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1.
Eur Heart J Case Rep ; 7(1): ytad021, 2023 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-36727131

RESUMEN

Background: Children with congenital heart disease remain at risk of being lost to follow-up. They may present at a later stage with complications related to underlying condition that usually require in-depth phenotyping for optimal management. We describe an unusual case of a double outlet right ventricle (DORV) who presented three decades after initial intervention. Case summary: A 32-year-old female sought attention for worsening breathlessness. Available data suggested that she had double outlet right ventricle (DORV) and underwent pulmonary artery (PA) banding in infancy. On examination, she was frail, clubbed and cyanosed with oxygen saturation reduced to 75% at rest. Blood tests demonstrated elevated haemoglobin (208 g/L) and thrombocytopenia (70 *109/L). Echocardiography was limited but demonstrated DORV with increased gradient across both outflow tracts. Cardiac magnetic resonance (CMR) imaging clearly demonstrated DORV with doubly committed interventricular communication, subvalvular aortic valve stenosis and supravalvular pulmonary stenosis due to a previous PA band. CMR was also remarkable for demonstrating severely impaired left ventricle and difficulty achieving optimal contrast between myocardium and blood pool on late enhancement images. She underwent relief of subaortic stenosis with immediate improvement in left ventricular (LV) function and PA plasty with application of a new PA band. Conclusion: Lost to follow-up congenital patients present a particular challenge due to complications of chronic unusual physiology. This requires careful assessment by a multidisciplinary team to define optimal management strategy.

2.
J Card Surg ; 35(3): 557-563, 2020 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-31945228

RESUMEN

Fontan procedure is the preferred palliation for patients with single ventricles. OBJECTIVES: To evaluate early morbidity and mortality after Fontan operation in 87 consecutive patients, between 2007 and 2017. METHODS: Early survival, duration of intensive care unit (ICU), and hospital stays were the main outcomes evaluated. Potential influencing factors evaluated included preoperative and intraoperative variables. RESULTS: Fontan procedure was performed at a median age of 4.2 years (range, 17 months-26 years), and a median weight of 15.5 kg (range, 8-72 kg). Extracardiac Fontan was the procedure of choice. The median cardiopulmonary bypass time was 122 minutes (range, 58-550 minutes). The majority had a fenestration (75 out of 87). Postoperatively, the median duration of ICU stay and total hospital stay were (4, 1-76 days) and (16, 1-85 days), respectively. Fontan failure occurred in one patient (1%). Overall early survival was 94%, resulting in a mortality rate of 6%. Univariate analysis showed that heterotaxy (odds ratio [OR], 2.222; confidence interval [CI], 1.345-6.250; P = .003) and decreased ventricular function (OR, 2.207; CI, 1.348-6.061; P = .002) significantly decreased survival. The same analysis failed to identify any statistically significant risk factors for prolonged hospital and ICU stays. CONCLUSION: Our reported mortality and morbidity rates compared favorably with the reported rates. Therefore, Fontan operation can be performed in a tertiary care center in the United Arab Emirates with favorable early postoperative outcomes.


Asunto(s)
Procedimiento de Fontan , Cardiopatías Congénitas/cirugía , Ventrículos Cardíacos/anomalías , Ventrículos Cardíacos/cirugía , Adolescente , Adulto , Niño , Preescolar , Femenino , Cardiopatías Congénitas/mortalidad , Humanos , Lactante , Tiempo de Internación , Masculino , Tasa de Supervivencia , Factores de Tiempo , Resultado del Tratamiento , Adulto Joven
3.
Angiology ; 71(1): 17-26, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31129986

RESUMEN

The Middle East and North Africa (MENA) region has a high burden of morbidity and mortality due to premature (≤55 years in men; ≤65 years in women) myocardial infarction (MI) and acute coronary syndrome (ACS). Despite this, the prevalence of risk factors in patients presenting with premature MI or ACS is incompletely described. We compared lifestyle, clinical risk factors, and biomarkers associated with premature MI/ACS in the MENA region with selected non-MENA high-income countries. We identified English-language, peer-reviewed publications through PubMed (up to March 2018). We used the World Bank classification system to categorize countries. Patients with premature MI/ACS in the MENA region had a higher prevalence of smoking than older patients with MI/ACS but a lower prevalence of diabetes, hypertension, and dyslipidemia. Men with premature MI/ACS had a higher prevalence of smoking than women but a lower prevalence of diabetes and hypertension. The MENA region had sparse data on lifestyle, diet, psychological stress, and physical activity. To address these knowledge gaps, we initiated the ongoing Gulf Population Risks and Epidemiology of Vascular Events and Treatment (Gulf PREVENT) case-control study to improve primary and secondary prevention of premature MI in the United Arab Emirates, a high-income country in the MENA region.


Asunto(s)
Síndrome Coronario Agudo/epidemiología , Infarto del Miocardio/epidemiología , Síndrome Coronario Agudo/mortalidad , Síndrome Coronario Agudo/prevención & control , África del Norte/epidemiología , Edad de Inicio , Anciano , Estudios de Casos y Controles , Comorbilidad , Diabetes Mellitus/epidemiología , Dislipidemias/epidemiología , Femenino , Humanos , Hipertensión/epidemiología , Masculino , Persona de Mediana Edad , Medio Oriente/epidemiología , Mortalidad Prematura , Infarto del Miocardio/mortalidad , Infarto del Miocardio/prevención & control , Prevalencia , Prevención Primaria , Pronóstico , Proyectos de Investigación , Factores de Riesgo , Prevención Secundaria , Factores Sexuales , Fumar/efectos adversos , Fumar/epidemiología
4.
J Cardiothorac Surg ; 14(1): 33, 2019 Feb 08.
Artículo en Inglés | MEDLINE | ID: mdl-30736865

RESUMEN

BACKGROUND: Congenital coronary artery fistula in association with aneurysm of the involved coronary artery in adults is rare. Moreover, the right coronary artery- left atrial fistula is also uncommon. Most of the cases are asymptomatic. However, symptomatic patients need therapeutic interventions. The potential complications associated with this anomaly are life-threatening, therefore, there is a need to explore more on differential diagnosis, investigations, management strategies and prevention of complications. CASE PRESENTATION: We present herewith a 26-year-old male patient with symptoms of chest pain and dyspnea. He was diagnosed with aneurysmal dilatation of the right coronary artery in its entire course which terminated as a fistulous communication into the left atrium. The closure of the fistula was done using autologous pericardial patch under cardiopulmonary bypass. Currently, the patient is being followed up after surgery and receiving anticoagulants. CONCLUSION: The advancement in the diagnostic imaging modalities have made it possible to find similar abnormalities more frequently. Due to rare nature of this anomaly, there is a need to explore and discuss management strategies that include medical management, surgical intervention or percutaneous interventions for a successful outcome.


Asunto(s)
Aneurisma Coronario/congénito , Aneurisma Coronario/diagnóstico por imagen , Fístula/congénito , Adulto , Puente Cardiopulmonar , Dolor en el Pecho/diagnóstico por imagen , Aneurisma Coronario/cirugía , Angiografía Coronaria , Anomalías de los Vasos Coronarios/diagnóstico por imagen , Anomalías de los Vasos Coronarios/cirugía , Diagnóstico Diferencial , Fístula/diagnóstico por imagen , Fístula/cirugía , Atrios Cardíacos/anomalías , Atrios Cardíacos/diagnóstico por imagen , Humanos , Masculino , Tomografía Computarizada por Rayos X , Fístula Vascular/congénito , Fístula Vascular/diagnóstico por imagen , Fístula Vascular/cirugía
5.
Pediatr Transplant ; 20(8): 1087-1092, 2016 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-27397767

RESUMEN

We performed a retrospective review of outcomes after heart transplantation during long-term follow-up of a surgical cohort of 1138 Fontan patients who were followed at the Mayo Clinic. Follow-up information was obtained from medical records and a clinical questionnaire that was mailed to patients not known to be deceased at the initiation of the study. Forty-four of 1138 Fontan patients with initial or subsequent evaluation at Mayo had cardiac transplantation between 1988 and 2014 (mean age at transplantation was 23.2 ± 12 yr, median was 19.8 yr; mean interval between Fontan and transplantation was 13.0 ± 7.7 yr, median was 13.1 yr). Two patients had combined organ transplantation (one heart-lung, one heart-liver). Twelve of the 44 (27%) patients had PLE prior to transplantation. There was no difference in post-bypass Fontan pressures or incidence of late reoperations for AVV repair/replacement between transplanted and non-transplanted patients. There were 16 (36%) deaths in the transplantation cohort; seven occurred within 30 days of transplantation. Overall one, five, 10, and 15 yr post-transplantation survival was 80%, 72%, 69%, and 55%, respectively. Although this is a challenging group of patients, intermediate-term results suggest that cardiac transplantation remains a reasonable option for patients with a failed Fontan circulation.


Asunto(s)
Procedimiento de Fontan , Trasplante de Corazón , Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Lactante , Trasplante de Hígado , Estudios Longitudinales , Trasplante de Pulmón , Masculino , Persona de Mediana Edad , Reoperación , Estudios Retrospectivos , Encuestas y Cuestionarios , Resultado del Tratamiento , Adulto Joven
6.
J Extra Corpor Technol ; 48(1): 23-6, 2016 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-27134305

RESUMEN

Patients needing the assistance of extracorporeal membrane oxygenation (ECMO) are at risk of hemodilution and, in some instances, may require exposure to large amounts of allogeneic blood products. Patient outcomes can be improved by taking steps to reduce transfusions and hemodilution. Currently, modified ultrafiltration (MUF) is used across the world to reduce hemodilution after cardiopulmonary bypass (CPB). Another common technique during bypass initiation is autologous priming. By applying modified versions of these techniques, ECMO patients may potentially benefit. Usually, patients requiring immediate transition from CPB to ECMO are not stable enough to tolerate MUF. Through alterations of the CPB and ECMO circuit tubing, MUF can be performed once on ECMO. Another technique to potentially lower the transfusion requirements for ECMO patients is a complete circuit blood transfer during an ECMO circuit exchange. While selective component changes are preferred if possible, occasionally a complete circuit change must be done. To minimize hemodilution or prevent priming with blood products, the original ECMO circuit's blood can be transferred to the new ECMO circuit before connecting to the patient. Both of these techniques, in our opinion, helped to reduce the number of transfusions that our ECMO patients have seen during these critical time periods.


Asunto(s)
Transfusión Sanguínea , Oxigenación por Membrana Extracorpórea/métodos , Hemodilución , Hemofiltración , Transfusión Sanguínea/estadística & datos numéricos , Puente Cardiopulmonar/métodos , Diseño de Equipo , Oxigenación por Membrana Extracorpórea/efectos adversos , Oxigenación por Membrana Extracorpórea/instrumentación , Hemodilución/estadística & datos numéricos , Hemofiltración/instrumentación , Hemofiltración/métodos , Humanos , Ultrafiltración/instrumentación , Ultrafiltración/métodos , Dispositivos de Acceso Vascular
7.
Artículo en Inglés | MEDLINE | ID: mdl-27060049

RESUMEN

Although most valve operations performed annually address lesions of the aortic or mitral valves, the frequency of pulmonary valve replacement (PVR) is increasing because most patients with congenital heart disease are surviving into the adult years. The vast majority of patients, especially children that require PVR, obtain a tissue valve because of the relative good durability and the lack of a need for anticoagulation. Because the need for repeat operation is inevitable for most patients, and the population of adults with congenital heart disease continues to grow, there are increasing situations in which a mechanical pulmonary prosthesis may be appropriate. Most patients being considered for mechanical PVR have a congenital diagnosis and require multi-valve procedures, and quality of life and need for repeat operation(s) are major issues. Mechanical valves are durable but require anticoagulation, which carries its own inherent set of risks. There are conflicting reports regarding the late outcome of mechanical PVR. There are few reports that indicate that, in the pulmonary position, bileaflet valves are at higher risk for complications compared with monodisc valves; however, the majority of these patients were not anticoagulated with warfarin, but simply maintained on aspirin. There is a growing body of literature documenting low rates of thrombosis or pulmonary prosthesis dysfunction when proper anticoagulation and monitoring are applied.


Asunto(s)
Cardiopatías Congénitas/cirugía , Implantación de Prótesis de Válvulas Cardíacas , Prótesis Valvulares Cardíacas , Válvula Pulmonar/anomalías , Adulto , Anticoagulantes/uso terapéutico , Humanos , Reoperación
9.
Expert Rev Cardiovasc Ther ; 13(10): 1101-9, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-26357983

RESUMEN

Ebstein anomaly accounts for 1% of all congenital heart disease. It is a right ventricular myopathy with failure of tricuspid valve delamination and highly variable tricuspid valve morphology that usually results in severe regurgitation. It is the only congenital heart lesion that has a range of clinical presentations, from the severely symptomatic neonate to an asymptomatic adult. Neonatal operation has high operative mortality, whereas operation performed beyond infancy and into adulthood has low operative mortality. Late survival and quality of life for hospital survivors are excellent for the majority of patients in all age brackets. Atrial tachyarrhythmias are the most common late complication. There have been more techniques of tricuspid repair reported in the literature than any other congenital or acquired cardiac lesion. This is largely due to the infinite anatomic variability encountered with this anomaly. The cone reconstruction of Ebstein anomaly can achieve near anatomic restoration of the tricuspid valve anatomy. Early and intermediate results with these repairs are promising. Reduced right ventricular function continues to be a challenge for some patients, as is the need for reoperation for recurrent tricuspid regurgitation. The purpose of this article is to outline the current standard of care for diagnosis and treatment of Ebstein anomaly and describe innovative strategies to address poor right ventricular function and associated right-sided heart failure.


Asunto(s)
Anomalía de Ebstein/cirugía , Cardiopatías Congénitas/fisiopatología , Calidad de Vida , Procedimientos Quirúrgicos Cardíacos/métodos , Insuficiencia Cardíaca/fisiopatología , Humanos , Reoperación , Válvula Tricúspide/patología , Insuficiencia de la Válvula Tricúspide/fisiopatología
10.
Curr Cardiol Rev ; 7(2): 92-101, 2011 May.
Artículo en Inglés | MEDLINE | ID: mdl-22548032

RESUMEN

Heart transplantation is an accepted therapeutic modality for end-stage congenital heart disease for both biventricular and univentricular anomalies. Many transplant centers have pushed the limits of transplantation to include patients with high pulmonary vascular resistance, high panel reactive antibodies, positive cross-matches, and ABO-incompatibility. Excellent results have been possible, particularly with the development of improved diagnostic and therapeutic algorithms to prevent and treat rejection, infection, and post-transplant lymphoproliferative disease. Late graft failure and chronic rejection remain vexing problems. The vast majority of patients with biventricular congenital heart disease have undergone prior cardiac surgical procedures. Indications for transplantation in this subgroup are primarily progressive refractory heart failure following prior cardiac surgical reconstructive procedures. Contraindications to transplantation mimic those for other forms of end-stage heart disease. A determination of pulmonary vascular resistance is important in listing patients with biventricular congenital heart disease for heart transplantation. Modifications in the implant technique are necessary and vary depending on underlying recipient anatomy. Risk factors for perioperative outcomes in patients with biventricular congenital heart disease include the need for reoperation, the degree of anatomic reconstruction necessary during the implant procedure, and the degree of antibody sensitization, in addition to a number of other recipient and donor factors. Postoperative outcomes and survival are very good but remain inferior to those with cardiomyopathy in most series. In conclusion, patients with end-stage biventricular congenital heart disease represent a complex group of patients for heart transplantation, and require careful evaluation and management to ensure optimal outcomes.


Asunto(s)
Cardiopatías/congénito , Cardiopatías/cirugía , Trasplante de Corazón/métodos , Contraindicaciones , Rechazo de Injerto/prevención & control , Cardiopatías Congénitas/cirugía , Cardiopatías/mortalidad , Cardiopatías/patología , Insuficiencia Cardíaca/cirugía , Trasplante de Corazón/inmunología , Trasplante de Corazón/mortalidad , Humanos , Lactante , Recién Nacido , Cuidados Paliativos/métodos , Factores de Riesgo , Apoyo Social , Recolección de Tejidos y Órganos/métodos , Trasplante Homólogo , Resistencia Vascular/fisiología
11.
Pediatr Cardiol ; 31(7): 1104-6, 2010 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-20697703

RESUMEN

An 8-year-old boy developed a large aneurysm of the aortic arch after neonatal complete repair of interrupted aortic arch type B and closure of a perimembranous ventricular septal defect. To our knowledge, this unusual complication has not been previously reported in the English literature.


Asunto(s)
Aorta Torácica/anomalías , Aorta Torácica/cirugía , Aneurisma de la Aorta Torácica/cirugía , Niño , Humanos , Masculino , Resultado del Tratamiento
12.
World J Pediatr Congenit Heart Surg ; 1(1): 78-86, 2010 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-23804726

RESUMEN

The traditional single-patch technique for repair of complete atrioventricular (AV) canal requires surgical division of the superior and inferior common leaflets. In the neonate and young infant, subsequent resuspension of very delicate AV valve tissue on the pericardial patch can be problematic. Selective application of the modified single-patch technique as described by Nunn (Australian technique) minimizes manipulation of the AV valve leaflet tissue. Previous reports have documented that since the late 1980s, the traditional single-patch approach with leaflet resuspension is possible with a mortality of 3% or less. A review of the initial 33 patients managed with the Australian technique was undertaken. The ventricular septal defect was moderate or large in 29 patients (88%). In the balanced canal subgroup, there was no early mortality; 1 patient underwent reoperative mitral repair for cleft dehiscence 1 year postoperatively, and 1 patient with heterotaxy required pacemaker implantation. In the unbalanced canal subgroup, 2 patients died perioperatively (22%). There have been no late deaths or new left ventricular outflow tract obstruction in either subgroup. Selective application of the single-patch technique currently allows excellent results for surgical repair in the neonatal period or early infancy. Even during the learning phase of the Australian technique, satisfactory results were achieved.

13.
Circ Cardiovasc Genet ; 2(5): 442-9, 2009 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-20031619

RESUMEN

BACKGROUND: Mutations in the genes encoding sarcomere proteins have been associated with both hypertrophic and dilated cardiomyopathy. Recently, mutations in myosin heavy chain (MYH7), cardiac actin (ACTC), and troponin T (TNNT2) were associated with left ventricular noncompaction, a form of cardiomyopathy characterized with hypertrabeculation that may also include reduced function of the left ventricle. METHODS AND RESULTS: We used clinically available genetic testing on 3 cases referred for evaluation of left ventricular dysfunction and noncompaction of the left ventricle and found that all 3 individuals carried sarcomere mutations. The first patient presented with neonatal heart failure and was referred for left ventricular noncompaction cardiomyopathy. Genetic testing found 2 different mutations in MYBPC3 in trans. The first mutation, 3776delA, Q1259fs, rendered a frame shift at 1259 of cardiac myosin-binding protein C and the second mutation was L1200P. The frameshift mutation was also found in this mother who displayed mild echocardiographic features of cardiomyopathy, with only subtle increase in trabeculation and an absence of hypertrophy. A second pediatric patient presented with heart failure and was found to carry a de novo MYH7 R369Q mutation. The third case was an adult patient with dilated cardiomyopathy referred for ventricular hypertrabeculation. This patient had a family history of congestive heart failure, including pediatric onset cardiomyopathy where 3 individuals in the family were found to have the MYH7 mutation R1250W. CONCLUSIONS: Genetic testing should be considered for cardiomyopathy with hypertrabeculation.


Asunto(s)
Hipertrofia Ventricular Izquierda/genética , Mutación , Sarcómeros/genética , Miosinas Cardíacas/genética , Proteínas Portadoras/genética , Niño , Preescolar , Femenino , Humanos , Hipertrofia Ventricular Izquierda/diagnóstico por imagen , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Cadenas Pesadas de Miosina/genética , Linaje , Ultrasonografía
14.
Catheter Cardiovasc Interv ; 70(1): 90-7, 2007 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-17585388

RESUMEN

OBJECTIVES: In this study, we evaluated the feasibility, safety, and outcome of device closure of muscular ventricular septal defects (mVSD) in infants less than 1 year of age using the Amplatzer devices. BACKGROUND: Closure of mVSDs continues to represent a surgical challenge with significant morbidity. Hence, device closure is becoming an attractive and reliable alternative. However, little is known about the feasibility of this procedure in small infants. METHODS: Between July 1999 and September 2006, device closure of mVSD was attempted in 20 infants ranging in age from 3 days to 12 months (median +/- SD; 4.6 +/- 3.8) and in weight from 3.2 to 8.9 kg (4.6 +/- 1.9) under TEE guidance by percutaneous or hybrid (perventricular) techniques. The size of the VSD as assessed by TEE ranged from 3 to 11 mm (6.0 +/- 2.2) and the Qp:Qs ratio ranged from 0.7 to 8.8 (2.8 +/- 2.3). RESULTS: The device was successfully placed in 19/20 infants and it ranged in size from 4 to 14 mm (8.0 +/- 2.6). It was percutaneously deployed in 11/19 and by the hybrid approach in 8/19. There were 30 devices placed in 19 infants with multiple devices placed in 5/19 infants. Fluoroscopy times ranged from 11 to 136 min (41 +/- 28) and procedure times ranged from 57 to 291 min (178 +/- 68). The success rate as defined by complete closure or a trivial shunt was 84% immediately and 100% at 1-year follow-up. Major complications occurred in 4/20 patients: wire perforation and hemopericardium (n = 1), device migration (n = 1), transient electromechanical dissociation (n = 1), and mediastinitis (n = 1). At a median follow-up of 3.8 years, all patients improved and had no hemodynamically significant VSDs. No major complications were encountered in patients with multiple devices. CONCLUSION: In infants less than 1 year of age, percutaneous and perventricular device closure of mVSDs is technically feasible and highly effective with low morbidity. Long term safety and efficacy needs to be assessed.


Asunto(s)
Defectos del Tabique Interventricular/terapia , Selección de Paciente , Implantación de Prótesis/instrumentación , Cateterismo Cardíaco , Cineangiografía , Ecocardiografía Doppler en Color , Ecocardiografía Transesofágica , Estudios de Factibilidad , Femenino , Fluoroscopía , Estudios de Seguimiento , Defectos del Tabique Interventricular/diagnóstico por imagen , Defectos del Tabique Interventricular/cirugía , Humanos , Lactante , Recién Nacido , Masculino , Diseño de Prótesis , Implantación de Prótesis/efectos adversos , Radiografía Intervencional , Proyectos de Investigación , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Factores de Tiempo , Resultado del Tratamiento , Ultrasonografía Intervencional
15.
Ann Thorac Surg ; 83(2): 678-80, 2007 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-17258014

RESUMEN

Distortion of septal and atrial anatomy in Ebstein's anomaly places the atrioventricular node and His bundle at risk for injury at operation. We present a novel technique for creating a robust neo-annulus, remote from conduction tissue, adding to the armamentarium of techniques available for the reconstruction or replacement of the tricuspid valve. Three cases are described. All the patients were in sinus rhythm, with competent native or bioprosthetic valves, at short-term follow-up.


Asunto(s)
Bioprótesis , Procedimientos Quirúrgicos Cardíacos/métodos , Anomalía de Ebstein/cirugía , Prótesis Valvulares Cardíacas , Válvula Tricúspide/anomalías , Válvula Tricúspide/cirugía , Adolescente , Adulto , Animales , Anomalía de Ebstein/fisiopatología , Femenino , Frecuencia Cardíaca , Humanos , Masculino , Porcinos , Resultado del Tratamiento
16.
Int J Pediatr Otorhinolaryngol ; 71(1): 57-62, 2007 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-17034866

RESUMEN

OBJECTIVE: To review the presentation and natural history of children with vascular rings and present management guidelines. METHODS: Retrospective study of tertiary care pediatric medical center charts from 1991 to 2002. RESULTS: There were 37 males and 27 females with a diagnosis of vascular rings. At presentation, 91% of patients had airway symptoms and 47% had esophageal symptoms. Airway symptoms included stridor (63%), recurrent respiratory infections (47%), respiratory distress (19%), and cough (17%). The most common esophageal symptom was dysphagia (27%). Pre-operative studies included: echocardiography (96%), chest X-ray (93%), barium swallow (75%), magnetic resonance imaging (MRI) (60%), and computerized tomography (CT) scan of the chest (59%). Surgical management included open (n=25) and thoracoscopic (n=39) approach. Complications included recurrent laryngeal nerve injury in five patients (8%). CONCLUSION: Children with vascular rings present with respiratory and/or feeding difficulty. The evaluation should include chest X-ray, echocardiography, and barium swallow. Direct laryngoscopy and bronchoscopy are recommended to assess the degree of compression of the airway and/or esophagus, tracheomalacia, and vocal fold motion prior to intervention. Indication for surgical release is given when the diagnosis is made and can be assisted by advanced radiology studies. Surgical options include minimally invasive techniques involving either thoracoscopic or robotic-assisted repairs, as well as open procedures involving thoracotomy.


Asunto(s)
Aorta Torácica/anomalías , Arteria Subclavia/anomalías , Aorta Torácica/patología , Aorta Torácica/cirugía , Preescolar , Trastornos de Deglución/etiología , Diagnóstico por Imagen , Insuficiencia de Crecimiento/etiología , Femenino , Humanos , Masculino , Traumatismos del Nervio Laríngeo Recurrente , Trastornos Respiratorios/etiología , Ruidos Respiratorios/etiología , Estudios Retrospectivos , Arteria Subclavia/patología , Arteria Subclavia/cirugía , Cirugía Torácica Asistida por Video/efectos adversos
17.
J Thorac Cardiovasc Surg ; 131(1): 163-171.e2, 2006 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-16399308

RESUMEN

BACKGROUND: Survival after stage I palliation for hypoplastic left heart syndrome or related anomalies remains poor in high-risk neonates. We hypothesized that a less invasive hybrid approach would be beneficial in this patient population. METHODS: The hybrid stage I procedure was performed in the catheterization laboratory. Via a median sternotomy, both branch pulmonary arteries were banded, and a ductal stent was delivered via a main pulmonary artery puncture and positioned under fluoroscopic guidance. RESULTS: Between October 2003 and June 2005, 14 high-risk neonates underwent a hybrid stage I procedure. Eleven of 14 had hypoplastic left heart syndrome. Two also underwent peratrial atrial septal stenting, and 5 required percutaneous atrial stenting later. Two neonates with an intact or highly restrictive atrial septum had emergency percutaneous atrial stent placement. Hospital survival was 11 (78.5%) of 14. One patient required extracorporeal membrane oxygenation support for intraoperative cardiac arrest. He underwent cardiac transplantation but died later of sepsis. One patient died of ductal stent embolization, and a third died of progressive cardiac dysfunction. The first 4 patients required pulmonary artery band revisions. There were none after we modified our technique and added branch pulmonary artery angiograms. There were 2 interstage deaths from atrial stent occlusion and from preductal retrograde coarctation. Eight patients underwent stage II procedures, consisting of aortic arch reconstruction, atrial septectomy, and cavopulmonary shunt. Two patients died after stage II. One patient is awaiting stage II. CONCLUSIONS: The hybrid stage I palliation is a valid option in high-risk neonates. As experience is accrued, it may become the preferred alternative. However, in aortic atresia, the development of preductal retrograde coarctation is a significant problem.


Asunto(s)
Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Procedimientos Quirúrgicos Cardíacos/métodos , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/complicaciones , Recién Nacido , Complicaciones Posoperatorias/epidemiología , Factores de Riesgo
18.
Ann Thorac Surg ; 80(6): 2309-13, 2005 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-16305894

RESUMEN

BACKGROUND: This study reports on our initial experience with robotically assisted patent ductus arteriosus (PDA) closure and vascular ring division in children. METHODS: From April 2002 to May 2004, 15 patients underwent PDA closure (n = 9) and vascular ring repair (n = 6) by a totally endoscopic approach, utilizing the Da Vinci robotic system. The mean age of the patients was 8.3 +/- 4.7 years (range, 3 to 18) and the mean weight, 35.5 +/- 19.0 kg (range, 14.1 to 77.0 kg). Three thoracoscopic trocars were used to accommodate the endoscopic camera and two surgical instruments with an additional small incision for lung retraction. After dissection by the surgeon seated at the master console, PDA ligation with clips or division of the atretic arch and ductal ligament was performed. RESULTS: Total operative times were 170 +/- 46 minutes (PDA) and 167 +/- 48 minutes (vascular ring). One patient with vascular ring was converted to thoracotomy because of dense adhesions due to previous surgery. Precise and easy surgical maneuver was possible with the articulated surgical instruments and three-dimensional visualization in 14 patients. Intraoperative transesophageal echocardiography confirmed no persistent shunt in all PDA patients. No laryngeal nerve injury and hemorrhage were noted. All patients were extubated in the operating room. Median length of postoperative hospital stay was 1.5 days. CONCLUSIONS: Robotically assisted PDA closure and vascular ring division is a feasible and safe procedure. Future technologic improvement, including smaller instrument size and incorporation of tactile feedback, may permit application of this technique to even younger infants and intracardiac repairs.


Asunto(s)
Aorta Torácica/anomalías , Aorta Torácica/cirugía , Conducto Arterioso Permeable/cirugía , Robótica , Toracoscopía , Adolescente , Procedimientos Quirúrgicos Cardíacos/instrumentación , Procedimientos Quirúrgicos Cardíacos/métodos , Niño , Preescolar , Diseño de Equipo , Humanos
19.
Eur J Cardiothorac Surg ; 27(3): 420-4, 2005 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-15740950

RESUMEN

OBJECTIVE: Review of surgical repair of aortic root aneurysms using composite graft or homograft in children. METHODS: A consecutive series of 34 children (mean age 10.8+/-5.4 years) who underwent elective aortic root replacement using composite graft or homograft from 1987 to 2003 (mean follow-up 5.7+/-3.7 years). RESULTS: Preoperatively, the aortic annulus and aortic root average z-scores were 4.1+/-2.2 and 9.4+/-4.7, respectively. Composite graft root replacement was performed in 22 patients, and cryopreserved aortic homograft root replacement in 12 patients. There was one perioperative death in the homograft group due to sudden cardiovascular collapse. There was one late death in the composite graft group due to acute aortic dissection, and two late deaths in the homograft root replacement group, one at 7 months postoperatively due to coronary artery thrombosis and one due to severe chronic myocardial dysfunction 5 years postoperatively. One patient who initially had a homograft died due to mechanical valve thrombosis following reoperative composite graft replacement. Five patients had reoperations at a median of 7.1 years after initial surgery. One patient in the composite graft group underwent arch replacement. There were no graft related reoperations after composite graft root replacement, but 4 patients in the homograft group had reoperative composite graft replacement. Predictors of reoperation included age at surgery, lower weight, and longer ICU time (P<0.05). CONCLUSIONS: In children with aortic root aneurysms, reoperation is more common after homograft root replacement than composite graft replacement. Composite graft root replacement provides more stable repair of the aortic root.


Asunto(s)
Aneurisma de la Aorta/cirugía , Válvula Aórtica/cirugía , Implantación de Prótesis de Válvulas Cardíacas/métodos , Prótesis Valvulares Cardíacas , Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Síndrome de Marfan/cirugía , Falla de Prótesis , Reoperación , Factores de Riesgo , Resultado del Tratamiento
20.
J Thorac Cardiovasc Surg ; 128(4): 579-87, 2004 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-15457159

RESUMEN

BACKGROUND: There is no current acceptable approach for intracardiac beating-heart interventions. We have adapted real-time 3-dimensional echocardiography with specialized instrumentation to facilitate beating-heart repair of atrial septal defects and mitral valve plasty to investigate the feasibility of real-time 3-dimensional echocardiography-guided cardiac surgery. METHODS: In experiment I a modified real-time 3-dimensional echocardiography system with x4 matrix transducer was compared with 2-dimensional echocardiography in the performance of common surgical tasks. Completion times, deviation from an ideal trajectory, and an echogenic target were measured. In experiment II porcine atrial septal defects were closed with an original semiautomatic suturing device (n = 4) and with a 5-mm endoscopic stapler and a pericardial or polytetrafluoroethylene patch (n = 4). In experiment III a pulsatile porcine mitral valve model was developed, and suture placement through the anterior and posterior mitral leaflets was performed (n = 8). During all experiments, the operator was blinded to the target and operated on only with ultrasonic guidance. RESULTS: In experiment I, compared with 2-dimensional echocardiographic guidance, completion times improved by 21% ( P <.01) with high-trajectory accuracy, and suture deviation was significantly smaller (2-dimensional echocardiography, 5.4 +/- 2.7 mm; 3-dimensional echocardiography, 1.7 +/- 0.7 mm; P <.05) in real-time 3-dimensional echocardiography-guided tasks. In experiments II and III in both atrial septal defect closure and mitral valve plasty, real-time 3-dimensional echocardiography provided satisfactory images and sufficient anatomic detail for suturing and patch deployment. All surgical tasks were successfully performed with accuracy. CONCLUSIONS: Real-time 3-dimensional echocardiography provides adequate imaging and anatomic detail to act as a sole guide for surgical task performance. These initial experiments demonstrate the feasibility of beating-heart direct or patch closure of atrial septal defects and mitral valve plasty without cardiopulmonary bypass.


Asunto(s)
Procedimientos Quirúrgicos Cardíacos , Ecocardiografía Tridimensional , Animales , Procedimientos Quirúrgicos Cardíacos/métodos , Puente Cardiopulmonar , Ecocardiografía , Ecocardiografía Tridimensional/instrumentación , Estudios de Factibilidad , Defectos del Tabique Interatrial/diagnóstico por imagen , Defectos del Tabique Interatrial/cirugía , Válvula Mitral/diagnóstico por imagen , Válvula Mitral/cirugía , Politetrafluoroetileno , Prótesis e Implantes , Instrumentos Quirúrgicos , Técnicas de Sutura , Porcinos
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