra/cirurgia , Procedimentos Cirúrgicos Cardíacos/mortalidade , Progressão da Doença , Procedimentos Endovasculares/efeitos adversos , Procedimentos Endovasculares/mortalidade , Europa (Continente)/epidemiologia , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Prevalência , Modelos de Riscos Proporcionais , Pneumopatia Veno-Oclusiva/diagnóstico , Pneumopatia Veno-Oclusiva/etiologia , Pneumopatia Veno-Oclusiva/mortalidade , Reoperação , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Síndrome de Cimitarra/mortalidade , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do TratamentoRESUMO
AIMS: Hypertrophic cardiomyopathy (HCM) is the commonest inherited cause of sudden cardiac death in children; current guidelines suggest HCM screening after 12-15 years of age. The study aims to establish the age range at highest risk. METHODS AND RESULTS: Cohort study from six regional centres of paediatric cardiology, including children presenting with sudden death; n = 150 (59% = male; 39% familial HCM). Age- and gender-specific mortality was calculated, and compared with rates calculated from the Swedish National Cause of Death Registry. There were 56 deaths within the cohort, 39 were sudden arrhythmia deaths, with 31 at <19 years of age. Between 9-13.9 years of age annual sudden death mortality averages 7.2%, vs. 1.7% after 16 years of age; P = 0.025, odds ratio for proportions 3.75 [95% confidence intervals (CI) 1.18-11.91], similar in both familial and idiopathic HCM. The risk for sudden death peaks earlier in girls (10-11 years), with male preponderance after the age of 15. National cause of death statistics confirm that the mortality rate from HCM is significantly higher in the 8-16 year olds (0.112 per 100,000 age-specific population) than in the 17-30 year olds (0.055 per 100,000; 95% CI 0.011-0.099). CONCLUSION: In families with HCM, children should be screened at an early age.
Assuntos
Cardiomiopatia Hipertrófica/mortalidade , Morte Súbita Cardíaca/etiologia , Obstrução do Fluxo Ventricular Externo/mortalidade , Adolescente , Antagonistas Adrenérgicos beta/uso terapêutico , Adulto , Distribuição por Idade , Fatores Etários , Androgênios/metabolismo , Cardiomiopatia Hipertrófica/tratamento farmacológico , Criança , Estudos de Coortes , Morte Súbita Cardíaca/epidemiologia , Eletrocardiografia , Feminino , Humanos , Masculino , Guias de Prática Clínica como Assunto , Medição de Risco , Fatores Sexuais , Obstrução do Fluxo Ventricular Externo/tratamento farmacológicoRESUMO
BACKGROUND: The failing Fontan circulation is associated with hepatic impairment. The nature of this liver injury is poorly defined. OBJECTIVE: To establish the gross and histological liver changes of patients with Fontan circulation relative to clinical, biochemical and haemodynamic findings. METHODS: Patients were retrospectively assessed for extracardiac Fontan conversion between September 2003 and June 2005, according to an established clinical protocol. Twelve patients, mean age 24.6 (range 15.8-43.4) years were identified. The mean duration since the initial Fontan procedure was 14.1 (range 6.9-26.4) years. RESULTS: Zonal enhancement of the liver (4/12) on CT was more common in patients with lower hepatic vein pressures (p = 0.007), and in those with absent cardiac cirrhosis on histological examination (p = 0.033). Gastro-oesophageal varices (4/12) were more common in patients with higher hepatic vein pressure (21 (6.3) vs 12.2 (2.2) mm Hg, p = 0.013) and associated with more advanced cirrhosis (p = 0.037). The extent of cirrhosis (7/12) was positively correlated with the hepatic vein pressure (r = 0.83, p = 0.003). A significant positive correlation was found between the Fontan duration and the degree of hepatic fibrosis (r = 0.75, p = 0.013), as well as presence of broad scars (r = 0.71, p = 0.021). Protein-losing enteropathy (5/12) occurred more frequently in patients with longer Fontan duration (11.7 (3.2) vs 17.9 (6.1) years, p = 0.038). CONCLUSIONS: Liver injury, which can be extensive in this patient group, is related to Fontan duration and hepatic vein pressures. CT scan assists non-invasive assessment. Cardiac cirrhosis with the risk of developing gastro-oesophageal varices and regenerative liver nodules, a precursor to hepatocellular carcinoma, is common in this patient group.
Assuntos
Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/fisiopatologia , Circulação Hepática/fisiologia , Hepatopatias/etiologia , Adolescente , Adulto , Pressão Sanguínea/fisiologia , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Humanos , Hepatopatias/patologia , Hepatopatias/fisiopatologia , Testes de Função Hepática , Masculino , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
OBJECTIVE: To define the anatomic characteristics of the congenitally malformed and severely stenotic aortic valve using trans-thoracic real time three-dimensional echocardiography, and to compare and contrast this with the valvar morphology as seen at surgery. DESIGN: Prospective cross-sectional observational study. SETTING: Tertiary centre for paediatric cardiology. METHODS: All patients requiring aortic valvotomy between December 2003 and July 2004 were evaluated prior to surgery with three-dimensional echocardiography. Full volume loop images were acquired using the Phillips Sonos 7500 system. A single observer analysed the images using "Q lab 4.1" software. The details were then compared with operative findings. RESULTS: We identified 8 consecutive patients, with a median age of 16 weeks, ranging from 1 day to 11 years, with median weight of 7.22 kilograms, ranging from 2.78 to 22 kilograms. The measured diameter of the valvar orifice, and the number of leaflets identified, corresponded closely with surgical assessment. The sites of fusion of the leaflets were correctly identified by the echocardiographic imaging in all cases. Fusion between the right and non-coronary leaflets was identified in half the patients. Dysplasia was observed in 3 patients, with 1 patient having nodules and 2 shown to have excrescences. At surgery, nodules were excised, and excrescences were trimmed. The dysplastic changes correlated well with operative findings, though statistically not significant. CONCLUSION: We recommend trans-thoracic real time three-dimensional echocardiography for the assessment of the congenitally malformed aortic valve, particularly to identify sites of fusion between leaflets and to measure the orificial diameter. The definition of nodularity, and the prognosis of nodules based on the mode of intervention, will need a comparative study of patients submitted to balloon dilation as well as those undergoing surgical valvotomy.
Assuntos
Estenose da Valva Aórtica , Procedimentos Cirúrgicos Cardíacos/métodos , Ecocardiografia Tridimensional/métodos , Estenose da Valva Aórtica/congênito , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/cirurgia , Criança , Pré-Escolar , Estudos Transversais , Seguimentos , Humanos , Lactente , Recém-Nascido , Estudos Prospectivos , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
BACKGROUND: Hypertrophic cardiomyopathy is a common cause of sudden death in children. In this study, we aimed to identify clinical measures for stratification of this risk in childhood. PATIENTS AND METHODS: By means of a retrospective cohort study from six regional centres of paediatric cardiology, we identified 128 patients with hypertrophic cardiomyopathy presenting below 19 years of age, with a mean follow-up of 10.8 years. Of the patients, 31 had died, 16 suddenly, with a median age at sudden death of 13.3 years. RESULTS: Cox regression shows that electrocardiographic voltages, analysed as the sum of the R and S waves in all six limb leads (p equal to 0.001), and septal thickness expressed as proportion of the 95th centile for age (p equal to 0.036), were independent predictors of sudden death. When the sum of the R and S waves is over 10 millivolts, the odds ratio for sudden death was 8.4, with 95% confidence intervals from 2.2 to 33.7 (p equal to 0.0012), and finding a septal thickness over 190% of 95th centile for age gives an odds ratio of 6.2, with confidence intervals from 1.5 to 25.1 (p equal to 0.011). Noonan's syndrome, with a p value equal to 0.043, and the ratio of the left ventricular wall to its cavity in diastole, with a p value equal to 0.005, were independent predictors of death in cardiac failure, with a ratio of the mural thickness to the dimension of the cavity over 0.30 giving an odds ratio of 36.0, with confidence limits from 4.2 to 311, and a p value equal to 0.00009. At follow-up, patients deemed to be at a high risk of dying suddenly were identified by the combination of the sum of the R and S waves greater than 10 millivolts and septal thickness over 190%, with a sensitivity of 91%, specificity of 78%, positive predictive value of 50%, and a negative predictive value of 97%. CONCLUSIONS: Children at high risk of dying suddenly with hypertrophic cardiomyopathy, with a subsequent annual mortality of 6.6%, can be distinguished at the time of diagnosis from those patients having a low risk of sudden death, the latter with an annual mortality of 0.27%.
Assuntos
Cardiomiopatia Hipertrófica/mortalidade , Morte Súbita Cardíaca , Ecocardiografia/métodos , Eletrocardiografia/métodos , Adolescente , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Cardiomiopatia Hipertrófica/terapia , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Valor Preditivo dos Testes , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Medição de Risco , Fatores de RiscoRESUMO
Acute myocarditis is characterized by the development of rapid life-threatening congestive heart failure and arrhythmias. In many cases with hemodynamic compromise, medical therapy and mechanical support alone are not sufficient. Various surgical procedures have been tried to bridge patients with myocarditis to both transplant and recovery. Mitral regurgitation is a frequent association with end stage cardiomyopathy and predicts poor outcome. Mitral annuloplasty is well-established in adults with ischemic and dilated cardiomyopathy and the results are superior to medical therapy alone and are comparable to cardiac transplantation. However, its effectiveness and use is not well-established in children with cardiomyopathy. We report our experience in two children.
Assuntos
Insuficiência da Valva Mitral/cirurgia , Miocardite/complicações , Feminino , Humanos , Hipertensão Pulmonar/etiologia , Lactente , Recém-Nascido , Masculino , Insuficiência da Valva Mitral/etiologia , Insuficiência da Valva Mitral/fisiopatologiaRESUMO
Patients with unpalliated complex cyanotic congenital heart may have significant morbidity resulting from severe pulmonary hypertension. In late stages, medical management is often difficult, and worsening right heart failure is resistant to medication. The risk of complications and early death can be averted by detailed evaluation and prompt intervention to identify the reversible elements that compound their physiology. It is vital to address any treatable issue to improve a patient's quality of life while awaiting heart-lung transplantation. Our case is a good example of such an approach, where the identification and treatment of reversible pulmonary venous hypertension in the background of irreversible pulmonary arterial hypertension, by offloading the left atrium by atrial septostomy and stent implantation, resulted in significant improvement in the quality of life for the patient.
Assuntos
Cateterismo Cardíaco/métodos , Cateterismo/métodos , Átrios do Coração/cirurgia , Cardiopatias Congênitas/terapia , Hipertensão Pulmonar/terapia , Stents , Adulto , Procedimentos Cirúrgicos Cardíacos , Eletrocardiografia , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/fisiopatologia , Septos Cardíacos/cirurgia , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/fisiopatologia , Qualidade de Vida , Resultado do TratamentoRESUMO
We present three cases of multiple interatrial communications to illustrate the extended use of the Amplatzer septal occluder and to highlight procedural techniques. The first case demonstrates how a single device can be used to close two adjacent defects. It is important to balloon size both defects and to note if balloon occlusion of one defect closes the other and vice versa. The second case demonstrates closure of multiple atrial septal defects using two devices. The technique needed to obtain an "interleaved" position of the two devices is detailed. This technique allows for a lower profile that reduces stress on the devices and surrounding structures and may decrease the risk of thromboembolism and device embolization. The third case describes device closure of a residual defect adjacent to a previous device implanted at an earlier sitting. In this situation, an overlapping position without the option for "interleaving" is inevitable.
Assuntos
Comunicação Interatrial/cirurgia , Próteses e Implantes , Implantação de Prótese/métodos , Criança , Pré-Escolar , Desenho de Equipamento , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
New data are emerging on the use of palivizumab as prophylaxis against infection with the respiratory syncytial virus in infants with congenital cardiac disease. Following a 4-year multicentre randomised trial, it was shown that prophylactic injections with palivizumab were effective and safe for such children. Prophylaxis consists of 5, monthly, intramuscular injections of palivizumab, at a dose of 15 mg/kg, given during the season for infection with the respiratory syncytial virus. Timing is at the discretion of the physician, depending on the onset of the season locally. It is suggested that, in the United Kingdom, this should be commenced in mid-September. To help clinicians to identify appropriate candidates for palivizumab, a working group of the British Paediatric Cardiac Association has developed recommendations. Infants, namely those under 1 year old, with congenital cardiac disease likely to benefit from prophylaxis include those with haemodynamically significant lesions, particularly increased pulmonary blood flow with or without cyanosis; pulmonary venous congestion, pulmonary hypertension or long-term pulmonary complications, residual haemodynamic abnormalities following medical or surgical intervention (patients who have undergone cardiopulmonary bypass should receive an injection as soon as they are medically stable), cardiomyopathy requiring treatment, and congenital cardiac disease likely to need hospital admission for medical or surgical intervention during the season of infection with the virus. Prophylaxis with palivizumab may also be indicated, at the discretion of the physician, in some children with complex cardiac disease over the age of 1 year. Children less likely to benefit from prophylaxis are those with haemodynamically insignificant disease, or those with lesions adequately corrected by medical or surgical intervention.