RESUMO
AIMS: We compared in vivo blood flow and pulsatility after different types of Fontan operation using magnetic resonance imaging. MATERIAL AND METHODS: A total of 37 consecutive patients (mean age 19+/-7.9 years, 7.3+/-3.2 years after Fontan operation), 7 with atriopulmonary anastomosis (APC), 18 with intra-atrial lateral tunnel (LTFO) and 12 with extracardiac Fontan (ECFO) were studied using magnetic resonance phase-contrast velocity mapping. Blood flow (volume flow) in the superior vena cava (SVC), inferior vena cava (IVC) and both pulmonary arteries were measured and a pulsatility index was calculated for each vessel. RESULTS: For all modifications, the blood flow distribution between the SVC and IVC was normal (1:2). Patients with APC had a normal pulsatility, a dilated right atrium, partial backward flow in the IVC and physiological blood flow distribution between the pulmonary arteries. LTFO and ECFO patients had no retrograde flow in the IVC, equal blood flow distribution between the pulmonary arteries and very low or absent pulsatility. CONCLUSIONS: MRI allows hemodynamic quantification and characterization of various types of Fontan modifications and may be a valuable tool to predict Fontan failure. Despite showing normal pulsatility, patients with APC have right atrial dilatation and partial backward flow in the IVC, demonstrating suboptimal Fontan circulation. LTFO and ECFO both produce unidirectional antegrade flow in the IVC but pulsatility is very low or absent, which may promote poor pulmonary artery growth and increase of pulmonary vascular resistance contributing to late Fontan failure.
Assuntos
Técnica de Fontan/métodos , Cardiopatias Congênitas/fisiopatologia , Imageamento por Ressonância Magnética , Artéria Pulmonar , Circulação Pulmonar , Veia Cava Inferior , Veia Cava Superior , Adolescente , Adulto , Velocidade do Fluxo Sanguíneo , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/cirurgia , Humanos , Lactente , Imageamento por Ressonância Magnética/métodos , Masculino , Período Pós-Operatório , Artéria Pulmonar/cirurgia , Fluxo Sanguíneo Regional , Reprodutibilidade dos Testes , Projetos de Pesquisa , Processamento de Sinais Assistido por Computador , Veia Cava Inferior/cirurgia , Veia Cava Superior/cirurgiaRESUMO
BACKGROUND: New oral substances such as beraprost, bosentan and sildenafil have proven effective in different forms of pulmonary arterial hypertension (PAH), both alone and in combination with standard treatment such as intravenous and inhaled prostacyclins. However, there are few reports so far on the effect of a combination of exclusively oral substances. In this paper, we present our initial findings of treatment using a combination of these oral substances in a heterogeneous group of patients with different forms of PAH. MATERIALS AND METHODS: Eleven patients with a median age of 12.9 years (5.5-54.7 years) with both idiopathic PAH and forms associated with congenital cardiac defects (PAH-CHD) with a mean pulmonary arterial pressure > 25 mmHg were enrolled in an observational, open-label, prospective, single-centre study. Either combination treatment with bosentan and sildenafil was started initially, or an existing bosentan treatment was complemented with sildenafil given as an add-on therapy. Mean doses given were 2.3 +/- 0.6 mg kg(-1) for bosentan and 2.1 +/- 0.9 mg kg(-1) for sildenafil. Clinical status, exercise capacity, and haemodynamics were assessed at baseline and at the end of the observation period after a mean follow-up time of 1.1 years (0.5-2.5 years). RESULTS: No major side effects regarding liver function and blood pressure regulation were noted. One patient died of sudden death elsewhere. Most patients were in New York Heart Association (NYHA) functional class III. Clinical improvement was about one NYHA class (mean 2.8 +/- 0.4-1.6 +/- 0.8, P = 0.001), which was associated with an increase of transcutaneous oxygen saturation (89.9 +/- 9.9-92.3 +/- 7.1%; P = 0.037), maximum oxygen uptake (18.1 +/- 6.8-22.8 +/- 10.4 mL kg(-1) x min; P = 0.043), and 6-minute walking distance (351 +/- 58-451 +/- 119 m; P = 0.039). Mean pulmonary arterial pressure measured invasively decreased (62 +/- 12-46 +/- 18 mmHg; P = 0.041). CONCLUSIONS: In our patient group, a combination of oral bosentan and sildenafil proved to be safe and effective. Clearly, randomized, double-blind, placebo-controlled studies are warranted to define the role and type of combination therapies in PAH.
Assuntos
Anti-Hipertensivos/administração & dosagem , Hipertensão Pulmonar/tratamento farmacológico , Inibidores de Fosfodiesterase/administração & dosagem , Piperazinas/administração & dosagem , Sulfonamidas/administração & dosagem , Administração Oral , Adolescente , Adulto , Bosentana , Criança , Pré-Escolar , Quimioterapia Combinada , Antagonistas dos Receptores de Endotelina , Teste de Esforço/métodos , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/fisiopatologia , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/fisiopatologia , Pessoa de Meia-Idade , Estudos Prospectivos , Purinas , Citrato de Sildenafila , Sulfonas , Resultado do TratamentoRESUMO
We compared the left ventricular Tei index measured by tissue Doppler imaging (TDI) to that obtained by pulsed Doppler (PW) in patients with congenital heart disease. In 40 consecutive patients with a variety of congenital and acquired heart diseases, the left ventricular (LV) PW Doppler-derived Tei index was assessed on-line as previously described. TDI-derived anatomic curved M-mode and the unprocessed velocity trace from the basal septum were used to time the opening and closure of the mitral and aortic valves in one cardiac cycle, respectively. The TDI Tei index was calculated off-line according to the equation (isovolumetric relaxation time + isovolumetric contraction time)/ ejection time. The Tei index calculated from TDI correlated significantly with that measured by pulsed Doppler (r = 0.92, p = 0.001). The mean difference (range) between pulsed Doppler-derived Tei index and TDI-derived Tei index was 0.005 (-0.07-0.06), which was within the limits of agreements. Interobserver variability for the TDI-derived Tei index was 5 +/- 3%. The TDI Tei index can be used to assess the global LV function in patients with congenital heart disease. In contrast to the PW Doppler-derived Tei index, the TDI-derived Tei index obtained from the same cardiac cycle may help to differentiate systolic from diastolic dysfunction by providing specific information on the isovolumetric intervals.
Assuntos
Ecocardiografia Doppler de Pulso , Cardiopatias Congênitas/diagnóstico por imagem , Ventrículos do Coração/diagnóstico por imagem , Função Ventricular Esquerda/fisiologia , Adolescente , Adulto , Criança , Pré-Escolar , Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/fisiopatologia , Humanos , Pessoa de Meia-Idade , Contração Miocárdica , Variações Dependentes do Observador , Índice de Gravidade de DoençaRESUMO
This study aimed to quantify paradoxical interventricular septal motion (PSM) among 20 patients following tetralogy of Fallot (TOF) repair without severe pulmonary regurgitation and 20 age-matched normal subjects. PSM was quantified using the echocardiography-derived paradox index. Tissue Doppler-derived strain rate was used to assess the longitudinal and radial systolic function of the interventricular septum (IVS). The tissue Doppler-derived Tei index was used to assess the global left ventricular function. Compared to the control group, the paradox index in patients after repair of TOF was significantly higher (p = 0.001), whereas the regional IVS longitudinal (p = 0.02) and radial (p = 0.001) systolic strain rate peaks were significantly reduced. The paradox index in the patient group correlated inversely with the IVS radial peak systolic strain rate (r = -0.64, p = 0.004) and positively with QRS duration (r = 0.50, p = 0.02). The left ventricular (LV) Tei-index correlated significantly with the paradox index (r = 0.71, p = 0.001) and with the septal radial systolic strain rate peak (r = 0.59, p = 0.004). We conclude that electrical delay and reduced regional septal systolic function were the main causes for paradoxical septal motion among patients following TOF repair without significant pulmonary regurgitation. The reduced LV systolic function among this group of patients is mainly secondary to diminished septal systolic function and the paradoxical septal motion.
Assuntos
Septos Cardíacos/fisiopatologia , Contração Miocárdica/fisiologia , Tetralogia de Fallot/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Ecocardiografia Doppler de Pulso , Seguimentos , Septos Cardíacos/diagnóstico por imagem , Humanos , Lactente , Período Pós-Operatório , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/fisiopatologia , Fatores de TempoRESUMO
BACKGROUND: In clinical settings an easy and reliable method for following up right ventricular (RV) function in patients after repair of tetralogy of Fallot (TOF) is needed. It is, however, unclear whether the novel modified short axis view from echocardiography is superior to the apical four chamber view in this aspect. MATERIALS AND METHODS: Thirty postoperative TOF patients with median age 17 years (range 6-45 years) and follow up period of 10 years (range 0.5-40 years) were examined echocardiographically using the apical four chamber view and the novel modified short axis view. RV areas in end-systole (Amin) and end-diastole (Amax) were measured and an area fraction [(Amax - Amin)/Amax * 100%] was calculated from the respective view. RV ejection fraction was assessed through magnetic resonance imaging (MRI). The RV area fractions from echocardiography were compared to the RV ejection fraction. RESULTS: The right ventricular area fraction derived from the modified short axis view was significantly lower than that from the apical four chamber view (34.3+/-9.1% vs. 42.5+/-10.2%, p=0.007). Both the RV area fractions obtained from the modified short axis view (r=0.674, p<0.001) and from the apical four chamber view (r=0.512, p=0.025) correlated significantly with the MRI derived RV ejection fraction. CONCLUSION: The novel modified short axis view from echocardiography may be superior to the apical four chamber view for routine follow up of patients after TOF repair, in whom the right ventricular outflow tract plays an important role in the right ventricular systolic function.
Assuntos
Tetralogia de Fallot/cirurgia , Disfunção Ventricular Direita/diagnóstico por imagem , Adolescente , Adulto , Criança , Ecocardiografia/métodos , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Disfunção Ventricular Direita/diagnósticoRESUMO
INTRODUCTION: Bosentan, a dual endothelin-receptor antagonist, has been shown to be an effective treatment option in patients with the idiopathic form of pulmonary arterial hypertension (PAH). We used bosentan as compassionate treatment in infants and young children with congenital heart disease (CHD) who had a) PAH preoperatively representing a contraindication to corrective surgery or b) persisting PAH after corrective surgery causing right heart failure and reduced exercise tolerance. METHODS: Seven children with PAH due to CHD (median age 3.8 years; range 1.5 to 6.4 years) received 3 mg/kg/d bosentan (Tracleer) orally. Clinical, echocardiographic and hemodynamic parameters were measured and laboratory tests performed before treatment and during steady state while on treatment. Routine liver function parameters were monitored monthly. RESULTS: Mean bosentan treatment time was 8.6+/-5 months. During bosentan therapy there were no significant adverse events. The clinical status remained stable or improved in all patients: NYHA class decreased from 2.6+/-0.6 to 1.7+/-0.6 (p<0.05). This was associated with a mean reduction of the right ventricular systolic pressure (RVSP) from 96+/-11 mmHg to 71+/-26 mmHg (p<0.05). CONCLUSIONS: Treatment with bosentan in infants and young children with PAH due to congenital heart disease was tolerated without significant side effects and resulted in stabilization of clinical status. A significant reduction in right ventricular systolic pressure (RVSP) could be demonstrated. These results suggest that the dose regimen used is appropriate and safe for the treatment of infants and children with PAH, resulting in a reduction of pathologically increased pulmonary vascular resistance.
Assuntos
Cardiopatias Congênitas/complicações , Hipertensão Pulmonar/tratamento farmacológico , Hipertensão Pulmonar/etiologia , Cuidados Paliativos/métodos , Sulfonamidas/uso terapêutico , Anti-Hipertensivos/uso terapêutico , Bosentana , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Projetos Piloto , Artéria Pulmonar/efeitos dos fármacos , Resultado do TratamentoRESUMO
OBJECTIVE: To compare the effects of surgical and device closure of atrial septal defects on atrial and ventricular performance assessed by the novel tissue Doppler derived strain rate. BACKGROUND: Despite the increasing number of transcatheter closures, there is no information comparing the effect of the transcatheter closure technique on atrial performance with that of conventional surgery. Tissue Doppler derived strain rate can effectively quantify local myocardial function independent of the overall heart motion. DESIGN AND PATIENTS: Twenty-four patients [aged 21.5 (6-70) years] with isolated atrial septal defect of the secondum type before and 1 week after surgical (n = 12) or Amplatzer Septal Occluder closure (n = 12) and 30 healthy controls [aged 26.0 (2-58) years] were studied. Atrial and ventricular strain rate curves were assessed in the middle of their corresponding lateral walls in an apical four-chamber view. The systolic, early diastolic, and late diastolic strain rates peaks were measured. RESULTS: Compared to preclosure condition, the right atrial late diastolic (P < 0.01), right ventricular systolic (P < 0.01), right ventricular early diastolic (P < 0.01), and left atrial late diastolic peak (P < 0.01) strain rates were reduced after surgery but not after Amplatzer Septal Occluder closure. The LV parameters did not significantly differ before and after atrial septal defect closure by either technique. CONCLUSIONS: In contrast to surgery, transcatheter closure of atrial septal defect preserves atrial and right ventricular function. Tissue Doppler derived strain rate can be applied to provide quantitative analysis of regional atrial and ventricular performance.
Assuntos
Ecocardiografia Doppler , Comunicação Interatrial/fisiopatologia , Comunicação Interatrial/terapia , Função Ventricular Esquerda , Função Ventricular Direita , Adolescente , Adulto , Idoso , Cateterismo Cardíaco , Criança , Comunicação Interatrial/diagnóstico por imagem , Humanos , Pessoa de Meia-IdadeRESUMO
INTRODUCTION: The long-term follow-up of patients with D-transposition of the great arteries after atrial switch operation shows specific problems such as tricuspid valve insufficiency, rhythm disturbances and failure of the morphologic right ventricle in systemic position. Assessment of the myocardial contractility of the subaortic right ventricle by conventional echocardiography is limited. The usage of tissue Doppler echocardiography with strain combined with strain rate imaging provides a new approach for quantitative analysis of longitudinal myocardial function. The aim of this study was to assess patterns of wall motion and regional contractile function of the systemic right ventricle in patients after atrial switch operation for D-transposition of the great arteries and to compare them to those of normal subjects. PATIENTS AND METHODS: Twenty-four patients with Dtransposition of the great arteries after atrial switch operation with a mean age of 21.3 (range, 13 to 31) years and a postoperative period of 16.9 years were examined and compared to 22 control individuals with a mean age of 21.5 (range, 3 to 43) years. Tissue Doppler studies were obtained from apical 4- chamber view to determine regional systolic (Syst(T)) and diastolic (E(T), A(T)) velocities as well as E(T)/A(T) ratio at the basal free wall. The presystolic isovolumic contraction peak was assessed and the ratio of the presystolic peak velocity to the isovolumic acceleration time as the IVA index was calculated. Strain and peak systolic and diastolic strain rates were assessed on basal, middle and apical segments of the right ventricular free wall. Data obtained from the morphologic right systemic ventricle in patients were compared to those derived from the left and the right ventricle in controls. RESULTS: The right ventricular free wall systolic velocities were significantly reduced in patients compared to velocities obtained from the normal right and left ventricle. On the other hand, the IVA index was only reduced in patients compared to the IVA index in the normal subpulmonary right ventricle. Compared to data obtained from the normal systemic left ventricle, the IVA index in patients was not significantly different. In contrast, strain and strain rate parameters in all analyzed segments mostly showed a highly significant reduction compared to normal right and left ventricular data. CONCLUSION: Tissue Doppler echocardiography is a promising tool for the evaluation of regional myocardial contractile function of the morphologic right systemic ventricle in patients following atrial switch operation for D-transposition of the great arteries. Presystolic, systolic and diastolic regional ventricular function was reduced in the systemic right ventricle. However, further comparative studies using other quantitative parameters of global and regional myocardial function derived from cardiac catheterization or MRI should be performed in order to evaluate the reliability of tissue Doppler echocardiography for the assessment of global right ventricular function in these patients.
Assuntos
Ecocardiografia Doppler , Átrios do Coração/cirurgia , Contração Miocárdica/fisiologia , Complicações Pós-Operatórias/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Disfunção Ventricular Direita/diagnóstico por imagem , Adolescente , Adulto , Procedimentos Cirúrgicos Cardíacos , Criança , Pré-Escolar , Diástole/fisiologia , Endocárdio/diagnóstico por imagem , Endocárdio/fisiopatologia , Feminino , Seguimentos , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/fisiopatologia , Hemodinâmica/fisiologia , Humanos , Masculino , Complicações Pós-Operatórias/fisiopatologia , Valores de Referência , Sístole/fisiologia , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/fisiopatologia , Disfunção Ventricular Direita/fisiopatologia , Função Ventricular Direita/fisiologiaRESUMO
OBJECTIVE: To evaluate the feasibility and usefulness of the Cheatham platinum (CP) stent in a broad spectrum of lesions. METHODS: Retrospective analysis of 60 implanted CP stents (11-80 mm lengths, 12 covered) between September 2001 and March 2004. PATIENTS: 53 patients aged 2.5-68 years (median 17 years). Body weight ranged from 12-95 kg (median 52 kg). Thirty six patients had aortic (re)coarctation; seven of them had functionally interrupted aortic arches. Thirteen patients had pulmonary artery stenosis and four had stenosis of caval veins or conduits in a total cavopulmonary connection (TCPC). RESULTS: Arterial pressure gradients dropped from 33 mm Hg (range 20-80 mm Hg) to 5 mm Hg (range 0-10 mm Hg) and pressure gradients in TCPC or caval veins dropped from 4 mm Hg (range 4-20 mm Hg) to 0 mm Hg (range 0-3 mm Hg). All stents were placed in the target lesion without complications. Three stent fractures without clinical instability were noted. CONCLUSIONS: The CP stent is suitable for the treatment of vessel stenosis in congenital heart diseases from childhood to adulthood. Whether these good results will be stable in the long term needs to be investigated.
Assuntos
Aorta Torácica/patologia , Coartação Aórtica/cirurgia , Doenças da Aorta/cirurgia , Artéria Pulmonar/patologia , Stents , Veias Cavas/patologia , Adolescente , Adulto , Idoso , Aorta Torácica/cirurgia , Coartação Aórtica/fisiopatologia , Doenças da Aorta/fisiopatologia , Pressão Sanguínea/fisiologia , Criança , Pré-Escolar , Constrição Patológica , Estudos de Viabilidade , Humanos , Pessoa de Meia-Idade , Desenho de Prótese , Falha de Prótese , Implantação de Prótese/métodos , Artéria Pulmonar/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Veias Cavas/cirurgiaRESUMO
Transcatheter closure of atrial septal defects (ASDs) is a safe and effective treatment. Over the past years, an increasing number of elderly patients (age > 60 years) have been admitted for transcatheter closure to prevent ongoing congestive heart failure from volume overload. However, recent data point to the risk of serious acute left ventricular dysfunction leading to pulmonary edema immediately after surgical or transcatheter ASD closure in some patients. In this study, we used a technique described before to recognize in advance patients at risk of left heart failure after ASD closure. Those patients at risk were then treated with preventive conditioning medication for 48-72 hr before definitive transcatheter ASD closure was performed. Fifty-nine patients aged over 60 years (range, 60-81.8 years; median, 68 years) were admitted to our institution for transcatheter closure of an atrial septal defect. All patients received evaluation of atrial pressures before and during temporary balloon occlusion of the ASD. Patients with left ventricular restriction due to increased mean atrial pressures (> 10 mm Hg) during ASD occlusion received anticongestive conditioning medication with i.v. dopamine, milrinone, and furosemide for 48-72 hr before definitive ASD closure with an Amplatzer septal occluder was performed. In 44 patients without any signs of left ventricular restriction, ASD closure was performed within the first session. Fifteen (25%) out of 59 patients showed left ventricular restriction. In the majority of patients with LV restriction, the mean left atrial pressures with occluded ASD were significantly decreased after 48-72 hr of conditioning medication. Definitive ASD closure was then performed in a second session. Only two patients received a fenestrated 32 mm Amplatzer occluder due to persistent increased atrial pressures > 10 mm Hg even after conditioning medication. There were no significant differences in shunt, device size, or defect size between the two groups. Balloon occlusion of atrial septal defects identifies patients with left ventricular restrictive physiology before ASD closure. Intravenous anticongestive conditioning medication seems to be highly effective in preventing congestive heart failure after interventional closure of an ASD in the elderly patient with a restrictive left ventricle.
Assuntos
Cateterismo Cardíaco/métodos , Cardiotônicos/uso terapêutico , Cateterismo/métodos , Diuréticos/uso terapêutico , Insuficiência Cardíaca/prevenção & controle , Comunicação Interatrial/terapia , Disfunção Ventricular Esquerda/tratamento farmacológico , Idoso , Idoso de 80 Anos ou mais , Cateterismo Cardíaco/efeitos adversos , Cardiotônicos/administração & dosagem , Cateterismo/efeitos adversos , Diuréticos/administração & dosagem , Dopamina/administração & dosagem , Dopamina/uso terapêutico , Quimioterapia Combinada , Furosemida/administração & dosagem , Furosemida/uso terapêutico , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/fisiopatologia , Comunicação Interatrial/fisiopatologia , Ventrículos do Coração/efeitos dos fármacos , Ventrículos do Coração/fisiopatologia , Humanos , Injeções Intravenosas , Pessoa de Meia-Idade , Milrinona/administração & dosagem , Milrinona/uso terapêutico , Estudos Retrospectivos , Resultado do Tratamento , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Esquerda/efeitos dos fármacos , Função Ventricular Esquerda/fisiologiaRESUMO
We report on a patient suffering from recurrent cerebrovascular events despite previous transcatheter closure of persistent foramen ovale (PFO) with a Helex occluder. There was evidence of persistent left-to-right atrial shunt shown by transesophageal contrast echocardiography and the patient was admitted to our institution for interventional closure of the supposed residual defect. However, the PFO was completely closed by the device and left pulmonary artery injections showed a pulmonary arteriovenous fistula in the left lower lobe. This rare malformation may well explain the recurrent paradoxical embolism. Transcatheter fistula closure with coils was performed successfully. This case underlines that the existence of an isolated pulmonary arteriovenous fistula as a right-to-left shunt in patients with cryptogenic stroke should not be overlooked, even if a PFO is present and pulmonary arteriovenous fistula is not suggested by the initial physical findings or chest X-ray.
Assuntos
Fístula Arteriovenosa/diagnóstico , Cateterismo/efeitos adversos , Embolia Paradoxal/etiologia , Comunicação Interatrial/terapia , Artéria Pulmonar/anormalidades , Embolia Pulmonar/etiologia , Veias Pulmonares/anormalidades , Adulto , Fístula Arteriovenosa/complicações , Fístula Arteriovenosa/terapia , Cateterismo Cardíaco/efeitos adversos , Diagnóstico Diferencial , Ecocardiografia Transesofagiana , Embolia Paradoxal/diagnóstico por imagem , Embolia Paradoxal/terapia , Embolização Terapêutica , Feminino , Comunicação Interatrial/complicações , Comunicação Interatrial/diagnóstico por imagem , Humanos , Pulmão/irrigação sanguínea , Artéria Pulmonar/diagnóstico por imagem , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/terapia , Veias Pulmonares/diagnóstico por imagem , Recidiva , Falha de TratamentoRESUMO
Despite increasing clinical impact of cardiac resynchronization therapy (CRT) with av-synchronous biventricular pacing in adults with dilated cardiomyopathy (DCMP), an ejection fraction (EF) of less than 35% and left bundle branch block (LBBB), there is still only little experience in children. We report on a 9-year-old boy with histologically proven DCMP and LBBB who had fulfilled the criteria for heart transplantation (HTX) after cardiac decompensation including catecholamine therapy. A transvenous CRT pacing system was implanted without technical difficulties. The healing process was uneventful. With optimized AV-interval invasive evaluation during implantation indicated a 16% pulse pressure increase and a 63% augmentation of LV dp/dt by pacing the LV 20 ms prior to the RV. Tissue Doppler imaging demonstrated complete LV resynchronization. Physical capacity increased and HTX could be delayed.
Assuntos
Bloqueio de Ramo/terapia , Baixo Débito Cardíaco/terapia , Cardiomiopatia Dilatada/terapia , Hipertrofia Ventricular Esquerda/terapia , Marca-Passo Artificial , Cateterismo Cardíaco , Criança , Ecocardiografia Doppler , Eletrocardiografia , Estudos de Viabilidade , Seguimentos , Transplante de Coração , Humanos , Masculino , Resultado do TratamentoAssuntos
Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Adolescente , Adulto , Antropometria , Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/etiologia , Prótese Vascular , Criança , Pré-Escolar , Seguimentos , Técnica de Fontan/efeitos adversos , Desenvolvimento Humano , Humanos , Incidência , Lactente , Análise de Sobrevida , Resultado do TratamentoRESUMO
OBJECTIVE: To study the impact of the maturational process of diastolic left ventricular function on trans-mitral Doppler flow parameters. METHODS AND PARTICIPANTS: In a survey we examined pulsed-wave Doppler signals and diastolic time intervals from 238 healthy neonates and infants. Using multiple linear regression analysis, we evaluated the impact of physiological determinants on parameter expression. RESULTS: Early-filling and atrial-filling peak velocities, early-filling acceleration and deceleration rates, and the atrial-filling time velocity integral reached a climax within 2 months after birth, while early-filling time velocity integral followed increasing throughout the study period. The isovolumic relaxation time was found to be significantly longer for neonates than for infants older than 2 months. The observed parameter movements could be attributed to changes of stroke volume and mitral valve area for early filling-time velocity integral (R2 = 0.93), and of heart rate, stroke volume, and mitral valve area for early filling peak velocity (R2 = 0.84), and atrial-filling time velocity integral (R2 = 0.65). Isovolumic relaxation time and atrial-filling peak velocity became heart rate dependent not before 3 months after birth. CONCLUSIONS: The observed parameter changes are powerful indicators for the maturational process in diastolic function. This process is mainly completed by 3 months of age.
Assuntos
Diástole/fisiologia , Função Ventricular Esquerda/fisiologia , Função Atrial , Ecocardiografia Doppler de Pulso , Feminino , Humanos , Lactente , Modelos Lineares , Masculino , Estudos Prospectivos , Valores de Referência , Volume SistólicoRESUMO
UNLABELLED: Between August 1989 and July 2003 14 Jehovah's Witness children with congenital heart defects (CHD) aged under 14 years (median 2.9 years) and with a median weight of 14 kg underwent 16 operations with cardiopulmonary bypass (CPB). Five children had been operated on previously between one to three times. Preoperatively, 7 children were prepared with oral iron supplementation and 10 received erythropoietin. Mean hemoglobin (Hb) at admission was 14.4 g/dl (range 10.9 - 19.2). The cardiopulmonary bypass (CPB) circuit was modified to reduce total priming volume. High doses of aprotinin were administered. The modified ultrafiltration (MUF) circuit, used in 7 patients, was parallel to the ECC circuit with continuous circulation of the blood through a small shunt between the arterial and venous lines. Operations performed consisted of VSD closure (3 pts.), ASD closure (3 pts.), Fontan operation (2 pts.), and complete AV canal correction, aortic commissurotomy, Ross operation, Glenn shunt, cor triatriatum correction, MV reconstruction combined with left outflow tract stenosis resection, correction of absent pulmonary valve syndrome, and correction of tetralogy of Fallot in one patient each. There were no deaths. Mean duration of CPB was 192 min and mean aortic cross-clamp time 40 min. The Hb value at the end of the operation was 4.9 - 14.5 g/dl (mean 9.6) and at discharge it was 7.1 - 14.5 g/dl (mean 15.5). No blood or blood products were used in any patient. CONCLUSION: Bloodless cardiac surgery with and without CPB can be safely performed in Jehovah's Witness infants and children.
Assuntos
Cardiopatias Congênitas/cirurgia , Testemunhas de Jeová , Aprotinina/administração & dosagem , Ponte Cardiopulmonar , Criança , Pré-Escolar , Desamino Arginina Vasopressina/administração & dosagem , Desamino Arginina Vasopressina/uso terapêutico , Eritropoetina/uso terapêutico , Comunicação Interatrial/cirurgia , Comunicação Interventricular/cirurgia , Hemoglobinas/análise , Hemostáticos/administração & dosagem , Hemostáticos/uso terapêutico , Humanos , LactenteRESUMO
BACKGROUND: The Fontan operation causes an acute decrease of volume overload of the univentricular heart followed by changes in ventricular geometry. The postoperative increase of myocardial mass-volume-index (MVI) may alter ventricular diastolic function. In this study, we analysed whether the increase in MVI and changes of the ventricular geometry have an effect on the decrease of the exercise capacity in patients with Fontan surgery. METHODS: We examined the cardiopulmonary function of 24 patients with functional single ventricle and Fontan operation 3.63 +/- 1.97 years after surgery (m = 14, w = 10, age: 14.57 +/- 9.74 years) using a bicycle cardiopulmonary exercise testing. The parameters of exercise capacity and cardiopulmonary function were correlated with Magnetic Resonance Imaging (MRI) parameters such as the MVI, enddiastolic ventricular muscle mass (EDMM) und endsystolic volume (ESV). RESULTS: The exercise capacity was 2.06 +/- 0.54 W/kg (60.63 +/- 15.75% of the norm) and VO(2)max was 27.41 +/- 8.87 ml/min/kg (60.91 +/- 19.7% of the norm). There was a positive correlation of exercise capacity and VO(2)max with ESV (r(2) = 0.2572, p = 0.033) and EDMM (r(2) = 0.2544, p = 0.024), but none with the MVI. CONCLUSION: Myocardial hypertrophy may influence the myocardial performance of the univentricular heart and thereby the physical performance in children and adults with Fontan circulation.
Assuntos
Volume Cardíaco/fisiologia , Cardiomiopatia Hipertrófica/fisiopatologia , Teste de Esforço , Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/anormalidades , Complicações Pós-Operatórias/fisiopatologia , Adolescente , Adulto , Débito Cardíaco/fisiologia , Criança , Feminino , Seguimentos , Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/fisiopatologia , Ventrículos do Coração/cirurgia , Hemodinâmica/fisiologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Miocárdio/patologia , Oxigênio/sangue , Resistência Física/fisiologiaRESUMO
An infant with myocardial infarction due to congenital stenosis of the left coronary artery with consecutive left ventricular dysfunction and mitral regurgitation developed refractory pulmonary hypertension (PHT) and recurrent PHT crises. Catecholamines to support cardiac function, or pulmonary vasodilators like inhaled nitric oxide showed no effect. Treatment with Levosimendan (Simdax), a new inodilator, combining both inotropic and pulmonary vasodilating effects, improved left ventricular dysfunction, increased cardiac index, decreased pulmonary vascular resistance and reduced frequency and extent of the PHT crises. This case may suggest the use of Levosimendan as a long-term inotropic agent and pulmonary vasodilator in children with depressed cardiac function.
Assuntos
Cardiotônicos/administração & dosagem , Estenose Coronária/congênito , Anomalias dos Vasos Coronários/cirurgia , Hidrazonas/administração & dosagem , Hipertensão Pulmonar/congênito , Infarto do Miocárdio/congênito , Complicações Pós-Operatórias/tratamento farmacológico , Piridazinas/administração & dosagem , Vasodilatadores/administração & dosagem , Baixo Débito Cardíaco/tratamento farmacológico , Estenose Coronária/cirurgia , Ecocardiografia Doppler/efeitos dos fármacos , Eletrocardiografia Ambulatorial/efeitos dos fármacos , Humanos , Hipertensão Pulmonar/tratamento farmacológico , Lactente , Recém-Nascido , Assistência de Longa Duração , Masculino , Infarto do Miocárdio/cirurgia , Artéria Pulmonar/efeitos dos fármacos , Pressão Propulsora Pulmonar , Simendana , Volume Sistólico/efeitos dos fármacos , Resultado do Tratamento , Resistência Vascular/efeitos dos fármacosRESUMO
We report on the transcatheter closure of ventricular septal defects (VSD) in 26 patients with Amplatzer Occluders and Nit- Occlud Coil Systems. Twenty-one patients had a perimembranous and 5 patients a muscular VSD. Patients' age range was 5 months to 59 years (median 8 years) and their body weight 4.5 kg to 167 kg (median 28 kg). Defect diameters were 3-11 mm (median 5 mm). Sixteen patients had left ventricular volume overload and 7 patients pulmonary hypertension (median 50% of systemic pressure). Seven patients suffered from trivial or mild aortic regurgitation. Twenty-eight devices (4-12 mm; median 8 mm) were implanted (16 Amplatzer, 12 Nit-Occlud) through sheaths of 4F to 9F (median 7F). Fluoroscopy times were 8.3- 56.5 min (median 26.2 min). One coil was surgically explanted directly after intervention. One patient needed pulmonary banding due to additional VSDs. After a follow-up of 7 months (1-12 months), 2 patients had a small and 9 a minimal residual shunt. Thirteen defects were completely closed. Transcatheter closure of VSDs with new devices seems to be a promising therapy for suitable defects in different hemodynamic conditions in patients of every age.
Assuntos
Cateterismo Cardíaco/instrumentação , Comunicação Interventricular/cirurgia , Implantação de Prótese , Adolescente , Adulto , Angiografia , Criança , Pré-Escolar , Feminino , Fluoroscopia , Seguimentos , Comunicação Interventricular/diagnóstico por imagem , Septos Cardíacos/diagnóstico por imagem , Septos Cardíacos/cirurgia , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia , Ajuste de PróteseRESUMO
We studied the influence of preload augmentation on Doppler-derived left ventricular diastolic function parameters in infants with a birth weight <1500 g. In 44 neonates with a closed duct and 29 neonates with an open ductus arteriosus a complete echocardiographic study including Doppler investigations of the mitral inflow signals and analysis of diastolic time intervals was performed. Neonates with an open duct had a lower median gestational age (27.1 vs. 29, P<0.01), a higher cardiac index (425 vs. 260 ml/min/kg, P<0.001), and a different M-mode left atrial to aortic diameter ratio (1.36 vs. 0.79, P<0.001), but birth weight, age at examination (6.6 vs. 7.9 days), and heart rate were similar compared to the neonates with a closed duct. Main differences in diastolic indexes existed in early and atrial filling integrals and peak velocities, early filling acceleration time, and isovolumic relaxation time. Most informative in neonates with an open ductus peak early filling velocity (41.2 vs. 30.4 cm, P<0.01) and peak atrial filling velocity (49.2 vs. 35.9, P<0.001) are higher, and isovolumic relaxation time is shorter (45 vs. 53 ms, P<0.001) than in closed duct peers. Isovolumic relaxation time is inversely correlated with the cardiac index (R = -0.78). These parameter changes can be interpreted as incipient left ventricular diastolic failure in extremely low birth weight infants in the presence of a preload challenge. The coincidence of higher peak velocities with a shortened isovolumic relaxation time is very likely a result of left atrial pressure elevation. Preload mismatch has to be considered in preterm infants with a persistent ductus arteriosus. The results of this study can be helpful to find the indication for PDA-closure and to adjust volume replacement therapy, and catecholamine medication to a level appropriate for the individual cardiac performance.