RESUMO
BACKGROUND: Limited data exist regarding the coronary revascularization procedures needed during the repair of several congenital and pediatric cardiac malformations. We aimed to determine risk factors for in-hospital mortality and long-term outcomes of various pediatric coronary revascularization procedures. METHODS: We retrospectively reviewed the records of 32 consecutive pediatric patients who underwent coronary revascularization procedures at our institution between May 1995 and June 2020. In-hospital mortality, risk factors, surgical indications, revascularization patency, and mid- and long-term follow-up data were investigated. Patients were categorized into the coronary artery bypass grafting (n = 11) and other coronary artery procedure (n = 21) groups. RESULTS: The median age and weight of patients at the time of surgery were 9 months and 4.8 kg, respectively. There were five in-hospital deaths (5/32, 15.6%). The mortality rates were 27.2% (3/11) in the coronary artery bypass grafting group and 9.5% (2/21) in the other coronary artery procedure group (p = .206; 95% confidence interval: 0.496-25.563). The mortality rates for planned and rescue procedures were 8.3% (2/24) and 37.5% (3/8) (p = .06), respectively. The median follow-up time was 12.5 years. Control imaging studies for coronary patency were performed in 70.3% (19/27) of surviving patients. The overall coronary patency rate was 94.7% (18/19). CONCLUSIONS: Pediatric coronary revascularization procedures with elective-planned indications can be performed with good outcomes. Young age and rescue and emergency procedures may carry an increased risk of in-hospital mortality, although not found to be statistically significant. Surviving patients require lifelong follow-up regarding the patency of reperfused coronary arteries.
Assuntos
Doença da Artéria Coronariana , Vasos Coronários , Criança , Ponte de Artéria Coronária , Doença da Artéria Coronariana/cirurgia , Vasos Coronários/diagnóstico por imagem , Vasos Coronários/cirurgia , Seguimentos , Humanos , Estudos Retrospectivos , Fatores de Risco , Resultado do TratamentoRESUMO
Pulmonary valve preservation in tetralogy of Fallot (TOF) repair is one of the most challenging issues. Herein, we describe a novel valve-sparing technique for TOF repair that primarily consists of skeletonization of the anterior part of the pulmonary annulus and gentle dilatation by preserving the pulmonary valve and annulus integrity. With encouraging early results, this technique is suggested to prevent severe pulmonary regurgitation and provide acceptable relief of pulmonary stenosis in patients with TOF.
Assuntos
Procedimentos Cirúrgicos Cardiovasculares/métodos , Valva Pulmonar/cirurgia , Tetralogia de Fallot/cirurgia , Pré-Escolar , Dilatação Patológica , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Resultado do TratamentoRESUMO
BACKGROUND: We present our clinical experience with coronary artery bypass grafting (CABG) in children. METHODS: Ten children who underwent CABG between July 1995 and August 2017 were retrospectively analyzed. Data including congenital cardiac malformations, previous surgical procedures, age and sex, type of coronary complications, ischemic events preceding surgery, and ventricular function before and after CABG were recorded. RESULTS: The study population consisted of five males and five females with a median age of 2.5 years (range, 88 days to 15 years). Eight internal mammary arteries (IMAs) and two saphenous veins were used for grafting. Indications for bypass grafting were coronary artery (CA) complications related to the post-arterial switch operation in six, CA complications during the Ross procedure in two, and an iatrogenic CA injury during complete repair of tetralogy of Fallot with abnormal CA, crossing the right ventricular outflow tract in two patients. Six of the grafts were performed as rescue procedures. Three patients died during hospitalization. The mean follow-up time was 6.8 years (range, 3 months to 18 years). Anastomoses were evaluated by coronary angiography in four patients, and were all patent. Echocardiography revealed normal myocardial function in all patients. CONCLUSION: Our study suggests that the IMA should be the graft of choice in children due to its growth potential and long-term patency.
Assuntos
Ponte de Artéria Coronária , Doença da Artéria Coronariana/cirurgia , Cardiopatias Congênitas/cirurgia , Artéria Torácica Interna/transplante , Complicações Pós-Operatórias/cirurgia , Adolescente , Transposição das Grandes Artérias , Criança , Pré-Escolar , Doença da Artéria Coronariana/fisiopatologia , Vasos Coronários/lesões , Vasos Coronários/cirurgia , Feminino , Seguimentos , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/fisiopatologia , Humanos , Doença Iatrogênica , Lactente , Masculino , Complicações Pós-Operatórias/fisiopatologia , Estudos Retrospectivos , Resultado do Tratamento , Grau de Desobstrução Vascular , Função VentricularRESUMO
Background/aim: Although pulmonary valve replacement (PVR) improves ventricular function and symptoms, the benefit and optimal timing of PVR are controversial. This study aimed to evaluate early response to PVR for right ventricle (RV) dilatation and QRS duration. Materials and methods: Retrospective analysis was performed for 32 patients with repaired tetralogy of Fallot (TOF) between March 2005 and October 2017. The differences between preoperative and postoperative changes in echocardiographic parameters, clinical symptoms, and QRS duration were evaluated. Results: There were no in-hospital or late deaths. Mean age at the time of PVR was 16.57 ± 7.97 years. The interval between TOF repair and PVR was 12.99 ± 7.06 years. Postoperative echocardiographic findings showed significant reduction in indexed RV end-diastolic diameter (RV-EDDI) and the ratio of RV/LV-EDDI (P = 0.001 and P = 0.001, respectively). Higher preoperative RV-EDDI was associated with decreased change in RV-EDDI after PVR (r = 0.63; P = 0.001). Normalization of RV diameters was found to be independent of age at PVR, interval between TOF repair and PVR, preoperative QRS duration, and preoperative RV-EDDI. Conclusion: Significant improvement in RV diameter and symptoms could be obtained with PVR in patients with severe pulmonary regurgitation.
RESUMO
An 8-year-old boy with previous shunt operation for corrected transposition of great arteries, ventricular septal defect, pulmonary stenosis and multiple aortopulmonary collateral arteries underwent corrective surgery. In the early post-operative period, there were clinical findings of superior vena cava obstruction. Cardiac catheterization at 72 h following surgery showed a systemic venous baffle stenosis between the vena cava and right atrium. A stent was successfully implanted in the vena cava percutaneously, and the stenosis was relieved. Her symptoms resolved in a short time period, and she was extubated rapidly. During the follow-up, excellent maintenance and patency of systemic venous baffle were observed.
Assuntos
Complicações Pós-Operatórias/terapia , Stents , Síndrome da Veia Cava Superior/terapia , Transposição dos Grandes Vasos/cirurgia , Criança , Angiografia Coronária , Diagnóstico Diferencial , Humanos , Masculino , Complicações Pós-Operatórias/diagnóstico por imagem , Síndrome da Veia Cava Superior/complicações , Síndrome da Veia Cava Superior/diagnóstico por imagem , Transposição dos Grandes Vasos/complicações , Transposição dos Grandes Vasos/diagnóstico por imagemRESUMO
The mortality and morbidity of fetal aortic stenosis (AS) depend on the degree of the hemodynamic effects of the stenosis, and left ventricular (LV) adaptation, development and function during fetal life. In the case of critical AS, the development of hydrops and death in utero are well recognized entities. A 23-week gestation fetus was diagnosed with critical severe AS, cardiomegaly, a dilated LV with very poor contractility, and mitral regurgitation. There was a reversal of flow in the aortic arch through the ductus arteriosis and a reversed a-wave in the ductus venosus on Doppler examination. The fetus had hydrops with ascites, and massive scalp, face and skin edema. Fetal amniocentesis was normal. Aortic valvuloplasty was performed under general anesthesia and echocardiographic guidance. Pericardial effusion was not observed after the procedure. However, LV function could not be ameliorated and continued to diminish. There was no cardiac activity in the fetus two hours after the intervention. Aortic valvuloplasty in utero for AS is technically feasible. Mortality is mainly associated with technical errors, LV function, and the degree of endofibroelastosis in the effected fetuses.
Assuntos
Estenose da Valva Aórtica/terapia , Valvuloplastia com Balão/métodos , Bradicardia/etiologia , Morte Fetal/etiologia , Doenças Fetais/terapia , Adulto , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/embriologia , Valvuloplastia com Balão/efeitos adversos , Bradicardia/embriologia , Fibroelastose Endocárdica/embriologia , Evolução Fatal , Feminino , Doenças Fetais/diagnóstico por imagem , Insuficiência Cardíaca/embriologia , Humanos , Hidropisia Fetal , Gravidez , Ultrassonografia Pré-NatalRESUMO
BACKGROUND: The peritoneal equilibration test (PET) is recommended in paediatric peritoneal dialysis (PD) patients to assist prescription management. Despite contradictory reports, high transporter status is associated with reduced survival rate in adults. Since cardiac disease is one of the main causes of mortality in paediatric PD patients, we aimed to evaluate whether transport features have any effect on biochemical data and cardiac function in this group. METHODS: One hundred and ten PD patients (13 +/- 5 years, PD vintage: 31 +/- 27 months) were enrolled into the study. Four-hour dialysate/plasma creatinine ratio was used for differentiating PET groups. Thirty-eight patients were high transporters, 29 were high-average transporters and 43 were low-average/low transporters. Echocardiography was performed in all subjects. RESULTS: Age, PD vintage, dialysate glucose concentration, ultrafiltration volume, urine volume and blood pressure levels were similar in all PET groups. No biochemical or echocardiographic data (ejection fraction, fractional shortening, left ventricular mass index, myocardial performance index, power Doppler E/tissue Doppler E ratio reflecting diastolic function) were different among PET groups except lower albumin (P = 0.025) levels in high transporters and higher high-sensitivity C-reactive protein (P = 0.026) levels in high and high-average transporters compared to other transport groups. CONCLUSIONS: Cardiac structural and functional abnormalities are highly prevalent among paediatric PD patients. Transport rates did not have a significant effect on biochemical parameters or cardiac structural/functional parameters. It might be suggested that being a high transporter does not provide a disadvantage in terms of atherogenic tendency and cardiac disease in paediatric PD patients. Oligoanuria, anaemia and hypertension were independent predictors of cardiac disease.
Assuntos
Coração/fisiopatologia , Falência Renal Crônica/fisiopatologia , Falência Renal Crônica/terapia , Diálise Peritoneal/métodos , Peritônio/fisiopatologia , Adolescente , Transporte Biológico/fisiologia , Doenças Cardiovasculares , Estudos de Casos e Controles , Criança , Ecocardiografia Doppler , Feminino , Humanos , Falência Renal Crônica/mortalidade , Masculino , Fatores de Risco , Taxa de Sobrevida , TurquiaRESUMO
We report two pediatric patients with IDC who underwent autologous PSCT. Both cases were referred to our clinic for cardiac transplantation because of end-stage heart failure resistant to conventional therapy with digoxin, diuretics, ACE inhibitors, and sympathomimetics. They had ejection fractions below 35%. In each case, autologous stem cell transplantation was performed via the coronary arteries, and five wk after the procedure transthoracic echocardiography showed a striking gain in their ejection fractions and an improvement in the left ventricular dimensions compared with the initial measurements. Although heart transplantation is the only option for children with IDC, stem cell transplantation can lessen the waiting list mortality and prolong the time for a patient to wait for a suitable donor.
Assuntos
Cardiomiopatia Dilatada/cirurgia , Insuficiência Cardíaca/cirurgia , Transplante de Células-Tronco de Sangue Periférico , Cardiomiopatia Dilatada/diagnóstico por imagem , Cardiomiopatia Dilatada/fisiopatologia , Criança , Ecocardiografia , Feminino , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/fisiopatologia , Humanos , Masculino , Volume Sistólico , Fatores de Tempo , Transplante AutólogoRESUMO
This article reviews our experience with 111 pediatric patients following open-heart surgery over 1-year period. Peritoneal dialysis was required in 34 of 111 children (30.6%). We randomly selected 33 patients who did not require peritoneal dialysis as control group. The indications of dialysis were oligoanuria and/or elevated serum creatinine level (19/34, 55.8%), fluid overload and/or hemodynamic alterations (10/34, 29.5%), and hyperkalemia and/or acidosis (5/34, 14.7%). Among the 34 dialyzed patients, 19 (55.6%) had acute renal failure (ARF). Cyanotic congenital heart disease was significantly higher in patients who required dialysis than the patients who did not require dialysis (67.6% and 22.6%, respectively, p < 0.001). Cardiopulmonary bypass time was significantly longer in patients with ARF than those without ARF (p < 0.05). Overall mortality rate was significantly higher in patients who required dialysis than control group (42.1% and 18.2%, respectively, p < 0.05). However, in the dialyzed group the mortality for patients who developed ARF was 68.4% and 6.7% for those who did not develop ARF [odds ratio (OR): 30.3, confidence interval (CI) 95%: 3.2-28.7, p < 0.001]. In conclusion in children high mortality rate following open-heart surgery was associated with ARF. Patients with cyanotic congenital heart disease and prolonged cardiopulmonary bypass time are at risk for ARF. The presence of these factors can be predicted in the early institution of peritoneal dialysis after cardiac surgery.
Assuntos
Injúria Renal Aguda/terapia , Cardiopatias/congênito , Cardiopatias/cirurgia , Diálise Peritoneal/estatística & dados numéricos , Injúria Renal Aguda/etiologia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/métodos , Pré-Escolar , Humanos , Lactente , Estudos Retrospectivos , Fatores de TempoRESUMO
Kearns-Sayre syndrome (KSS) is a rare mitochondrial disease in which neuromuscular structures, endocrine glands, and cardiac conduction systems are most commonly involved. An 11-year-old boy was admitted with blurred consciousness, respiratory instability, and bradycardia of two-hour onset. He was immediately intubated. His medical history included growth retardation and myopic refractive defect for six years, therapy for somatomedin C deficiency for 15 months, and bilateral ptosis for three months. On physical examination, he was unconscious, had a peak heart rate of 40/min, blood pressure of 60/20 mmHg, and a weak pulse. Laboratory findings showed elevated blood lactate and blood pyruvate levels and an increased lactate/pyruvate ratio. The electrocardiogram showed complete atrioventricular block and echocardiography showed mitral valve prolapse. Following implantation of a temporary transvenous cardiac pacemaker, his heart rate and clinical condition improved. Further analysis with cranial magnetic resonance (MR) imaging demonstrated hyperintense signal changes in the subcortical white matter of the two cerebral hemispheres, bilateral thalamus, putamen, cerebral peduncles, dorsal medulla, and midbrain. The typical clinical and MR findings confirmed the initial diagnosis of KSS. A permanent cardiac pacemaker was implanted into the right ventricle.
Assuntos
Bloqueio Cardíaco/diagnóstico , Fator de Crescimento Insulin-Like I/deficiência , Síndrome de Kearns-Sayre/diagnóstico , Marca-Passo Artificial , Criança , Diagnóstico Diferencial , Eletrocardiografia , Bloqueio Cardíaco/etiologia , Bloqueio Cardíaco/terapia , Humanos , Síndrome de Kearns-Sayre/terapia , Imageamento por Ressonância Magnética , Masculino , Prolapso da Valva Mitral/diagnóstico , Prolapso da Valva Mitral/etiologia , Prolapso da Valva Mitral/terapiaRESUMO
Left ventricular pseudoaneurysm (LVPA) is a rare but lethal complication of mitral valve replacement (MVR) or myocardial infarction. Early correction is necessary for patients with extensive and expanding LVPA. We report a transcatheter closure of LVPA after MVR. A 63-year-old woman presented with an LVPA 2 months after MVR. The repeated computed tomographic scan and transthoracic echocardiography showed enhancement of pseudoaneurysm. The LVPA was closed successfully with Amplatzer Vascular Plug using a transcatheter approach.
Assuntos
Falso Aneurisma/cirurgia , Aneurisma Cardíaco/cirurgia , Implante de Prótese de Valva Cardíaca , Ventrículos do Coração , Valva Mitral/cirurgia , Complicações Pós-Operatórias/cirurgia , Cateterismo Cardíaco , Procedimentos Cirúrgicos Cardíacos/métodos , Feminino , Humanos , Pessoa de Meia-Idade , Dispositivo para Oclusão SeptalRESUMO
METHODS: We aimed to clarify arteriosclerotic risk and to document possible relationships between cardiovascular risk factors and echocardiographic parameters in paediatric peritoneal dialysis (PD) patients. M-mode/Doppler/tissue Doppler echocardiographic studies and lipid/lipoproteins, homocysteine, high-sensitivity C-reactive protein (HS-CRP) levels and carotid intima-media thickness (CIMT) were determined in 59 patients (age: 14.2 +/- 4.5 years) and in 36 healthy subjects. RESULTS: Structural and functional cardiac abnormalities were observed in patients on maintenance dialysis. Increased left ventricular mass index (LVMI, P = 0.000), relative wall thickness (P = 0.000), myocardial performance index (MPI, P = 0.000) were documented in the patients. Lipoprotein (a) (P = 0.000), homocysteine (P = 0.001), HS-CRP (P = 0.000) and CIMT (P = 0.000) were significantly elevated in the patients. Left ventricular hypertrophy (LVH) was prevalent in 68% of the patients. Patients with LVH had higher levels of HS-CRP (P = 0.001) and CIMT (P = 0.028) than those without LVH. Haemoglobin was an independent predictor of LVMI (beta: -8.9, P = 0.001), while residual diuresis and CIMT were independent predictors of diastolic dysfunction (beta: -0.45, P = 0.034 and beta: 5.90, P = 0.008, respectively). Albumin (beta: -0.72, P = 0.018) and Kt/V urea (beta: -0.48, P = 0.012) were significant predictors of CIMT. There were positive correlations between LVMI and CIMT. HS-CRP was positively correlated with LVMI as well as CIMT. CONCLUSIONS: Elevated levels of atherosclerotic/ inflammatory risk factors, low haemoglobin levels and loss of residual renal function and their negative effects on heart are of remarkable importance in paediatric patients on maintenance peritoneal dialysis. Achieving recommended targets for haemoglobin, blood pressure and Kt/V urea, preserving residual renal function as well as managing inflammation and subsequent arteriosclerosis is obviously essential to improve the patients' prognosis.
Assuntos
Doenças Cardiovasculares/etiologia , Diálise Peritoneal/efeitos adversos , Adolescente , Adulto , Pressão Sanguínea , Proteína C-Reativa/análise , Artérias Carótidas/patologia , Criança , Ecocardiografia , Feminino , Humanos , Falência Renal Crônica/etiologia , Masculino , Miocárdio/patologia , Fatores de Risco , Adulto JovemRESUMO
OBJECTIVES: We evaluated patients who underwent complete or partial surgical correction for atrioventricular septal defect (AVSD) with regard to surgical techniques and early and midterm results. STUDY DESIGN: Forty-six patients were treated for complete (n=28) or partial (n=18) AVSD between 2000 and 2007. There were nine boys and 19 girls (mean age 5.5 months; range 1.5 to 11 months) with complete AVSD. Of these, 17 patients underwent total repair, while 11 patients underwent palliative procedures. Five males and 13 females (mean age 11 years; range 1 to 50 years) with partial AVSD were treated with total repair. Down syndrome was seen in nine patients (32.1%) and one patient (5.6%) in complete and partial AVSD groups, respectively. Twenty-one patients (75%) and 14 patients (77.8%) could be followed-up for a mean of 26.3 months (range 1-72) and 21.8 months (range 2 to 71) in the two groups, respectively. RESULTS: Total repair of partial AVSD resulted in no mortality or significant morbidity. Early postoperative mortality occurred in three cases (10.7%) after repair of complete AVSD, one of which had Down syndrome. Six patients required prolonged mechanical ventilation beyond one week. Two patients without Down syndrome underwent reoperation due to severe atrioventricular (AV) valve insufficiency in the early postoperative period. None of the patients required permanent pacemaker implantation. Clinical and echocardiographic monitoring showed moderate left AV valve insufficiency in three patients in each group, while the remaining patients had no or minimal insufficiency. CONCLUSION: Total repair of complete AVSD should be the procedure of choice in early infancy. Left AV valve insufficiency continues to be the most important cause of postoperative morbidity in these cases.
Assuntos
Defeitos dos Septos Cardíacos/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Síndrome de Down/complicações , Feminino , Defeitos dos Septos Cardíacos/etiologia , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Insuficiência da Valva Mitral/etiologia , Insuficiência da Valva Mitral/cirurgia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia , Reoperação , Resultado do TratamentoRESUMO
OBJECTIVES: Complete atrioventricular septal defect (AVSD) with tetralogy of Fallot (TOF) is a rare congenital heart anomaly. We evaluated surgical results of total repair in patients with TOF and AVSD. STUDY DESIGN: Seven patients (5 girls, 2 boys; age range 2.5 to 14 years) underwent total repair for AVSD and TOF. Three patients had Down syndrome. Three patients had previous systemic-to-pulmonary shunt operations. Accompanying anomalies were left superior vena cava (n=2), left atrial isomerism (n=1), muscular VSD (n=1), and double outlet right ventricle (n=1). Preoperative diagnoses were based on echocardiographic examinations. The two-patch technique was used for surgical repair. Reconstruction of the right ventricular outflow tract was performed using transannular and infundibular patches in four and three patients, respectively. The follow-up period ranged from six months to nine years (mean 3.4+/-2.9 years). RESULTS: No mortality occurred throughout the follow-up period. Two patients had prolonged hospitalization (>1 month) due to pulmonary infection and sepsis. At the latest follow-up, functional capacity was NYHA class I in five patients, and class II in two patients. Final echocardiographic examinations showed mild left atrioventricular (AV) valve insufficiency in five patients, and mild (n=3) or moderate (n=1) right AV valve insufficiency. Two patients had mild residual pulmonary stenosis, and three patients with a transannular patch had free pulmonary insufficiency. All the patients had proper right and left ventricular functions and all were in sinus rhythm. CONCLUSION: With a proper surgical strategy and technique, AVSD and TOF can be corrected successfully. Long-term follow-up is necessary for AV valve dysfunction and pulmonary insufficiency.
Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Cardiopatias Congênitas/cirurgia , Comunicação Interatrial/cirurgia , Comunicação Interventricular/cirurgia , Tetralogia de Fallot/cirurgia , Adolescente , Criança , Feminino , Seguimentos , Humanos , Masculino , Fatores de Tempo , Resultado do TratamentoRESUMO
A 28-week-old preterm infant with a birth weight of 1250 g had a broken segment of umbilical vein catheter (UVC) lodged in the right atrium. It was observed that a 7-cm fragment of catheter had migrated to the right atrium through the inferior vena cava. The catheter was successfully retrieved by fluoroscopically guided percutaneous cardiac catheterization, using a cobra catheter and snare wire, without complication. Fracture and embolization of UVC is a rare but serious complication. Presently described was a case of fractured and embolized UVC in a very low-birth-weight preterm infant, and its successful retrieval via percutaneous endovascular approach.
Assuntos
Cateterismo Venoso Central/efeitos adversos , Migração de Corpo Estranho/terapia , Átrios do Coração , Recém-Nascido Prematuro , Recém-Nascido de muito Baixo Peso , Veias Umbilicais , Remoção de Dispositivo , Diagnóstico Diferencial , Falha de Equipamento , Migração de Corpo Estranho/diagnóstico , Migração de Corpo Estranho/diagnóstico por imagem , Migração de Corpo Estranho/etiologia , Humanos , Recém-NascidoRESUMO
Transcatheter device implantation has become an attractive alternative to surgery in the closure of atrial septal defects in selected patients. However, it can lead to early and late sequelae, some of them life threatening. For example, 79 days before her admission to our emergency department with sudden-onset respiratory distress and respiratory arrest (leading to cardiac tamponade and rupture), a 22-year-old woman had undergone percutaneous closure of an atrial septal defect. We describe the damage and its treatment. Although the adverse effects of transcatheter device implantation are rare, physicians should know that these events can be life threatening. Further data are needed to prevent such sequelae and to design new devices. It is of utmost importance that patients and their family members be informed both of possible sequelae and of life-saving interventions to be administered at early diagnosis.
Assuntos
Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/instrumentação , Traumatismos Cardíacos/etiologia , Comunicação Interatrial/terapia , Dispositivo para Oclusão Septal/efeitos adversos , Tamponamento Cardíaco/etiologia , Remoção de Dispositivo , Feminino , Traumatismos Cardíacos/diagnóstico , Traumatismos Cardíacos/cirurgia , Comunicação Interatrial/diagnóstico , Humanos , Desenho de Prótese , Fatores de Tempo , Resultado do Tratamento , Adulto JovemRESUMO
One of the options for the management of borderline/failing extracardiac Fontan circulation is surgical creation of an atrial fenestration to decompress the systemic venous compartment and improve cardiac output. Depending on the body surface area of the patient, a 5- to 10-mm polytetrafluoroethylene (PTFE) tube graft can be used. When fenestration is required in a patient with failing Fontan circulation, particularly in redo cases, application of a side-biting clamp may be challenging because of adhesions and a thickened atrial wall. In this article, we present our off-pump technique of atrial-side anastomosis of PTFE graft interposition between an extracardiac Fontan conduit and the atrium without using a side-biting clamp.
Assuntos
Prótese Vascular , Técnica de Fontan , Átrios do Coração/cirurgia , Politetrafluoretileno , Complicações Pós-Operatórias/cirurgia , Humanos , Masculino , Adulto JovemRESUMO
This report describes a patient who had some phenotypic features of Down syndrome (DS) as well as severe conotruncal cardiac anomalies, including pulmonary atresia with ventricular septal defect (tetralogy of Fallot with pulmonary atresia), confluent pulmonary arteries, a large left-sided ductus arteriosus, left aortic arch, aberrant right subclavian artery, and secundum atrial septal defect. Cytogenetic and fluorescence in situ hybridization (FISH) analysis was carried out on peripheral blood lymphocytes and skin fibroblasts using probes specific for the chromosomal loci 21q22.13 to 21q22.2 and locus 22q11.2. This revealed 47,XX+21/46,XX mosaicism at a rate of 15:85 and the micro-deletion 22q11.2 (del22q11.2). Some patients'congenital cardiac anomalies are atypical for the type of mosaicism or aneuploidy. The case suggests that association of del22q11.2 should be considered in patients with chromosomal mosaicism or aneuploidy who also have particular conotruncal cardiac defects.
Assuntos
Cromossomos Humanos Par 22 , Síndrome de Down , Mosaicismo , Humanos , Hibridização in Situ Fluorescente , Recém-Nascido , MasculinoRESUMO
Prenatal echocardiographic diagnosis of heart defects is important because it permits counseling of the parents with regard to prognosis and treatment options and prepares the medical team for the treatment postnatally. A male infant with absent pulmonary valve syndrome diagnosed prenatally at 22 weeks' gestation is reported. This congenital anomaly is characterized by absent or rudimentary pulmonary valve cusps, conoventricular septal defect, and massive dilatation of the pulmonary arteries. Soon after delivery the infant developed cyanosis and respiratory distress. The infant was placed in prone position for the relief of bronchial compression and nasal continuous positive airway pressure was (CPAP) started. Although the clinical status of the infant improved after supportive treatment, he deteriorated acutely and died at the age of five days. Fetal diagnosis remains an integral part of successful management of children with heart disease. Despite the potential benefits of prenatal diagnosis, it is hard to show significant improvement in mortality, especially in severely affected cases.
Assuntos
Valva Pulmonar/anormalidades , Ultrassonografia Pré-Natal , Adulto , Ecocardiografia , Evolução Fatal , Feminino , Humanos , Recém-Nascido , Masculino , Gravidez , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Valva Pulmonar/diagnóstico por imagemRESUMO
Bronchial compression due to pulmonary artery dilation is an important problem in infants with congenital heart disease, and can complicate the postoperative course. In recent years, airway stenting has become a popular treatment for these cases. We achieved success with endobronchial stenting in a two-month-old infant with bronchial compression caused by a dilated pulmonary artery.