RESUMEN
BACKGROUND: Cyanotic CHD is a life-threatening condition that presents with low oxygen saturation in the newborn period. Hypoxemia might cause alterations in the metabolic pathways. In the present study, we aimed to evaluate the early postnatal amino acid and carnitine/acylcarnitine profiles of newborn infants with cyanotic CHD. METHODS: A single centre case-control study was conducted. Twenty-seven patients with cyanotic CHD and 54 healthy newborn controls were enrolled. As part of the neonatal screening programme, results of amino acid and carnitine/acylcarnitine were recorded and compared between groups. RESULTS: Twenty-seven neonates with cyanotic CHD and 54 healthy newborns as controls were enrolled in the study. Cyanotic CHD neonates had higher levels of alanine, phenylalanine, leucine/isoleucine, citrulline, ornithine, C5, C5-OH; but lower levels of C3, C10, C12, C14, C14:1, C16, C16.1, C18, C5-DC, C6-DC, C16-OH, C16:1-OH when compared with the healthy controls. CONCLUSION: This study showed that there are differences between patients with cyanotic CHD and healthy controls in terms of postnatal amino acid and carnitine/acylcarnitine profiles.
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Aminoácidos , Carnitina , Lactante , Humanos , Recién Nacido , Estudios de Casos y Controles , Carnitina/metabolismo , MetabolomaRESUMEN
Extracorporeal membrane oxygenation (ECMO) is widely used after congenital heart surgery. The purpose of this study is to analyze the factors influencing mortality and morbidity in patients who require ECMO support after congenital cardiac surgery. All 109 patients (5.8% of total cases) who underwent ECMO support after congenital heart surgery between January 2014 and 2021 were included in this single-center study. The mean age was 10.13 ± 20.55 months, and the mean weight was 6.41 ± 6.79 kg. 87 (79.8%) of the patients were under 1 year of age. A total of 54 patients (49.5%) were weaned successfully from ECMO support, and 27 of them (24.8%) were discharged. The childhood age group had the best outcomes. Seventy-seven percent of the children were weaned successfully, and 50% were discharged. 69 patients (63.3%) had biventricular physiology; weaning and survival outcomes were better than single ventricle patients (P-value 0.002 and < 0.001, respectively). Low cardiac output (n = 49; 44.9%) as an ECMO indication had better outcomes than extracorporeal cardiopulmonary resuscitation (n = 31; 28.4%) (P = 0.05). Most of the patients had ≥ 4 Modified Aristotle Comprehensive Complexity (MACC) levels, and higher MACC levels were associated with a higher mortality rate. The most common procedure was the Norwood operation (16.5%), with the worst outcome (5.5% survival). Bleeding and renal complications were the most common complications affecting outcomes. Results were more satisfactory in patients with biventricular repair, childhood, and lower MACC levels. Early initiation of ECMO in borderline patients without experiencing cardiac arrest or multiorgan failure may improve outcomes.
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Procedimientos Quirúrgicos Cardíacos , Oxigenación por Membrana Extracorpórea , Cardiopatías Congénitas , Niño , Humanos , Lactante , Preescolar , Oxigenación por Membrana Extracorpórea/efectos adversos , Resultado del Tratamiento , Estudios Retrospectivos , Cardiopatías Congénitas/complicaciones , Procedimientos Quirúrgicos Cardíacos/métodosRESUMEN
The congenital left ventricular diverticulum is a rare cardiac malformation, and it may associate with Cantrell's Pentalogy with other cardiac defects. However, isolated ventricular diverticulum without any other cardiac defect in complete Cantrell's syndrome is very rare. We describe a 6-year-old male patient with a complete Cantrell's syndrome with isolated left ventricular diverticulum.
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Divertículo , Cardiopatías Congénitas , Pentalogía de Cantrell , Masculino , Humanos , Niño , Pentalogía de Cantrell/complicaciones , Pentalogía de Cantrell/diagnóstico , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/diagnóstico por imagen , Divertículo/diagnóstico , Divertículo/diagnóstico por imagen , SíndromeRESUMEN
OBJECTIVE: Laterality anomalies are almost always associated with severe cardiac anomalies. Demographic properties, type of the procedures, associated anomalies, and early and mid-term prognosis of four types of laterality anomalies were analysed. METHODS: A total of 64 consecutive patients with laterality anomalies were enrolled between July 2014 and July 2020. We grouped the patients as situs solitus dextrocardia (SSD) (n = 12; 18.7%); situs inversus (SI) (n = 16; 25%); right atrial isomerism (RAI) (n = 29; 45.3%); and left atrial isomerism (LAI) (n = 7; 10.9%). TAPVC was only present in the RAI group (31%). Incidence of mitral or tricuspid atresia was higher in the SSD group (25%). All the patients were followed up with a mean of 19.06 ± 17.6 (0.1-72) months. RESULTS: Early postoperative mortality was 17 patients, among 107 procedures (15.8%). Twelve patients were in the neonatal period. All ten patients survived after isolated ductal stenting. Fourteen of the deaths were in the RAI group (48.3%). The 3-year survival rates were 85% in LAI, 78.7% in SI, 55.8% in SSD, and 38% in RAI groups. According to the multivariable Cox regression model, mechanical ventilation, kidney injury, RAI, and complex surgery in the neonatal period were independent risk factors for early mortality. CONCLUSION: Laterality anomalies are one of the most challenging patients who commonly had univentricular physiology. The most prevalent anomaly was RAI, and RAI had the worst outcome and survival. Ductal stent is an acceptable first intervention during the neonatal period in suitable patients. Complex procedures may carry a high risk of death in the neonatal period.
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Cardiopatías Congénitas , Síndrome de Heterotaxia , Situs Inversus , Cardiopatías Congénitas/epidemiología , Cardiopatías Congénitas/cirugía , Síndrome de Heterotaxia/epidemiología , Síndrome de Heterotaxia/cirugía , Humanos , Recién Nacido , Pronóstico , Factores de Riesgo , Situs Inversus/complicaciones , Tasa de SupervivenciaRESUMEN
BACKGROUND: The effect of prenatal diagnosis on prognosis in patients with transposition of the great arteries is not clear. In this study, we compared the outcomes after arterial switch operation. METHODS: Outcome of 112 patients who had arterial switch operation in the neonatal period were analysed. The patients were divided into two groups: those who had prenatal diagnosis (Group 1; n = 34) and those who did not (Group 2; n = 78). The patients were also classified based on their diagnosis: simple transposition, transposition with ventricular septal defect and/or aortic arch hypoplasia, and Taussig-Bing anomaly. RESULTS: In Group 1, the C-section delivery rate was higher (82% vs. 44%; p = 0.004), and it was observed that patients in Group 1 were more often intubated upon admission to the neonatal ICU (38% vs. 9%; p = 0.005). No differences were found between the two groups in terms of operation time, cardiopulmonary bypass time, post-operative invasive respiratory support duration, or extracorporeal membrane oxygenation support. It was observed that those who had Taussig-Bing anomaly had a higher mortality. CONCLUSIONS: Timely treatment have a positive effect on neonatal mortality and morbidity. That's why all families with prenatal diagnosis of critical CHD should be recommended to have the delivery in a tertiary care hospital. Although it could not be demonstrated in this study, prenatal diagnosis has a potential to improve surgical results especially in countries or cities, which does not have enough resources for transfer and surgical units. Further efforts are needed to improve prenatal screening programmes.
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Operación de Switch Arterial , Ventrículo Derecho con Doble Salida , Transposición de los Grandes Vasos , Humanos , Recién Nacido , Embarazo , Lactante , Femenino , Operación de Switch Arterial/métodos , Ventrículo Derecho con Doble Salida/cirugía , Transposición de los Grandes Vasos/diagnóstico , Transposición de los Grandes Vasos/cirugía , Estudios de Seguimiento , Estudios Retrospectivos , Morbilidad , Diagnóstico Prenatal , Resultado del TratamientoRESUMEN
BACKGROUND: Acute kidney injury (AKI) is a common complication of congenital heart diseases (CHDs) after cardiac surgery. This study aimed to define the frequency and critical course, risk factors and short-term outcomes of AKI in postoperative CHD neonates. METHODS: Postoperatively followed term CHD newborn infants were enrolled in the study. Infants with congenital anomalies of the urinary tract and other major congenital anomalies were excluded. Neonatal modified KDIGO criteria were used to assess AKI. RESULTS: A total of 199 postoperatively followed newborn infants were included in the study. Acute kidney injury was detected in 71 (35.6%) patients. Of these patients, 24 (33.8%) were in stage 1, 14 (19.7%) in stage 2, and 33 (46.5%) in stage 3. Acute kidney injury occurred within the first week (median 1 day [IQR 1-2 days]) of cardiac surgery in 93% of the patients. The duration of invasive respiratory support and extracorporeal membrane oxygenation (ECMO) and mortality were significantly higher in stage 3 patients. Higher vasoactive-inotropic score (OR, 1.02; 95% CI, 1.0-1.04; p = 0.008) and receiving ECMO (OR, 7.9; 95% CI, 2.6-24.4; p = 0.001) were associated with risk for the development of AKI. The mortality rate was 52.1% in the AKI (+) patients, and having AKI (OR 7.1; 95% CI, 3.5-14.18) was significantly associated with mortality. CONCLUSION: Acute kidney injury, a common early complication after critical neonatal CHD cardiac surgery, is associated with increased morbidity and mortality. Stage 3 AKI is associated with significantly higher mortality rates.
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Lesión Renal Aguda , Procedimientos Quirúrgicos Cardíacos , Oxigenación por Membrana Extracorpórea , Cardiopatías Congénitas , Lesión Renal Aguda/diagnóstico , Lesión Renal Aguda/epidemiología , Lesión Renal Aguda/etiología , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Oxigenación por Membrana Extracorpórea/efectos adversos , Cardiopatías Congénitas/cirugía , Humanos , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Estudios Retrospectivos , Factores de RiesgoRESUMEN
OBJECTIVES: Surgical management of aortic arch hypoplasia (AAH) with associated intracardiac anomalies is a challenge in newborns. We reviewed the characteristics and outcomes of neonates and infants who underwent pulmonary artery banding concomitant to arch repair and single-stage total repair at our institution. METHODS: Medical records of 60 patients undergoing aortic arch reconstruction for AAH from 2014 to 2019 were retrospectively reviewed. Twenty-five patients were female (41.6%), and the age of the patients ranged from 4 to 120 days (median, 19.5 days). The patients were divided into two groups: Group 1 (23 patients) underwent pulmonary artery banding concomitant to arch repair, and Group 2 (37 patients) underwent single-stage total repair in addition to arch repair. All arch repair procedures consisted of an extended (to the midportion of the ascending aorta) patch aortoplasty. RESULTS: Postoperative early mortality occurred in 12 patients, eight in Group 1 (34.8%) and four in Group 2 (10.8%). There was an early survival advantage in Group 2 (p = .019). Recoarctation occurred in 13 cases (21.6%), and 11 (18.3%) of them required reintervention (balloon angioplasty: 7, reoperation: 4). On univariate analysis, risk factors associated with death were pulmonary artery banding (hazard ratio [HR], 0.44; confidence interval [CI], 0.09-2; p = .019), prematurity (HR, 4.67; CI, 1.34-16.18; p = <.001), preoperative mechanical ventilation support requirement (HR, 0.048; CI, 0.52-6.39; p = .048), and functional single ventricle (HR, 0.43; CI, 0.1-1.86; p = .006). The mean duration of follow-up was 21.9 ± 15.1 months, and there was no late death in either group. CONCLUSION: Single-stage repair of AAH with intracardiac pathologies has better results than palliation, according to survival rates and postoperative results. The use of the patch augmentation technique in AAH is valid and associated with an acceptable incidence of recurrent arch obstruction.
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Aorta Torácica , Coartación Aórtica , Aorta , Aorta Torácica/cirugía , Coartación Aórtica/cirugía , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Reoperación , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: Transannular patch, which results in pulmonary insufficiency (PI), is usually required during repair of tetralogy of fallot (TOF). In this study, we compared three types of pulmonary valve reconstruction techniques during transannular repair of TOF. METHODS: Between February 2014 and January 2018, 50 patients with TOF underwent primary repair with transannular patch. These patients were divided into three groups. In Group 1, (n = 15), a single gluteraldehyde-treated autologous pericardial patch (standard method) was reconstructed as monocusp. In Group 2, (n = 16) Nunn's bileaflet pulmonary valve reconstruction technique was used with pericardial patch. In Group 3, (n = 19), Nunn's bileaflet technique was performed with expanded polytetrafluoroethylene membrane. The outcomes of the patients and early and midterm competency of the pulmonary valves were analyzed. RESULTS: These techniques were significantly effective in early postoperative period. Freedom from moderate to severe PI were 73.3%; 100% and 89.4%, respectively. Mortality, duration of intensive care unit and hospital stay were similar between the groups. The mean follow-up period was 17.5 ± 13.0 (3-57) months. Freedom from moderate to severe PI decreased to 40%; 81.2% and 73.7%, respectively at the end of the follow-up period. Presence of moderate to severe PI was significantly higher in Group 1 (p = .018 between Groups 1 and 2, p = .048 between Groups 1 and 3). CONCLUSION: All three pulmonary valve reconstruction techniques provided competent pulmonary valves. Nunn's bileaflet technique had better outcome at midterm. It has a potential to delay right ventricular dysfunction at long-term.
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Procedimientos Quirúrgicos Cardíacos , Insuficiencia de la Válvula Pulmonar , Válvula Pulmonar , Tetralogía de Fallot , Humanos , Lactante , Politetrafluoroetileno , Válvula Pulmonar/cirugía , Insuficiencia de la Válvula Pulmonar/cirugía , Estudios Retrospectivos , Tetralogía de Fallot/cirugía , Resultado del TratamientoRESUMEN
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.
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Enfermedad de la Arteria Coronaria , Vasos Coronarios , Niño , Puente de Arteria Coronaria , Enfermedad de la Arteria Coronaria/cirugía , Vasos Coronarios/diagnóstico por imagen , Vasos Coronarios/cirugía , Estudios de Seguimiento , Humanos , Estudios Retrospectivos , Factores de Riesgo , Resultado del TratamientoRESUMEN
OBJECTIVE: After congenital heart surgery, some patients may need long-term mechanical ventilation because of chronic respiratory failure. In this study, we analysed outcomes of the patients who need tracheostomy and home mechanical ventilation. METHODS: Amongst 1343 patients who underwent congenital heart surgery between January, 2014 and June, 2018, 45 needed tracheostomy and HMV. The median age of these patients was 6.4 months (12 days-6.5 years). Nineteen patients underwent palliation while 26 patients underwent total repair. Post-operative diaphragm plication was performed in five patients (11%). Median duration of mechanical ventilation before tracheostomy was 32 days (8-154 days). The patients were followed up with their home ventilators in ward and at home. Mean follow-up time was 36.24 ± 11.61 months. RESULTS: The median duration of ICU stay after tracheostomy was 27 days (range 2-93 days). Follow-up time in ward was median 30 days (2-156 days). A total of 12 patients (26.6%) were separated from the ventilator and underwent decannulation during hospital stay. Thirty-two patients (71.1%) were discharged home with home ventilator support. Of them, 15 patients (46.9%) were separated from the respiratory support in median of 6 weeks (1 week-11 months) and decannulations were performed. Total mortality was 31.1%. in which four patients are still HMV dependent. There was no significant difference for decannulation between total repair and palliation patients. CONCLUSION: HMV via tracheostomy is a useful option for the treatment of children who are dependent on long-term ventilation after congenital heart surgery although there are potential risks.
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Cardiopatías Congénitas , Traqueostomía , Niño , Cardiopatías Congénitas/cirugía , Humanos , Tiempo de Internación , Respiración Artificial , Estudios RetrospectivosRESUMEN
OBJECTIVE: We aimed to determine the early and midterm outcomes of ductal stenting in neonates with ductal-dependent pulmonary blood flow. METHODS: Between January, 2014 and July, 2018, 102 patients who underwent 115 cardiac catheterisation procedures for ductal stent implantation in our department were retrospectively reviewed. The age of the neonates ranged from 3 to 30 days (median: 11 days) and their weights ranged from 1.8 to 5.8 kg (mean, 2.8 ± 0.53 kg). Fifty-two patients had functional single ventricle and 50 had biventricular physiology. Thirty-one patients' weights were <2,500 g (30.3%). The patent ductus arteriosus was vertical in 60 patients (58.8%). The mean ductal length was 12.4 ± 4.1 mm (range, 7.8-23 mm), and the mean narrowest ductal diameter was 2.1 ± 0.7 mm (range, 1.2-3.4 mm). RESULTS: The technical success rate was 85.2%. Procedure-related mortality occurred in three patients (2.9%). After the procedure, the aortic oxygen saturation increased from a mean of 73.1 ± 6.2% to a mean of 90.4 ± 4.3% (p < 0.001), and the ductus diameter increased from a mean of 2.1 ± 0.7 mm to a mean of 4.2 ± 0.9 mm (p < 0.001). Either transcatheter or surgical reinterventions were required in 35 patients (34.3%) during the follow-up period after a median of 101 days (2-356 days). Thirty-three patients (32.3%) were bridged to surgical repair after a median of 288 days (163-650 days). The median duration of palliation with ductal stents was 210 days (range, 2-525 days). CONCLUSION: Ductus arteriosus stenting may be a reasonable and effective alternative to surgery for the initial palliation procedure in neonates with ductus-dependent pulmonary flow.
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Conducto Arterioso Permeable , Circulación Pulmonar , Cateterismo Cardíaco , Conducto Arterioso Permeable/cirugía , Humanos , Recién Nacido , Estudios Retrospectivos , Stents , Resultado del TratamientoRESUMEN
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.
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Puente de Arteria Coronaria , Enfermedad de la Arteria Coronaria/cirugía , Cardiopatías Congénitas/cirugía , Arterias Mamarias/trasplante , Complicaciones Posoperatorias/cirugía , Adolescente , Operación de Switch Arterial , Niño , Preescolar , Enfermedad de la Arteria Coronaria/fisiopatología , Vasos Coronarios/lesiones , Vasos Coronarios/cirugía , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/fisiopatología , Humanos , Enfermedad Iatrogénica , Lactante , Masculino , Complicaciones Posoperatorias/fisiopatología , Estudios Retrospectivos , Resultado del Tratamiento , Grado de Desobstrucción Vascular , Función VentricularRESUMEN
BACKGROUND: Vascular rings may cause pressure on the trachea and/or esophagus of varying degree, resulting in symptoms. This study assessed the presentation symptoms, diagnostic methods and treatment results after surgery in children with vascular ring. METHODS: Symptomatic vascular ring patients undergoing surgery between January 2010 and August 2014 at Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, were retrospectively assessed. The presentation symptoms, demographic characteristics, and diagnostic tests were evaluated. Operative data and postoperative follow up, complications and problems were also examined in detail. RESULTS: Twenty-one patients underwent surgery, 13 (63%) of whom were male. Median age was 12 months (range, 1 month-8 years). Among these patients, 62% (n = 13) had double aortic arch, 24% (n = 5) had right-sided aortic arch + ligamentum arteriosum, 10% (n = 2) had left-sided aortic arch + aberrant right subclavian artery, and 4% (n = 1) had pulmonary sling abnormality. Computed tomography (CT) angiography was performed in 17/21 patients. All the patients were successfully operated on. In the early postoperative period, two patients had chylothorax and one had nerve paralysis of nervus laryngeus recurrens. One patient died in the early period, and two patients had ongoing stridor on follow up. CONCLUSIONS: CT angiography is effective for the differential diagnosis and visualization of vascular ring abnormality. In such cases, full recovery can be assured with early diagnosis and surgery.
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Aorta Torácica/anomalías , Malformaciones Vasculares , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/cirugía , Niño , Preescolar , Angiografía por Tomografía Computarizada , Diagnóstico Diferencial , Estenosis Esofágica/etiología , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Complicaciones Posoperatorias/epidemiología , Estudios Retrospectivos , Estenosis Traqueal/etiología , Resultado del Tratamiento , Malformaciones Vasculares/complicaciones , Malformaciones Vasculares/diagnóstico , Malformaciones Vasculares/cirugíaRESUMEN
There are different surgical methods for the repair of persistent left superior vena cava that connects directly to the left atrium. We describe an extracardiac surgical technique that includes direct anastomosis of persistent left superior vena cava to the right atrium with right atrial flap and autologous pericardium. We have performed this procedure in four cases and there is no obstruction at postoperative control studies. Right atrial flap repair is a feasible extracardiac technique that offers growth potential.
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Procedimientos Quirúrgicos Cardíacos/métodos , Atrios Cardíacos/cirugía , Cardiopatías Congénitas/cirugía , Colgajos Quirúrgicos , Vena Cava Superior/cirugía , Anastomosis Quirúrgica , Preescolar , Atrios Cardíacos/anomalías , Atrios Cardíacos/crecimiento & desarrollo , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/fisiopatología , Humanos , Lactante , Pericardio/trasplante , Trasplante Autólogo , Resultado del Tratamiento , Vena Cava Superior/anomalías , Vena Cava Superior/crecimiento & desarrolloRESUMEN
Interrupted aortic arch is a very rare but well-described congenital anomaly. Concomitant presentation of interrupted aortic arch and giant subclavian artery aneurysm is an unusual event in adulthood. To the best of our knowledge, hybrid intervention for type A aortic arch interruption with a giant aneurysm of the subclavian artery is an alternative approach for the management of these concomitant pathologies in adults.
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Aneurisma/diagnóstico por imagen , Aneurisma/cirugía , Aorta Torácica/anomalías , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/cirugía , Arteria Subclavia/anomalías , Implantación de Prótesis Vascular , Angiografía por Tomografía Computarizada , Humanos , Masculino , Adulto JovenRESUMEN
Non-syndromic congenital supravalvular aortic stenosis (SVAS) leads to ventricular hypertrophy and increased oxygen consumption, and when combined with other factors reduces coronary blood flow, potentially resulting in myocardial ischemia and sudden cardiac death. While the anatomic obstruction of coronary circulation is as common in non-syndromic SVAS as in Williams syndrome, it often remains unacknowledged. Extracorporeal membrane oxygenation (ECMO) is an elective procedure that can be used to support patients with cardiac arrest during diagnosis as a way to reduce cardiopulmonary load in preparation for surgery or further treatment. In this report, we describe the rare case of an infant with severe SVAS and mild valvular pulmonary and left main coronary artery stenosis, as well as breath-holding spells. After multiple cardiac arrests, the infant underwent diagnostic catheter angiography on ECMO and had the pathology surgically corrected.
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Reanimación Cardiopulmonar/métodos , Estenosis Coronaria/complicaciones , Oxigenación por Membrana Extracorpórea , Paro Cardíaco/terapia , Angiografía Coronaria , Estenosis Coronaria/diagnóstico , Estenosis Coronaria/terapia , Electrocardiografía , Paro Cardíaco/etiología , Humanos , Lactante , MasculinoRESUMEN
BACKGROUND: We evaluated the preoperative, operative and postoperative risk factors affecting early mortality in patients who underwent total correction of tetralogy of Fallot (TOF). METHODS: One hundred and twenty-two TOF patients who underwent reparative surgery between January 2010 and November 2013 were enrolled in the study. RESULTS: Mean patient age and weight was 2.3 ± 2.5 years and 11.3 ± 6.4 kg, respectively. Cardiac catheterization was performed in 101 patients (83%),and coronary anomalies were found in 11 patients. Mean McGoon index, pulmonary annulus z-score, main pulmonary artery z-score, left pulmonary artery z-score and right pulmonary artery z-score were 2.0 ± 0.4, -1.85 ± 1.54, -2.84 ± 2.06, 1.17 ± 1.73, and 0.74 ± 1.57, respectively. Total reparative surgery with a transannular patch was performed in 97 patients (79.6%); the rest underwent valve-sparing surgery. Median duration of postoperative mechanical ventilation, intensive care and hospital stay were 19 h, 3 days and 9 days, respectively. Extracorporeal membrane oxygenation (ECMO) was required in 10 patients in the postoperative early period. Arrhythmias occurring in the early postoperative period were junctional ectopic tachycardia (n = 13), complete atrioventricular block(n = 10; permanent epicardial pacemaker implanted in four) and ventricular tachycardia (n = 4). Nine patients died in the early postoperative period (7.3%). Parameters found to be associated with increased mortality were low preoperative oxygen saturation; high right ventricular/aortic pressure ratio immediately after surgery; presence of coronary anomaly; requirement of postoperative ECMO; and pacemaker (P = 0.02, P = 0.04, P = 0.01, P = 0.0001, P = 0.03, respectively). CONCLUSIONS: Poor preoperative oxygenation, presence of coronary anomaly, complete AV block in the early postoperative period, high RV pressure and requirement of ECMO appear to be the most significant factors that affect early mortality in the surgical treatment of TOF. Appropriate preoperative assessment, correct surgical strategies and attentive intensive care monitoring are required in order to reduce mortality.
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Procedimientos Quirúrgicos Cardíacos/mortalidad , Tetralogía de Fallot/cirugía , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Periodo Perioperatorio , Estudios Retrospectivos , Factores de Riesgo , Tetralogía de Fallot/mortalidad , Factores de Tiempo , Turquía/epidemiologíaRESUMEN
BACKGROUND: Systemic-pulmonary shunts are widely used in initial palliation in cyanotic congenital heart disease. The incidence and the relationship between acute shunt obstruction and thrombophilia are not precisely defined. The aim of this study was to determine the frequency of shunt obstruction in the early postoperative period, and to define the frequency and presence of thrombophilia factors in patients treated for acute shunt thrombosis. METHODS: Between October 2010 and October 2012, 77 patients who had systemic-pulmonary shunt operation were included in this prospective study. Patients who developed shunt obstruction were examined in terms of inherited and acquired thrombophilia factors. RESULTS: Median patient age was 61 days and median weight was 4.3 kg. Thirty-three patients were neonates. Diameter of the Gore-Tex grafts used for the shunt ranged from 3 mm to 5 mm. Acute shunt occlusion rate was 10% (8/77), and all of these occurred in the first 24 h. Thrombophilia was found in three of eight patients who underwent intervention (surgical and/or transcatheter) due to shunt thrombosis (presence of anti-phospholipid antibodies, n = 1; protein C deficiency, n = 1; and factor V Leiden mutation, n = 1) and only one patient died. CONCLUSIONS: Acute shunt obstruction developed in 10% of patients who underwent systemic-pulmonary shunt, and emergency surgery or transcatheter intervention can be life saving in this context. Acute shunt obstruction can occur due to mechanical and hemodynamic problems, but clinicians should also consider and evaluate thrombophilia factors.
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Servicios Médicos de Urgencia , Oclusión de Injerto Vascular/etiología , Arteria Pulmonar/cirugía , Arteria Subclavia/cirugía , Trombofilia/complicaciones , Trombosis/etiología , Anastomosis Quirúrgica/efectos adversos , Estudios de Factibilidad , Femenino , Estudios de Seguimiento , Oclusión de Injerto Vascular/epidemiología , Humanos , Incidencia , Recién Nacido , Masculino , Estudios Prospectivos , Arteria Pulmonar/anomalías , Estudios Retrospectivos , Trombosis/epidemiología , Turquía/epidemiologíaRESUMEN
We present the use of Konno's procedure for the reconstruction of a huge left ventricular outflow tract pseudoaneurysm formed after aortic valve replacement.
Asunto(s)
Aneurisma Falso/cirugía , Procedimientos Quirúrgicos Cardiovasculares/métodos , Aneurisma Cardíaco/cirugía , Complicaciones Posoperatorias/cirugía , Obstrucción del Flujo Ventricular Externo/cirugía , Adulto , Válvula Aórtica/cirugía , Insuficiencia de la Válvula Aórtica/cirugía , Implantación de Prótesis de Válvulas Cardíacas , Humanos , Masculino , Factores de Tiempo , Resultado del TratamientoRESUMEN
Behçet's disease is a chronic multisystem inflammatory disorder. There are limited data about cardiac involvement, but it is seen rarely. Herein, we present a 33-year-old male patient with heart failure secondary to a right ventricular mass. It was first diagnosed as inflammatory myofibroblastic tumor (IMT) histopathologically. During the postoperative follow-up, a thrombus was detected at the interatrial septum, and the patient was reevaluated. The diagnosis was possible Behçet's disease, and the mass, previously reported as IMT, was determined to be an organizing thrombus with a mixture of granulation tissue and thrombotic material.