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1.
Heart ; 104(22): 1864-1870, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-29720396

RESUMO

OBJECTIVES: Treatment of infants with tetralogy of Fallot (ToF) has evolved in the last two decades with increasing use of primary surgical repair (PrR) and transcatheter right ventricular outflow tract palliation (RVOTd), and fewer systemic-to-pulmonary shunts (SPS). We aim to report contemporary results using these treatment options in a comparative study. METHODS: This a retrospective study using data from the UK National Congenital Heart Disease Audit. All infants (n=1662, median age 181 days) with ToF and no other complex defects undergoing repair or palliation between 2000 and 2013 were considered. Matching algorithms were used to minimise confounding due to lower age and weight in those palliated. RESULTS: Patients underwent PrR (n=1244), SPS (n=311) or RVOTd (n=107). Mortality at 12 years was higher when repair or palliation was performed before the age of 60 days rather than after, most significantly for primary repair (18.7% vs 2.2%, P<0.001), less so for RVOTd (10.8% vs 0%, P=0.06) or SPS (12.4% vs 8.3%, P=0.2). In the matched groups of patients, RVOTd was associated with more right ventricular outflow tract (RVOT) reinterventions (HR=2.3, P=0.05 vs PrR, HR=7.2, P=0.001 vs SPS) and fewer pulmonary valve replacements (PVR) (HR=0.3 vs PrR, P=0.05) at 12 years, with lower mortality after complete repair (HR=0.2 versus PrR, P=0.09). CONCLUSIONS: We found that RVOTd was associated with more RVOT reinterventions, fewer PVR and fewer deaths when compared with PrR in comparable, young infants, especially so in those under 60 days at the time of the first procedure.


Assuntos
Cateterismo Cardíaco , Procedimentos Cirúrgicos Cardíacos , Cuidados Paliativos/métodos , Tetralogia de Fallot/terapia , Fatores Etários , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/mortalidade , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Criança , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Auditoria Médica , Estudos Retrospectivos , Fatores de Risco , Tetralogia de Fallot/diagnóstico , Tetralogia de Fallot/mortalidade , Tetralogia de Fallot/cirurgia , Fatores de Tempo , Resultado do Tratamento , Reino Unido
2.
Catheter Cardiovasc Interv ; 90(7): 1135-1144, 2017 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-28799706

RESUMO

OBJECTIVES: This study aimed to report our national experience with transcatheter patent ductus arteriosus (PDA) occlusion in infants weighing <6 kg. BACKGROUND: The technique of transcatheter PDA closure has evolved in the past two decades and is increasingly used in smaller patients but data on safety and efficacy are limited. METHODS: Patients weighing < 6 kg in whom transcatheter PDA occlusion was attempted in 13 tertiary paediatric cardiology units in the United Kingdom and Ireland were retrospectively analyzed to review the outcome and complications. RESULTS: A total of 408 patients underwent attempted transcatheter PDA closure between January 2004 and December 2014. The mean weight at catheterization was 4.9 ± 1.0 kg and mean age was 5.7 ± 3.0 months. Successful device implantation was achieved in 374 (92%) patients without major complication and of these, complete occlusion was achieved in 356 (95%) patients at last available follow-up. Device embolization occurred in 20 cases (5%). The incidence of device related obstruction to the left pulmonary artery or aorta and access related peripheral vascular injury were low. There were no deaths related to the procedure. CONCLUSIONS: Transcatheter closure of PDA can be accomplished in selected infants weighing <6 kg despite the manufacturer's recommended weight limit of 6 kg for most ductal occluders. The embolization rate is higher than previously reported in larger patients. Retrievability of the occluder and duct morphology needs careful consideration before deciding whether surgical ligation or transcatheter therapy is the better treatment option.


Assuntos
Peso Corporal , Cateterismo Cardíaco/métodos , Permeabilidade do Canal Arterial/terapia , Fatores Etários , Cateterismo Cardíaco/efeitos adversos , Tomada de Decisão Clínica , Permeabilidade do Canal Arterial/diagnóstico por imagem , Humanos , Lactente , Irlanda , Estudos Retrospectivos , Fatores de Risco , Centros de Atenção Terciária , Fatores de Tempo , Resultado do Tratamento , Reino Unido
3.
JACC Cardiovasc Interv ; 10(12): 1233-1243, 2017 06 26.
Artigo em Inglês | MEDLINE | ID: mdl-28641844

RESUMO

OBJECTIVES: The aim of this study was to define the dynamic in vivo morphology of post-infarct ventricular septal defect (PIVSD), which has not been previously described in living patients. BACKGROUND: PIVSD is a devastating complication of acute myocardial infarction. METHODS: The anatomic features of PIVSD, as demonstrated by computed tomography or magnetic resonance imaging, were retrospectively reviewed. RESULTS: Thirty-two PIVSDs were assessed, 16 left coronary artery and 16 right coronary artery PIVSDs. PIVSDs were large (mean maximum dimension 26.5 ± 11.5 mm, mean area 5.2 ± 4.2 cm2) and oval (mean eccentricity index 1.7 ± 0.5), with thin margins (diastolic mean thickness 5 mm from the edge of the PIVSD 6.4 ± 3.0mm), and only 22% of PIVSDs were entirely confined to the septum. The defects could be larger in diastole or systole. The stem of the largest available Amplatzer occluder stem (St. Jude Medical, St. Paul, Minnesota) filled only 50% of defects. Patients with small defects may survive without closure. Without closure, those with large defects die. If accepted for closure, PIVSD size and coronary territory did not predict survival >1 year (overall 60%). CONCLUSIONS: This is the first detailed anatomic description of PIVSD in living patients. Defects may be larger in systole or diastole, meaning that single-phase measurement is unsuitable. Its complex nature means that the most commonly available occluder device is frequently unsuitable. Successful closure leads to prolonged survival and should be attempted where possible. This study may lead to improved patient selection, closure techniques, and device design.


Assuntos
Imagem Cinética por Ressonância Magnética , Tomografia Computadorizada por Raios X , Ruptura do Septo Ventricular/diagnóstico por imagem , Idoso , Idoso de 80 Anos ou mais , Cateterismo Cardíaco/instrumentação , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Desenho de Prótese , Estudos Retrospectivos , Dispositivo para Oclusão Septal , Fatores de Tempo , Resultado do Tratamento , Ruptura do Septo Ventricular/mortalidade , Ruptura do Septo Ventricular/fisiopatologia , Ruptura do Septo Ventricular/terapia
4.
J Thorac Cardiovasc Surg ; 148(6): 2618-26, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-25156466

RESUMO

OBJECTIVE: To identify the outcomes of surgically treated subaortic stenosis in a national population. METHODS: From 2000 to 2013, 1047 patients aged < 40 years underwent 1142 subaortic stenosis procedures. Of the 1047 patients, 484 (46.2%) were considered to have complex stenosis (CS) because at or before the first operation they had mitral valve (MV) disease, aortic valve disease, aortic coarctation or an interrupted aortic arch. RESULTS: The 30-day mortality was 0.7% for simple stenosis (SS), 2.3% for CS (P = .06), and 1.6% overall. Age < 1 year (P < .01), MV procedure (P = .02) and an interrupted aortic arch at the index procedure (P < .01) were risk factors for early death. Konno-type procedure early mortality was 2.4%. The 12-year survival was 97.1%, with a significant difference between SS and CS (hazard ratio [HR], 4.53; P = .02). Having MV disease alone (HR, 4.11; P = .02), MV disease plus aortic coarctation (HR, 6.73; P = .008), and age < 1 year (HR, 6.72; P < .001) were risk factors for late mortality. Freedom from subaortic reintervention overall was 92.3% and 88.5% at 5 and 12 years, respectively, much greater with CS than with SS (HR, 4.91; P < .0001). The independent risk factors for reintervention were younger age at the index procedure (HR, 0.1/y; P = .002), concomitant MV procedure (HR, 2.68; P = .019), ventricular septal defect plus interrupted aortic arch (HR, 3.19; P = .014), and ventricular septal defect plus aortic coarctation (HR, 2.41; P = .023). Undergoing a concomitant aortic valve procedure at the index procedure was protective (HR, 0.29; P = .025). CONCLUSIONS: Patients with SS had excellent outcomes. However, those with CS had worse long-term survival and freedom from reintervention, with morbidity and mortality greatest in young patients with multiple lesions. Additional evaluation in large-scale prospective studies is warranted.


Assuntos
Estenose Aórtica Subvalvar/cirurgia , Procedimentos Cirúrgicos Cardíacos , Adolescente , Adulto , Fatores Etários , Estenose Aórtica Subvalvar/diagnóstico , Estenose Aórtica Subvalvar/mortalidade , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Criança , Pré-Escolar , Bases de Dados Factuais , Mortalidade Hospitalar , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/cirurgia , Modelos de Riscos Proporcionais , Reoperação , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Reino Unido , Adulto Jovem
5.
Orphanet J Rare Dis ; 8: 23, 2013 Feb 12.
Artigo em Inglês | MEDLINE | ID: mdl-23398819

RESUMO

First described in 1983, Barth syndrome (BTHS) is widely regarded as a rare X-linked genetic disease characterised by cardiomyopathy (CM), skeletal myopathy, growth delay, neutropenia and increased urinary excretion of 3-methylglutaconic acid (3-MGCA). Fewer than 200 living males are known worldwide, but evidence is accumulating that the disorder is substantially under-diagnosed. Clinical features include variable combinations of the following wide spectrum: dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), endocardial fibroelastosis (EFE), left ventricular non-compaction (LVNC), ventricular arrhythmia, sudden cardiac death, prolonged QTc interval, delayed motor milestones, proximal myopathy, lethargy and fatigue, neutropenia (absent to severe; persistent, intermittent or perfectly cyclical), compensatory monocytosis, recurrent bacterial infection, hypoglycaemia, lactic acidosis, growth and pubertal delay, feeding problems, failure to thrive, episodic diarrhoea, characteristic facies, and X-linked family history. Historically regarded as a cardiac disease, BTHS is now considered a multi-system disorder which may be first seen by many different specialists or generalists. Phenotypic breadth and variability present a major challenge to the diagnostician: some children with BTHS have never been neutropenic, whereas others lack increased 3-MGCA and a minority has occult or absent CM. Furthermore, BTHS was first described in 2010 as an unrecognised cause of fetal death. Disabling mutations or deletions of the tafazzin (TAZ) gene, located at Xq28, cause the disorder by reducing remodeling of cardiolipin, a principal phospholipid of the inner mitochondrial membrane. A definitive biochemical test, based on detecting abnormal ratios of different cardiolipin species, was first described in 2008. Key areas of differential diagnosis include metabolic and viral cardiomyopathies, mitochondrial diseases, and many causes of neutropenia and recurrent male miscarriage and stillbirth. Cardiolipin testing and TAZ sequencing now provide relatively rapid diagnostic testing, both prospectively and retrospectively, from a range of fresh or stored tissues, blood or neonatal bloodspots. TAZ sequencing also allows female carrier detection and antenatal screening. Management of BTHS includes medical therapy of CM, cardiac transplantation (in 14% of patients), antibiotic prophylaxis and granulocyte colony-stimulating factor (G-CSF) therapy. Multidisciplinary teams/clinics are essential for minimising hospital attendances and allowing many more individuals with BTHS to live into adulthood.


Assuntos
Síndrome de Barth/genética , Síndrome de Barth/complicações , Síndrome de Barth/diagnóstico , Síndrome de Barth/fisiopatologia , Cardiopatias/complicações , Humanos , Masculino
6.
Congenit Heart Dis ; 8(6): E192-5, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23350955

RESUMO

Loeys-Dietz is a multisystem congenital syndrome that comprises craniofacial and cutaneous abnormalities as well as structural cardiac defects. One of its key pathological features is an aggressive widespread vasculopathy that can manifest as aortic or cerebral aneurysms, which is prone to dissection and rupture. We report a case of a large aneurysm of the ductus arteriosus in a patient with Loeys-Dietz syndrome, successfully occluded by interventional catheterization.


Assuntos
Aneurisma/diagnóstico por imagem , Aneurisma/terapia , Aortografia/métodos , Cateterismo Cardíaco , Canal Arterial/diagnóstico por imagem , Cardiopatias/diagnóstico por imagem , Cardiopatias/terapia , Síndrome de Loeys-Dietz/complicações , Tomografia Computadorizada por Raios X , Aneurisma/complicações , Cateterismo Cardíaco/instrumentação , Cardiopatias/complicações , Humanos , Lactente , Síndrome de Loeys-Dietz/diagnóstico , Síndrome de Loeys-Dietz/genética , Masculino , Valor Preditivo dos Testes , Resultado do Tratamento
7.
Interv Cardiol Clin ; 2(1): 173-180, 2013 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-28581981

RESUMO

Percutaneous transcatheter device closure for post-myocardial infarction ventricular septal defect is a feasible alternative to open surgical patch repair. Patient selection, imaging, timing of intervention, technique, and results are discussed.

8.
Am Heart J ; 162(2): 398-404, 2011 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-21835303

RESUMO

BACKGROUND: Increased aortic stiffness and reduced baroreceptor reflex sensitivity have been described independently after coarctation of the aorta (CoA) repair. This study sought to determine the relationship between these variables and blood pressure control in adolescents after early CoA repair. METHODS: Spontaneous baroreceptor reflex sensitivity (sBRS) and aortic pulse wave velocity (PWV) were measured in 29 adolescents after CoA repair and compared with 20 age-matched controls. Patients treated for hypertension or having residual aortic narrowing were excluded. Ambulatory blood pressure (ABP), heart rate variability, and cardiac output were also recorded. After ABP measurement, CoA subjects were classified as normotensive or hypertensive. RESULTS: Nine patients (31%) were hypertensive according to standard definitions, and this subgroup had higher aortic PWV than the normotensive subgroup (P = .004). There was a significant positive correlation between ABP and PWV seen in the whole CoA group (r(2) = 0.5, P < .01). The normotensive subgroup had increased sBRS compared with controls (P = .02). This difference was not seen between the hypertensive subgroup and controls. There was a significant inverse relationship between sBRS and aortic PWV in the whole CoA group (r(2) = 0.25, P = .01). The normotensive subgroup had a significant reduction in stroke index compared with controls (P = .02), which was not seen in the hypertensive subgroup (P = .96). CONCLUSIONS: Adolescents with hypertension after CoA repair have increased aortic PWV and a relative reduction in sBRS compared with normotensive CoA patients. Thus, failure of the baroreceptor reflex to compensate for increasing arterial stiffness may herald the onset of hypertension in these patients.


Assuntos
Aorta Torácica/fisiopatologia , Coartação Aórtica/fisiopatologia , Barorreflexo/fisiologia , Pressão Sanguínea/fisiologia , Resistência Vascular/fisiologia , Adolescente , Progressão da Doença , Feminino , Humanos , Masculino , Índice de Gravidade de Doença
9.
Hypertens Res ; 34(5): 543-7, 2011 May.
Artigo em Inglês | MEDLINE | ID: mdl-21412243

RESUMO

Patients with coarctation of the aorta develop early onset hypertension in spite of early effective repair. This is associated with significant morbidity and is arguably the single most important outcome variable in this patient group. We discuss the potential pathophysiological mechanisms involved in the development of hypertension with clinical reference to monozygotic twins, and review potential strategies for therapy and prevention in this setting.


Assuntos
Aorta/crescimento & desenvolvimento , Coartação Aórtica/complicações , Hipertensão/etiologia , Hipertensão/fisiopatologia , Aorta/fisiopatologia , Aorta/cirurgia , Coartação Aórtica/cirurgia , Feminino , Humanos , Hipertensão/genética , Lactente , Masculino , Gêmeos Monozigóticos
10.
Pediatr Cardiol ; 32(2): 202-5, 2011 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-21188374

RESUMO

Hypertension after repair of coarctation of the aorta (CoA) is the outcome variable most closely associated with adverse long-term events such as stroke and myocardial infarction. This study sought to evaluate the outpatient management of casual blood pressure (BP) measurements in young children after early repair of CoA. A retrospective analysis was performed of clinical findings, echocardiographic data, casual BP recordings, and subsequent BP management of 114 children with CoA repair aged 1-13 years during 338 outpatient visits managed at two congenital cardiac centers. Children with associated significant congenital heart disease or corrective surgery after the age of 6 months were excluded from the study. Blood pressure was documented at 233 clinic visits (69%), and systolic BP (SBP) was above 95th percentile for age and sex in 45 instances (19%). This represented an elevated SBP recording for 31 children (27%), with two or more successive elevated recordings for 11 children (10%). Of 12 subjects receiving antihypertensive medication, three had inadequate BP control. Blood pressure is not documented at approximately 30% of outpatient visits of children with repaired CoA. When elevated BP is documented, in all cases no recorded action was taken. This may have significant implications for cardiovascular outcomes in this cohort of patients.


Assuntos
Coartação Aórtica/cirurgia , Pressão Sanguínea , Hipertensão/diagnóstico , Complicações Pós-Operatórias , Adolescente , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/patologia , Criança , Proteção da Criança , Pré-Escolar , Erros de Diagnóstico , Feminino , Humanos , Hipertensão/diagnóstico por imagem , Hipertensão/etiologia , Lactente , Masculino , Pacientes Ambulatoriais , Pediatria , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Ultrassonografia
11.
Congenit Heart Dis ; 5(5): 444-9, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-21087430

RESUMO

AIMS: To outline the etiology, clinical course, short-term survival to discharge and neurological outcome of infants (<1 yr) with new cardiac diagnoses presenting to a pediatric intensive care (PICU) unit with acute cardiac compromise. METHODS: Retrospective search of a computerized database and medical case notes for all acute cardiac admissions to PICU from June 2001 to 2006. Pre-existing hospital-based patients with new cardiac diagnoses were excluded. RESULTS: Seventy patients were identified, 38 (54%) of whom were male. There were six main subgroups: obstructive left heart lesions (n= 20), transposition of the great arteries (TGA) (n= 9), total anomalous pulmonary venous drainage (TAPVD) (n= 7), dilated cardiomyopathy (n= 11), arrhythmia (n= 12), and others (n= 11). Fifty-nine patients (84%) were external referrals to our center. The median age at presentation was 13.5 days (0-272) with median duration of symptoms of 1 day (0-21). The median base deficit at presentation was -7.6 mEq/L (-43 to +4.2). Fifty-three patients (76%) required respiratory support with a median duration of ventilation of 4 days (1-49). Fifty-six patients (80%) required inotropic support. The median PICU stay was 7 days (1-64) with a median total hospital stay of 16 days (1-71). Six patients (9%) died prior to discharge. Of the survivors 7 (11%) had seizure activity or evolving clinical neurological abnormalities. CONCLUSIONS: Cardiovascular compromise due to previously unrecognized congenital or acquired heart disease is associated with clinically significant morbidity and mortality. Longer term follow-up is required to evaluate the initial effect of poor cardiac output and hypoxia on long-term neurodevelopmental outcome.


Assuntos
Cardiopatias/diagnóstico , Cardiopatias/terapia , Unidades de Terapia Intensiva Pediátrica , Avaliação de Processos e Resultados em Cuidados de Saúde , Cardiotônicos/uso terapêutico , Desenvolvimento Infantil , Diagnóstico Tardio , Inglaterra , Feminino , Cardiopatias/complicações , Cardiopatias/mortalidade , Cardiopatias/fisiopatologia , Mortalidade Hospitalar , Humanos , Lactente , Recém-Nascido , Unidades de Terapia Intensiva Pediátrica/estatística & dados numéricos , Tempo de Internação , Masculino , Sistema Nervoso/crescimento & desenvolvimento , Avaliação de Processos e Resultados em Cuidados de Saúde/estatística & dados numéricos , Alta do Paciente , Respiração Artificial , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Convulsões/etiologia , Convulsões/fisiopatologia , Fatores de Tempo , Resultado do Tratamento
12.
Ann Thorac Surg ; 90(2): 600-4, 2010 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-20667357

RESUMO

BACKGROUND: Increased arterial stiffness is linked to hypertension in adults after surgical repair for coarctation of the aorta. We evaluated the influence of surgical approaches, namely, subclavian flap repair (SFR) and end-to-end anastomosis (EEA), on arterial stiffness, blood pressure, cardiac output, and cardiac baroreceptor function in a cohort of young children after coarctation repair to determine if the surgical approach influenced longer term blood pressure control. METHODS: We measured pulse wave velocity in 21 children with a mean age of 5 years, after early (less than 6 months) coarctation repair (SFR, n = 11; EEA, n = 10), and compared these with 18 matched controls. Blood pressure was recorded on three occasions from the right arm. Cardiac output was recorded using a transthoracic bioimpedence technique. We measured spontaneous baroreceptor reflex sensitivity to evaluate whether increased arterial stiffness was associated with reduced aortic baroreflex sensitivity. RESULTS: Right arm systolic blood pressure (108.3 + or - 3.5 mm Hg SFR versus 97.8 + or - 2.9 mm Hg EEA, p = 0.03) and pulse wave velocity (6.0 + or - 0.2 ms(-1) SFR versus 5.2 + or - 0.2 ms(-1) EEA, p = 0.02) were significantly greater in the SFR compared with EEA group. Blood pressure and pulse wave velocity were also higher in the SFR group compared with controls. These differences were not demonstrated when comparing the EEA group with controls. There was no difference in stroke volume, spontaneous baroreceptor reflex sensitivity, or heart rate or blood pressure variability between the groups. CONCLUSIONS: Young children undergoing SFR have higher blood pressure and stiffer upper limb arteries compared with matched children undergoing EEA. Our data suggest that better longer-term cardiovascular outcome is to be expected with the EEA surgical approach.


Assuntos
Coartação Aórtica/cirurgia , Anastomose Cirúrgica , Coartação Aórtica/fisiopatologia , Artérias/fisiopatologia , Pressão Sanguínea , Procedimentos Cirúrgicos Cardíacos/métodos , Criança , Pré-Escolar , Complacência (Medida de Distensibilidade) , Feminino , Humanos , Masculino , Estudos Retrospectivos , Artéria Subclávia/cirurgia , Retalhos Cirúrgicos
13.
Heart ; 96(15): 1212-6, 2010 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-19744966

RESUMO

BACKGROUND: Stenting for aortic coarctation is known to be effective in the medium term. Aneurysm formation following stent implantation is a recognised complication. However, data regarding aortic wall injury and stent integrity following stent placement are sparse. OBJECTIVES: We report comprehensive clinical, echocardiographic and prospective CT follow-up data following stenting for aortic coarctation from a single centre. Methods Full data analysis of all patients undergoing balloon expandable stent implantation and follow-up procedures in a single tertiary congenital cardiac unit. RESULTS: Between October 2002 and April 2008, we performed 102 coarctation stent procedures on 88 patients. Median age was 20.6 years (range 8.5-65) and median weight 65 kg (range 34-101). 94 stents (26 covered) were implanted. 12 procedures were re-dilatations. Stenting resulted in a reduction of the gradient across the site of coarctation, from a median of 20 mm Hg to 4 mm Hg. There were no procedure-related deaths. Four patients had immediate complications (one requiring emergency surgery). During median follow-up of 34.5 months (range 4.2-72.8), two patients had late complications requiring additional stent procedures. Follow-up CT data are available in 84 patients with MRI in one patient (96.5%). Only one patient developed a procedure-related aortic aneurysm. All stent fractures (n=7) occurred with a single stent design. CONCLUSIONS: Stenting for aortic coarctation and re-coarctation is effective with low immediate complication rates. CT is useful in the longer term for assessment of stent integrity and post-procedural aneurysm formation. Overall incidence of post-procedural aneurysm is rare and stent fractures were not seen with newer generation stents.


Assuntos
Aneurisma Aórtico/etiologia , Coartação Aórtica/terapia , Cateterismo/métodos , Stents , Doença Aguda , Adolescente , Adulto , Idoso , Aorta/lesões , Aneurisma Aórtico/diagnóstico por imagem , Cateterismo/efeitos adversos , Criança , Falha de Equipamento , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Recidiva , Stents/efeitos adversos , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Adulto Jovem
15.
Pacing Clin Electrophysiol ; 32(9): 1123-9, 2009 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-19719487

RESUMO

BACKGROUND: Endocardial pacemaker lead placement can be challenging after Mustard and Senning operations for transposition of the great arteries (D-TGA), if there is atresia of the systemic venous pathways and because the coronary sinus cannot be used for cardiac resynchronization therapy. Radiofrequency (RF)-assisted perforation techniques have been used in congenital heart disease but have not been reported for use in pacemaker implantation. METHODS AND RESULTS: We describe RF perforation of an atretic superior systemic venous pathway and systemic venous baffles in three patients after Senning and Mustard operations to implant endocardial pacing systems to achieve conventional or biventricular pacing. CONCLUSIONS: RF-energy-assisted perforation is feasible and effective tool to facilitate endocardial lead placement during dual-chamber and biventricular pacemaker implantation in patients with Mustard or Senning operations for D-TGA.


Assuntos
Estimulação Cardíaca Artificial/métodos , Ablação por Cateter/métodos , Eletrodos Implantados , Implantação de Prótese/métodos , Punções/métodos , Transposição dos Grandes Vasos/cirurgia , Disfunção Ventricular Esquerda/etiologia , Adolescente , Adulto , Humanos , Masculino , Transposição dos Grandes Vasos/complicações , Resultado do Tratamento , Disfunção Ventricular Esquerda/prevenção & controle
16.
Pacing Clin Electrophysiol ; 32(11): 1428-33, 2009 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-19769583

RESUMO

BACKGROUND: Long-term pacing is frequently necessary in patients with congenital heart disease (CHD). Preservation of ventricular function and avoidance of venous occlusion is important in these patients. Site-selective pacing with a smaller diameter lead is achievable with the model 3830 lead (SelectSecure, Medtronic Inc., Minneapolis, MN, USA), which was specifically designed to target these complications. We describe our initial experience with the Model 3830 lead in patients with CHD. METHODS: Retrospective analysis of all patients undergoing site-selective implantation of a Model 3830 lead(s) from two congenital heart centers (Bristol, UK, and Dublin, Ireland) from October 2004 until February 2008. RESULTS: We implanted 139 SelectSecure leads (atrial n=70; ventricular n=69) in 90 patients (57 male) with CHD. Median age at implantation: 13.4 years (1.1-59.2 years), median weight: 43 kg. Sixty-nine patients (76%) were children (<18 years). Indications for lead implantation included atrioventricular block (n=55), sinus node disease (n=18), implantable cardiac defibrillator (n=12), antitachycardia pacing (n=4), and cardiac resynchronization (n=1). Twenty-two patients underwent pre-existing lead extraction during the same procedure. All the attempted procedures resulted in successful pacing. One patient had a significantly raised threshold at implantation. There was no procedural mortality. There were two procedural complications. Three patients required lead repositioning for increasing thresholds early postprocedure (<6 weeks). Four leads (2.9%) had displaced on median follow-up of 21.8 months (0.5-42 months). CONCLUSIONS: The Model 3830 lead is safe and effective in patients with CHD. This is a technically challenging patient group yet procedural complication and lead displacement rates are acceptable.


Assuntos
Eletrodos Implantados , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/terapia , Insuficiência Cardíaca/prevenção & controle , Insuficiência Cardíaca/terapia , Marca-Passo Artificial , Adolescente , Adulto , Criança , Pré-Escolar , Desenho de Equipamento , Análise de Falha de Equipamento , Feminino , Cardiopatias Congênitas/complicações , Insuficiência Cardíaca/etiologia , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Estudos Retrospectivos , Adulto Jovem
18.
Orphanet J Rare Dis ; 4: 17, 2009 Jul 10.
Artigo em Inglês | MEDLINE | ID: mdl-19591690

RESUMO

Patent arterial duct (PAD) is a congenital heart abnormality defined as persistent patency in term infants older than three months. Isolated PAD is found in around 1 in 2000 full term infants. A higher prevalence is found in preterm infants, especially those with low birth weight. The female to male ratio is 2:1. Most patients are asymptomatic when the duct is small. With a moderate-to-large duct, a characteristic continuous heart murmur (loudest in the left upper chest or infraclavicular area) is typical. The precordium may be hyperactive and peripheral pulses are bounding with a wide pulse pressure. Tachycardia, exertional dyspnoea, laboured breathing, fatigue or poor growth are common. Large shunts may lead to failure to thrive, recurrent infection of the upper respiratory tract and congestive heart failure. In the majority of cases of PAD there is no identifiable cause. Persistence of the duct is associated with chromosomal aberrations, asphyxia at birth, birth at high altitude and congenital rubella. Occasional cases are associated with specific genetic defects (trisomy 21 and 18, and the Rubinstein-Taybi and CHARGE syndromes). Familial occurrence of PAD is uncommon and the usual mechanism of inheritance is considered to be polygenic with a recurrence risk of 3%. Rare families with isolated PAD have been described in which the mode of inheritance appears to be dominant or recessive. Familial incidence of PAD has also been linked to Char syndrome, familial thoracic aortic aneurysm/dissection associated with patent arterial duct, and familial patent arterial duct and bicuspid aortic valve associated with hand abnormalities. Diagnosis is based on clinical examination and confirmed with transthoracic echocardiography. Assessment of ductal blood flow can be made using colour flow mapping and pulsed wave Doppler. Antenatal diagnosis is not possible, as PAD is a normal structure during antenatal life. Conditions with signs and symptoms of pulmonary overcirculation secondary to a left-to-right shunt must be excluded. Coronary, systemic and pulmonary arteriovenous fistula, peripheral pulmonary stenosis and ventricular septal defect with aortic regurgitation and collateral vessels must be differentiated from PAD on echocardiogram. In preterm infants with symptomatic heart failure secondary to PAD, treatment may be achieved by surgical ligation or with medical therapy blocking prostaglandin synthesis (indomethacin or ibuprofen). Transcatheter closure of the duct is usually indicated in older children. PAD in preterm and low birth weight infants is associated with significant co-morbidity and mortality due to haemodynamic instability. Asymptomatic patients with a small duct have a normal vital prognosis but have a lifetime risk of endocarditis. Patients with moderate-to-large ducts with significant haemodynamic alterations may develop irreversible changes to pulmonary vascularity and pulmonary hypertension.


Assuntos
Permeabilidade do Canal Arterial , Doenças do Prematuro , Procedimentos Cirúrgicos Cardíacos , Permeabilidade do Canal Arterial/diagnóstico , Permeabilidade do Canal Arterial/epidemiologia , Permeabilidade do Canal Arterial/patologia , Permeabilidade do Canal Arterial/cirurgia , Feminino , Humanos , Recém-Nascido de Baixo Peso , Recém-Nascido , Recém-Nascido Prematuro , Doenças do Prematuro/diagnóstico , Doenças do Prematuro/epidemiologia , Doenças do Prematuro/patologia , Doenças do Prematuro/cirurgia , Masculino , Prevalência
19.
Catheter Cardiovasc Interv ; 73(4): 576-80, 2009 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-19235246

RESUMO

Despite the wide variety of occlusion devices available for closing the patent ductus arteriosus; some defects with unusual morphology still remain a challenge. The drive for increasingly user friendly and lower caliber delivery systems is also ongoing. The new amplatzer ductal occluder II may prove to be a useful additional tool in these circumstances. We describe our initial experiences with this new device in three patients with long tubular (Type C) ducts.


Assuntos
Cateterismo Cardíaco/instrumentação , Permeabilidade do Canal Arterial/terapia , Permeabilidade do Canal Arterial/diagnóstico por imagem , Desenho de Equipamento , Humanos , Lactente , Radiografia Intervencionista , Resultado do Tratamento
20.
Catheter Cardiovasc Interv ; 72(1): 65-71, 2008 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-18412235

RESUMO

OBJECTIVES: We describe our complete experience with covered stent implantation for aortic coarctation including short- to medium-term outcomes. BACKGROUND: Coarctation of the aorta is a heterogeneous disease process with multiple associated complications both with and without treatment. Covered stents have evolved to provide greater support to the aortic wall and a varied approach with choice of stent tailored to the anatomy of the patient is required. METHODS: We retrospectively analyzed consecutive patient data from two congenital heart centers from March 2003 to October 2007. RESULTS: We implanted 38 covered stents in 37 patients (20 female) of which three were self-expanding stents. Median age was 29.6 years (9-65) with median weight of 71.5 kg (35-95). The indications for stent placement were native coarctation (n = 13); recurrent coarctation following surgical treatment (n = 11); aneurysm associated with previous coarctation surgery (n = 7); aorto-bronchial fistula leading to acute hemoptysis (n = 2); stent fracture (n = 1); associated arterial duct (n = 2). One patient had a combination of acquired coarctation and false aneurysm. The median screening time was 10 min (3-22). The median systolic gradient of 26 mm Hg (10-60) was reduced to 4 mm Hg (0-20) postprocedure (P < 0.001). There were no deaths on median follow up of 11.5 months (1-56). One patient developed aortic rupture during the procedure and required emergency surgery. CONCLUSION: Covered stent implantation for treatment of aortic coarctation is safe and highly effective in selected patients. Self-expanding stent grafts may be preferable to balloon expandable stents when there is aneurysm formation in the setting of aortopathy.


Assuntos
Angioplastia Coronária com Balão , Aneurisma Aórtico/complicações , Coartação Aórtica/complicações , Coartação Aórtica/terapia , Materiais Revestidos Biocompatíveis , Stents , Adolescente , Adulto , Idoso , Aneurisma Aórtico/terapia , Criança , Estudos de Coortes , Desenho de Equipamento , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Poliésteres , Politetrafluoretileno , Estudos Retrospectivos , Resultado do Tratamento
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